CRYSTAL STRUCTURE OF CLOSTRIDIUM BOTULINUM NEUROTOXIN SEROTYPE B.

DOE Office of Scientific and Technical Information (OSTI.GOV)

SWAMINATHAN,S.; ESWARAMOORTHY,S.

2001-11-19

The toxigenic strains of Clostridium botulinum produce seven serologically distinct types of neurotoxins labeled A - G (EC 3.4.24.69), while Clostridium tetani produces tetanus neurotoxin (EC 3.4.24.68). Botulinum and tetanus neurotoxins (BoNTs and TeNT) are produced as single inactive chains of molecular mass of approximately 150 kDa. Most of these neurotoxins are released after being cleaved into two chains, a heavy chain (HI) of 100 kDa and a light chain (L) of 50 kDa held together by an interchain disulfide bond, by tissue proteinases. BoNT/E is released as a single chain but cleaved by host proteinases [1]. Clostvidium botulinum neurotoxinsmore » are extremely poisonous proteins with their LD{sub 50} for humans in the range of 0.1 - 1 ng kg{sup -1} [2]. Botulinum neurotoxins are responsible for neuroparalytic syndromes of botulism characterized by serious neurological disorders and flaccid paralysis. BoNTs block the release of acetylcholine at the neuromuscular junction causing flaccid paralysis while TeNT blocks the release of neurotransmitters like glycine and {gamma}-aminobutyric acid (GABA) in the inhibitory interneurons of the spinal cord resulting in spastic paralysis. In spite of different clinical symptoms, their aetiological agents intoxicate neuronal cells in the same way and these toxins have similar structural organization [3].« less

[Achievements and enlightenment of modern acupuncture therapy for stroke based on the neuroanatomy].

PubMed

Chen, Li-Fang; Fang, Jian-Qiao; Chen, Lu-Ni; Wang, Chao

2014-04-01

Up to now, in the treatment of stroke patients by acupuncture therapy, three main representative achievements involving scalp acupuncture intervention, "Xing Nao Kai Qiao" (restoring consciousness and inducing resuscitation) acupuncture technique and nape acupuncture therapy have been got. Regarding their neurobiological mechanisms, the scalp acupuncture therapy is based on the functional localization of the cerebral cortex, "Xing Nao Kai Qiao" acupuncture therapy is closely related to nerve stem stimulation, and the nape acupuncture therapy is based on the nerve innervation of the regional neck-nape area in obtaining therapeutic effects. In fact, effects of these three acupuncture interventions are all closely associated with the modern neuroanatomy. In the treatment of post-stroke spastic paralysis, cognitive disorder and depression with acupuncture therapy, modern neuroanatomical knowledge should be one of the key theoretical basis and new therapeutic techniques should be explored and developed continuously.

Quadriceps femoris spasticity in children with cerebral palsy: measurement with the pendulum test and relationship with gait abnormalities.

PubMed

Szopa, Andrzej; Domagalska-Szopa, Małgorzata; Kidoń, Zenon; Syczewska, Małgorzata

2014-12-16

Development of a reliable and objective test of spasticity is important for assessment and treatment of children with cerebral palsy. The pendulum test has been reported to yield reliable measurements of spasticity and to be sensitive to variations in spasticity in these children. However, the relationship between the pendulum test scores and other objective measures of spasticity has not been studied. The present study aimed to assess the effectiveness of an accelerometer-based pendulum test as a measurement of spasticity in CP, and to explore the correlation between the measurements of this test and the global index of deviation from normal gait in in children with cerebral palsy. We studied thirty-six children with cerebral palsy, including 18 with spastic hemiplegia and 18 with spastic diplegia, and a group of 18 typically-developing children. Knee extensor spasticity was assessed bilaterally using the accelerometer-based pendulum test and three-dimensional gait analysis. The Gillette Gait Index was calculated from the results of the gait analysis. The data from the accelerometer-based pendulum test could be used to distinguish between able-bodied children and children with cerebral palsy. Additionally, two of the measurements, first swing excursion and relaxation index, could be used to differentiate the degree of knee extensor spasticity in the children with cerebral palsy. Only a few moderate correlations were found between the Gillette Gait Index and the pendulum test data. This study demonstrates that the pendulum test can be used to discriminate between typically developing children and children with CP, as well as between various degrees of spasticity, such as spastic hemiplegia and spastic diplegia, in the knee extensor muscle of children with CP. Deviations from normal gait in children with CP were not correlated with the results of the pendulum test.

Botulinum Toxin Type a as a Therapeutic Agent against Headache and Related Disorders.

PubMed

Luvisetto, Siro; Gazerani, Parisa; Cianchetti, Carlo; Pavone, Flaminia

2015-09-23

Botulinum neurotoxin A (BoNT/A) is a toxin produced by the naturally-occurring Clostridium botulinum that causes botulism. The potential of BoNT/A as a useful medical intervention was discovered by scientists developing a vaccine to protect against botulism. They found that, when injected into a muscle, BoNT/A causes a flaccid paralysis. Following this discovery, BoNT/A has been used for many years in the treatment of conditions of pathological muscle hyperactivity, like dystonias and spasticities. In parallel, the toxin has become a "glamour" drug due to its power to ward off facial wrinkles, particularly frontal, due to the activity of the mimic muscles. After the discovery that the drug also appeared to have a preventive effect on headache, scientists spent many efforts to study the potentially-therapeutic action of BoNT/A against pain. BoNT/A is effective at reducing pain in a number of disease states, including cervical dystonia, neuropathic pain, lower back pain, spasticity, myofascial pain and bladder pain. In 2010, regulatory approval for the treatment of chronic migraine with BoNT/A was given, notwithstanding the fact that the mechanism of action is still not completely elucidated. In the present review, we summarize experimental evidence that may help to clarify the mechanisms of action of BoNT/A in relation to the alleviation of headache pain, with particular emphasis on preclinical studies, both in animals and humans. Moreover, we summarize the latest clinical trials that show evidence on headache conditions that may obtain benefits from therapy with BoNT/A.

Botulinum Toxin Type A as a Therapeutic Agent against Headache and Related Disorders

PubMed Central

Luvisetto, Siro; Gazerani, Parisa; Cianchetti, Carlo; Pavone, Flaminia

2015-01-01

Botulinum neurotoxin A (BoNT/A) is a toxin produced by the naturally-occurring Clostridium botulinum that causes botulism. The potential of BoNT/A as a useful medical intervention was discovered by scientists developing a vaccine to protect against botulism. They found that, when injected into a muscle, BoNT/A causes a flaccid paralysis. Following this discovery, BoNT/A has been used for many years in the treatment of conditions of pathological muscle hyperactivity, like dystonias and spasticities. In parallel, the toxin has become a “glamour” drug due to its power to ward off facial wrinkles, particularly frontal, due to the activity of the mimic muscles. After the discovery that the drug also appeared to have a preventive effect on headache, scientists spent many efforts to study the potentially-therapeutic action of BoNT/A against pain. BoNT/A is effective at reducing pain in a number of disease states, including cervical dystonia, neuropathic pain, lower back pain, spasticity, myofascial pain and bladder pain. In 2010, regulatory approval for the treatment of chronic migraine with BoNT/A was given, notwithstanding the fact that the mechanism of action is still not completely elucidated. In the present review, we summarize experimental evidence that may help to clarify the mechanisms of action of BoNT/A in relation to the alleviation of headache pain, with particular emphasis on preclinical studies, both in animals and humans. Moreover, we summarize the latest clinical trials that show evidence on headache conditions that may obtain benefits from therapy with BoNT/A. PMID:26404377

Neuromodulation of lower limb motor control in restorative neurology.

PubMed

Minassian, Karen; Hofstoetter, Ursula; Tansey, Keith; Mayr, Winfried

2012-06-01

One consequence of central nervous system injury or disease is the impairment of neural control of movement, resulting in spasticity and paralysis. To enhance recovery, restorative neurology procedures modify altered, yet preserved nervous system function. This review focuses on functional electrical stimulation (FES) and spinal cord stimulation (SCS) that utilize remaining capabilities of the distal apparatus of spinal cord, peripheral nerves and muscles in upper motor neuron dysfunctions. FES for the immediate generation of lower limb movement along with current rehabilitative techniques is reviewed. The potential of SCS for controlling spinal spasticity and enhancing lower limb function in multiple sclerosis and spinal cord injury is discussed. The necessity for precise electrode placement and appropriate stimulation parameter settings to achieve therapeutic specificity is elaborated. This will lead to our human work of epidural and transcutaneous stimulation targeting the lumbar spinal cord for enhancing motor functions in spinal cord injured people, supplemented by pertinent human research of other investigators. We conclude that the concept of restorative neurology recently received new appreciation by accumulated evidence for locomotor circuits residing in the human spinal cord. Technological and clinical advancements need to follow for a major impact on the functional recovery in individuals with severe damage to their motor system. Copyright © 2012 Elsevier B.V. All rights reserved.

Neuromodulation of lower limb motor control in restorative neurology

PubMed Central

Minassian, Karen; Hofstoetter, Ursula; Tansey, Keith; Mayr, Winfried

2012-01-01

One consequence of central nervous system injury or disease is the impairment of neural control of movement, resulting in spasticity and paralysis. To enhance recovery, restorative neurology procedures modify altered, yet preserved nervous system function. This review focuses on functional electrical stimulation (FES) and spinal cord stimulation (SCS) that utilize remaining capabilities of the distal apparatus of spinal cord, peripheral nerves and muscles in upper motor neuron dysfunctions. FES for the immediate generation of lower limb movement along with current rehabilitative techniques is reviewed. The potential of SCS for controlling spinal spasticity and enhancing lower limb function in multiple sclerosis and spinal cord injury is discussed. The necessity for precise electrode placement and appropriate stimulation parameter settings to achieve therapeutic specificity is elaborated. This will lead to our human work of epidural and transcutaneous stimulation targeting the lumbar spinal cord for enhancing motor functions in spinal cord injured people, supplemented by pertinent human research of other investigators. We conclude that the concept of restorative neurology recently received new appreciation by accumulated evidence for locomotor circuits residing in the human spinal cord. Technological and clinical advancements need to follow for a major impact on the functional recovery in individuals with severe damage to their motor system. PMID:22464657

Amblyopia Associated with Congenital Facial Nerve Paralysis.

PubMed

Iwamura, Hitoshi; Kondo, Kenji; Sawamura, Hiromasa; Baba, Shintaro; Yasuhara, Kazuo; Yamasoba, Tatsuya

2016-01-01

The association between congenital facial paralysis and visual development has not been thoroughly studied. Of 27 pediatric cases of congenital facial paralysis, we identified 3 patients who developed amblyopia, a visual acuity decrease caused by abnormal visual development, as comorbidity. These 3 patients had facial paralysis in the periocular region and developed amblyopia on the paralyzed side. They started treatment by wearing an eye patch immediately after diagnosis and before the critical visual developmental period; all patients responded to the treatment. Our findings suggest that the incidence of amblyopia in the cases of congenital facial paralysis, particularly the paralysis in the periocular region, is higher than that in the general pediatric population. Interestingly, 2 of the 3 patients developed anisometropic amblyopia due to the hyperopia of the affected eye, implying that the periocular facial paralysis may have affected the refraction of the eye through yet unspecified mechanisms. Therefore, the physicians who manage facial paralysis should keep this pathology in mind, and when they see pediatric patients with congenital facial paralysis involving the periocular region, they should consult an ophthalmologist as soon as possible. © 2016 S. Karger AG, Basel.

Cyanide and the human brain: perspectives from a model of food (cassava) poisoning.

PubMed

Tshala-Katumbay, Desire D; Ngombe, Nadege N; Okitundu, Daniel; David, Larry; Westaway, Shawn K; Boivin, Michael J; Mumba, Ngoyi D; Banea, Jean-Pierre

2016-08-01

Threats by fundamentalist leaders to use chemical weapons have resulted in renewed interest in cyanide toxicity. Relevant insights may be gained from studies on cyanide mass intoxication in populations relying on cyanogenic cassava as the main source of food. In these populations, sublethal concentrations (up to 80 μmol/l) of cyanide in the blood are commonplace and lead to signs of acute toxicity. Long-term toxicity signs include a distinct and irreversible spastic paralysis, known as konzo, and cognition deficits, mainly in sequential processing (visual-spatial analysis) domains. Toxic culprits include cyanide (mitochondrial toxicant), thiocyanate (AMPA-receptor chaotropic cyanide metabolite), cyanate (protein-carbamoylating cyanide metabolite), and 2-iminothiazolidine-4-carboxylic acid (seizure inducer). Factors of susceptibility include younger age, female gender, protein-deficient diet, and, possibly, the gut functional metagenome. The existence of uniquely exposed and neurologically affected populations offers invaluable research opportunities to develop a comprehensive understanding of cyanide toxicity and test or validate point-of-care diagnostic tools and treatment options to be included in preparedness kits in response to cyanide-related threats. © 2016 New York Academy of Sciences.

Cyanide and the human brain: perspectives from a model of food (cassava) poisoning

PubMed Central

Tshala-Katumbay, Desire D.; Ngombe, Nadege N.; Okitundu, Daniel; David, Larry; Westaway, Shawn K.; Boivin, Michael J.; Mumba, Ngoyi D.; Banea, Jean-Pierre

2016-01-01

Threats by fundamentalist leaders to use chemical weapons have resulted in renewed interest in cyanide toxicity. Relevant insights may be gained from studies on cyanide mass intoxication in populations relying on cyanogenic cassava as the main source of food. In these populations, sublethal concentrations (up to 80 µmol/L) of cyanide in the blood are commonplace and lead to signs of acute toxicity. Long-term toxicity signs include a distinct and irreversible spastic paralysis, known as konzo, and cognition deficits, mainly in sequential processing (visual–spatial analysis) domains. Toxic culprits include cyanide (mitochondrial toxicant), thiocyanate (AMPA-receptor chaotropic cyanide metabolite), cyanate (protein-carbamoylating cyanide metabolite), and 2-iminothiazolidine-4-carboxylic acid (seizure inducer). Factors of susceptibility include younger age, female gender, protein-deficient diet, and, possibly, the gut functional metagenome. The existence of uniquely exposed and neurologically affected populations offers invaluable research opportunities to develop a comprehensive understanding of cyanide toxicity and test or validate point-of-care diagnostic tools and treatment options to be included in preparedness kits in response to cyanide-related threats. PMID:27450775

[Tuberculous meningoencephalitis--own observations].

PubMed

Garlicki, Aleksander; Kluba-Wojewoda, Urszula; Bociaga-Jasik, Monika; Kalinowska-Nowak, Anna; Sobczyk-Krupiarz, Iwona; Mach, Tomasz

2003-01-01

Nine cases of the tuberculous meningoencephalitis in adult men and women treated in years 1993-2000 have been presented. The diagnosis was established on the basis of clinical picture and examination of the cerebrospinal fluid (CSF). Routine analysis of CSF was done, as well as the microscopic examination, biological assay, and culture on the Lowenstein-Jensena medium and using Bactec 460 TB system. Only in one case Mycobacterium tuberculosis was found in the smear of CSF stained by Ziehl-Nielsen method. In one case the biologic assay was positive and Mycobacterium tuberculosis was cultured on Lowenstein-Jensen medium. In another 4 cases the pathogen was detected by Bactec 460 TB technique. In 4 cases Mycobacterium tuberculosis was also cultured from the secretion of the respiratory tract, and in one patient urine. On chest X-ray characteristic changes for pulmonary tuberculosis were observed in 5 cases (56%). Seven patients (78%) had hydrocephalus detected on CT scan of the head. One patient died, one (11%) developed persistent complication in form of spastic paralysis of lower extremities and sphincters dysfunction. Seven patients (78%) recovered completely after the 12 months therapy with standard chemotherapeutic regimen for tuberculosis.

The swimming test is effective for evaluating spasticity after contusive spinal cord injury

PubMed Central

Ryu, Youngjae; Ogata, Toru; Nagao, Motoshi; Kitamura, Taku; Morioka, Kazuhito; Ichihara, Yoshinori; Doi, Toru; Sawada, Yasuhiro; Akai, Masami; Nishimura, Ryohei; Fujita, Naoki

2017-01-01

Spasticity is a frequent chronic complication in individuals with spinal cord injury (SCI). However, the severity of spasticity varies in patients with SCI. Therefore, an evaluation method is needed to determine the severity of spasticity. We used a contusive SCI model that is suitable for clinical translation. In this study, we examined the feasibility of the swimming test and an EMG for evaluating spasticity in a contusive SCI rat model. Sprague-Dawley rats received an injury at the 8th thoracic vertebra. Swimming tests were performed 3 to 6 weeks after SCI induction. We placed the SCI rats into spasticity-strong or spasticity-weak groups based on the frequency of spastic behavior during the swimming test. Subsequently, we recorded the Hoffman reflex (H-reflex) and examined the immunoreactivity of serotonin (5-HT) and its receptor (5-HT2A) in the spinal tissues of the SCI rats. The spasticity-strong group had significantly decreased rate-dependent depression of the H-reflex compared to the spasticity-weak group. The area of 5-HT2A receptor immunoreactivity was significantly increased in the spasticity-strong group. Thus, both electrophysiological and histological evaluations indicate that the spasticity-strong group presented with a more severe upper motor neuron syndrome. We also observed the groups in their cages for 20 hours. Our results suggest that the swimming test provides an accurate evaluation of spasticity in this contusive SCI model. We believe that the swimming test is an effective method for evaluating spastic behaviors and developing treatments targeting spasticity after SCI. PMID:28182676

The swimming test is effective for evaluating spasticity after contusive spinal cord injury.

PubMed

Ryu, Youngjae; Ogata, Toru; Nagao, Motoshi; Kitamura, Taku; Morioka, Kazuhito; Ichihara, Yoshinori; Doi, Toru; Sawada, Yasuhiro; Akai, Masami; Nishimura, Ryohei; Fujita, Naoki

2017-01-01

Spasticity is a frequent chronic complication in individuals with spinal cord injury (SCI). However, the severity of spasticity varies in patients with SCI. Therefore, an evaluation method is needed to determine the severity of spasticity. We used a contusive SCI model that is suitable for clinical translation. In this study, we examined the feasibility of the swimming test and an EMG for evaluating spasticity in a contusive SCI rat model. Sprague-Dawley rats received an injury at the 8th thoracic vertebra. Swimming tests were performed 3 to 6 weeks after SCI induction. We placed the SCI rats into spasticity-strong or spasticity-weak groups based on the frequency of spastic behavior during the swimming test. Subsequently, we recorded the Hoffman reflex (H-reflex) and examined the immunoreactivity of serotonin (5-HT) and its receptor (5-HT2A) in the spinal tissues of the SCI rats. The spasticity-strong group had significantly decreased rate-dependent depression of the H-reflex compared to the spasticity-weak group. The area of 5-HT2A receptor immunoreactivity was significantly increased in the spasticity-strong group. Thus, both electrophysiological and histological evaluations indicate that the spasticity-strong group presented with a more severe upper motor neuron syndrome. We also observed the groups in their cages for 20 hours. Our results suggest that the swimming test provides an accurate evaluation of spasticity in this contusive SCI model. We believe that the swimming test is an effective method for evaluating spastic behaviors and developing treatments targeting spasticity after SCI.

Medial gastrocnemius structure and gait kinetics in spastic cerebral palsy and typically developing children: A cross-sectional study.

PubMed

Martín Lorenzo, Teresa; Rocon, Eduardo; Martínez Caballero, Ignacio; Lerma Lara, Sergio

2018-05-01

To compare medial gastrocnemius muscle-tendon structure, gait propulsive forces, and ankle joint gait kinetics between typically developing children and those with spastic cerebral palsy, and to describe significant associations between structure and function in children with spastic cerebral palsy.A sample of typically developing children (n = 9 /16 limbs) and a sample of children with spastic cerebral palsy (n = 29 /43 limbs) were recruited. Ultrasound and 3-dimensional motion capture were used to assess muscle-tendon structure, and propulsive forces and ankle joint kinetics during gait, respectively.Children with spastic cerebral palsy had shorter fascicles and muscles, and longer Achilles tendons than typically developing children. Furthermore, total negative power and peak negative power at the ankle were greater, while total positive power, peak positive power, net power, total vertical ground reaction force, and peak vertical and anterior ground reaction forces were smaller compared to typically developing children. Correlation analyses revealed that smaller resting ankle joint angles and greater maximum dorsiflexion in children with spastic cerebral palsy accounted for a significant decrease in peak negative power. Furthermore, short fascicles, small fascicle to belly ratios, and large tendon to fascicle ratios accounted for a decrease in propulsive force generation.Alterations observed in the medial gastrocnemius muscle-tendon structure of children with spastic cerebral palsy may impair propulsive mechanisms during gait. Therefore, conventional treatments should be revised on the basis of muscle-tendon adaptations.

A Screening Tool to Identify Spasticity in Need of Treatment

PubMed Central

Zorowitz, Richard D.; Wein, Theodore H.; Dunning, Kari; Deltombe, Thierry; Olver, John H.; Davé, Shashank J.; Dimyan, Michael A.; Kelemen, John; Pagan, Fernando L.; Evans, Christopher J.; Gillard, Patrick J.; Kissela, Brett M.

2017-01-01

Objective To develop a clinically useful patient-reported screening tool for health care providers to identify patients with spasticity in need of treatment regardless of etiology. Design Eleven spasticity experts participated in a modified Delphi panel and reviewed and revised 2 iterations of a screening tool designed to identify spasticity symptoms and impact on daily function and sleep. Spasticity expert panelists evaluated items pooled from existing questionnaires to gain consensus on the screening tool content. The study also included cognitive interviews of 20 patients with varying spasticity etiologies to determine if the draft screening tool was understandable and relevant to patients with spasticity. Results The Delphi panel reached an initial consensus on 21 of 47 items for the screening tool and determined that the tool should have no more than 11 to 15 items and a 1-month recall period for symptom and impact items. After 2 rounds of review, 13 items were selected and modified by the expert panelists. Most patients (n = 16 [80%]) completed the cognitive interview and interpreted the items as intended. Conclusions Through the use of a Delphi panel and patient interviews, a 13-item spasticity screening tool was developed that will be practical and easy to use in routine clinical practice. PMID:27552355

Evaluation of the Effects of Sativex (THC BDS: CBD BDS) on Inhibition of Spasticity in a Chronic Relapsing Experimental Allergic Autoimmune Encephalomyelitis: A Model of Multiple Sclerosis

PubMed Central

Hilliard, A.; Stott, C.; Wright, S.; Guy, G.; Pryce, G.; Al-Izki, S.; Bolton, C.; Giovannoni, G.

2012-01-01

This study investigated the antispasticity potential of Sativex in mice. Chronic relapsing experimental allergic encephalomyelitis was induced in adult ABH mice resulting in hind limb spasticity development. Vehicle, Sativex, and baclofen (as a positive control) were injected intravenously and the “stiffness” of limbs assessed by the resistance force against hind limb flexion. Vehicle alone caused no significant change in spasticity. Baclofen (5 mg/kg) induced approximately a 40% peak reduction in spasticity. Sativex dose dependently reduced spasticity; 5 mg/kg THC + 5 mg/kg CBD induced approximately a 20% peak reduction; 10 mg/kg THC + 10 mg/kg CBD produced approximately a 40% peak reduction in spasticity. Sativex has the potential to reduce spasticity in an experimental mouse model of multiple sclerosis (MS). Baclofen reduced spasticity and served as a positive control. Sativex (10 mg/kg) was just as effective as baclofen, providing supportive evidence for Sativex use in the treatment of spasticity in MS. PMID:22928118

Outcome assessment for spasticity management in the patient with traumatic brain injury: the state of the art.

PubMed

Elovic, Elie P; Simone, Lisa K; Zafonte, Ross

2004-01-01

The objective of this article was to (1) review the engineering and medical literature to structure the available information concerning the assessment of spasticity in the neurological population; (2) to discuss the strengths and weaknesses of the different methods currently in use in spasticity assessment; and (3) make recommendations for future efforts in spasticity outcome assessment. Spasticity textbooks, Web sites, and OVID, IEEE, and Medline searches from 1966 through 2003 of spasticity, quantitative measure, or outcome assessment in the rehabilitation population were used as data sources. Over 500 articles were reviewed. Articles that discussed outcome measures used to assess interventions and evaluation of spasticity were included. Authors reviewed the articles looking at inclusion criteria, data collection, methodology, assessment methods, and conclusions for validity and relevance to this article. Issues such as clinical relevance, real-world function and lack of objectivity, and time consumed during performance are important issues for spasticity assessment. Some measures such as the Ashworth Scale remain in common use secondary to ease of use despite their obvious functional limitations. More functional outcome goals are plagued by being more time consuming and a general inability to demonstrate changes after an intervention. This may be secondary to the other factors that combine with spasticity to cause dysfunction at that level. Quantitative metrics can provide more objective measurements but their clinical relevance is sometimes problematic. The assessment of spasticity outcome is still somewhat problematic. Further work is necessary to develop measures that have real-world functional significance to both the individuals being treated and the clinicians. A lack of objectivity is still a problem. In the future it is important for clinicians and the engineers to work together in the development of better outcome measures.

Aculaser therapy for the treatment of cerebral palsy

NASA Astrophysics Data System (ADS)

Anwar, Shahzad; Nazir Khan, Malik M.; Nadeem Khan, Malik M.; Qazi, Faiza M.; Awan, Abid H.; Ammad, Haseeb U.

2012-03-01

A single, open and non comparative study was conducted at Anwar Shah Trust for C.P. & Paralysis in collaboration with the Departments of Neurology and Neurosurgery, Children Hospital Lahore, Pakistan to evaluate the effects of ACULASER THERAPY in childern suffering from Cerebral Palsy (C.P.) and associated Neurological Disorders like epilepsy, cortical blindness, spasticity, hemiplegia, paraplegia, diplegia, quadriplegia, monoplegia, sensoryneural deafness and speech disorders. In all 500 children were treated and the data was gathered during a period of 4 years from December 2006 till December 2010. These children were further classified according to the type of C.P. (spastic, athetoid, mixed) they suffered from and associated Neurological Disorders. This article shows results in C.P. childern who were treated with ACULASER THERAPY for a minimum of 08 weeks and more or had minimum of 15 treatment sessions and more. This article also shows that those childern who were given a break in the treatment for 1 month to 1 year did not show any reversal of the signs and symptoms. Analysis of the data showed that out of 342 children with Spasticity and Stiffness 294 showed marked improvement showing 87% success rate, out of 252 children with Epileptic fits, there was a significant reduction in the intensity, frequency and duration of Epileptic fits in 182 children showing 72% success rate, out of 96 children with Cortical Blindness 60 children showed improvement accounting for 63% efficacy rate, out of 210 children with Hearing Difficulties, 126 showed marked improvement accounting for 60% improvement rate, out of 380 children with Speech Disorders 244 showed improvement reflecting 64 % improvement rate, out of 192 children with Hemiplegia 142 showed improvement in movement, tone and power accounting for 74% improvement rate, out of 152 children with Quadriplegia 104 showed improvement in gross and fine motor functions showing 69% success rate and out of 116 children with Paraplegia of lower limbs 88 showed improvement in weight bearing, standing and movement accounting for 76% improvement rate.

Treating cerebral palsy with aculaser therapy

NASA Astrophysics Data System (ADS)

Anwar, Shahzad; Nazir Khan, Malik M.; Nadeem Khan, Malik M.; Qazi, Faiza M.; Awan, Abid H.; Dar, Irfan

2008-03-01

A single, open and non comparative study was conducted at Anwar Shah Trust for C.P. & Paralysis in collaboration with the Departments of Neurology and Neurosurgery, Children Hospital Lahore, Pakistan to evaluate the effects of ACULASER THERAPY in childern suffering from Cerebral Palsy (C.P.) and associated Neurological Disorders like epilepsy, cortical blindness, spasticity, hemiplegia, paraplegia, diplegia, quadriplegia, monoplegia, sensory-neural deafness and speech disorders. In all 250 childern were treated and the data was gathered during a period of 3 years from December 2003 till December 2006. These children were further classified according to the type of C.P. (spastic, athetoid, mixed) they suffered from and associated Neurological Disorders. This article shows results in C.P. childern who were treated with ACULASER THERAPY for minimum 6 weeks and more or had minimum of 15 treatment sessions and more. This article also shows that those childern who were given a break in the treatment for 1 month to 1 year did not show any reversal of the signs and symptoms. Analysis of the data showed that out of 171 children with Spasticity and Stiffness 147 showed marked improvement showing 87% success rate, out of 126 children with Epileptic fits, there was a significant reduction in the intensity, frequency and duration of Epileptic fits in 91 children showing 72% success rate, out of 48 children with Cortical Blindness 30 children showed improvement accounting for 63% efficacy rate, out of 105 children with Hearing Difficulties, 63 showed marked improvement accounting for 60% improvement rate, out of 190 children with Speech Disorders 122 showed improvement reflecting 64% improvement rate, out of 96 children with Hemiplegia 71 showed improvement in movement, tone and power accounting for 74% improvement rate, out of 76 children with Quadriplegia 52 showed improvement in gross and fine motor functions showing 69% success rate and out of 58 children with Paraplegia of lower limbs 44 showed improvement in weight bearing, standing and movement accounting for 76% improvement rate.

Using the international classification of functioning, disability and health to expand understanding of paralysis in the United States through improved surveillance.

PubMed

Fox, Michael H; Krahn, Gloria L; Sinclair, Lisa B; Cahill, Anthony

2015-07-01

Surveillance on paralysis prevalence has been conceptually and methodologically challenging. Numerous methods have been used to approximate population-level paralysis prevalence estimates leading to widely divergent prevalence estimates. To describe three phases in use of the International Classification of Functioning, Disability and Health (ICF) as a framework and planning tool for defining paralysis and developing public health surveillance of this condition. Description of the surveillance methodology covers four steps: an assessment of prior data collection efforts that included a review of existing surveys, registries and other data collection efforts designed to capture both case definitions in use and prevalence of paralysis; use of a consensus conference of experts to develop a case definition of paralysis based on the ICF rather than medical diagnostic criteria; explanation of use of the ICF framework for domains of interest to develop, cognitively test, validate and administer a brief self-report questionnaire for telephone administration on a population; and development and administration of a Paralysis Prevalence and Health Disparities Survey that used content mapping to back code items from existing national surveys to operationalize key domains. ICF coding led to a national population-based survey of paralysis that produced accurate estimates of prevalence and identification of factors related to the health of people in the U.S. living with paralysis. The ICF can be a useful tool for developing valid and reliable surveillance strategies targeting subgroups of individuals with functional disabilities such as people with paralysis and others. Published by Elsevier Inc.

Myelopathy associated with melorheostosis: a case report.

PubMed

Reznik, Mark; Fried, Guy W

2005-07-01

A man in his mid thirties presented with lower-extremity weakness and spasticity because of a myelopathy caused by a rare disorder of bone known as melorheostosis. The primary pathology involved was compression of the cord at the cervicothoracic levels by dystrophic osseous formation within the vertebral bodies. Based on a review of existing literature, it was evident that the spine is an uncommon location to find melorheostosis, making this disease entity a unique cause of myelopathy. The patient's progress was closely observed during his inpatient rehabilitation program, after he underwent spinal decompression surgery. Starting from the level of complete paralysis, he was able to regain functional strength in his legs by the end of his 2-month course. Despite the lack of reported outcomes in cases of myelopathy associated with melorheostosis, our report describes a favorable prognosis with good recovery of both strength and function.

Phaeohyphomycosis in a snow leopard (Uncia uncia) due to Cladophialophora bantiana.

PubMed

Janovsky, M; Gröne, A; Ciardo, D; Völlm, J; Burnens, A; Fatzer, R; Bacciarini, L N

2006-01-01

Phaeohyphomycosis caused by Cladophialophora bantiana was diagnosed in a 5-month-old snow leopard with spastic paralysis of the hind legs and inability to defaecate or urinate. At post-mortem examination, a greenish soft mass resembling an abscess was found on one side of the epidural space at the fourth lumbar vertebral body. Histological examination revealed a purulent meningitis with myelomalacia. Dematiaceous fungal hyphae, present within the inflammatory infiltrate, were identified as C. bantiana by culture and sequence analysis of the 18S ribosomal RNA gene. This neurotropic fungus rarely affects organs other than the brain in human beings and cats, and has been reported only occasionally in Europe. The case described suggests that phaeohyphomycosis due to C. bantiana infection may be recognized more frequently in the future and the possible involvement of organs other than the brain should be borne in mind.

Vocal cord paralysis after surgery to the descending thoracic aorta via left posterolateral thoracotomy.

PubMed

Ohta, Noriyuki; Mori, Takahiko

2007-11-01

Vocal cord paralysis is one of the frequently encountered complications after aortic surgery. However, reports of vocal cord paralysis after aortic surgery have been limited. In a retrospective cohort study of vocal cord paralysis after aortic surgery at a general hospital, we sought factors related to its development after aortic surgery to the descending thoracic aorta via left posterolateral thoracotomy. We reviewed data for a total of 69 patients who, between 1989 and 1995, underwent aortic surgery to the descending thoracic aorta. We assessed factors associated with the development of vocal cord paralysis and postoperative complications. Postoperative vocal cord paralysis appeared in 19 patients. Multiple logistic regression analysis revealed two risk factors for vocal cord paralysis: chronic dilatation of the aorta at the left subclavian artery (odds ratio = 8.67) and anastomosis proximal to the left subclavian artery (odds ratio = 17.7). The duration of mechanical ventilation was significantly prolonged for patients with vocal cord paralysis. Certain surgical factors associated with left subclavian artery increase the risk of vocal cord paralysis after surgery on the descending thoracic aorta. Vocal cord paralysis after aortic surgery did not increase aspiration pneumonia but was associated with pulmonary complications.

Quantitative evaluations of ankle spasticity and stiffness in neurological disorders using manual spasticity evaluator.

PubMed

Peng, Qiyu; Park, Hyung-Soon; Shah, Parag; Wilson, Nicole; Ren, Yupeng; Wu, Yi-Ning; Liu, Jie; Gaebler-Spira, Deborah J; Zhang, Li-Qun

2011-01-01

Spasticity and contracture are major sources of disability in people with neurological impairments that have been evaluated using various instruments: the Modified Ashworth Scale, tendon reflex scale, pendulum test, mechanical perturbations, and passive joint range of motion (ROM). These measures generally are either convenient to use in clinics but not quantitative or they are quantitative but difficult to use conveniently in clinics. We have developed a manual spasticity evaluator (MSE) to evaluate spasticity/contracture quantitatively and conveniently, with ankle ROM and stiffness measured at a controlled low velocity and joint resistance and Tardieu catch angle measured at several higher velocities. We found that the Tardieu catch angle was linearly related to the velocity, indicating that increased resistance at higher velocities was felt at further stiffer positions and, thus, that the velocity dependence of spasticity may also be position-dependent. This finding indicates the need to control velocity in spasticity evaluation, which is achieved with the MSE. Quantitative measurements of spasticity, stiffness, and ROM can lead to more accurate characterizations of pathological conditions and outcome evaluations of interventions, potentially contributing to better healthcare services for patients with neurological disorders such as cerebral palsy, spinal cord injury, traumatic brain injury, and stroke.

Modified Ashworth Scale (MAS) Model based on Clinical Data Measurement towards Quantitative Evaluation of Upper Limb Spasticity

NASA Astrophysics Data System (ADS)

Puzi, A. Ahmad; Sidek, S. N.; Mat Rosly, H.; Daud, N.; Yusof, H. Md

2017-11-01

Spasticity is common symptom presented amongst people with sensorimotor disabilities. Imbalanced signals from the central nervous systems (CNS) which are composed of the brain and spinal cord to the muscles ultimately leading to the injury and death of motor neurons. In clinical practice, the therapist assesses muscle spasticity using a standard assessment tool like Modified Ashworth Scale (MAS), Modified Tardiue Scale (MTS) or Fugl-Meyer Assessment (FMA). This is done subjectively based on the experience and perception of the therapist subjected to the patient fatigue level and body posture. However, the inconsistency in the assessment is prevalent and could affect the efficacy of the rehabilitation process. Thus, the aim of this paper is to describe the methodology of data collection and the quantitative model of MAS developed to satisfy its description. Two subjects with MAS of 2 and 3 spasticity levels were involved in the clinical data measurement. Their level of spasticity was verified by expert therapist using current practice. Data collection was established using mechanical system equipped with data acquisition system and LABVIEW software. The procedure engaged repeated series of flexion of the affected arm that was moved against the platform using a lever mechanism and performed by the therapist. The data was then analyzed to investigate the characteristics of spasticity signal in correspondence to the MAS description. Experimental results revealed that the methodology used to quantify spasticity satisfied the MAS tool requirement according to the description. Therefore, the result is crucial and useful towards the development of formal spasticity quantification model.

Haptic Recreation of Elbow Spasticity

PubMed Central

Kim, Jonghyun; Damiano, Diane L.

2013-01-01

The aim of this paper is to develop a haptic device capable of presenting standardized recreation of elbow spasticity. Using the haptic device, clinicians will be able to repeatedly practice the assessment of spasticity without requiring patient involvement, and these practice opportunities will help improve accuracy and reliability of the assessment itself. Haptic elbow spasticity simulator (HESS) was designed and prototyped according to mechanical requirements to recreate the feel of elbow spasticity. Based on the data collected from subjects with elbow spasticity, a mathematical model representing elbow spasticity is proposed. As an attempt to differentiate the feel of each score in Modified Ashworth Scale (MAS), parameters of the model were obtained respectively for three different MAS scores 1, 1+, and 2. The implemented haptic recreation was evaluated by experienced clinicians who were asked to give MAS scores by manipulating the haptic device. The clinicians who participated in the study were blinded to each other’s scores and to the given models. They distinguished the three models and the MAS scores given to the recreated models matched 100% with the original MAS scores from the patients. PMID:22275660

Passive Range of Motion in a Population-Based Sample of Children with Spastic Cerebral Palsy Who Walk

ERIC Educational Resources Information Center

McDowell, Brona C.; Salazar-Torres, Jose J.; Kerr, Claire; Cosgrove, Aidan P.

2012-01-01

-While passive range of motion (PROM) is commonly used to inform decisions on therapeutic management, knowledge of PROM of children with spastic cerebral palsy (CP) is limited. A population-based sample of 178 children with spastic CP (110 male; unilateral, n = 94; bilateral, n = 84; age range 4-17 years) and 68 typically developing children (24…

High-frequency transcutaneous electrical nerve stimulation alleviates spasticity after spinal contusion by inhibiting activated microglia in rats.

PubMed

Hahm, Suk-Chan; Yoon, Young Wook; Kim, Junesun

2015-05-01

Transcutaneous electrical nerve stimulation (TENS) can be used as a physical therapy for spasticity, but the effects of TENS on spasticity and its underlying mechanisms remain unclear. The purpose of this study was to test the effects of TENS on spasticity and the role of activated microglia as underlying mechanisms of TENS treatment for spasticity in rats with a 50-mm contusive spinal cord injury (SCI). A spinal contusion was made at the T12 spinal segment in adult male Sprague-Dawley rats using the NYU impactor. Behavioral tests for motor function were conducted before and after SCI and before and after TENS application. To assess spasticity, the modified Ashworth scale (MAS) was used before and after SCI, high-frequency (HF)/low-frequency (LF) TENS application at 3 different intensities (motor threshold [MT], 50% and 90% MT) or minocycline administration. Immunohistochemistry for microglia was performed at the lumbar spinal segments. Motor recovery reached a plateau approximately 28 days after SCI. Spasticity was well developed and was sustained above the MAS grade of 3, beginning at 28 days after SCI. HF-TENS at 90% MT significantly alleviated spasticity. Motor function did not show any significant changes with LF- or HF-TENS treatment. HF-TENS significantly reduced the proportion of activated microglia observed after SCI. Minocycline, the microglia inhibitor, also significantly alleviated spasticity with the reduction of activated microglia expression. These results suggest that HF-TENS at 90% MT alleviates spasticity in rats with SCI by inhibiting activated microglia. © The Author(s) 2014.

Barium-induced skeletal muscle paralysis in the rat, and its relationship to human familial periodic paralysis

PubMed Central

Schott, G. D.; McArdle, B.

1974-01-01

An in vivo study of skeletal muscle paralysis induced by intravenous barium chloride has been made in curarized and non-curarized rats. The influence of potassium and calcium chlorides, propranolol, ouabain, and prior adrenalectomy on the paralysis has also been studied. Paralysis is found to be due to a direct effect on skeletal muscle, and to correlate well with the development of hypokalaemia. Possible mechanisms of action of barium are discussed, and attention is drawn to the similarity between barium poisoning and hypokalaemic familial periodic paralysis. PMID:4813426

Vocal cord paralysis after aortic arch surgery: predictors and clinical outcome.

PubMed

Ohta, Noriyuki; Kuratani, Toru; Hagihira, Satoshi; Kazumi, Ken-Ichiro; Kaneko, Mitsunori; Mori, Takahiko

2006-04-01

This study is retrospective cohort study of data on vocal cord paralysis after aortic arch surgery collected during 14 years at a general hospital. We investigated factors in the development of vocal cord paralysis after aortic arch surgery and the effect of vocal cord paralysis on clinical course and outcome. We reviewed data for 182 patients who underwent aortic arch surgery for aortic arch aneurysm and aortic dissection between 1989 and 2003, of whom 58 patients had proximal aortic repair, 62 had distal arch repair, and 62 had total arch repair. We assessed factors associated with the development of vocal cord paralysis and examined in detail the clinical outcome of patients with vocal cord paralysis. Postoperative vocal cord paralysis occurred in 40 patients. Multiple logistic regression analysis revealed the following risk factors with odds ratios (OR) for vocal cord paralysis: extension of procedures into distal arch (OR, 17.0), chronic dilatation of the aorta at the left subclavian artery (OR, 9.14), and total arch repair (OR, 4.24). Adoption of open-style stent-grafts reduced the incidence of vocal cord paralysis (OR, 0.031). The postoperative occurrence of vocal cord paralysis itself emerges as an independent predictor of pulmonary complications (OR, 4.12) and leads to a longer duration of hospital stay. The risk of vocal cord paralysis after aortic arch surgery depends on surgical factors, such as aneurysmal involvement of the distal arch, or the application of newer, less invasive surgical procedures. Vocal cord paralysis after aortic arch surgery itself, under aggressive postoperative respiratory management, did not increase aspiration pneumonia but was associated with postoperative complications leading to higher hospital mortality and prolonged hospitalization.

Development of a clinical spasticity scale for evaluation of dogs with chronic thoracolumbar spinal cord injury.

PubMed

Lewis, Melissa J; Olby, Natasha J

2017-07-01

OBJECTIVE To develop a spasticity scale for dogs with chronic deficits following severe spinal cord injury (SCI) for use in clinical assessment and outcome measurement in clinical trials. ANIMALS 20 chronically paralyzed dogs with a persistent lack of hind limb pain perception caused by an acute SCI at least 3 months previously. PROCEDURES Spasticity was assessed in both hind limbs via tests of muscle tone, clonus, and flexor and extensor spasms adapted from human scales. Measurement of patellar clonus duration and flexor spasm duration and degree was feasible. These components were used to create a canine spasticity scale (CSS; overall score range, 0 to 18). Temporal variation for individual dogs and interrater reliability were evaluated. Gait was quantified with published gait scales, and CSS scores were compared with gait scores and clinical variables. Owners were questioned regarding spasticity observed at home. RESULTS 20 dogs were enrolled: 18 with no apparent hind limb pain perception and 2 with blunted responses; 5 were ambulatory. Testing was well tolerated, and scores were repeatable between raters. Median overall CSS score was 7 (range, 3 to 11), and flexor spasms were the most prominent finding. Overall CSS score was not associated with age, SCI duration, lesion location, or owner-reported spasticity. Overall CSS score and flexor spasm duration were associated with gait scores. CONCLUSIONS AND CLINICAL RELEVANCE The CSS could be used to quantify hind limb spasticity in dogs with chronic thoracolumbar SCI and might be a useful outcome measure. Flexor spasms may represent an integral part of stepping in dogs with severe SCI.

Vocal Cord Paralysis

MedlinePlus

... paralysis. Known causes may include: Injury to the vocal cord during surgery. Surgery on or near your neck or upper ... Factors that may increase your risk of developing vocal cord paralysis include: Undergoing throat or chest surgery. People who need surgery on their thyroid, throat ...

Thoracic 9 Spinal Transection-Induced Model of Muscle Spasticity in the Rat: A Systematic Electrophysiological and Histopathological Characterization

PubMed Central

Corleto, Jose A.; Bravo-Hernández, Mariana; Kamizato, Kota; Kakinohana, Osamu; Santucci, Camila; Navarro, Michael R.; Platoshyn, Oleksandr; Cizkova, Dasa; Lukacova, Nadezda; Taylor, Julian; Marsala, Martin

2015-01-01

The development of spinal hyper-reflexia as part of the spasticity syndrome represents one of the major complications associated with chronic spinal traumatic injury (SCI). The primary mechanism leading to progressive appearance of muscle spasticity is multimodal and may include loss of descending inhibitory tone, alteration of segmental interneuron-mediated inhibition and/or increased reflex activity to sensory input. Here, we characterized a chronic thoracic (Th 9) complete transection model of muscle spasticity in Sprague-Dawley (SD) rats. Isoflurane-anesthetized rats received a Th9 laminectomy and the spinal cord was transected using a scalpel blade. After the transection the presence of muscle spasticity quantified as stretch and cutaneous hyper-reflexia was identified and quantified as time-dependent changes in: i) ankle-rotation-evoked peripheral muscle resistance (PMR) and corresponding electromyography (EMG) activity, ii) Hoffmann reflex, and iii) EMG responses in gastrocnemius muscle after paw tactile stimulation for up to 8 months after injury. To validate the clinical relevance of this model, the treatment potency after systemic treatment with the clinically established anti-spastic agents baclofen (GABAB receptor agonist), tizanidine (α2-adrenergic agonist) and NGX424 (AMPA receptor antagonist) was also tested. During the first 3 months post spinal transection, a progressive increase in ankle rotation-evoked muscle resistance, Hoffmann reflex amplitude and increased EMG responses to peripherally applied tactile stimuli were consistently measured. These changes, indicative of the spasticity syndrome, then remained relatively stable for up to 8 months post injury. Systemic treatment with baclofen, tizanidine and NGX424 led to a significant but transient suppression of spinal hyper-reflexia. These data demonstrate that a chronic Th9 spinal transection model in adult SD rat represents a reliable experimental platform to be used in studying the pathophysiology of chronic spinal injury-induced spasticity. In addition a consistent anti-spastic effect measured after treatment with clinically effective anti-spastic agents indicate that this model can effectively be used in screening new anti-spasticity compounds or procedures aimed at modulating chronic spinal trauma-associated muscle spasticity. PMID:26713446

Aspects of the pharmacology of a new anthelmintic

PubMed Central

Aubry, M. L.; Cowell, Pauline; Davey, M. J.; Shevde, S.

1970-01-01

1. The pharmacological properties of an anthelmintic, pyrantel, and some of its analogues have been described and compared with piperazine in a variety of vertebrate and helminth preparations. 2. Pyrantel and its analogues in common with nicotine and decamethonium cause spastic paralysis in chicks and contracture of the chick semispinalis and toad rectus abdominis muscles. 3. In the soleus and anterior tibialis muscles of the cat, pyrantel in large amounts caused a short-lived neuromuscular block that was preceded by initial depolarization. 4. In preparations from cat and rat, pyrantel showed properties common to both competitive and depolarizing neuromuscular blocking drugs. 5. Pyrantel blocked the contracture evoked by transmural stimulation and caused a marked contracture of the worm. Piperazine caused a gradually developing reduction in the responses to transmural stimulation and no contracture. 6. Pyrantel and its analogues caused a slowly developing contracture of strip preparations of Ascaris, being more than 100 times more active than acetylcholine in this respect. Piperazine caused a relaxation of Ascaris strip preparations and in common with (+)-tubocurarine blocked the responses to acetylcholine and pyrantel analogues on this preparation. 7. Pyrantel caused depolarization and increased spike discharge frequency in single muscle cells of Ascaris, these changes being accompanied by increase in tension. Piperazine, on the other hand, caused hyperpolarization and reduction in spike discharge frequency and relaxation, and antagonized the effects of pyrantel. PMID:5417856

[Usefulness of Bolus Administration Using the FLEX Mode(Bolus Infusion Mode)for Baclofen Tolerance].

PubMed

Tanaka, Kazunori

2017-02-01

Intrathecal baclofen(ITB)is used to treat intractable spasticity of various etiologies and can provide better control of spasticity through the adjustment of the dose administered through the pump. However, in patients who develop tolerance to baclofen with the standard simple continuous mode, a sharp increase in dose becomes necessary, and spasticity can become harder to control. We investigated whether switching from the simple continuous mode to the bolus infusion mode was effective in controlling spasticity in patients with baclofen tolerance. We reported four patients undergoing ITB therapy at our facility who were considered to have developed baclofen tolerance. We observed the number of bolus infusions and total dose suitable for maintaining spasticity control after switching from the simple continuous mode to the bolus infusion mode. After switching to the bolus infusion mode, the total dose could be reduced in the short term; however, in the long term, the frequency of bolus infusions had to be increased to maintain spasticity control. Two years after changing to bolus infusion six times a day, the total dose was higher than that in the simple continuous mode for two of the four patients, and was the same level in the other two patients. Our four cases suggest that bolus infusion is effective in patients with baclofen tolerance during ITB therapy. Therefore, the conditions of bolus infusion should be further investigated.

Peripheral magnetic stimulation to decrease spasticity in cerebral palsy.

PubMed

Flamand, Véronique H; Beaulieu, Louis-David; Nadeau, Line; Schneider, Cyril

2012-11-01

Muscle spasticity in pediatric cerebral palsy limits movement and disrupts motor performance, thus its reduction is important in rehabilitation to optimize functional motor development. Our pilot study used repetitive peripheral magnetic stimulation, because this emerging technology influences spinal and cerebral synaptic transmission, and its antispastic effects were reported in adult neurologic populations. We tested whether five sessions of tibial and common peroneal nerve stimulation exerted acute and long-term effects on spasticity of the ankle plantar flexor muscles in five spastic diparetic children (mean age, 8 years and 3 months; standard deviation, 1 year and 10 months). Muscle resistance to fast stretching was measured with a manual dynamometer as a spasticity indicator. A progressive decrease was observed for the more impaired leg, reaching significance at the third session. This sustained reduction of spasticity may reflect that the peripheral stimulation improved the controls over the spinal circuitry. It thus suggests that a massive stimulation-induced recruitment of sensory afferents may be able to influence central nervous system plasticity in pediatric cerebral palsy. Copyright © 2012 Elsevier Inc. All rights reserved.

Balneotherapy in treatment of spastic upper limb after stroke.

PubMed

Erceg-Rukavina, Tatjana; Stefanovski, Mihajlo

2015-02-01

After stroke, spasticity is often the main problem that prevents functional recovery. Pain occurs in up to 70% of patients during the first year post-stroke. A total of 70 patients (30 female and 45 male) mean age (65.67) participated in prospective, controlled study. ischaemic stroke, developed spasticity of upper limb, post-stroke interval <6 months. contraindications for balneotherapy and inability to follow commands. Experimental group (Ex) (n=35) was treated with sulphurous baths (31°-33°C) and controlled group (Co) with taped water baths, during 21 days. All patients were additionally treated with kinesitherapy and cryotherapy. The outcome was evaluated using Modified Ashworth scale for spasticity and VAS scale for pain. The significance value was sat at p<0.05. To find out the effects of balneotherapy with sulphurous bath on spasticity and pain in affected upper limb. Reduction in tone of affected upper limb muscles was significant in Ex group (p<0.05). Pain decreased significantly in Ex-group (p<0.01). Our results show that balneotherapy with sulphurous water reduces spasticity and pain significantly and can help in treatment of post-stroke patients.

Probing conformational states of glutaryl-CoA dehydrogenase by fragment screening

DOE Office of Scientific and Technical Information (OSTI.GOV)

Begley, Darren W.; Davies, Douglas R.; Hartley, Robert C.

Glutaric acidemia type 1 is an inherited metabolic disorder which can cause macrocephaly, muscular rigidity, spastic paralysis and other progressive movement disorders in humans. The defects in glutaryl-CoA dehydrogenase (GCDH) associated with this disease are thought to increase holoenzyme instability and reduce cofactor binding. Here, the first structural analysis of a GCDH enzyme in the absence of the cofactor flavin adenine dinucleotide (FAD) is reported. The apo structure of GCDH from Burkholderia pseudomallei reveals a loss of secondary structure and increased disorder in the FAD-binding pocket relative to the ternary complex of the highly homologous human GCDH. After conducting amore » fragment-based screen, four small molecules were identified which bind to GCDH from B. pseudomallei. Complex structures were determined for these fragments, which cause backbone and side-chain perturbations to key active-site residues. Structural insights from this investigation highlight differences from apo GCDH and the utility of small-molecular fragments as chemical probes for capturing alternative conformational states of preformed protein crystals.« less

Mutations in PNPLA6 are linked to photoreceptor degeneration and various forms of childhood blindness

PubMed Central

Kmoch, S.; Majewski, J.; Ramamurthy, V.; Cao, S.; Fahiminiya, S.; Ren, H.; MacDonald, I.M.; Lopez, I.; Sun, V.; Keser, V.; Khan, A.; Stránecký, V.; Hartmannová, H.; Přistoupilová, A.; Hodaňová, K.; Piherová, L.; Kuchař, L.; Baxová, A.; Chen, R.; Barsottini, O.G.P.; Pyle, A.; Griffin, H.; Splitt, M.; Sallum, J.; Tolmie, J.L.; Sampson, J.R.; Chinnery, P.; Canada, Care4Rare; Banin, E.; Sharon, D.; Dutta, S.; Grebler, R.; Helfrich-Foerster, C.; Pedroso, J.L.; Kretzschmar, D.; Cayouette, M.; Koenekoop, R.K.

2015-01-01

Blindness due to retinal degeneration affects millions of people worldwide, but many disease-causing mutations remain unknown. PNPLA6 encodes the patatin-like phospholipase domain containing protein 6, also known as neuropathy target esterase (NTE), which is the target of toxic organophosphates that induce human paralysis due to severe axonopathy of large neurons. Mutations in PNPLA6 also cause human spastic paraplegia characterized by motor neuron degeneration. Here we identify PNPLA6 mutations in childhood blindness in seven families with retinal degeneration, including Leber congenital amaurosis and Oliver McFarlane syndrome. PNPLA6 localizes mostly at the inner segment plasma membrane in photo-receptors and mutations in Drosophila PNPLA6 lead to photoreceptor cell death. We also report that lysophosphatidylcholine and lysophosphatidic acid levels are elevated in mutant Drosophila. These findings show a role for PNPLA6 in photoreceptor survival and identify phospholipid metabolism as a potential therapeutic target for some forms of blindness. PMID:25574898

KAPS (kinematic assessment of passive stretch): a tool to assess elbow flexor and extensor spasticity after stroke using a robotic exoskeleton.

PubMed

Centen, Andrew; Lowrey, Catherine R; Scott, Stephen H; Yeh, Ting-Ting; Mochizuki, George

2017-06-19

Spasticity is a common sequela of stroke. Traditional assessment methods include relatively coarse scales that may not capture all characteristics of elevated muscle tone. Thus, the aim of this study was to develop a tool to quantitatively assess post-stroke spasticity in the upper extremity. Ninety-six healthy individuals and 46 individuals with stroke participated in this study. The kinematic assessment of passive stretch (KAPS) protocol consisted of passive elbow stretch in flexion and extension across an 80° range in 5 movement durations. Seven parameters were identified and assessed to characterize spasticity (peak velocity, final angle, creep (or release), between-arm peak velocity difference, between-arm final angle, between-arm creep, and between-arm catch angle). The fastest movement duration (600 ms) was most effective at identifying impairment in each parameter associated with spasticity. A decrease in peak velocity during passive stretch between the affected and unaffected limb was most effective at identifying individuals as impaired. Spasticity was also associated with a decreased passive range (final angle) and a classic 'catch and release' as seen through between-arm catch and creep metrics. The KAPS protocol and robotic technology can provide a sensitive and quantitative assessment of post-stroke elbow spasticity not currently attainable through traditional measures.

Role Of Stretching Exercises In The Management Of Constipation In Spastic Cerebral Palsy.

PubMed

Awan, Waqar Ahmed; Masood, Tahir

2016-01-01

Constipation is considered as one of the most common non-motor manifestations in cerebral palsy (CP). Along with other reasons, spasticity also contributes in developing constipation in CP, by decreasing mobility of trunk and lower extremities and abdominal viscera. Stretching exercises of upper extremities, trunk and lower extremities are routine management of spasticity in CP children. The objective of the study was to determine the role of stretching exercises in improving constipation symptoms in children with spastic cerebral palsy and to explore the association between spasticity and constipation among cerebral palsy children. Single-group Pretest-Posttest Design (Quasi Experimental Study Design). The study was conducted at Physiotherapy Department of National Institute of Rehabilitation Medicine (NIRM) Islamabad. Thirty spastic CP children - both male and female - with complaints of constipation were recruited through non-probability, convenience sampling. The mean age of the children was 7.55±1.33 years. Each child was assessed for defecation frequency (DF), constipation severity by constipation assessment scale (CAS) and level of spasticity by modified ash worth scale for spasticity (MASS) at baseline. Stretching exercises were performed for 30 seconds with five repetitions and at least once a day for six week, followed by positioning of patients in reflex inhibiting posture. Final data was collected using the same tools as done at the baseline. Paired samples t-test was used to analyse the rehabilitation-induced changes after 6 weeks. To determine association between spasticity and constipation Pearson product-moment correlation coefficient was used. The data was analysed through SPSS 20. Significant changes, compared to the baseline scores, were observed after 6 weeks of stretching exercises in MASS (2.53±0.62 Vs 1.53±0.77), DF (2.43±0.67 Vs 3.70±1.02) and CAS (7.23±1.50 Vs 5.43±1.73) with p≤0.05. The results also showed significant correlation between changes in levels of spasticity and severity of constipation (r = 0.37; p=0.04). Finally, significant correlation was present between improvement in spasticity and defecation frequency (r =-0.39; p=0.02). Stretching exercises administered for the management of spasticity in CP can significantly improve the symptoms of constipation in such children. The results of the study showed that constipation is strongly associated with level of spasticity in CP children.

Clinical analysis of cervical radiculopathy causing deltoid paralysis.

PubMed

Chang, Han; Park, Jong-Beom; Hwang, Jin-Yeun; Song, Kyung-Jin

2003-10-01

In general, deltoid paralysis develops in patients with cervical disc herniation (CDH) or cervical spondylotic radiculopathy (CSR) at the level of C4/5, resulting in compression of the C5 nerve root. Therefore, little attention has been paid to CDH or CSR at other levels as the possible cause of deltoid paralysis. In addition, the surgical outcomes for deltoid paralysis have not been fully described. Fourteen patients with single-level CDH or CSR, who had undergone anterior cervical decompression and fusion for deltoid paralysis, were included in this study. The severity of deltoid paralysis was classified into five grades according to manual motor power test, and the severity of radiculopathy was recorded on a visual analog scale (zero to ten points). The degree of improvement in both the severity of deltoid paralysis and radiculopathy following surgery was evaluated. Of 14 patients, one had C3/4 CDH, four had C4/5 CDH, three had C4/5 CSR, one had C5/6 CDH, and five had C5/6 CSR. Both deltoid paralysis and radiculopathy improved significantly with surgery (2.57+/-0.51 grades vs 4.14+/-0.66, P=0.001, and 7.64+/-1.65 points vs 3.21+/-0.58, P=0.001, respectively). In conclusion, the current study demonstrates that deltoid paralysis can develop due to CDH or CSR not only C4/5, but also at the levels of C3/4 and C5/6, and that surgical decompression significantly improves the degree of deltoid paralysis due to cervical radiculopathy.

Subarachnoid-to-Subarachnoid Shunt for Correction of Nonfunctioning Baclofen Pump in a Severe Case of Chronic Debilitating Post-Spinal Cord Injury Spasticity.

PubMed

Bakare, Adewale A; Weyhenmeyer, Jonathan; Lee, Albert

2018-02-01

Perhaps the most disabling condition seen in patients with spinal cord injury (SCI) is spasticity. Spasticity is characterized as hyperreflexia and hypertonicity as a result of damage to the supraspinal tracts in the aftermath of SCI. Intrathecal baclofen (ITB) is the mainstay therapy for spasticity unresponsive to oral baclofen. One of the problems associated with post-SCI spasticity unresponsive to ITB is the development of scar tissue that prevents the diffusion of baclofen in the desired spinal cord area. This case offers a unique strategy to deal with multilevel scar tissue. This 46-year-old paraplegic male with a T8 SCI whose spasticity had been well managed with ITB therapy for many years recently suffered intractable spasticity necessitating multiple reoperations for a nonfunctioning ITB catheter secondary to extensive scar tissue and intrathecal adhesions. Placement of a subarachnoid-to-subarachnoid shunt eliminated the problem of extensive scar tissue preventing adequate baclofen therapy. After undergoing multilevel thoracic and lumbar laminectomies with subarachnoid-to-subarachnoid spinal shunt, the patient's spasticity was finally brought under control with adequate daily baclofen infusion. This case demonstrates a creative way to address ITB catheter failure before considering other measures, such as neuroablative procedures (e.g., rhizotomy, myelotomy). This case reinforces the recommendation that ablative procedures, which have far greater complications, should be reserved for patients who have failed medical or other nonablative therapies. Copyright © 2017 Elsevier Inc. All rights reserved.

[Persistent Bilateral Vocal Cord Paralysis after General Anesthesia in a Patient with Multiple System Atrophy: A Case Report].

PubMed

Konishi, Hanako; Mizota, Toshiyuki; Fukuda, Kazuhiko

2015-06-01

We report a case of persistent bilateral vocal cord paralysis which developed after spine surgery under general anesthesia in a patient with multiple system atrophy. A 64-year-old woman was scheduled to receive spinal fusion surgery for kyphoscoliosis. She did not have apparent symptoms of vocal cord paralysis such as hoarseness before surgery. The surgery was performed smoothly under general anesthesia with endotracheal intubation. However, immediately after extubation, the patient developed severe upper airway obstruction and was re-intubated. Fiberoptic laryngoscopy revealed bilateral vocal cord abductor paralysis. Vocal cord paralysis did not improve and she received tracheotomy on the 12th day after surgery. She also showed symptoms of autonomic nervous system dysfunction and cerebellar ataxia, and was diagnosed as multiple system atrophy on postoperative day 64. We discuss differential diagnosis of persistent vocal cord paralysis after general anesthesia, and anesthetic management of a patient with multiple system atrophy.

Facial paralysis due to an occult parotid abscess.

PubMed

Orhan, Kadir Serkan; Demirel, Tayfun; Kocasoy-Orhan, Elif; Yenigül, Kubilay

2008-01-01

Facial paralysis associated with benign diseases of the parotid gland is very rare. It has been reported in approximately 16 cases of acute suppurative parotitis or parotid abscess. We presented a 45-year-old woman who developed facial paralysis secondary to an occult parotid abscess. Initially, there was no facial paralysis and the signs and symptoms were suggestive of acute parotitis, for which medical treatment was initiated. Three days later, left-sided facial palsy of HB (House-Brackmann) grade 5 developed. Ultrasonography revealed a pretragal, hypoechoic mass, 10x8 mm in size, causing inflammation in the surrounding tissue. Fine needle aspiration biopsy obtained from the mass revealed polymorphonuclear leukocytes and lymphocytes. No malignant cells were observed. The lesion was diagnosed as an occult parotid abscess. After a week, the mass disappeared and facial paralysis improved to HB grade 4. At the end of the first month, facial paralysis improved to HB grade 1. At three months, facial nerve function was nearly normal.

Preexisting severe cervical spinal cord compression is a significant risk factor for severe paralysis development in patients with traumatic cervical spinal cord injury without bone injury: a retrospective cohort study.

PubMed

Oichi, Takeshi; Oshima, Yasushi; Okazaki, Rentaro; Azuma, Seiichi

2016-01-01

The objective of this study is to investigate whether preexisting severe cervical spinal cord compression affects the severity of paralysis once patients develop traumatic cervical spinal cord injury (CSCI) without bone injury. We retrospectively investigated 122 consecutive patients with traumatic CSCI without bone injury. The severity of paralysis on admission was assessed by the American Spinal Injury Association impairment scale (AIS). The degree of preexisting cervical spinal cord compression was evaluated by the maximum spinal cord compression (MSCC) and was divided into three categories: minor compression (MSCC ≤ 20 %), moderate compression (20 % < MSCC ≤ 40 %), and severe compression (40 % < MSCC). We investigated soft-tissue damage on magnetic resonance imaging to estimate the external force applied. Other potential risk factors, including age, sex, fused vertebra, and ossification of longitudinal ligament, were also reviewed. A multivariate logistic regression analysis was performed to investigate the risk factors for developing severe paralysis (AIS A-C) on admission. Our study included 103 males and 19 females with mean age of 65 years. Sixty-one patients showed severe paralysis (AIS A-C) on admission. The average MSCC was 22 %. Moderate compression was observed in 41, and severe in 20. Soft-tissue damage was observed in 91. A multivariate analysis showed that severe cervical spinal cord compression significantly affected the severity of paralysis at the time of injury, whereas both mild and moderate compression did not affect it. Soft-tissue damage was also significantly associated with severe paralysis on admission. Preexisting severe cervical cord compression is an independent risk factor for severe paralysis once patients develop traumatic CSCI without bone injury.

Balneotherapy in Treatment of Spastic Upper Limb after Stroke

PubMed Central

Erceg-Rukavina, Tatjana; Stefanovski, Mihajlo

2015-01-01

Introduction: After stroke, spasticity is often the main problem that prevents functional recovery. Pain occurs in up to 70% of patients during the first year post-stroke. Materials and methods: A total of 70 patients (30 female and 45 male) mean age (65.67) participated in prospective, controlled study. Inclusion criteria: ischaemic stroke, developed spasticity of upper limb, post-stroke interval <6 months. Exclusion criteria: contraindications for balneotherapy and inability to follow commands. Experimental group (Ex) (n=35) was treated with sulphurous baths (31°-33°C) and controlled group (Co) with taped water baths, during 21 days. All patients were additionally treated with kinesitherapy and cryotherapy. The outcome was evaluated using Modified Ashworth scale for spasticity and VAS scale for pain. The significance value was sat at p<0.05. Goal: To find out the effects of balneotherapy with sulphurous bath on spasticity and pain in affected upper limb. Results: Reduction in tone of affected upper limb muscles was significant in Ex group (p<0.05). Pain decreased significantly in Ex-group (p<0.01). Conclusion: Our results show that balneotherapy with sulphurous water reduces spasticity and pain significantly and can help in treatment of post-stroke patients. PMID:25870474

Poststroke chronic disease management: towards improved identification and interventions for poststroke spasticity-related complications.

PubMed

Brainin, Michael; Norrving, Bo; Sunnerhagen, Katharina S; Goldstein, Larry B; Cramer, Steven C; Donnan, Geoffrey A; Duncan, Pamela W; Francisco, Gerard; Good, David; Graham, Glenn; Kissela, Brett M; Olver, John; Ward, Anthony; Wissel, Jörg; Zorowitz, Richard

2011-02-01

This paper represents the opinion of a group of researchers and clinicians with an established interest in poststroke care and is based on the recognised need for long-term care following stroke, especially in view of the global increase of disability due to stroke. Among the more frequent long-term complications following stroke are spasticity-related disabilities. Although spasticity alone occurs in up to 60% of stroke survivors, disabling spasticity affects only 4-10%. Spasticity further interferes with important functions of daily life when it occurs in association with pain, motor impairment, and overall declines of cognitive and neurological function. It is proposed that the aftermath of stroke be considered a chronic disease requiring a multifactorial and multilevel approach. There are, however, knowledge gaps related to the prediction and recognition of poststroke disability. Interventions to prevent or minimise such disabilities require further development and evaluation. Poststroke spasticity research should focus on reducing disability and be considered as part of a continuum of chronic care requirements and should be recognised as a part of a comprehensive poststroke disease management programme. © 2011 The Authors. International Journal of Stroke © 2011 World Stroke Organization.

Transverse forces versus modified ashworth scale for upper limb flexion/extension in para-sagittal plane.

PubMed

Seth, Nitin; Johnson, Denise; Abdullah, Hussein A

2017-07-01

Spasticity is a common impairment following an upper motor neuron lesion in conditions such as stroke and brain injury. A clinical issue is how to best quantify and measure spasticity. Recently, research has been performed to develop new methods of spasticity quantification using various systems. This paper follows up on previous work taking a closer look at the role of transversal forces obtained via rehabilitation robot for motions in the para-sagittal plane. Results from 45 healthy individuals and 40 individuals with acquired brain injury demonstrate that although the passive upper motions are vertical, horizontal forces into and away from the individual's body demonstrate a relationship with the Modified Ashworth Scale. This finding leads the way to new avenues of spasticity quantification and monitoring.

Further Studies on the Prevalence of Isolated Sleep Paralysis in Black Subjects

PubMed Central

Bell, Carl C.; Dixie-Bell, Dora D.; Thompson, Belinda

1986-01-01

In a previous study, one of the authors (C.C.B.) found isolated sleep paralysis was common in blacks. In this study, conducted by interviews, a recurrent pattern (one or more episodes per month) of isolated sleep paralysis episodes in blacks was described by at least 25 percent of the afflicted sample studied. Frequent episodes were associated with stress, and subjects with isolated sleep paralysis had an unusually high prevalence of panic disorder (15.5 percent). The genetic transmission of sleep paralysis was studied in a large black family, and in addition to stressful environmental factors being associated with the condition, there appears to be a dominant genetic factor associated with the predisposition for developing sleep paralysis. The implications of these findings for stress, anxiety, sleep, and psychophysiologic disorders are discussed. PMID:3746934

Pathophysiology, assessment and management of multiple sclerosis spasticity: an update.

PubMed

Haas, Judith

2011-04-01

Spasticity is one of the most common and disabling symptoms associated with multiple sclerosis (MS). MS spasticity occurs through both myelin and nerve fiber (axonal) degradation, which commence in the early stages of the disease. More than 80% of MS patients experienced spasticity in a large UK survey, with more than 50% of patients reporting their spasticity to be `moderate' or `severe'. Data from a large US registry show that patients with moderate-to-severe MS spasticity experience levels of disability that correlate closely with being wheelchair-bound and/or bedridden. The Ashworth scale is the most commonly used scale for assessing the degree of MS spasticity. However, the validity, reliability and sensitivity of this scale have been challenged and it is not considered an ideal scale for assessing the severity of MS spasticity. The numerical rating scale, a well-established standard pain assessment tool, provides a reliable, valid and simplified scale for patient self-rated assessment of the mean level of spasticity over the previous 24 h (0 = no spasticity, 10 = worst possible spasticity). According to data from the German MS Register, almost a third of MS patients with spasticity were untreated. Despite the availability of oral agents for generalized spasticity (often used in conjunction with physical/rehabilitation management strategies), including baclofen, tizanidine, dantrolene and gabapentin, there is limited clinical evidence to support their use and there is a need for improved and better tolerated pharmacological therapies for MS spasticity. The endocannabinoid system modulator, Sativex(®) (nabiximols, USAN name), provides an alternative therapeutic approach in the management of MS spasticity.

The efficacy of diazepam in enhancing motor function in children with spastic cerebral palsy.

PubMed

Mathew, Anna; Mathew, M C; Thomas, Molly; Antonisamy, B

2005-04-01

Muscle spasm and hypertonia limit mobility in children with spastic cerebral palsy. This double-blind, placebo-controlled, randomized controlled clinical trial studies the clinical efficacy of a low dose of diazepam in enhancing movement in children with spastic cerebral palsy. One hundred and eighty children fulfilled the criteria and were randomly allocated to receive one of two doses of diazepam or placebo at bedtime; 173 completed the study. There was a significant reduction of hypertonia, improvement in the range of passive movement, and an increase in spontaneous movement in the children who received diazepam. There was no report of daytime drowsiness. In developing countries, where cost factors often determine choice of drug, diazepam is a cheap and effective way of relieving spasm and stiffness, optimizing physical therapy and facilitating movement in children with spasticity.

Communication skills in individuals with spastic diplegia.

PubMed

Lamônica, Dionísia Aparecida Cusin; Paiva, Cora Sofia Takaya; Abramides, Dagma Venturini Marques; Biazon, Jamile Lozano

2015-01-01

To assess communication skills in children with spastic diplegia. The study included 20 subjects, 10 preschool children with spastic diplegia and 10 typical matched according to gender, mental age, and socioeconomic status. Assessment procedures were the following: interviews with parents, Stanford - Binet method, Gross Motor Function Classification System, Observing the Communicative Behavior, Vocabulary Test by Peabody Picture, Denver Developmental Screening Test II, MacArthur Development Inventory on Communicative Skills. Statistical analysis was performed using the values of mean, median, minimum and maximum value, and using Student's t-test, Mann-Whitney test, and Paired t-test. Individuals with spastic diplegia, when compared to their peers of the same mental age, presented no significant difference in relation to receptive and expressive vocabulary, fine motor skills, adaptive, personal-social, and language. The most affected area was the gross motor skills in individuals with spastic cerebral palsy. The participation in intervention procedures and the pairing of participants according to mental age may have approximated the performance between groups. There was no statistically significant difference in the comparison between groups, showing appropriate communication skills, although the experimental group has not behaved homogeneously.

A cost-effectiveness model for the use of a cannabis-derived oromucosal spray for the treatment of spasticity in multiple sclerosis.

PubMed

Gras, Adrien; Broughton, Julie

2016-12-01

Severity of spasticity in multiple sclerosis (MS) directly correlates with the level and cost of care required. This study assessed whether a tetrahydrocannabinol/cannabidiol (THC/CBD) oromucosal spray for treatment of moderate-severe MS spasticity is a cost-effective use of healthcare resources in Wales. A Markov model was developed to compare THC/CBD plus standard of care (SoC) treatments with SoC alone. At 30 years, total incremental cost for THC/CBD plus SoC treatment was estimated at £3,836/patient (ICER: £10,891/quality-adjusted life year [QALY]). Hospital admission costs had the greatest effect on the base case ICER. Inclusion of carer cost led to incremental cost of -£33,609/patient (ICER: -£95,423/QALY). The THC/CBD spray was found to be cost-effective for the treatment of spasticity in MS, and dominant, if home carer costs were included. Use of THC/CBD has the potential to generate cost savings by significantly improving the symptoms of moderate to severe MS spasticity.

Lower sulfurtransferase detoxification rates of cyanide in konzo-A tropical spastic paralysis linked to cassava cyanogenic poisoning.

PubMed

Kambale, K J; Ali, E R; Sadiki, N H; Kayembe, K P; Mvumbi, L G; Yandju, D L; Boivin, M J; Boss, G R; Stadler, D D; Lambert, W E; Lasarev, M R; Okitundu, L A; Mumba Ngoyi, D; Banea, J P; Tshala-Katumbay, D D

2017-03-01

Using a matched case-control design, we sought to determine whether the odds of konzo, a distinct spastic paraparesis associated with food (cassava) cyanogenic exposure in the tropics, were associated with lower cyanide detoxification rates (CDR) and malnutrition. Children with konzo (N=122, 5-17 years of age) were age- and sex-matched with presumably healthy controls (N=87) and assessed for motor and cognition performances, cyanogenic exposure, nutritional status, and cyanide detoxification rates (CDR). Cyanogenic exposure was ascertained by thiocyanate (SCN) concentrations in plasma (P-SCN) and urine (U-SCN). Children with a height-for-age z-score (HAZNCHS)<-2 were classified as nutritionally stunted. CDR was measured as time required to convert cyanide to SCN, and expressed as ms/μmol SCN/mg protein or as mmolSCN/ml plasma/min. Mean (SD) U-SCN in children with konzo was 521.9 (353.6) μmol/l and was, significantly higher than 384.6 (223.7) μmol/l in those without konzo. Conditional regression analysis of data for age- and sex- matched case-control pairs showed that konzo was associated with stunting (OR: 5.8; 95% CI: 2.7-12.8; p<0.01; N=83 paired groups) and higher U-SCN (OR: 1.1; 95% CI: 1.02-1.20 per 50-μmol increase in U-SCN; p=0.02; N=47 paired groups). After adjusting for stunting and U-SCN, the odds of developing konzo was reduced by 63% (95% CI: 11-85%, p=0.03; N=41 paired groups) for each 5mmol SCN/(ml plasma/min)-increase in CDR. Linear regression analysis indicated a significant association between BOT-2 or KABC-II scores and both the HAZNCHS z-score and the U-SCN concentration, but not the CDR. Our findings provide evidence in support of interventions to remove cyanogenic compounds from cassava prior to human consumption or, peharps, enhance the detoxification of cyanide in those relying on the cassava as the main source of food. Copyright © 2016 Elsevier B.V. All rights reserved.

Lower sulfurtransferase detoxification rates of cyanide in konzo—A tropical spastic paralysis linked to cassava cyanogenic poisoning

PubMed Central

Kambale, K.J.; Ali, E.R.; Sadiki, N.H.; Kayembe, K.P.; Mvumbi, L.G.; Yandju, D.L.; Boivin, M.J.; Boss, G.R.; Stadler, D.D.; Lambert, W.E.; Lasarev, M.R.; Okitundu, L.A.; Ngoyi, D. Mumba; Banea, J.P.; Tshala-Katumbay, D.D.

2016-01-01

Using a matched case-control design, we sought to determine whether the odds of konzo, a distinct spastic paraparesis associated with food (cassava) cyanogenic exposure in the tropics, were associated with lower cyanide detoxification rates (CDR) and malnutrition. Children with konzo (N= 122, 5–17 years of age) were age- and sex-matched with presumably healthy controls (N = 87) and assessed for motor and cognition performances, cyanogenic exposure, nutritional status, and cyanide detoxification rates (CDR). Cyanogenic exposure was ascertained by thiocyanate (SCN) concentrations in plasma (P-SCN) and urine (U-SCN). Children with a height-for-age z-score (HAZNCHS) < −2 were classified as nutritionally stunted. CDR was measured as time required to convert cyanide to SCN, and expressed as ms/μmol SCN/mg protein or as mmolSCN/ml plasma/min. Mean (SD) U-SCN in children with konzo was 521.9 (353.6) μmol/l and was, significantly higher than 384.6 (223.7) μmol/l in those without konzo. Conditional regression analysis of data for age- and sex- matched case-control pairs showed that konzo was associated with stunting (OR: 5.8; 95% CI: 2.7–12.8; p <0.01; N = 83 paired groups) and higher U-SCN (OR: 1.1; 95% CI: 1.02–1.20 per 50-μmol increase in U-SCN; p = 0.02; N = 47 paired groups). After adjusting for stunting and U-SCN, the odds of developing konzo was reduced by 63% (95% CI: 11–85%, p = 0.03; N = 41 paired groups) for each 5 mmol SCN/(ml plasma/min)-increase in CDR. Linear regression analysis indicated a significant association between BOT-2 or KABC-II scores and both the HAZNCHS z-score and the U-SCN concentration, but not the CDR. Our findings provide evidence in support of interventions to remove cyanogenic compounds from cassava prior to human consumption or, peharps, enhance the detoxification of cyanide in those relying on the cassava as the main source of food. PMID:27246648

Advances in the management of multiple sclerosis symptoms: pathophysiology and assessment of spasticity in multiple sclerosis.

PubMed

Tintoré, Mar

2015-01-01

Spasticity is a prevalent and troublesome symptom for people with multiple sclerosis (MS). Common instruments to measure MS spasticity include the clinician-rated (modified) Ashworth scale and the patient-rated 0-10 spasticity Numerical Rating Scale (NRS). Current opinion is that measurement of MS spasticity should incorporate the patient's perspective. Other instruments to assess spasticity-associated symptoms such as the Penn spasms frequency scale, sleep quality NRS and pain NRS can assist in tracking MS spasticity evolution and inform management choices. Worsening spasticity reduces patient autonomy, impacts negatively on quality of life and increases health resource utilization and costs. Despite the wide range of issues associated with MS spasticity, undertreatment is common and standard treatment options (physiotherapy and classical oral therapies) often fail to provide adequate symptomatic control.

Sit-to-stand movement changes in preschool-aged children with spastic diplegia following one neurodevelopmental treatment session--a pilot study.

PubMed

Yonetsu, Ryo; Iwata, Akira; Surya, John; Unase, Kazunori; Shimizu, Junichi

2015-01-01

This study was designed to provide a better understanding of how a single neurodevelopmental treatment (NDT) session affects sit-to-stand (STS) movements in children with cerebral palsy (CP). Eight children with spastic diplegia and five typically developing children, aged 4-6 years, participated in this study. The CP participants performed STS movements immediately before and after a 40-min NDT session. Using a three-dimensional, four-camera analysis system, angular movements involving the hip, knee and ankle joints of the participants were obtained. During forward tilt of the trunk, the maximum and final angles after the NDT session significantly decreased compared with those before the session (p < 0.05, p < 0.01). Moreover, the final hip flexion after the session also significantly decreased compared with that before the session (p < 0.01). On the other hand, the initial, maximum and final ankle dorsiflexion angles after the session were significantly greater (p < 0.05, p < 0.01 and p < 0.05, respectively) than before the session. These findings suggest that a single NDT session enables children with CP to stand from a seated position without using some atypical movement patterns. Preschool-aged children with spastic diplegia, with limited ability to independently transfer from a sitting position, and dependent on a wheelchair for mobility experience obstacles to enhanced activities of daily life and social participation. A single neurodevelopmental treatment session would enable children with spastic diplegia to perform sit-to-stand movements more efficiently, with selective muscle control. Understanding how a single neurodevelopmental treatment session affects sit-to-stand movements in children with spastic diplegia is invaluable for therapists planning more efficient therapeutic programs and may enable children with spastic diplegia to develop improved mobility.

Summary of evidence-based guideline: complementary and alternative medicine in multiple sclerosis: report of the guideline development subcommittee of the American Academy of Neurology.

PubMed

Yadav, Vijayshree; Bever, Christopher; Bowen, James; Bowling, Allen; Weinstock-Guttman, Bianca; Cameron, Michelle; Bourdette, Dennis; Gronseth, Gary S; Narayanaswami, Pushpa

2014-03-25

To develop evidence-based recommendations for complementary and alternative medicine (CAM) in multiple sclerosis (MS). We searched the literature (1970-March 2011; March 2011-September 2013 MEDLINE search), classified articles, and linked recommendations to evidence. Clinicians might offer oral cannabis extract for spasticity symptoms and pain (excluding central neuropathic pain) (Level A). Clinicians might offer tetrahydrocannabinol for spasticity symptoms and pain (excluding central neuropathic pain) (Level B). Clinicians should counsel patients that these agents are probably ineffective for objective spasticity (short-term)/tremor (Level B) and possibly effective for spasticity and pain (long-term) (Level C). Clinicians might offer Sativex oromucosal cannabinoid spray (nabiximols) for spasticity symptoms, pain, and urinary frequency (Level B). Clinicians should counsel patients that these agents are probably ineffective for objective spasticity/urinary incontinence (Level B). Clinicians might choose not to offer these agents for tremor (Level C). Clinicians might counsel patients that magnetic therapy is probably effective for fatigue and probably ineffective for depression (Level B); fish oil is probably ineffective for relapses, disability, fatigue, MRI lesions, and quality of life (QOL) (Level B); ginkgo biloba is ineffective for cognition (Level A) and possibly effective for fatigue (Level C); reflexology is possibly effective for paresthesia (Level C); Cari Loder regimen is possibly ineffective for disability, symptoms, depression, and fatigue (Level C); and bee sting therapy is possibly ineffective for relapses, disability, fatigue, lesion burden/volume, and health-related QOL (Level C). Cannabinoids may cause adverse effects. Clinicians should exercise caution regarding standardized vs nonstandardized cannabis extracts and overall CAM quality control/nonregulation. Safety/efficacy of other CAM/CAM interaction with MS disease-modifying therapies is unknown.

[Management of symptoms associated with spasticity in patients with multiple sclerosis].

PubMed

Fernández-Pablos, María Asunción; Costa-Frossard, Lucienne; García-Hernández, Carlos; García-Montes, Inmaculada; Escutia-Roig, Matilde

To describe the role of nurses in the management of symptoms related to spasticity in patients with multiple sclerosis (MS). A descriptive study was developed based on a questionnaire on spasticity in MS patients. The questionnarie was completed through an anonymous tele-voting system at a national meeting with nurses involved in the management of these patients. Apart from fatigue, according to the opinion of the participants, the spasticity symptom associated with MS most notified by patients was difficulty in walking, followed by spasms and pain. Participants thought that it is important that nursing takes: 1) a role in identifying these symptoms, 2) should focus on the detection of the triggering or aggravating factors, and 3) on providing support in the assessment of the level of spasticity. It is important to inform about the correct use of anti-spasticity drugs, how to adjust the dosage and side effects of treatments, including cannabinoids via an oromucosal spray, titrating its doses according to each patient, and monitoring its tolerability, efficacy and adherence. Although there are usually resources to follow up these patients, there are still important gaps, including the lack of a specific follow-up protocol. Although all the participants are experts in the management of patients with MS, there is still diversity in the functions they perform, and the available resources they have in their hospitals. Nurses act as a key element in the process of identification of symptoms, training and monitoring of these patients with spasticity in EM. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

Evaluation of Stiffness of the Spastic Lower Extremity Muscles in Early Spinal Cord Injury by Acoustic Radiation Force Impulse Imaging

PubMed Central

Cho, Kang Hee

2015-01-01

Objective To investigate intrinsic viscoelastic changes using shear wave velocities (SWVs) of spastic lower extremity muscles in patients with early spinal cord injury (SCI) via acoustic radiation force impulse (ARFI) imaging and to evaluate correlation between the SWV values and spasticity. Methods Eighteen patients with SCI within 3 months and 10 healthy adults participated. We applied the ARFI technique to measure SWV of gastrocnemius muscle (GCM) and long head of biceps femoris muscle. Spasticity of ankle and knee joint was assessed by original Ashworth Scale. Results Ten patients with SCI had spasticity. Patients with spasticity had significantly faster SWV for GCM and biceps femoris muscle than those without spasticity (Mann-Whitney U test, p=0.007 and p=0.008) and normal control (p=0.011 and p=0.037, respectively). The SWV values of GCM correlated with the ankle spasticity (Spearman rank teat, p=0.026). There was significant correlation between the SWV values for long head of biceps femoris muscle and knee spasticity (Spearman rank teat, p=0.022). Conclusion ARFI demonstrated a difference in muscle stiffness in the GCM between patients with spastic SCI and those without spasticity. This finding suggested that stiffness of muscles increased in spastic lower extremity of early SCI patients. ARFI imaging is a valuable tool for noninvasive assessment of the stiffness of the spastic muscle and has the potential to identify pathomechanical changes of the tissue associated with SCI. PMID:26161345

Management of Spasticity in Children with Cerebral Palsy

PubMed Central

Shamsoddini, Alireza; Amirsalari, Susan; Hollisaz, Mohammad-Taghi; Rahimnia, Alireza; Khatibi-Aghda, Amideddin

2014-01-01

Cerebral palsy is the most common cause of spasticity and physical disability in children and spasticity is one of the commonest problems in those with neurological disease. The management of spasticity in children with cerebral palsy requires a multidisciplinary effort and should be started as early as possible. There are a number of treatments available for the management of spasticity. This article reviews the variety of options available for the clinical management of spasticity. PMID:25755853

The relationship between spasticity and gross motor capability in nonambulatory children with spastic cerebral palsy.

PubMed

Katusic, Ana; Alimovic, Sonja

2013-09-01

Spasticity has been considered as a major impairment in cerebral palsy (CP), but the relationship between this impairment and motor functions is still unclear, especially in the same group of patients with CP. The aim of this investigation is to determine the relationship between spasticity and gross motor capability in nonambulatory children with spastic CP. Seventy-one children (30 boys, 41 girls) with bilateral spastic cerebral palsy and with Gross Motor Function Classification System (GMFCS) levels IV (n=34) and V (n=37) were included in the study. The spasticity level in lower limbs was evaluated using the Modified Modified Ashworth Scale and the gross motor function with the Gross Motor Function Measure (GMFM-88). Spearman's correlation analysis was used to determine the nature and the strength of the relationship. The results showed a moderate correlation between spasticity and gross motor skills (ρ=0.52 for the GMFCS level; ρ=0.57 for the GMFM-88), accounting for less than 30% of the explained variance. It seems that spasticity is just one factor among many others that could interfere with gross motor skills, even in children with severe forms of spastic CP. Knowledge of the impact of spasticity on motor skills may be useful in the setting of adequate rehabilitation strategies for nonambulatory children with spastic CP.

The Place of Operations upon the Sympathetic System in the Treatment of Poliomyelitis.

PubMed

Ogilvie, W H

1933-02-01

Revived interest in sympathetic surgery originated in orthopaedics. Royle's theories and operations. Their trial, failure and final abandonment. Value of sympathetic operations widely investigated; while finality has not been reached they have proved effective for three main purposes: (1) Relief of pain especially in bladder diseases. (2) Removal of inhibition and sphincteric spasm in alimentary, anal and bladder diseases. (3) Production of vaso-dilatation in (a) vaso-spastic diseases; (b) vaso-degenerative diseases; (c) conditions not due to arterial disease in which increased blood supply is beneficial.Poliomyelitis falls into the last group.-Cause of poor blood supply uncertain; ? lack of function; ? upset of some reflex; ? paralysis of vaso-dilators.TWO PROBLEMS ARISE, BOTH OF WHICH MAY BE TREATED BY OPERATIONS ON THE SYMPATHETIC: (1) The cold, blue limb, which develops chilblains, sores, or even deep ulcers every winter, often stopping treatment and requiring patient to be confined to bed. (2) The limb with considerable and rapidly increasing shortening. Sometimes these limbs show a fair return of power, and were it not for the heavy boot made necessary by the shortening, the patient could be made to walk well.Method of attack.-(1) Periarterial sympathectomy; (2) ramisectomy; (3) ganglionectomy. Physiological basis of each. Criticism of (1) and (2).Details of the operation for ganglionectomy.-Alternative approaches and their advantages. The immediate and late results of the procedure.Five cases discussed briefly.

[Myths and evidence on the use of botulinum toxin: spasticity in adults and in children with cerebral palsy].

PubMed

Martinez-Castrillo, J C; Pena-Segura, J L; Sanz-Cartagena, P; Alonso-Curco, X; Arbelo-Gonzalez, J M; Arriola-Pereda, G; Coll-Bosch, M D; Conejero-Casares, J A; Garcia-Ribes, A; Jauma-Classen, S; Pagonabarraga, J; Vidal-Valls, J; Garcia-Ruiz, P J

2017-05-16

Spasticity is a medical problem with a high incidence that significantly impact on the quality of life of patients and their families. To analyze and to answer different questions about the use of botulinum toxin type A (BTA) in our clinical practice. A group of experts in neurology develop a list of topics related with the use of BTA. Two big groups were considered: spasticity in adults and in children with cerebral palsy. A literature search at PubMed for English, French, and Spanish language articles published up to June 2016 was performed. The manuscript was structured as a questionnaire that includes those questions that, according to the panel opinion, could generate more controversy or doubt. The initial draft was reviewed by the expert panel members to allow for modifications, and after subsequent revisions for achieving the highest degree of consensus, the final text was then validated. Different questions about diverse aspects of spasticity in adults, such as methods for evaluating spasticity, infiltration techniques, doses, number of infiltration points, etc. Regarding spasticity in children with cerebral palsy, the document included questions about minimum age of infiltration, methods of analgesia, etc. This review is a tool for continuous training for neurologist and rehabilitation specialist and residents of both specialties, about different specific areas of the management of BTA.

Botulinum toxin therapy for treatment of spasticity in multiple sclerosis: review and recommendations of the IAB-Interdisciplinary Working Group for Movement Disorders task force.

PubMed

Dressler, Dirk; Bhidayasiri, Roongroj; Bohlega, Saeed; Chahidi, Abderrahmane; Chung, Tae Mo; Ebke, Markus; Jacinto, L Jorge; Kaji, Ryuji; Koçer, Serdar; Kanovsky, Petr; Micheli, Federico; Orlova, Olga; Paus, Sebastian; Pirtosek, Zvezdan; Relja, Maja; Rosales, Raymond L; Sagástegui-Rodríguez, José Alberto; Schoenle, Paul W; Shahidi, Gholam Ali; Timerbaeva, Sofia; Walter, Uwe; Saberi, Fereshte Adib

2017-01-01

Botulinum toxin (BT) therapy is an established treatment of spasticity due to stroke. For multiple sclerosis (MS) spasticity this is not the case. IAB-Interdisciplinary Working Group for Movement Disorders formed a task force to explore the use of BT therapy for treatment of MS spasticity. A formalised PubMed literature search produced 55 publications (3 randomised controlled trials, 3 interventional studies, 11 observational studies, 2 case studies, 35 reviews, 1 guideline) all unanimously favouring the use of BT therapy for MS spasticity. There is no reason to believe that BT should be less effective and safe in MS spasticity than it is in stroke spasticity. Recommendations include an update of the current prevalence of MS spasticity and its clinical features according to classifications used in movement disorders. Immunological data on MS patients already treated should be analysed with respect to frequencies of MS relapses and BT antibody formation. Registration authorities should expand registration of BT therapy for spasticity regardless of its aetiology. MS specialists should consider BT therapy for symptomatic treatment of spasticity.

Classification of Posture in Poststroke Upper Limb Spasticity: A Potential Decision Tool for Botulinum Toxin A Treatment?

ERIC Educational Resources Information Center

Hefter, Harald; Jost, Wolfgang H.; Reissig, Andrea; Zakine, Benjamin; Bakheit, Abdel Magid; Wissel, Jorg

2012-01-01

A significant percentage of patients suffering from a stroke involving motor-relevant central nervous system regions will develop a spastic movement disorder. Hyperactivity of different muscle combinations forces the limbs affected into abnormal postures or movement patterns. As muscular hyperactivity can effectively and safely be treated with…

Effect of Task Constraint on Reaching Performance in Children with Spastic Diplegic Cerebral Palsy

ERIC Educational Resources Information Center

Ju, Yun-Huei; You, Jia-Yuan; Cherng, Rong-Ju

2010-01-01

The purposes of the study were to examine the effect of task constraint on the reaching performance in children with spastic cerebral palsy (CP) and to examine the correlations between the reaching performance and postural control. Eight children with CP and 16 typically developing (TD) children participated in the study. They performed a…

Location and severity of spasticity in the first 1-2 weeks and at 3 and 18 months after stroke.

PubMed

Welmer, A-K; Widén Holmqvist, L; Sommerfeld, D K

2010-05-01

There is no consensus concerning the location or severity of spasticity, or how this changes with time after stroke. The purpose was to describe: the location and severity of spasticity, in different muscle groups, during the first 1-2 weeks and at 3 and 18 months after stroke; the association between the severity of spasticity and control of voluntary movements; and the occurrence of spasticity in younger versus older patients. In a cohort of consecutive patients, the following parameters were assessed during the first 1-2 weeks (n = 109) and at 3 (n = 95) and 18 (n = 66) months after first-ever stroke: spasticity, by the Modified Ashworth Scale in different muscle groups; plantar-flexor clonus, by physical examination; and movement function, by the Lindmark Motor Assessment Scale. During the first 1-2 weeks and at 3 months after stroke, spasticity was most common in the anti-gravity muscles. The severity of upper extremity spasticity increased over time (P < 0.05). Upper extremity spasticity and movement scores were moderately associated (r = -0.61, P < 0.05). At 3 months, spasticity was more common amongst the younger patients (P < 0.05). The results confirm that spasticity is most common in the anti-gravity muscles and is associated with the control of voluntary movements. As the severity of spasticity also increased after 3 months, when neurally mediated spasticity is expected to have passed its peak, intrinsic muscle changes may play a larger role than neural components with the passage of time after stroke.

Spasticity therapy reacts to astrocyte GluA1 receptor upregulation following spinal cord injury

PubMed Central

Gómez-Soriano, Julio; Goiriena, Eider; Taylor, Julian

2010-01-01

For almost three decades intrathecal baclofen therapy has been the standard treatment for spinal cord injury spasticity when oral medication is ineffective or produces serious side effects. Although intrathecal baclofen therapy has a good clinical benefit-risk ratio for spinal spasticity, tolerance and the life-threatening withdrawal syndrome present serious problems for its management. Now, in an experimental model of spinal cord injury spasticity, AMPA receptor blockade with NGX424 (Tezampanel) has been shown to reduce stretch reflex activity alone and during tolerance to intrathecal baclofen therapy. These results stem from the observation that GluA1 receptors are overexpressed on reactive astrocytes following experimental ischaemic spinal cord injury. Although further validation is required, the appropriate choice of AMPA receptor antagonists for treatment of stretch hyperreflexia based on our recent understanding of reactive astrocyte neurobiology following spinal cord injury may lead to the development of a better adjunct clinical therapy for spasticity without the side effects of intrathecal baclofen therapy. LINKED ARTICLE This article is a commentary on Oshiro et al., pp. 976–985 of this issue. To view this paper visit http://dx.doi.org/10.1111/j.1476-5381.2010.00954.x PMID:20662840

Site-specific transmission of a floor-based, high-frequency, low-magnitude vibration stimulus in children with spastic cerebral palsy

PubMed Central

Singh, Harshvardhan; Whitney, Daniel G; Knight, Christopher A; Miller, Freeman; Manal, Kurt; Kolm, Paul; Modlesky, Christopher M

2016-01-01

Objective To determine the degree to which a high-frequency, low-magnitude vibration (HLV) signal emitted by a floor-based platform transmits to the distal tibia and distal femur of children with spastic cerebral palsy (CP) during standing. Design Cross-sectional study Setting University research laboratory Participants 4 to 12 year-old children with spastic CP who could stand independently (n=18) and typically developing children (n=10) participated in the study. Intervention Not applicable Main outcome measures The vibration signal at the HLV platform (~33 Hz and 0.3 g), distal tibia and distal femur was measured using accelerometers. Degree of plantar flexor spasticity was assessed using the Modified Ashworth Scale. Results The HLV signal was greater (p<0.001) at the distal tibia than at the platform in children with CP (0.36±0.06 vs. 0.29±0.05 g) and controls (0.40 ± 0.09 vs. 0.24 ± 0.07 g). Although the HLV signal was also higher at the distal femur (0.35±0.09 g, p<0.001) than at the platform in controls, it was lower in children with CP (0.20±0.07 g, p<0.001). The degree of spasticity was negatively related to the HLV signal transmitted to the distal tibia (rs=−0.547) and distal femur (rs=−0.566) in children with CP (both p<0.05). Conclusions An HLV signal from a floor-based platform was amplified at the distal tibia, attenuated at the distal femur and inversely related to the degree of muscle spasticity in children with spastic CP. Whether this transmission pattern affects the adaptation of their bones to HLV requires further investigation. PMID:26392035

Deficits in Upper Limb Position Sense of Children with Spastic Hemiparetic Cerebral Palsy Are Distance-Dependent

ERIC Educational Resources Information Center

Smorenburg, Ana R. P.; Ledebt, Annick; Deconinck, Frederik J. A.; Savelsbergh, Geert J. P.

2012-01-01

This study examined the arm position sense in children with Spastic Hemiparetic Cerebral Palsy (SHCP) and typically developing children (TD) by means of a contralateral matching task. This task required participants to match the position of one arm with the position of the other arm for different target distances and from different starting…

Amyotrophic lateral sclerosis

PubMed Central

Wijesekera, Lokesh C; Leigh, P Nigel

2009-01-01

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterised by progressive muscular paralysis reflecting degeneration of motor neurones in the primary motor cortex, corticospinal tracts, brainstem and spinal cord. Incidence (average 1.89 per 100,000/year) and prevalence (average 5.2 per100,000) are relatively uniform in Western countries, although foci of higher frequency occur in the Western Pacific. The mean age of onset for sporadic ALS is about 60 years. Overall, there is a slight male prevalence (M:F ratio~1.5:1). Approximately two thirds of patients with typical ALS have a spinal form of the disease (limb onset) and present with symptoms related to focal muscle weakness and wasting, where the symptoms may start either distally or proximally in the upper and lower limbs. Gradually, spasticity may develop in the weakened atrophic limbs, affecting manual dexterity and gait. Patients with bulbar onset ALS usually present with dysarthria and dysphagia for solid or liquids, and limbs symptoms can develop almost simultaneously with bulbar symptoms, and in the vast majority of cases will occur within 1–2 years. Paralysis is progressive and leads to death due to respiratory failure within 2–3 years for bulbar onset cases and 3–5 years for limb onset ALS cases. Most ALS cases are sporadic but 5–10% of cases are familial, and of these 20% have a mutation of the SOD1 gene and about 2–5% have mutations of the TARDBP (TDP-43) gene. Two percent of apparently sporadic patients have SOD1 mutations, and TARDBP mutations also occur in sporadic cases. The diagnosis is based on clinical history, examination, electromyography, and exclusion of 'ALS-mimics' (e.g. cervical spondylotic myelopathies, multifocal motor neuropathy, Kennedy's disease) by appropriate investigations. The pathological hallmarks comprise loss of motor neurones with intraneuronal ubiquitin-immunoreactive inclusions in upper motor neurones and TDP-43 immunoreactive inclusions in degenerating lower motor neurones. Signs of upper motor neurone and lower motor neurone damage not explained by any other disease process are suggestive of ALS. The management of ALS is supportive, palliative, and multidisciplinary. Non-invasive ventilation prolongs survival and improves quality of life. Riluzole is the only drug that has been shown to extend survival. PMID:19192301

Adolescent Spinal Cord Injury and Paralysis--Understanding the Psychosocial Aspects.

ERIC Educational Resources Information Center

Smith, Craig D.

1985-01-01

A model developed by S. Fink is presented for understanding and predicting the phases through which an adolescent passes when facing paralysis. Implications for treatment based on this model are suggested. (Author/CL)

Chlamydia pneumoniae infection-related hemophagocytic lymphohistiocytosis and acute encephalitis and poliomyelitis-like flaccid paralysis.

PubMed

Yagi, Kanae; Kano, Gen; Shibata, Mayumi; Sakamoto, Izumi; Matsui, Hirofumi; Imashuku, Shinsaku

2011-05-01

A 3-year-old male presented with Chlamydia pneumoniae infection-related hemophagocytic lymphohistiocytosis (HLH). The patient developed an episode of HLH with severe skin eruption following C. pneumoniae pneumonia. Symptoms responded to steroid/cyclosporine A therapy, but the patient slowly lost consciousness and developed systemic flaccid paralysis. He was diagnosed with encephalitis/myelitis by brain and spinal MRI. Neurological symptoms and signs gradually resolved. We thought that the immune response to C. pneumoniae infection triggered the development of HLH, associated with unusual neurological complications. This report describes a novel case of C. pneumoniae-associated HLH and with poliomyelitis like flaccid paralysis. Copyright © 2010 Wiley-Liss, Inc.

New insights into the pathophysiology of post-stroke spasticity.

PubMed

Li, Sheng; Francisco, Gerard E

2015-01-01

Spasticity is one of many consequences after stroke. It is characterized by a velocity-dependent increase in resistance during passive stretch, resulting from hyperexcitability of the stretch reflex. The underlying mechanism of the hyperexcitable stretch reflex, however, remains poorly understood. Accumulated experimental evidence has supported supraspinal origins of spasticity, likely from an imbalance between descending inhibitory and facilitatory regulation of spinal stretch reflexes secondary to cortical disinhibition after stroke. The excitability of reticulospinal (RST) and vestibulospinal tracts (VSTs) has been assessed in stroke survivors with spasticity using non-invasive indirect measures. There are strong experimental findings that support the RST hyperexcitability as a prominent underlying mechanism of post-stroke spasticity. This mechanism can at least partly account for clinical features associated with spasticity and provide insightful guidance for clinical assessment and management of spasticity. However, the possible role of VST hyperexcitability cannot be ruled out from indirect measures. In vivo measure of individual brainstem nuclei in stroke survivors with spasticity using advanced fMRI techniques in the future is probably able to provide direct evidence of pathogenesis of post-stroke spasticity.

New insights into the pathophysiology of post-stroke spasticity

PubMed Central

Li, Sheng; Francisco, Gerard E.

2015-01-01

Spasticity is one of many consequences after stroke. It is characterized by a velocity-dependent increase in resistance during passive stretch, resulting from hyperexcitability of the stretch reflex. The underlying mechanism of the hyperexcitable stretch reflex, however, remains poorly understood. Accumulated experimental evidence has supported supraspinal origins of spasticity, likely from an imbalance between descending inhibitory and facilitatory regulation of spinal stretch reflexes secondary to cortical disinhibition after stroke. The excitability of reticulospinal (RST) and vestibulospinal tracts (VSTs) has been assessed in stroke survivors with spasticity using non-invasive indirect measures. There are strong experimental findings that support the RST hyperexcitability as a prominent underlying mechanism of post-stroke spasticity. This mechanism can at least partly account for clinical features associated with spasticity and provide insightful guidance for clinical assessment and management of spasticity. However, the possible role of VST hyperexcitability cannot be ruled out from indirect measures. In vivo measure of individual brainstem nuclei in stroke survivors with spasticity using advanced fMRI techniques in the future is probably able to provide direct evidence of pathogenesis of post-stroke spasticity. PMID:25914638

Treatment of os odontoideum in a patient with spastic quadriplegic cerebral palsy.

PubMed

Akpolat, Yusuf T; Fegale, Ben; Cheng, Wayne K

2015-08-01

Severe atlantoaxial instability due to os odontoideum in a patient with spastic cerebral palsy has not been well described. There is no consensus on treatment, particularly with regard to conservative or surgical options. Our patient was a 9-year-old girl with spastic cerebral palsy and unstable os odontoideum as an incidental finding. During the waiting period for elective surgical treatment, the patient developed respiratory compromise. Surgery was performed to reduce the subluxation and for C1-C2 arthrodesis and the girl regained baseline respiratory function. A CT scan was obtained 1 year after the initial surgery and revealed adequate maintenance of reduction and patency of the spinal canal. This patient highlights the fact that unstable os odontoideum can cause mortality due to respiratory distress in patients with spastic cerebral palsy. This is an important factor in deciding treatment options for cerebral palsy patients with low functional demand. We review the relevant literature. Copyright © 2015 Elsevier Ltd. All rights reserved.

Effect of Seat Surface Inclination on Postural Stability and forward Reaching Efficiency in Children with Spastic Cerebral Palsy

ERIC Educational Resources Information Center

Cherng, Rong-Ju; Lin, Hui-Chen; Ju, Yun-Huei; Ho, Chin-Shan

2009-01-01

The purpose of this study was to examine the effect of seat surface inclination on postural stability and forward reaching efficiency in 10 children with spastic cerebral palsy (CP) and 16 typically developing (TD) children. The children performed a static sitting and a forward reaching task while sitting on a height- and inclination-adjustable…

The Use of Botulinum Toxin for the Treatment of Muscle Spasticity in the First 2 Years of Life

ERIC Educational Resources Information Center

Bakheit, Abdel Magid

2010-01-01

Although there are sound theoretical reasons for the use of botulinum toxin (Btx) as early as possible in the management of severe childhood muscle spasticity, the experience with its safety in children younger than 2 years of age is limited and information about its possible effects on the development and maturation of the human motor system…

Hypokalemic paralysis in a young obese female.

PubMed

Chiang, Wen-Fang; Hsu, Yu-Juei; Chang, Chin-Chun; Lin, Shih-Hua

2012-08-16

Profound hypokalemia with paralysis usually poses a diagnostic and therapeutic challenge. We report on a 28-y-old obese Chinese female presenting with sudden onset of flaccid quadriparesis upon awaking in the morning. There is no family history of hyperthyroidism. She experienced body weight loss of 7 kg in 2 months. The most conspicuous blood biochemistry is marked hypokalemia (1.8 mmol/l) and hypophosphatemia (0.5 mmol/l) associated with low urine K(+) and phosphate excretion. Surreptitious laxatives and/or diuretics abuse-related hypokalemic paralysis were tentatively made. However, her relatively normal blood acid-base status and the absence of low urine Na(+) and/or Cl(-) excretion made these diagnoses unlikely. Furthermore, she developed rebound hyperkalemia (5.7 mmol/l) after only 80 mmol K(+) supplementation. Thyroid function test confirmed hyperthyroidism due to Graves' disease. Control of the hyperthyroidism completely abolished her periodic paralysis. Thyrotoxic periodic paralysis (TPP) should be kept in mind as a cause of paralysis in female, even with obesity, despite its predominance in adult males. Copyright © 2012 Elsevier B.V. All rights reserved.

Data condensed synthesis regarding kinesiotherapeutic procedures used in spasticity therapy

PubMed Central

Moraru, E; Onose, G

2014-01-01

Abstract Spasticity represents an important feature of the upper motoneuron syndrome (UMNS). The clinical signs, such as the abnormal movement models, the unwanted muscular co-contractions, the muscular and joint rigidity with a consecutive deformity can be signs of spasticity and, also of upper motoneuron lesion. The different therapeutic options applied in the management of spasticity are a basic component of UMNS treatment scheme. This article presents the main kinesiotherapeutic procedures used in spasticity therapy. PMID:25408747

Pendulum test measure correlates with gait parameters in children with cerebral palsy.

PubMed

Lotfian, M; Mirbagheri, M M; Kharazi, M R; Dadashi, F; Nourian, R; Irani, A; Mirbagheri, A

2016-08-01

Individuals with cerebral palsy (CP) usually suffer from different impairments including gait impairment and spasticity. Spastic hypertonia is a defining feature of spasticity and manifests as a mechanical abnormality. The objective of this study was to determine the relationship between spastic hypertonia and gait impairments in spastic children with CP, addressing an important controversial issue. Spastic hypertonia was quantified using the pendulum test. The gait impairments were evaluated using the motion capture system in a gait laboratory. Our results showed significant correlations among gait parameters; i.e. walking speed, step length, and the pendulum test measures. This indicates that neuromuscular abnormalities are associated with spasticity and may contribute to gait impairments. The clinical implication is that the impaired gait in children with CP may be improved with the treatment of neuromuscular abnormalities.

The effect of selective tibial neurotomy and rehabilitation in a quadriplegic patient with ankle spasticity following traumatic brain injury.

PubMed

Jang, Sung Ho; Park, Sung-Min; Kim, Seong Ho; Ahn, Sang Ho; Cho, Yun Woo; Ahn, Mi Ok

2004-08-31

Ankle spasticity following brain injury leads to abnormal posture and joint contracture; making standing or walking impossible. This study investigates the efficacy of selective tibial neurotomy (STN) and intensive rehabilitation in a patient who suffered ankle spasticity after brain injury. This case describes a 37-year-old man whose traumatic brain injury (TBI) resulted in severe right ankle spasticity and contracture. He was unable to stand due to severe right ankle spasticity and contracture. Intensive rehabilitation and STN allowed him to walk without brace at 6 months and run at 12 months after STN. STN is an effective procedure to resolve localized spasticity of the ankle and it may be considered as a management strategy after local injection to alleviate ankle spasticity and/or contracture prior to orthopaedic surgery.

Immediate post-dosing paralysis following severe soman and VX toxicosis in guinea pigs.

PubMed

Bide, R W; Schofield, L; Risk, D J

2005-01-01

There have been numerous studies of the central nervous system (CNS) involvement in organophosphate (OP) poisoning showing status epilepticus and/or 'electrographic seizures'. Brain damage has been demonstrated as 'neuronal necrosis' primarily in the cortex, thalamus and hippocampus. To the authors' knowledge there have been no reports of partial/total paralysis following close upon OP exposure although delayed paralysis has been reported. This report summarizes the immediate, OP induced paralytic events recorded in guinea pigs during development of the Canadian reactive skin decontaminant lotion (RSDL). As part of the development work, supra-lethal cutaneous doses of OP were applied to large numbers of guinea pigs followed by decontamination with the RSDL or predecessor lotions and solvents. Soman (pinacolyl methylphosphonofluoridate; GD) challenges were applied to 1277 animals and S-(2-diisopropyl-aminoethyl) methylphosphorothiolate (VX) challenges to 108. The classic sequence of clinical signs--ptyalism, tremors, fasciculations, convulsions, apnea and flaccid paralysis before death--was seen in the 658 animals that died and in many of the survivors. Eighty-four of 688 survivors of GD and 4 of 39 survivors of VX showed random paralysis of various distal regions following recovery from an insult which produced convulsions and/or flaccid paralysis. Because the experiments were designed to assess the decontamination procedures, there were no apparent relationships between the amounts of OP applied and the sequellae recorded. The observations of paralysis were also incidental to the prime focus of the experiments. Because of this, only ten animals paralysed following GD exposure were examined for histological effects. The pathologist diagnosed 'encephalomalacia' and 'focal necrotic lesions' in the cerebral cortex and 'focal necrotic lesions' in one spinal cord. Of the 84 guinea pigs paralysed after GD challenge, one was not decontaminated and the decontaminants used on the remainder were sufficiently varied that there appeared to be no relationship between the type of decontaminant and the resulting paralysis. 2005 John Wiley & Sons, Ltd.

Botulinum toxin in the management of facial paralysis.

PubMed

Cabin, Jonathan A; Massry, Guy G; Azizzadeh, Babak

2015-08-01

Complete flaccid facial paralysis, as well as the synkinetic and hyperkinetic sequelae of partial recovery, has significant impact on quality of life. Patients suffer from functional deficiencies, cosmetic deformity, discomfort and social consequences leading to emotional distress. Despite an extensive and sophisticated array of available interventions for facial reanimation, most patients have persistent issues that require consistent follow-up. In long-term management, botulinum toxin (BT) injection remains a critical tool in the treatment of the facial paralysis patient, particularly in the case of synkinesis, hyperkinesis and imbalance. We review the recent scientific literature and highlight key principles and developments in the use of BT in the management of facial paralysis, including less common applications for acute facial paralysis, hyperlacrimation and pseudoptosis. We reviewed the literature for the latest advances in the use of BT in facial paralysis, including applications and technique, as well as measurement tools and adjunct exercises. We also share our experience in treating our own patient population. BT continues to be a well tolerated and effective tool in the long-term management of facial paralysis, specifically in treating synkinesis, imbalance and hyperkinesis, as well as hyperlacrimation and pseudoptosis. Consistent measurement tools and adjunct neuromuscular retraining are crucial in the successful deployment of BT. Controversy exists as to whether BT should be used to manage facial paralysis during the acute phase, and whether BT application to the nonparalyzed face can improve long-term recovery in the paralyzed side.

Using Telemedicine to Improve Spasticity Diagnosis Rates

ClinicalTrials.gov

2018-02-20

Spasticity, Muscle; Muscular Diseases; Musculoskeletal Disease; Muscle Hypertonia; Muscle Spasticity; Neuromuscular Manifestations; Signs and Symptoms; Nervous System Diseases; Neurologic Manifestations

Relations among hypnagogic and hypnopompic experiences associated with sleep paralysis.

PubMed

Cheyne, J A; Newby-Clark, I R; Rueffer, S D

1999-12-01

The Waterloo Sleep Experiences Scale was developed to assess the prevalence of sleep paralysis and a variety of associated hypnagogic and hypnopompic hallucinoid experiences: sensed presence, felt pressure, floating sensations, auditory and visual hallucinations, and fear. Consistent with results of recent surveys, almost 30% of 870 university students reported at least one experience of sleep paralysis. Approximately three-quarters of those also reported at least one hallucinoid experience, and slightly more than 10% experienced three or more. Fear was positively associated with hallucinoid experiences, most clearly with sensed presence. Regression analyses lend support to the hypothesis that sensed presence and fear are primitive associates of sleep paralysis and contribute to the elaboration of further hallucinoid experiences, especially those involving visual experiences.

Comprehensive Quantification of the Spastic Catch in Children with Cerebral Palsy

ERIC Educational Resources Information Center

Lynn, Bar-On; Erwin, Aertbelien; Guy, Molenaers; Herman, Bruyninckx; Davide, Monari; Ellen, Jaspers; Anne, Cazaerck; Kaat, Desloovere

2013-01-01

In clinical settings, the spastic catch is judged subjectively. This study assessed the psychometric properties of objective parameters that define and quantify the severity of the spastic catch in children with cerebral palsy (CP). A convenience sample of children with spastic CP (N = 46; age range: 4-16 years) underwent objective spasticity…

Costs and quality of life in multiple sclerosis patients with spasticity.

PubMed

Svensson, J; Borg, S; Nilsson, P

2014-01-01

The resource use and health-related quality of life (HRQoL) of patients with multiple sclerosis (MS) spasticity are not well known. The purpose of this study was to obtain estimates of resource utilization, costs, and HRQoL, for patients with different levels of MS spasticity in southern Sweden. Cross-sectional data on spasticity severity (using a Numerical Rating Scale, NRS), resource use and HRQoL (using EQ-5D) were collected using a patient questionnaire and chart review. Patients were recruited through a clinic in southern Sweden. The study reviews direct medical, direct non-medical and indirect costs. Total costs were estimated to €114,293 per patient and year. Direct medical costs (€7898) accounted for 7% of total costs. Direct non-medical costs (€68,509) accounted for 60% of total costs. Total costs increased with severity of spasticity: for patients with severe spasticity, the total cost was 2.4 times greater than those for patients with mild spasticity. HRQoL decreased as spasticity increases. The results of this study show that MS spasticity is associated with a substantial burden on society in terms of costs and HRQoL. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Delta-9-Tetrahydrocannabinol/Cannabidiol Oromucosal Spray (Sativex®): A Review in Multiple Sclerosis-Related Spasticity.

PubMed

Keating, Gillian M

2017-04-01

Delta-9-tetrahydrocannabinol (THC)/cannabidiol (CBD) oromucosal spray (THC/CBD, Sativex ® , nabiximols) is available in numerous countries worldwide for the treatment of multiple sclerosis (MS)-related moderate to severe spasticity in patients who have not responded adequately to other anti-spasticity medication and who demonstrate clinically significant improvement in spasticity-related symptoms during an initial trial of therapy. Twelve weeks' therapy with THC/CBD improved MS-related spasticity in patients with an inadequate response to other anti-spasticity agents who had undergone a successful initial trial of THC/CBD therapy, according to the results of a pivotal phase 3 trial. Improvements in spasticity were maintained in the longer term with THC/CBD with no evidence of dose tolerance, and results of real-world studies confirm the effectiveness of THC/CBD in everyday clinical practice. Improvements in health-related quality of life and activities of daily living were also seen with THC/CBD. THC/CBD is generally well tolerated; adverse effects such as dizziness may occur whilst the THC/CBD dosage is being optimized. THC/CBD has low abuse potential and a low risk of psychoactive effects. In conclusion, THC/CBD oromucosal spray is a useful option for the treatment of MS-related spasticity not completely relieved with current anti-spasticity medication.

Influence of spasticity on mobility and balance in persons with multiple sclerosis.

PubMed

Sosnoff, Jacob J; Gappmaier, Eduard; Frame, Amy; Motl, Robert W

2011-09-01

Spasticity is a motor disorder characterized by a velocity-dependent increase in tonic stretch reflexes that presumably affects mobility and balance. This investigation examined the hypothesis that persons with multiple sclerosis (MS) who have spasticity of the lower legs would have more impairment of mobility and balance compared to those without spasticity. Participants were 34 ambulatory persons with a definite diagnosis of MS. The expanded disability status scale (EDSS) was used to characterize disability in the study sample. All participants underwent measurements of spasticity in the gastroc-soleus muscles of both legs (modified Ashworth scale), walking speed (timed 25-foot walk), mobility (Timed Up and Go), walking endurance (6-minute walk test), self-reported impact of MS on walking ability (Multiple Sclerosis Walking Scale-12), and balance (Berg Balance Test and Activities-specific Balance Confidence Scale). Fifteen participants had spasticity of the gastroc-soleus muscles based on modified Ashworth scale scores. The spasticity group had lower median EDSS scores indicating greater disability (P=0.03). Mobility and balance were significantly more impaired in the group with spasticity compared to the group without spasticity: timed 25-foot walk (P = 0.02, d = -0.74), Timed Up and Go (P = 0.01, d = -0.84), 6-minute walk test (P < 0.01, d = 1.03), Multiple Sclerosis Walking Scale-12 (P = 0.04, d = -0.76), Berg Balance Test (P = 0.02, d = -0.84) and Activities-specific Balance Confidence Scale (P = 0.04, d = -0.59). Spasticity in the gastroc-soleus muscles appears to have negative effect on mobility and balance in persons with MS. The relationship between spasticity and disability in persons with MS requires further exploration.

The meaning of spasticity to people with multiple sclerosis: what can health professionals learn?

PubMed

Morley, Alex; Tod, Angela; Cramp, Mary; Mawson, Sue

2013-07-01

Multiple sclerosis (MS) is the most common disabling neurological condition affecting young adults. One third of people on an American registry of people with MS (PWMS) reported having activities affected by spasticity. The psychosocial effects of spasticity in people with MS have been shown to be distressing and detrimental to emotional and social relationships when investigated from a psychology perspective. This paper investigates the impact of spasticity on the lives of people living with MS from a physiotherapeutic perspective. This study involved 12 semi-structured interviews with individuals experiencing MS-related spasticity. Ten sets of data were analyzed following framework analysis principles. Results suggest spasticity effects life experience of these PWMS in diverse and complex ways. Physical, psychological and social consequences of spasticity are closely linked and can be far reaching. Therapists need to be aware of links between specific physical symptoms and their psychosocial consequences if they want to improve peoples' quality of life. This paper provides in depth qualitative research evidence for the complexity of the spasticity experience for each individual, strengthening the argument for a patient-centred approach to treatment. These results also support the case for targeted interventions with effectiveness recorded in a patient-centred way. • Spasticity is suggested here to affect the lives of individuals with multiple sclerosis in diverse and far reaching ways. Therapists need to investigate this fully in subjective assessment to impact on people's quality of life. • Direct links were identified between treatable physical symptoms and far reaching consequences of spasticity. • Knowledge about the complexity of the spasticity experience for each individual will allow therapists to target interventions appropriately and accurately record effectiveness in a patient-centred way.

Simultaneous characterizations of reflex and nonreflex dynamic and static changes in spastic hemiparesis

PubMed Central

Chung, Sun G.; Ren, Yupeng; Liu, Lin; Roth, Elliot J.; Rymer, W. Zev

2013-01-01

This study characterizes tonic and phasic stretch reflex and stiffness and viscosity changes associated with spastic hemiparesis. Perturbations were applied to the ankle of 27 hemiparetic and 36 healthy subjects under relaxed or active contracting conditions. A nonlinear delay differential equation model characterized phasic and tonic stretch reflex gains, elastic stiffness, and viscous damping. Tendon reflex was characterized with reflex gain and threshold. Reflexively, tonic reflex gain was increased in spastic ankles at rest (P < 0.038) and was not regulated with muscle contraction, indicating impaired tonic stretch reflex. Phasic-reflex gain in spastic plantar flexors was higher and increased faster with plantar flexor contraction (P < 0.012) than controls (P < 0.023) and higher in dorsi-flexors at lower torques (P < 0.038), primarily because of its increase at rest (P = 0.045), indicating exaggerated phasic stretch reflex especially in more spastic plantar flexors, which showed higher phasic stretch reflex gain than dorsi-flexors (P < 0.032). Spasticity was associated with increased tendon reflex gain (P = 0.002) and decreased threshold (P < 0.001). Mechanically, stiffness in spastic ankles was higher than that in controls across plantar flexion/dorsi-flexion torque levels (P < 0.032), and the more spastic plantar flexors were stiffer than dorsi-flexors at comparable torques (P < 0.031). Increased stiffness in spastic ankles was mainly due to passive stiffness increase (P < 0.001), indicating increased connective tissues/shortened fascicles. Viscous damping in spastic ankles was increased across the plantar flexion torque levels and at lower dorsi-flexion torques, reflecting increased passive viscous damping (P = 0.033). The more spastic plantar flexors showed higher viscous damping than dorsi-flexors at comparable torque levels (P < 0.047). Simultaneous characterizations of reflex and nonreflex changes in spastic hemiparesis may help to evaluate and treat them more effectively. PMID:23636726

Site-Specific Transmission of a Floor-Based, High-Frequency, Low-Magnitude Vibration Stimulus in Children With Spastic Cerebral Palsy.

PubMed

Singh, Harshvardhan; Whitney, Daniel G; Knight, Christopher A; Miller, Freeman; Manal, Kurt; Kolm, Paul; Modlesky, Christopher M

2016-02-01

To determine the degree to which a high-frequency, low-magnitude vibration signal emitted by a floor-based platform transmits to the distal tibia and distal femur of children with spastic cerebral palsy (CP) during standing. Cross-sectional study. University research laboratory. Children with spastic CP who could stand independently (n=18) and typically developing children (n=10) (age range, 4-12y) participated in the study (N=28). Not applicable. The vibration signal at the high-frequency, low-magnitude vibration platform (approximately 33Hz and 0.3g), distal tibia, and distal femur was measured using accelerometers. The degree of plantar flexor spasticity was assessed using the Modified Ashworth Scale. The high-frequency, low-magnitude vibration signal was greater (P<.001) at the distal tibia than at the platform in children with CP (.36±.06g vs .29±.05g) and controls (.40±.09g vs .24±.07g). Although the vibration signal was also higher at the distal femur (.35±.09g, P<.001) than at the platform in controls, it was lower in children with CP (.20±.07g, P<.001). The degree of spasticity was negatively related to the vibration signal transmitted to the distal tibia (Spearman ρ=-.547) and distal femur (Spearman ρ=-.566) in children with CP (both P<.05). A high-frequency, low-magnitude vibration signal from a floor-based platform was amplified at the distal tibia, attenuated at the distal femur, and inversely related to the degree of muscle spasticity in children with spastic CP. Whether this transmission pattern affects the adaptation of the bones of children with CP to high-frequency, low-magnitude vibration requires further investigation. Copyright © 2016 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

Time-dependent, bidirectional, anti- and pro-spinal hyper-reflexia and muscle spasticity effect after chronic spinal glycine transporter 2 (GlyT2) oligonucleotide-induced downregulation.

PubMed

Kamizato, Kota; Marsala, Silvia; Navarro, Michael; Kakinohana, Manabu; Platoshyn, Oleksandr; Yoshizumi, Tetsuya; Lukacova, Nadezda; Wancewicz, Ed; Powers, Berit; Mazur, Curt; Marsala, Martin

2018-07-01

The loss of local spinal glycine-ergic tone has been postulated as one of the mechanisms contributing to the development of spinal injury-induced spasticity. In our present study using a model of spinal transection-induced muscle spasticity, we characterize the effect of spinally-targeted GlyT2 downregulation once initiated at chronic stages after induction of spasticity in rats. In animals with identified hyper-reflexia, the anti-spasticity effect was studied after intrathecal treatment with: i) glycine, ii) GlyT2 inhibitor (ALX 1393), and iii) GlyT2 antisense oligonucleotide (GlyT2-ASO). Administration of glycine and GlyT2 inhibitor led to significant suppression of spasticity lasting for a minimum of 45-60 min. Treatment with GlyT2-ASO led to progressive suppression of muscle spasticity seen at 2-3 weeks after treatment. Over the subsequent 4-12 weeks, however, the gradual appearance of profound spinal hyper-reflexia was seen. This was presented as spontaneous or slight-tactile stimulus-evoked muscle oscillations in the hind limbs (but not in upper limbs) with individual hyper-reflexive episodes lasting between 3 and 5 min. Chronic hyper-reflexia induced by GlyT2-ASO treatment was effectively blocked by intrathecal glycine. Immunofluorescence staining and Q-PCR analysis of the lumbar spinal cord region showed a significant (>90%) decrease in GlyT2 mRNA and GlyT2 protein. These data demonstrate that spinal GlyT2 downregulation provides only a time-limited therapeutic benefit and that subsequent loss of glycine vesicular synthesis resulting from chronic GlyT2 downregulation near completely eliminates the tonic glycine-ergic activity and is functionally expressed as profound spinal hyper-reflexia. These characteristics also suggest that chronic spinal GlyT2 silencing may be associated with pro-nociceptive activity. Copyright © 2018 Elsevier Inc. All rights reserved.

Aculaser therapy: a comprehensive approach for the treatment of cerebral palsy

NASA Astrophysics Data System (ADS)

Anwar, Shahzad; Nazir Khan, Malik Muhammed; Nadeem Khan, Malik Mohammad; Munir Qazi, Faiza; Ahmed, Imtiaz; Awan, Abid Hareef

2006-10-01

A single, open and non comparative study was conducted at Anwar Shah's First C.P. & Paralysis Clinic and Research Center in collaboration with the Departments of Neurology and Neurosurgery, Children Hospital Lahore, Pakistan to evaluate the effects of ACULASER THERAPY in childern suffering from Cerebral Palsy (CP) and associated Neurological Disorders like epilepsy, cortical blindness, spasticity, hemiplegia, paraplegia, quadriplegia, paraplegia, monoplegia, sensory-neural deafness and speech disorders. In all 100 childern were treated and the data was gathered during a period of 18 months from December 2003 till June 2005. This article shows results of the treatment with ACULASER THERAPY in CP childern who were treated for minimum 6 weeks and more or had minimum of 10 treatment sessions and more. This paper also shows that those childern who were given a break in the treatment for 4-12 weeks did not show any reversal of the symptoms. These children were classified according to the associated Neurological Disorders. Analysis of the data showed that out of 81 children with Spasticity and Stiffness 69 showed marked improvement showing 85% improvement rate, out of 54 children with Epileptic fits there was a significant reduction in the intensity, frequency and duration of Epileptic fits in 34 children showing 63% success rate, out of 18 children with Cortical Blindness 13 children showed improvement accounting for 72% efficacy rate, out of 45 children with Hearing Difficulties, 31 showed marked improvement accounting for 69% improvement rate, out of 100 children with Speech Disorders 67 showed improvement reflecting 67 % improvement rate, out of 46 children with Hemiplegia 32 showed improvement in movement, tone and power accounting for 69% improvement rate, out of 36 children with Quadriplegia 25 showed improvement in gross and fine motor functions showing 69% success rate and out of 18 children with Paraplegia of lower limbs 12 showed improvement in weight bearing, standing and movement accounting for 67% improvement rate .

Spasticity secondary to Leigh syndrome managed with selective dorsal rhizotomy: a case report.

PubMed

Mazarakis, N K; Vloeberghs, M H

2016-09-01

Selective dorsal rhizotomy (SDR) is a surgical technique used to treat spasticity in children secondary to cerebral palsy (CP). We report, to the best of our knowledge for the first time, the case of a child who underwent SDR for the management of spasticity secondary to Leigh syndrome. SDR resulted in excellent functional outcome with significant improvement in spasticity. This result contributes to the mounting evidence that SDR could be used to alleviate spasticity secondary not only to CP but also to other pathologies as well.

Validity and Inter-Rater Reliability of a Novel Bedside Referral Tool for Spasticity

ClinicalTrials.gov

2018-02-20

Spasticity, Muscle; Muscular Diseases; Musculoskeletal Disease; Muscle Hypertonia; Muscle Spasticity; Neuromuscular Manifestations; Signs and Symptoms; Nervous System Diseases; Neurologic Manifestations

[Vocal cord paralysis after endotracheal intubation: an uncommon complication of general anesthesia].

PubMed

Hurtado Nazal, Claudia; Araneda Vilches, Andrea; Vergara Marín, Carolina; García Contreras, Karen; Napolitano Valenzuela, Carla; Badía Ventí, Pedro

2018-04-05

General anesthesia is a safe, frequent procedure in clinical practice. Although it is very unusual in procedures not related to head and or neck surgery, vocal cord paralysis is a serious and important complication. Incidence has been associated with patient age and comorbidities, as well as the position of the endotracheal tube and cuff. It can become a dangerous scenario because it predisposes aspiration. To present a case and analyze the risk factors associated with increased risk of vocal cord paralysis described in the literature. 53 year-old diabetic man, who developed hoarseness in the postoperative period after receiving general anesthesia for an elective abdominal laparoscopic surgery. Otolaryngological evaluation showed left vocal cord paralysis. Vocal cord paralysis can be a serious complication of general anesthesia because of important voice dysfunction and risk of aspiration. The management is not yet fully established, so prevention and early diagnosis is essential. Copyright © 2018 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

Treatment with botulinum toxin in children with cerebral palsy: a qualitative study of parents' experiences.

PubMed

Lorin, K; Forsberg, A

2016-07-01

In children with cerebral palsy everyday movements such as walking, standing and using one's hands can be difficult to perform because of spasticity. Botulinum neurotoxin type A (BoNT-A) are often used to reduce spasticity. The aim of this study was to describe how parents of children with cerebral palsy experienced the child's treatment with BoNT-A, how the child was affected by the treatment and how spasticity affected the child. A qualitative study in which 15 parents of children (6-13 years old) with cerebral palsy were interviewed about their experiences of the BoNT-A treatment. The children had received several BoNT-A treatments. An interview guide was used with topics: the child's functions before and after the treatment, the outcomes of the treatment and how they valued the BoNT-A treatment. Content analysis was used to analyse the interviews. The analyses resulted in two themes: 'When softness comes and goes' and 'Both want and do not want'. The reduction of spasticity - softness - was described to promote motor functions, and facilitate the next step in motor development. The children were described as being more active out of their own initiative and having a happier mood. Spasticity, described as stiffness, was described to make walking more strenuous as well as interfering with activities. The BoNT-A injection procedure was perceived as troublesome and painful for the child, and sometimes traumatic for both children and parents. Treatment with BoNT-A was described as facilitating motor development and activity. The children's and the parents' negative experiences of the injection procedure should be addressed. © 2016 John Wiley & Sons Ltd.

The early use of botulinum toxin in post-stroke spasticity: study protocol for a randomised controlled trial.

PubMed

Lindsay, Cameron; Simpson, Julie; Ispoglou, Sissi; Sturman, Steve G; Pandyan, Anand D

2014-01-08

Patients surviving stroke but who have significant impairment of function in the affected arm are at more risk of developing pain, stiffness and contractures. The abnormal muscle activity, associated with post-stroke spasticity, is thought to be causally associated with the development of these complications. Treatment of spasticity is currently delayed until a patient develops signs of these complications. This protocol is for a phase II study that aims to identify whether using OnabotulinumtoxinA (BoNT-A) in combination with physiotherapy early post stroke when initial abnormal muscle activity is neurophysiologically identified can prevent loss of range at joints and improve functional outcomes.The trial uses a screening phase to identify which people are appropriate to be included in a double blind randomised placebo-controlled trial. All patients admitted to Sandwell and West Birmingham NHS Trust Hospitals with a diagnosis of stroke will be screened to identify functional activity in the arm. Those who have no function will be appropriate for further screening. Patients who are screened and have abnormal muscle activity identified on EMG will be given electrical stimulation to forearm extensors for 3 months and randomised to have either injections of BoNT-A or normal saline. The primary outcome measure is the action research arm test - a measure of arm function. Further measures include spasticity, stiffness, muscle strength and fatigue as well as measures of quality of life, participation and caregiver strain. ISRCTN57435427, EudraCT2010-021257-39, NCT01882556.

Changes to medial gastrocnemius architecture after surgical intervention in spastic diplegia.

PubMed

Shortland, Adam P; Fry, Nicola R; Eve, Linda C; Gough, Martin

2004-10-01

We assessed the architecture of the medial gastrocnemius in nine children (five males, four females; age range 6 to 15 years; mean 10 years 10 months, SD 3 years 6 months) with spastic diplegia by ultrasound imaging before and after a gastrocnemius recession. The children were ambulant (seven independent, one with a posterior walker, one using crutches) before and after surgical intervention. We compared values for fascicle lengths and deep fascicular-aponeurosis angles with those from a group of normally developing children (five males, five females; age range 6 to 11 years; mean 8 years 4 months, SD 1 year 4 months). Despite a variable interval between assessments (from 56 to 610 days), fascicles were shorter (p=0.00226) and the deep fascicular-aponeurosis angle increased (p=0.0152) after intervention. Fascicle lengths of patients were similar to those in the group of normally developing children before surgery. After surgery, fascicles in the group of children with spastic diplegia were shorter than in their normally developing peers (p=0.00109). The gastrocnemius recession procedure alters muscle architecture, though the degree of fascicular shortening varied, with four of the participants in our study losing less than 10% of their original fascicular length at maximum dorsiflexion. Increases in ankle-joint power in walking, observed after surgical intervention in children with spastic diplegia, may be due to a more normal ankle position rather than to improvements in the active mechanical performance of the gastrocnemius.

[Evaluation of pendulum testing of spasticity].

PubMed

Le Cavorzin, P; Hernot, X; Bartier, O; Carrault, G; Chagneau, F; Gallien, P; Allain, H; Rochcongar, P

2002-11-01

To identify valid measurements of spasticity derived from the pendulum test of the leg in a representative population of spastic patients. Pendulum testing was performed in 15 spastic and 10 matched healthy subjects. The reflex-mediated torque evoked in quadriceps femoris, as well as muscle mechanical parameters (viscosity and elasticity), were calculated using mathematical modelling. Correlation with the two main measures derived from the pendulum test reported in the literature (the Relaxation Index and the area under the curve) was calculated in order to select the most valid. Among mechanical parameters, only viscosity was found to be significantly higher in the spastic group. As expected, the computed integral of the reflex-mediated torque was found to be larger in spastics than in healthy subjects. A significant non-linear (logarithmic) correlation was found between the clinically-assessed muscle spasticity (Ashworth grading) and the computed reflex-mediated torque, emphasising the non-linear behaviour of this scale. Among measurements derived from the pendulum test which are proposed in the literature for routine estimation of spasticity, the Relaxation Index exhibited an unsuitable U-shaped pattern of variation with increasing reflex-mediated torque. On the opposite, the area under the curve revealed a linear regression, which is more convenient for routine estimation of spasticity. The pendulum test of the leg is a simple technique for the assessment of spastic hypertonia. However, the measurement generally used in the literature (the Relaxation Index) exhibits serious limitations, and would benefit to be replaced by more valid measures, such as the area under the goniometric curve, especially for the assessment of therapeutics.

Identifying the brain regions associated with acute spasticity in patients diagnosed with an ischemic stroke.

PubMed

Barlow, Susan J

2016-06-01

Spasticity is a common impairment found in patients that have been diagnosed with a stroke. Little is known about the pathophysiology of spasticity at the level of the brain. This retrospective study was performed to identify an association between the area of the brain affected by an ischemic stroke and the presence of acute spasticity. Physical and occupational therapy assessments from all patients (n = 441) that had suffered a stroke and were admitted into a local hospital over a 4-year period were screened for inclusion in this study. Subjects that fit the inclusion criteria were grouped according to the presence (n = 42) or absence (n = 129) of acute spasticity by the Modified Ashworth Scale score given during the hospital admission assessment. Magnetic resonance images from 20 subjects in the spasticity group and 52 from the control group were then compared using lesion density plots and voxel-based lesion-symptom mapping. An association of acute spasticity with the gray matter regions of the insula, basal ganglia, and thalamus was found in this study. White matter tracts including the pontine crossing tract, corticospinal tract, internal capsule, corona radiata, external capsule, and the superior fronto-occipital fasciculus were also found to be significantly associated with acute spasticity. This is the first study to describe an association between a region of the brain affected by an infarct and the presence of acute spasticity. Understanding the regions associated with acute spasticity will aid in understanding the pathophysiology of this musculoskeletal impairment at the level of the brain.

Intramedullary Solitary Fibrous Tumor--A Benign Form of Hemangiopericytoma? Case Report and Review of the Literature.

PubMed

Bruder, Markus; Tews, Dominique; Mittelbronn, Michel; Capper, David; Seifert, Volker; Marquardt, Gerhard

2015-07-01

Solitary fibrous tumors (SFTs) are benign tumors of the soft tissue occurring anywhere in the human body but arise predominantly in the visceral pleura. SFTs of the central nervous system are rare, especially when they occur within the spinal cord. We present a case of an 83-year-old female patient presenting with acute spastic paralysis of the lower extremities after a history of progressive weakness and incontinence. Magnetic resonance imaging (MRI) findings were typical for meningioma, but an intradural, mainly intramedullary tumor was found at the first operation. A second operation was performed under neurophysiological monitoring, and complete removal of the tumor was achieved. Neurological conditions improved after the procedure, but the patient remained paraparetic. Histological and immunohistochemical findings revealed an SFT. There is evidence that SFTs and hemangiopericytomas (HPCs) are not different entities but should be considered as different graduations of a common spectrum. The extent of resection is a prognostic factor for recurrence-free survival in SFT; therefore we recommend surgery with complete resection whenever possible depending on the results of mandatory intraoperative neurophysiological monitoring in these cases. Copyright © 2015 Elsevier Inc. All rights reserved.

Prevalence of Comorbid Spasticity and Urinary Incontinence in Residents of a Long-Term Care Facility

ClinicalTrials.gov

2018-02-20

Spasticity, Muscle; Muscular Diseases; Musculoskeletal Disease; Muscle Hypertonia; Muscle Spasticity; Neuromuscular Manifestations; Signs and Symptoms; Nervous System Diseases; Neurologic Manifestations

Effects of a Nintendo Wii exercise program on spasticity and static standing balance in spastic cerebral palsy.

PubMed

Gatica-Rojas, Valeska; Cartes-Velásquez, Ricardo; Méndez-Rebolledo, Guillermo; Guzman-Muñoz, Eduardo; Lizama, L Eduardo Cofré

2017-08-01

This study sought to evaluate the effects of a Nintendo Wii Balance Board (NWBB) intervention on ankle spasticity and static standing balance in young people with spastic cerebral palsy (SCP). Ten children and adolescents (aged 72-204 months) with SCP participated in an exercise program with NWBB. The intervention lasted 6 weeks, 3 sessions per week, 25 minutes for each session. Ankle spasticity was assessed using the Modified Modified Ashworth Scale (MMAS), and static standing balance was quantified using posturographic measures (center-of-pressure [CoP] measures). Pre- and post-intervention measures were compared. Significant decreases of spasticity in the ankle plantar flexor muscles (p < 0.01). There was also a significant reduction in the CoP sway area (p = 0.04), CoP mediolateral velocity (p =0.03), and CoP anterior-posterior velocity (p = 0.03). A 6-session NWBB program reduces the spasticity at the ankle plantar flexors and improves the static standing balance in young people with SCP.

Genetics Home Reference: spastic paraplegia type 7

MedlinePlus

... 000 people worldwide. Spastic paraplegia type 7 likely accounts for only a small percentage of all spastic paraplegia cases. Related Information What information about a genetic condition can statistics ...

Genetics Home Reference: spastic paraplegia type 2

MedlinePlus

... 000 people worldwide. Spastic paraplegia type 2 likely accounts for only a small percentage of all spastic paraplegia cases. Related Information What information about a genetic condition can statistics ...

Genetics Home Reference: spastic paraplegia type 8

MedlinePlus

... 000 people worldwide. Spastic paraplegia type 8 likely accounts for only a small percentage of all spastic paraplegia cases. Related Information What information about a genetic condition can statistics ...

Summary of evidence-based guideline: Complementary and alternative medicine in multiple sclerosis

PubMed Central

Yadav, Vijayshree; Bever, Christopher; Bowen, James; Bowling, Allen; Weinstock-Guttman, Bianca; Cameron, Michelle; Bourdette, Dennis; Gronseth, Gary S.; Narayanaswami, Pushpa

2014-01-01

Objective: To develop evidence-based recommendations for complementary and alternative medicine (CAM) in multiple sclerosis (MS). Methods: We searched the literature (1970–March 2011; March 2011−September 2013 MEDLINE search), classified articles, and linked recommendations to evidence. Results and recommendations: Clinicians might offer oral cannabis extract for spasticity symptoms and pain (excluding central neuropathic pain) (Level A). Clinicians might offer tetrahydrocannabinol for spasticity symptoms and pain (excluding central neuropathic pain) (Level B). Clinicians should counsel patients that these agents are probably ineffective for objective spasticity (short-term)/tremor (Level B) and possibly effective for spasticity and pain (long-term) (Level C). Clinicians might offer Sativex oromucosal cannabinoid spray (nabiximols) for spasticity symptoms, pain, and urinary frequency (Level B). Clinicians should counsel patients that these agents are probably ineffective for objective spasticity/urinary incontinence (Level B). Clinicians might choose not to offer these agents for tremor (Level C). Clinicians might counsel patients that magnetic therapy is probably effective for fatigue and probably ineffective for depression (Level B); fish oil is probably ineffective for relapses, disability, fatigue, MRI lesions, and quality of life (QOL) (Level B); ginkgo biloba is ineffective for cognition (Level A) and possibly effective for fatigue (Level C); reflexology is possibly effective for paresthesia (Level C); Cari Loder regimen is possibly ineffective for disability, symptoms, depression, and fatigue (Level C); and bee sting therapy is possibly ineffective for relapses, disability, fatigue, lesion burden/volume, and health-related QOL (Level C). Cannabinoids may cause adverse effects. Clinicians should exercise caution regarding standardized vs nonstandardized cannabis extracts and overall CAM quality control/nonregulation. Safety/efficacy of other CAM/CAM interaction with MS disease-modifying therapies is unknown. PMID:24663230

Spasticity management in multiple sclerosis.

PubMed

Hughes, Christina; Howard, Ileana M

2013-11-01

Spasticity is a prevalent and potentially disabling symptom common in individuals with multiple sclerosis. Adequate evaluation and management of spasticity requires a careful assessment of the patient's history to determine functional impact of spasticity and potential exacerbating factors, and physical examination to determine the extent of the condition and culpable muscles. A host of options for spasticity management are available: therapeutic exercise, physical modalities, complementary/alternative medicine interventions, oral medications, chemodenervation, and implantation of an intrathecal baclofen pump. Choice of treatment hinges on a combination of the extent of symptoms, patient preference, and availability of services. Copyright © 2013 Elsevier Inc. All rights reserved.

Development of GABA-sensitive spasticity and rigidity in rats after transient spinal cord ischemia: a qualitative and quantitative electrophysiological and histopathological study.

PubMed

Kakinohana, O; Hefferan, M P; Nakamura, S; Kakinohana, M; Galik, J; Tomori, Z; Marsala, J; Yaksh, T L; Marsala, M

2006-09-01

Transient spinal cord ischemia may lead to a progressive degeneration of spinal interneurons and subsequently to increased hind limb motor tone. In the present work we sought to characterize the rigidity and spasticity components of this altered motor function by: i) tonic electromyographic activity measured in gastrocnemius muscle before and after ischemia, ii) measurement of muscle resistance during the period of ankle flexion and corresponding changes in electromyographic activity, iii) changes in Hoffmann reflex, and, iv) motor evoked potentials. In addition the effect of intrathecal treatment with baclofen (GABAB receptor agonist; 1 microg), nipecotic acid (GABA uptake inhibitor; 300 microg) and dorsal L2-L5 rhizotomy on spasticity and rigidity was studied. Finally, the changes in spinal choline acetyltransferase (ChAT) and vesicular glutamate transporter 2 and 1 (VGLUT2 and VGLUT1) expression were characterized using immunofluorescence and confocal microscopy. At 3-7 days after ischemia an increase in tonic electromyographic activity with a variable degree of rigidity was seen. In animals with modest rigidity a velocity-dependent increase in muscle resistance and corresponding appearance in electromyographic activity (consistent with the presence of spasticity) was measured during ankle rotation (4-612 degrees /s rotation). Measurement of the H-reflex revealed a significant increase in Hmax/Mmax ratio and a significant loss of rate-dependent inhibition. In the same animals a potent increase in motor evoked potential amplitudes was measured and this change correlated positively with the increased H-reflex responses. Spasticity and rigidity were consistently present for a minimum of 3 months after ischemia. Intrathecal treatment with baclofen (GABA B receptor agonist) and nipecotic acid (GABA uptake inhibitor) provided a significant suppression of spasticity, rigidity, H-reflex or motor evoked potentials. Dorsal L2-L5 rhizotomy significantly decreased muscle resistance but had no effect on increased amplitudes of motor evoked potentials. Confocal analysis of spinal cord sections at 8 weeks-12 months after ischemia revealed a continuing presence of ChAT positive alpha-motoneurons, Ia afferents and VGLUT2 and VGLUT1-positive terminals but a selective loss of small presumably inhibitory interneurons between laminae V-VII. These data demonstrate that brief transient spinal cord ischemia in rat leads to a consistent development of spasticity and rigidity. The lack of significant suppressive effect of dorsal L2-L5 rhizotomy on motor evoked potentials response indicates that descending motor input into alpha-motoneurons is independent on Ia afferent couplings and can independently contribute to increased alpha-motoneuronal excitability. The pharmacology of this effect emphasizes the potent role of GABAergic type B receptors in regulating both the spasticity and rigidity.

Hereditary Spastic Paraplegia

MedlinePlus

... Mail: 800-999-NORD (6673) Spastic Paraplegia Foundation 1605 Goularte Place Fremont CA Fremont, CA 94539-7241 ... Mail: 800-999-NORD (6673) Spastic Paraplegia Foundation 1605 Goularte Place Fremont CA Fremont, CA 94539-7241 ...

Evaluating the functional outcomes of ultrasound-guided botulinum toxin type A injections using the Euro-musculus approach for upper limb spasticity treatment in post-stroke patients; an observational study.

PubMed

Buyukavci, Raikan; Akturk, Semra; Ersoy, Yüksel

2018-02-07

Ultrasound-guided botulinum toxin type A injection is an effective treatment for spasticity. Euro-musculus spasticity approach is a new method for administering injections to the correct point of the correct muscle. The clinical outcomes of this practical approach is not yet available in the literature. The purpose of this study was to evaluate the effects on spasticity and the functional outcomes of ultrasound guided botulinum toxin type A injections via the Euro-musculus spasticity approach to treat upper limb spasticity in post-stroke patients. An observational study. Inpatient post-stroke patients. Twenty five post-stroke patients with post-stroke upper limb spasticity were recruited. The ultrasound-guided botulinum toxin type A injections were administered into the spastic target muscles using the Euro-musculus spasticity approach, and all of the patients were enrolled in rehabilitation programmes after the injections. This research included the innervation zone and injection site figures and ultrasound images of each muscle in the upper limb. The degree of spasticity was assessed via the Modified Ashworth Scale and the upper limb motor function via the Fugl Meyer Upper Extremity Scale at the baseline and 4 and 12 weeks after the botulinum toxin type A injection. Significant decreases in the Modified Ashworth Scale scores of the upper limb flexor muscle tone measured 4 and 12 weeks after the botulinum toxin type A injection were found when compared to the baseline scores (p<0.025). When compared with the baseline Fugl Meyer Upper Extremity subgroup scores, the sitting position, wrist and total scores at 4 and 12 weeks were significantly improved (p<0.025). However, only the Fugl Meyer Upper Extremity hand scores were significantly improved 12 weeks after the injection (p<0.025). Ultrasound-guided botulinum toxin type A injection via the Euro- musculus spasticity approach is a practical and effective method for administering injections to the correct point of the correct muscle. Ultrasound-guided botulinum toxin type A injections combined with rehabilitation programmes decrease spasticity and improve the upper extremity motor functions in stroke patients. This new approach for ultrasound- guided botulinum toxin type A injection is very practical and effective method for upper extremity spasticity.

Spastic long-lasting reflexes in the awake rat after sacral spinal cord injury.

PubMed

Bennett, D J; Sanelli, L; Cooke, C L; Harvey, P J; Gorassini, M A

2004-05-01

Following chronic sacral spinal cord transection in rats the affected tail muscles exhibit marked spasticity, with characteristic long-lasting tail spasms evoked by mild stimulation. The purpose of the present paper was to characterize the long-lasting reflex seen in tail muscles in response to electrical stimulation of the tail nerves in the awake spastic rat, including its development with time and relation to spasticity. Before and after sacral spinal transection, surface electrodes were placed on the tail for electrical stimulation of the caudal nerve trunk (mixed nerve) and for recording EMG from segmental tail muscles. In normal and acute spinal rats caudal nerve trunk stimulation evoked little or no EMG reflex. By 2 wk after injury, the same stimulation evoked long-lasting reflexes that were 1) very low threshold, 2) evoked from rest without prior EMG activity, 3) of polysynaptic latency with >6 ms central delay, 4) about 2 s long, and 5) enhanced by repeated stimulation (windup). These reflexes produced powerful whole tail contractions (spasms) and developed gradually over the weeks after the injury (< or =52 wk tested), in close parallel to the development of spasticity. Pure low-threshold cutaneous stimulation, from electrical stimulation of the tip of the tail, also evoked long-lasting spastic reflexes, not seen in acute spinal or normal rats. In acute spinal rats a strong C-fiber stimulation of the tip of the tail (20 x T) could evoke a weak EMG response lasting about 1 s. Interestingly, when this C-fiber stimulation was used as a conditioning stimulation to depolarize the motoneuron pool in acute spinal rats, a subsequent low-threshold stimulation of the caudal nerve trunk evoked a 300-500 ms long reflex, similar to the onset of the long-lasting reflex in chronic spinal rats. A similar conditioned reflex was not seen in normal rats. Thus there is an unusually long low-threshold polysynaptic input to the motoneurons (pEPSP) that is normally inhibited by descending control. This pEPSP is released from inhibition immediately after injury but does not produce a long-lasting reflex because of a lack of motoneuron excitability. With chronic injury the motoneuron excitability is increased markedly, and the pEPSP then triggers sustained motoneuron discharges associated with long-lasting reflexes and muscle spasms.

Characterization of Spasticity in Cerebral Palsy: Dependence of Catch Angle on Velocity

ERIC Educational Resources Information Center

Wu, Yi-Ning; Ren, Yupeng; Goldsmith, Ashlee; Gaebler, Deborah; Liu, Shu Q.; Zhang, Li-Qun

2010-01-01

Aim: To evaluate spasticity under controlled velocities and torques in children with cerebral palsy (CP) using a manual spasticity evaluator. Method: The study involved 10 children with spastic CP (six males, four females; mean age 10y 1mo, SD 2y 9mo, range 7-16y; one with quadriplegia, six with right hemiplegia, three with left hemiplegia; Gross…

Full Body Gait Analysis May Improve Diagnostic Discrimination Between Hereditary Spastic Paraplegia and Spastic Diplegia: A Preliminary Study

ERIC Educational Resources Information Center

Bonnefoy-Mazure, A.; Turcot, K.; Kaelin, A.; De Coulon, G.; Armand, S.

2013-01-01

Hereditary spastic paraplegia (HSP) and spastic diplegia (SD) patients share a strong clinical resemblance. Thus, HSP patients are frequently misdiagnosed with a mild form of SD. Clinical gait analysis (CGA) has been highlighted as a possible tool to support the differential diagnosis of HSP and SD. Previous analysis has focused on the lower-body…

The negative impact of spasticity on the health-related quality of life of stroke survivors: a longitudinal cohort study.

PubMed

Gillard, Patrick J; Sucharew, Heidi; Kleindorfer, Dawn; Belagaje, Samir; Varon, Sepideh; Alwell, Kathleen; Moomaw, Charles J; Woo, Daniel; Khatri, Pooja; Flaherty, Matthew L; Adeoye, Opeolu; Ferioli, Simona; Kissela, Brett

2015-09-29

Spasticity often leads to symptomatic and functional problems that can cause disability for stroke survivors. We studied whether spasticity has a negative impact on health-related quality of life (HRQoL). As part of the Greater Cincinnati/Northern Kentucky Stroke Study (NCT00642213), 460 ischemic stroke patients were interviewed during hospitalization and then followed over time. HRQoL was measured by the Physical Component Summary (PCS) and Mental Component Summary (MCS) scores of the Short Form-12 (SF-12), EuroQol-5 dimension (EQ-5D), and Stroke-Specific Quality of Life (SSQOL) instruments, with lower scores indicating worse health. HRQoL differences between stroke survivors with and without spasticity were compared, adjusting for age, race, stroke severity, pre-stroke function, and comorbidities. Of the 460 ischemic stroke patients, 328 had spasticity data available 3 months after their stroke (mean age of 66 years, 49% were female, and 26% were black). Of these patients, 54 (16%) reported having spasticity. Three months following their stroke, patients who reported spasticity had lower mean scores on the PCS (29.6 ± 1.4 vs 37.3 ± 0.6; P < .001), EQ-5D (0.59 ± 0.03 vs 0.71 ± 0.01; P < .001), and SSQOL (3.57 ± 0.08 versus 3.78 ± 0.03; P = .03) compared with patients who did not report spasticity. Lower HRQoL scores were also observed at the 1-year (PCS, EQ-5D, and SSQOL) and 2-year (EQ-5D and SSQOL) interviews in those with spasticity compared with those without spasticity. Statistically and clinically meaningful differences in HRQoL exist between stroke survivors with and without spasticity.

Genetics Home Reference: spastic paraplegia type 31

MedlinePlus

... per 100,000 individuals. Spastic paraplegia type 31 accounts for 3 to 9 percent of all autosomal dominant hereditary spastic paraplegia cases. Related Information What information about a genetic condition can statistics ...

[Neuromotor assessment of patients with spastic cerebral palsy treated with orthopedic surgery at the National Rehabilitation Institute].

PubMed

Piana, A R; Viñals, C L; Del Valle, M C; Arellano, M S; Redón, A T; Peralta, S C; León, S L

2010-01-01

Cerebral palsy (CP) is a static neurologic condition resulting from a brain lesion occurring before the completion of brain development. The goal of management is not cure, but increasing patients' functionality and improving their capabilities and maintaining their locomotion, cognitive development, social interaction and independence. The best results are obtained with an early and intensive management that includes physical and occupational therapy, medical and surgical treatments, mechanical aids and the management of concomitant conditions. To assess the neuromotor improvement in patients with spastic CP after surgical treatment at the National Rehabilitation Institute. Patients with a diagnosis of spastic CP who presented at the Pediatric Rehabilitation outpatient service were referred to the Joint CP Clinic from January 2007 to January 2008, and underwent surgical treatment of the pelvic limbs. They were assessed 3 times and underwent neuromotor tests with gross motor function measure (GMFM), which was rated with the gross motor function classification system (GMFCS). Most of the patients had improvement in the muscle tone and contracture assessments as well as in the GMFM, and their self-mobility increased one level. Significant improvements were seen in the muscle tone and contractures after surgery; the GMFM and the self-mobility levels in the GMFCS also improved. Multiple level surgery together with a postoperative physical therapy program results in considerable improvements in the gross motor function measure of patients with spastic CP.

Epidemiologic Overview of Synkinesis in 353 Patients with Longstanding Facial Paralysis under Treatment with Botulinum Toxin for 11 Years.

PubMed

Salles, Alessandra Grassi; da Costa, Eduardo Fernandes; Ferreira, Marcus Castro; Remigio, Adelina Fatima do Nascimento; Moraes, Luciana Borsoi; Gemperli, Rolf

2015-12-01

Patients with longstanding facial paralysis often exhibit synkinesis. Few reports describe the prevalence and factors related to the development of synkinesis after facial paralysis. Botulinum toxin type A injection is an important adjunct treatment for facial paralysis-induced asymmetry and synkinesis. The authors assessed the clinical and epidemiologic characteristics of patients with sequelae of facial paralysis treated with botulinum toxin type A injections to evaluate the prevalence of synkinesis and related factors. A total of 353 patients (age, 4 to 84 years; 245 female patients) with longstanding facial paralysis underwent 2312 botulinum toxin type A injections during an 11-year follow-up. Doses used over the years, previous treatments (electrical stimulation, operations), and how they correlated to postparalysis and postreanimation synkinesis were analyzed. There was a significant association between cause and surgery. Most patients with facial paralysis caused by a congenital defect, trauma, or a tumor underwent reanimation. There were no sex- or synkinesis-related differences in the doses used, but the doses were higher in the reanimation group than in the no-surgery group. Synkinesis was found in 196 patients; 148 (41.9 percent) presented with postparalysis synkinesis (oro-ocular, oculo-oral) and 58 (16.4 percent) presented with postreanimation synkinesis. Ten patients presented with both types. This study determined the high prevalence (55.5 percent) of synkinesis in patients with longstanding facial paralysis. Postparalysis synkinesis was positively associated with infectious and idiopathic causes, electrical stimulation, facial nerve decompression, and no requirement for surgery. Postreanimation synkinesis was present in 28.2 percent of reanimated patients and was significantly associated with microsurgical flaps, transfacial nerve grafting, masseteric-facial anastomosis, and temporalis muscle transfers.

Effects of vibration on spasticity in individuals with spinal cord injury: a scoping systematic review.

PubMed

Sadeghi, Mahsa; Sawatzky, Bonita

2014-11-01

The objective of this systematic review was to evaluate how whole-body vibration (WBV) or focal vibration (FV) would change spasticity in individuals with spinal cord injury (SCI). A search was conducted of MEDLINE, EMBASE, CINAHL, and PsycINFO electronic databases. A hand search was conducted of the bibliographies of articles and journals relevant to the research question. The inclusion criteria were three or more individuals, 17 yrs or older, with SCI who experience spasticity, and WBV or FV application. The evidence level of all ten identified studies (195 SCI subjects) was low on the basis of Centre for Evidence Based Medicine level of evidence. WBV (n = 1) and FV (n = 9) were applied to assess the effects of vibration on different measures of spasticity in individuals with SCI. FV application resulted in a short-term spasticity reduction lasting for a maximum of 24 hrs. Neurophysiologic measures showed H-reflex inhibition in individuals with SCI after FV application. WBV resulted in a decrease in spasticity lasting for 6-8 days after the last vibration session. WBV and FV might decrease spasticity for a short period, but no evidence-based recommendation can be drawn from the literature to guide rehabilitation medicine clinicians to manage spasticity with vibration application.

Treatment with Botulinum Toxin for Refractory Fever Caused by Severe Spasticity: A Case Series.

PubMed

Lester, Jacobo; Alvarez-Resendiz, Gerardo Esteban; Klériga, Enrique; Videgaray, Fernando; Zambito, Gerardo

2018-06-01

Brain and spinal cord injuries may cause very severe spasticity that occasionally may be associated with persistent fever. We present 14 patients with spasticity and persistent fever, treated with botulinum toxin type A. Their spasticity improved and the fever resolved within a period no greater than 48 h. In all cases, infectious and other non-infectious causes were ruled out. When sustained tonic muscular activity is associated with a significant increase in body temperature and is refractory to the usual drugs used for hyperpyrexia, type A botulinum toxin may be an effective treatment option to control both spasticity and fever.

Ankle Plantarflexor Spasticity Does Not Restrict the Recovery of Ankle Plantarflexor Strength or Ankle Power Generation for Push-Off During Walking Following Traumatic Brain Injury.

PubMed

Williams, Gavin; Banky, Megan; Olver, John

2016-01-01

The main aim of this project was to determine the impact of plantarflexor spasticity on muscle performance for ambulant people with traumatic brain injury (TBI). A large metropolitan rehabilitation hospital. Seventy-two ambulant people with TBI who were attending physiotherapy for mobility limitations. Twenty-four participants returned for a 6-month follow-up reassessment. Cross-sectional cohort study. Self-selected walking speed, Tardieu scale, ankle plantarflexor strength, and ankle power generation (APG). Participants with ankle plantarflexor spasticity had significantly lower self-selected walking speed; however, there was no significant difference in ankle plantarflexor strength or APG. Participants with ankle plantarflexor spasticity were not restricted in the recovery of self-selected walking speed, ankle plantarflexor strength, or APG, indicating equivalent ability to improve their mobility over time despite the presence of spasticity. Following TBI, people with ankle plantarflexor spasticity have significantly greater mobility limitations than those without spasticity, yet retain the capacity for recovery of self-selected walking speed, ankle plantarflexor strength, and APG.

Spasticity in multiple sclerosis and role of glatiramer acetate treatment

PubMed Central

Meca-Lallana, Jose Eustasio; Hernández-Clares, Rocío; Carreón-Guarnizo, Ester

2015-01-01

Introduction Spasticity is one of the most disabling and difficult-to-treat symptoms shown by patients with multiple sclerosis, who often show a suboptimal and unsatisfactory response to classic treatment and new available nonpharmacological alternatives. Due to the progressive nature of this condition, the early management should be essential to improve long-term outcomes. Methods We performed a narrative literature review of the contribution of spasticity to the burden of multiple sclerosis and the potential role of classic disease-modifying drugs. Results Added to the underlying pathophysiology of spasticity, certain external factors and drugs such as interferon may exacerbate the existing condition, hence their awareness is crucial as part of an effective management of spasticity. Furthermore, the evidence for the effectiveness of glatiramer acetate in preventing spasticity in naïve patients and in those switching from interferon should not be ignored. Conclusions This literature review proposes the examination of spasticity and the influence of classic disease-modifying agents on the level of existing condition among the variables to be considered when deciding on therapy for multiple sclerosis in clinical practice. PMID:26445705

[Paralysis, organic brain syndrome, and cardiac dysrhythmias caused by chronic laxative abuse (author's transl)].

PubMed

Dahlmann, W; Volles, E; Lüderitz, B

1977-10-28

A 39-year-old woman developed generalised paralysis, reversible organic brain syndrome, and cardiac dysrhythmias after 15 years of laxative abuse. Under continuous and cautious administration of potassium the cardiac rhythm became normal within four days and two days later the paralysis and organic brain syndrome almost disappeared. The cause of the psychiatric symptoms is thought to be cerebral potassium deficiency and an abnormal sodium/potassium equilibrium. Other clinical signs and symptoms due to extreme potassium depletion are presented. The importance of Na+/K+-activated membrane ATP-ase in myocardium and CNS is discussed.

Right diaphragmatic paralysis following endocardial cryothermal ablation of inappropriate sinus tachycardia.

PubMed

Vatasescu, Radu; Shalganov, Tchavdar; Kardos, Attila; Jalabadze, Khatuna; Paprika, Dora; Gyorgy, Margit; Szili-Torok, Tamas

2006-10-01

Inappropriate sinus tachycardia (IST) is a rare disorder amenable to catheter ablation when refractory to medical therapy. Radiofrequency (RF) catheter modification/ablation of the sinus node (SN) is the usual approach, although it can be complicated by right phrenic nerve paralysis. We describe a patient with IST, who had symptomatic recurrences despite previous acutely successful RF SN modifications, including the use of electroanatomical mapping/navigation system. We decided to try transvenous cryothermal modification of the SN. We used 2 min applications at -85 degrees C at sites of the earliest atrial activation guided by activation mapping during isoprenaline infusion. Every application was preceded by high output stimulation to reveal phrenic nerve proximity. During the last application, heart rate slowly and persistently fell below 85 bpm despite isoprenaline infusion, but right diaphragmatic paralysis developed. At 6 months follow-up, the patient was asymptomatic and the diaphragmatic paralysis had partially resolved. This is the first report, we believe, of successful SN modification for IST by endocardial cryoablation, although this case also demonstrates the considerable risk of right phrenic nerve paralysis even with this ablation energy.

The madness of Dionysus -- six hypotheses on the illness of Nietzsche.

PubMed

Tényi, Tamás

2012-01-01

Friedrich Nietzsche (1844-1900) is considered as one of the most influential modern thinkers of the last two centuries. The great philosopher and poet developed a mental illness at the age of 44 and died at the age of 56. Pathological examination was not undertaken. At that time Nietzsche was diagnosed as having atypical paralysis progressiva, however recently five other probable diagnoses appeared in literature. Literature search in MEDLINE and Web of Science on the illness of Nietzsche. Six hypotheses were identified: 1. Paralysis progressiva (General paralysis of the insane) 2. Bipolar affective disorder followed by vascular dementia 3. Hereditary form of frontotemporal dementia 4. Brain tumor 5. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) 6. Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome. Developments in neurology and molecular genetics give new perspectives to the secret of Nietzsche's illness and also there is a consensus on the questioning of the original paralysis progressiva concept. As there was no postmortem, the clinical speculations on the medical problems of the great philosopher remain a challenge.

Use of Botulinum Neurotoxin Injections to Treat Spasticity

MedlinePlus

... walking. These problems, called spasticity, are common in cerebral palsy, traumatic brain injury, stroke, multiple sclerosis, and spinal ... How does BoNT control spasticity in children with cerebral palsy (CP)? There is strong evidence that BoNT injections ...

Muscle torque of healthy individuals and individuals with spastic hemiparesis after passive static streching.

PubMed

Tatsukawa DE Freitas, Sérgio Takeshi; DE Carvalho Abreu, Elizângela Márcia; Dos Reis, Mariane Cecilia; DE Souza Cunha, Bruna; Souza Moreira Prianti, Tamires; Pupio Silva Lima, Fernanda; Oliveira Lima, Mário

2016-01-01

Spasticity is one of the main causes of contracture, muscle weakness and subsequent functional incapacity. The passive static stretching can be included as having the purpose of increasing musculoskeletal flexibility, however, it also can influence the muscle torque. The objective is to verify the immediate effect of passive static stretching in the muscle strength of healthy and those who present spastic hemiparesis. There were assessed 20 subjects, 10 spastic hemiparetic (EG) and 10 healthy individuals (CG), including both sexes, aged between 22 and 78 years. The torque of extensor muscles of the knee was analyzed using isokinetic dynamometer. Results have shown that EG has less muscle torque compared to CG ( p < 0.01). In addition, EG presented a decrease in significance of muscle torque after stretching ( p < 0.05), however, it has not shown significant alteration in muscle torque of CG after performing the program that was prescribed. Immediately after the passive stretch, a significant torque decrease can be seen in hypertonic muscle; it is believed that this reduction may be associated with the physiological overlap between actin and myosin filaments and so preventing the muscle to develop a maximum contraction.

An order in Lewy body disorders: Retrograde degeneration in hyperbranching axons as a fundamental structural template accounting for focal/multifocal Lewy body disease.

PubMed

Uchihara, Toshiki

2017-04-01

Initial clinical recognition of "paralysis agitans" by James Parkinson was expanded by Jean-Martin Charcot, who recognized additional clinical findings of his own, such as slowness (distinct from paralysis), rigidity (distinct from spasticity) and characteristic countenance. Charcot assembled these findings under the umbrella of "Parkinson disease (PD)". This purely clinical concept was so prescient and penetrating that subsequent neuropathological and biochemical evidences were ordered along this axis to establish the nigra-central trinity of PD (dopamine depletion, nigral lesion with Lewy bodies: LBs). Although dramatic efficacy of levodopa boosted an enthusiasm for this nigra-centralism, extranigral lesions were identified, especially after identification of alpha-synuclein (αS) as a major constituent of LBs. Frequent αS lesions in the lower brainstem with their presumed upward spread were coupled with the self-propagating property of αS molecule, as a molecular template, to constitute the prion-Braak hypothesis. This hybrid concept might expectedly explain clinical, structural and biochemical features of PD/dementia with Lewy bodies (DLB) as if they were stereotypic. In spite of this ordered explanation, recent studies have demonstrated unexpectedly that αS lesions in the human brain, as well as their corresponding clinical manifestations, are much more disordered. Even with such a chaos of LB disorders, affected neuronal groups are uniformly characterized by hyperbranching axons, which may facilitate distal-dominant degeneration and retrograde progression of LB-related degeneration along axons as a fundamental structural order to template LB disorders. This "structural template" hypothesis may explain why: (i) some selective groups are prone to develop Lewy pathology; (ii) their clinical manifestations (especially non-motor components) are vague and generalized without somatotopic accentuation; (iii) distal axons and terminals are preferentially affected early, which is clinically detectable as reduced myocardial uptake of meta-iodobenzylguanidine in PD/DLB. Because each Lewy-prone system develops LBs independently, their isolated presentation as "focal LB disease" or their whatever combinations as "multifocal LB disease" are a more plausible framework to explain clinicopathological diversities of LB disorders. Clinical criteria are now being revised to integrate these clinicopathological disorders of PD/DLB. To gain closer access to the reality of the human brain, it is necessary to facilitate more interactions between clinicopathological and experimental fields so that both are mutually critical and complementary for improved diagnosis and treatment. © 2016 Japanese Society of Neuropathology.

Dynamometer-based measure of spasticity confirms limited association between plantarflexor spasticity and walking function in persons with multiple sclerosis

PubMed Central

Kremer, Theodore R.; Van Dillen, Linda R.; Wagner, Joanne M.

2017-01-01

The literature shows inconsistent evidence regarding the association between clinically assessed plantarflexor (PF) spasticity and walking function in ambulatory persons with multiple sclerosis (pwMS). The use of a dynamometer-based spasticity measure (DSM) may help to clarify this association. Our cohort included 42 pwMS (27 female, 15 male; age: 42.9 +/− 10.2 yr) with mild clinical disability (Expanded Disability Status Scale score: 3.6 +/− 1.6). PF spasticity was assessed using a clinical measure, the modified Ashworth Scale (MAS), and an instrumented measure, the DSM. Walking function was assessed by the timed 25-foot walk test (T25FWT), the 6-minute walk test (6MWT), and the 12-item Multiple Sclerosis Walking Scale (MSWS-12). Spearman rho correlations were used to evaluate relationships between spasticity measures, measures of walking speed and endurance, and self-perceived limitations in walking. The correlation was small between PF spasticity and the T25FWT (PF maximum [Max] MAS rho = 0.27, PF Max DSM rho = 0.26), the 6MWT (PF Max MAS rho = −0.20, PF Max DSM rho = −0.21), and the MSWS-12 (PF Max MAS rho = 0.11, PF Max DSM rho = 0.26). Our results are similar to reports in other neurologic clinical populations, wherein spasticity has a limited association with walking dysfunction. PMID:25356797

Procedure-Oriented Torsional Anatomy of the Hand for Spasticity Injection.

PubMed

John, Joslyn; Cianca, John; Chiou-Tan, Faye; Pandit, Sindhu; Furr-Stimming, Erin; Taber, Katherine H

To provide musculoskeletal ultrasound (MSKUS) images of hand anatomy in the position of hemiparetic flexion as a reference for spasticity injections. After a stroke, spasticity can result in anatomic distortion of the hand. Spasticity may require treatment with botulinum toxin or phenol injections. Anatomic distortion may decrease the accuracy of injections. Standard anatomic references are of limited utility because they are not in this spastic hemiparetic position. There presently is no anatomic reference in the literature for these spastic postures. This study is part three of a series examining torsional anatomy of the body. Ultrasound (US) images were obtained in a healthy subject. The muscles examined included the lumbricals and the flexor pollicis brevis. A marker dot was placed at each dorsal and palmar anatomic injection site for these muscles. The US probe was placed on these dots to obtain a cross-sectional view. A pair of US images was recorded with and without power Doppler imaging: the first in anatomic neutral and second in hemiparetic spastic positions. In addition, a video recording of the movement of the muscles during this rotation was made at each site. On the palmar view, the lumbricals rotated medially. On dorsal view, the lumbricals can be seen deep to the dorsal interossei muscles, with spastic position, and they become difficult to identify. The flexor pollicis brevis (FPB) muscle contracts with torsion, making abductor pollicis brevis (APB) predominately in view. The anatomic location of the lumbrical muscles makes them difficult to inject even with ultrasound guidance. However, recognizing the nearby digital vasculature allows for improved identification of the musculature for injection purposes. The FPB muscle also can be identified by its adjacent radial artery lateral to the flexor pollicus longus tendon. Normal anatomy of hand can become distorted in spastic hemiparesis. Diagnostic ultrasound is able to discern these anatomic locations if the sonographer is competent in recognizing the appearance of normal anatomy and is skilled in resolving the visual changes that occur in spastic hemiparesis. The authors hope this series of images will increase the accuracy, safety, and efficacy of spasticity injections in the hand.

Degeneration of serotonergic neurons in amyotrophic lateral sclerosis: a link to spasticity.

PubMed

Dentel, Christel; Palamiuc, Lavinia; Henriques, Alexandre; Lannes, Béatrice; Spreux-Varoquaux, Odile; Gutknecht, Lise; René, Frédérique; Echaniz-Laguna, Andoni; Gonzalez de Aguilar, Jose-Luis; Lesch, Klaus Peter; Meininger, Vincent; Loeffler, Jean-Philippe; Dupuis, Luc

2013-02-01

Spasticity is a common and disabling symptom observed in patients with central nervous system diseases, including amyotrophic lateral sclerosis, a disease affecting both upper and lower motor neurons. In amyotrophic lateral sclerosis, spasticity is traditionally thought to be the result of degeneration of the upper motor neurons in the cerebral cortex, although degeneration of other neuronal types, in particular serotonergic neurons, might also represent a cause of spasticity. We performed a pathology study in seven patients with amyotrophic lateral sclerosis and six control subjects and observed that central serotonergic neurons suffer from a degenerative process with prominent neuritic degeneration, and sometimes loss of cell bodies in patients with amyotrophic lateral sclerosis. Moreover, distal serotonergic projections to spinal cord motor neurons and hippocampus systematically degenerated in patients with amyotrophic lateral sclerosis. In SOD1 (G86R) mice, a transgenic model of amyotrophic lateral sclerosis, serotonin levels were decreased in brainstem and spinal cord before onset of motor symptoms. Furthermore, there was noticeable atrophy of serotonin neuronal cell bodies along with neuritic degeneration at disease onset. We hypothesized that degeneration of serotonergic neurons could underlie spasticity in amyotrophic lateral sclerosis and investigated this hypothesis in vivo using tail muscle spastic-like contractions in response to mechanical stimulation as a measure of spasticity. In SOD1 (G86R) mice, tail muscle spastic-like contractions were observed at end-stage. Importantly, they were abolished by 5-hydroxytryptamine-2b/c receptors inverse agonists. In line with this, 5-hydroxytryptamine-2b receptor expression was strongly increased at disease onset. In all, we show that serotonergic neurons degenerate during amyotrophic lateral sclerosis, and that this might underlie spasticity in mice. Further research is needed to determine whether inverse agonists of 5-hydroxytryptamine-2b/c receptors could be of interest in treating spasticity in patients with amyotrophic lateral sclerosis.

Lowland copperhead (Austrelaps superbus) envenomation causing severe neuromuscular paralysis in a dog.

PubMed

Wright, L V; Indrawirawan, Y H

2017-06-01

A case of lowland copperhead snake (Austrelaps superbus) envenomation in a dog is described. The dog developed severe and prolonged neuromuscular paralysis, including ventilatory failure. The dog was treated successfully with antivenom, intravenous fluids and mechanical ventilation. The toxic components of lowland copperhead snake venom are reviewed. © 2017 Australian Veterinary Association.

Prevalence and Causes of Paralysis-United States, 2013.

PubMed

Armour, Brian S; Courtney-Long, Elizabeth A; Fox, Michael H; Fredine, Heidi; Cahill, Anthony

2016-10-01

To estimate the prevalence and causes of functional paralysis in the United States. We used the 2013 US Paralysis Prevalence & Health Disparities Survey to estimate the prevalence of paralysis, its causes, associated sociodemographic characteristics, and health effects among this population. Nearly 5.4 million persons live with paralysis. Most persons with paralysis were younger than 65 years (72.1%), female (51.7%), White (71.4%), high school graduates (64.8%), married or living with a partner (47.4%), and unable to work (41.8%). Stroke is the leading cause of paralysis, affecting 33.7% of the population with paralysis, followed by spinal cord injury (27.3%), multiple sclerosis (18.6%), and cerebral palsy (8.3%). According to the functional definition, persons living with paralysis represent a large segment of the US population, and two thirds of them are between ages 18 and 64 years. Targeted health promotion that uses inclusion strategies to account for functional limitations related to paralysis can be undertaken in partnership with state and local health departments.

Diversity of ARSACS mutations in French-Canadians.

PubMed

Thiffault, I; Dicaire, M J; Tetreault, M; Huang, K N; Demers-Lamarche, J; Bernard, G; Duquette, A; Larivière, R; Gehring, K; Montpetit, A; McPherson, P S; Richter, A; Montermini, L; Mercier, J; Mitchell, G A; Dupré, N; Prévost, C; Bouchard, J P; Mathieu, J; Brais, B

2013-01-01

The growing number of spastic ataxia of Charlevoix-Saguenay (SACS) gene mutations reported worldwide has broadened the clinical phenotype of autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). The identification of Quebec ARSACS cases without two known SACS mutation led to the development of a multi-modal genomic strategy to uncover mutations in this large gene and explore phenotype variability. Search for SACS mutations by combining various methods on 20 cases with a classical French-Canadian ARSACS phenotype without two mutations and a group of 104 sporadic or recessive spastic ataxia cases of unknown cause. Western blot on lymphoblast protein from cases with different genotypes was probed to establish if they still expressed sacsin. A total of 12 mutations, including 7 novels, were uncovered in Quebec ARSACS cases. The screening of 104 spastic ataxia cases of unknown cause for 98 SACS mutations did not uncover carriers of two mutations. Compounds heterozygotes for one missense SACS mutation were found to minimally express sacsin. The large number of SACS mutations present even in Quebec suggests that the size of the gene alone may explain the great genotypic diversity. This study does not support an expanding ARSACS phenotype in the French-Canadian population. Most mutations lead to loss of function, though phenotypic variability in other populations may reflect partial loss of function with preservation of some sacsin expression. Our results also highlight the challenge of SACS mutation screening and the necessity to develop new generation sequencing methods to ensure low cost complete gene sequencing.

Classification of upper limb disability levels of children with spastic unilateral cerebral palsy using K-means algorithm.

PubMed

Raouafi, Sana; Achiche, Sofiane; Begon, Mickael; Sarcher, Aurélie; Raison, Maxime

2018-01-01

Treatment for cerebral palsy depends upon the severity of the child's condition and requires knowledge about upper limb disability. The aim of this study was to develop a systematic quantitative classification method of the upper limb disability levels for children with spastic unilateral cerebral palsy based on upper limb movements and muscle activation. Thirteen children with spastic unilateral cerebral palsy and six typically developing children participated in this study. Patients were matched on age and manual ability classification system levels I to III. Twenty-three kinematic and electromyographic variables were collected from two tasks. Discriminative analysis and K-means clustering algorithm were applied using 23 kinematic and EMG variables of each participant. Among the 23 kinematic and electromyographic variables, only two variables containing the most relevant information for the prediction of the four levels of severity of spastic unilateral cerebral palsy, which are fixed by manual ability classification system, were identified by discriminant analysis: (1) the Falconer index (CAI E ) which represents the ratio of biceps to triceps brachii activity during extension and (2) the maximal angle extension (θ Extension,max ). A good correlation (Kendall Rank correlation coefficient = -0.53, p = 0.01) was found between levels fixed by manual ability classification system and the obtained classes. These findings suggest that the cost and effort needed to assess and characterize the disability level of a child can be further reduced.

Effect of Transcutaneous Electrical Nerve Stimulation on Plantar Flexor Muscle Spasticity and Walking Speed in Stroke Patients.

PubMed

Laddha, Darshan; Ganesh, G Shankar; Pattnaik, Monalisa; Mohanty, Patitapaban; Mishra, Chittaranjan

2016-12-01

Spasticity is a major disabling symptom in patients post stroke. Although studies have demonstrated that transcutaneous electrical nerve stimulation (TENS) can reduce spasticity, the duration of single session TENS is a subject of debate. The purpose of this study was to determine the sustainability of the effects of TENS applied over common peroneal nerve in the reduction of ankle plantar-flexor spasticity and improving gait speed in patients post stroke. Thirty patients (11 women and 19 men) (mean age of 46.46 years) were randomly assigned to group 1 (task oriented exercises), group 2 (TENS for 30 min and task oriented exercises) and group 3 (TENS for 60 min and task oriented exercises) for a period of five sessions per week for 6 weeks. All patients were assessed for ankle plantar-flexor spasticity, passive ankle dorsi-flexion range of motion, clonus and timed up and go test at the time of recruitment to study, at 3 and 6 weeks of therapeutic intervention. The overall results of the study suggest that there was a decrease in ankle plantar flexor spasticity, ankle clonus and timed up and go score in all the groups. A greater reduction of spasticity was seen in TENS groups (groups 2 and 3) when compared to control. No significant improvement was found in timed up and go test (TUG) scores between groups. Both 30 min and 60 min of application of TENS are effective in reducing spasticity of ankle plantar flexors, improving walking ability and increase the effectiveness of task related training. Based on the effect size, we would recommend a longer duration application for the reduction of spasticity. Copyright © 2015 John Wiley & Sons, Ltd. Copyright © 2015 John Wiley & Sons, Ltd.

Does spasticity contribute to walking dysfunction after stroke?

PubMed Central

Ada, L.; Vattanasilp, W.; O'Dwyer, N.; Crosbie, J.

1998-01-01

OBJECTIVES—Clinically, it is assumed that spasticity of the calf muscles interferes with walking after stroke. The aim was to examine this assumption by evaluating the contribution of spasticity in the gastrocnemius muscle to walking dysfunction in an ambulant stroke population several months after stroke.
METHODS—Fourteen stroke patients who were able to walk independently and 15 neurologically normal control subjects were recruited. Both resting and action stretch reflexes of the gastrocnemius muscle were investigated under conditions that simulated walking. Resting tonic stretch reflexes were measured to assess spasticity whereas action tonic stretch reflexes were measured to assess the possible contribution of spasticity to gait dysfunction.
RESULTS—Two thirds of the stroke patients exhibited resting tonic stretch reflexes which indicate spasticity, whereas none of the control subjects did. However, the stroke patients exhibited action tonic stretch reflexes that were of similar magnitude to the control subjects, suggesting that their reflex activity during walking was not different from that of control subjects. Furthermore, there was no evidence that the action stretch reflex in the stroke patients contributed a higher resistance to stretch than the control subjects.
CONCLUSIONS—Whereas most of the stroke patients exhibited spasticity when measured both clinically and physiologically, they did not exhibit an increase in resistance to dorsiflexion due to exaggerated action tonic stretch reflexes. It is concluded that it is unlikely that spasticity causes problems in walking after stroke in ambulant patients. Therefore, it seems inappropriate to routinely reduce or inhibit the reflex response to improve functional movement in stroke rehabilitation. Factors other than spasticity should be considered when analysing walking after stroke, so that appropriate treatment is provided to patients.

 PMID:9598679

Does reducing spasticity translate into functional benefit? An exploratory meta-analysis

PubMed Central

Francis, H; Wade, D; Turner-Stokes, L; Kingswell, R; Dott, C; Coxon, E

2004-01-01

Background: Spasticity and loss of function in an affected arm are common after stroke. Although botulinum toxin is used to reduce spasticity, its functional benefits are less easily demonstrated. This paper reports an exploratory meta-analysis to investigate the relationship between reduced arm spasticity and improved arm function. Method: Individual data from stroke patients in two randomised controlled trials of intra-muscular botulinum toxin were pooled. The Modified Ashworth Scale (elbow, wrist, fingers) was used to calculate a "Composite Spasticity Index". Data from the arm section of the Barthel Activities of Daily Living Index (dressing, grooming, and feeding) and three subjective measures (putting arm through sleeve, cleaning palm, cutting fingernails) were summed to give a "Composite Functional Index". Change scores and the time of maximum change were also calculated. Results: Maximum changes in both composite measures occurred concurrently in 47 patients. In 26 patients the improvement in spasticity preceded the improvement in function with 18 showing the reverse. There was a definite relationship between the maximum change in spasticity and the maximum change in arm function, independent of treatment (ρ = –0.2822, p = 0.0008, n = 137). There was a clear relationship between the changes in spasticity and in arm function in patients treated with botulinum toxin (Dysport) at 500 or 1000 units (ρ = –0.5679, p = 0.0090, n = 22; ρ = –0.4430, p = 0.0018, n = 47), but not in those treated with placebo or 1500 units. Conclusions: Using a targeted meta-analytic approach, it is possible to demonstrate that reducing spasticity in the arm is associated with a significant improvement in arm function. PMID:15489384

Botulinum toxin type a injections to salivary glands: combination with single event multilevel chemoneurolysis in 2 children with severe spastic quadriplegic cerebral palsy.

PubMed

Kim, Heakyung; Lee, Yung; Weiner, Daniel; Kaye, Robin; Cahill, Anne Marie; Yudkoff, Marc

2006-01-01

We describe 2 children with severe spastic quadriplegic cerebral palsy (CP) who have significant drooling and frequent aspiration pneumonia. They underwent simultaneous botulinum toxin type A (BTX-A) injections to salivary glands for drooling and prevention of aspiration pneumonia along with single-event multilevel chemoneurolysis (SEMLC) with BTX-A and 5% phenol for severe diffuse spasticity. There was significant improvement in drooling, frequency of aspiration pneumonia, and spasticity without adverse effect. BTX-A injections into the salivary glands, in addition to SEMLC, for these 2 children with medically complicated severe spastic quadriplegic CP, were safe and highly successful procedures, which improved their health-related quality of life.

West Nile virus encephalomyelitis with polio-like paralysis & nigral degeneration.

PubMed

Schafernak, Kristian T; Bigio, Eileen H

2006-11-01

Patients infected with West Nile virus (WNV) may develop acute neurologic disease, which can be severe or even fatal, including WNV meningitis, encephalitis, and an irreversible acute flaccid paralysis or poliomyelitis-like syndrome. Movement disorders have also been described. We report combined neuronal loss, gliosis, and neurofibrillary tangle formation in the substantia nigra of a 41-year-old man with a history of WNV encephalomyelitis and poliomyelitis-like paralysis. Clinically our patient did not display parkinsonism, however, it is interesting to speculate whether, in the absence of the residual subacute poliomyelitis-like syndrome, the neuropathologic findings could have eventually evolved clinically into WNV-associated postencephalitic parkinsonism.

Hereditary spastic paraplegia.

PubMed

Blackstone, Craig

2018-01-01

The hereditary spastic paraplegias (HSPs) are a heterogeneous group of neurologic disorders with the common feature of prominent lower-extremity spasticity, resulting from a length-dependent axonopathy of corticospinal upper motor neurons. The HSPs exist not only in "pure" forms but also in "complex" forms that are associated with additional neurologic and extraneurologic features. The HSPs are among the most genetically diverse neurologic disorders, with well over 70 distinct genetic loci, for which about 60 mutated genes have already been identified. Numerous studies elucidating the molecular pathogenesis underlying HSPs have highlighted the importance of basic cellular functions - especially membrane trafficking, mitochondrial function, organelle shaping and biogenesis, axon transport, and lipid/cholesterol metabolism - in axon development and maintenance. An encouragingly small number of converging cellular pathogenic themes have been identified for the most common HSPs, and some of these pathways present compelling targets for future therapies. Copyright © 2018 Elsevier B.V. All rights reserved.

Tonic vibration reflex in spasticity, Parkinson's disease, and normal subjects

PubMed Central

Burke, David; Andrews, Colin J.; Lance, James W.

1972-01-01

The tonic vibration reflex (TVR) has been studied in the quadriceps and triceps surae muscles of 34 spastic, 15 Parkinsonism, and 10 normal subjects. The TVR of spasticity develops rapidly, reaching a plateau level within 2-4 sec of the onset of vibration. The tonic contraction was often preceded by a phasic spike which appeared to be a vibration-induced equivalent of the tendon jerk. The initial phasic spike was usually followed by a silent period, and induced clonus in some patients. No correlation was found between the shape of the TVR and the site of the lesion in the central nervous system. The TVR of normal subjects and patients with Parkinsonism developed slowly, starting some seconds after the onset of vibration, and reaching a plateau level in 20-60 sec. A phasic spike was recorded occasionally in these subjects, but the subsequent tonic contraction followed the usual time course. Muscle stretch increased the quadriceps TVR of all subjects, including those with spasticity in whom the quadriceps stretch reflex decreased with increasing stretch. It is suggested that this difference between the tonic vibration reflex and the tonic stretch reflex arises from the selective activation of spindle primary endings by vibration, while both the primary and the secondary endings are responsive to muscle stretch. The TVR could be potentiated by reinforcement in some subjects. Potentiation outlasted the reinforcing manoeuvre, and was most apparent at short muscle lengths. As muscle stretch increased, thus producing a larger TVR, the degree of potentiation decreased. It is therefore suggested that the effects of reinforcement result at least partially from the activation of the fusimotor system. Since reinforcement potentiated the TVR of patients with spinal spasticity in whom a prominent clasp-knife phenomenon could be demonstrated, it is suggested that the effects of reinforcement are mediated by a descending pathway that traverses the anterior quadrant of the spinal cord. PMID:4261955

TREATMENT OF THE SPASTICITY IN CHILDREN WITH CEREBRAL PALSY

PubMed Central

Meholjić-Fetahović, Ajša

2007-01-01

Botulinum toxin is a natural purified protein and one of the strongest biological poisons - neurotoxin. It is produced by the bacterium Clostridium botulinum. Its medical usage started in USA in 1981 and in Europe in 1992. There are seven different immune types of the toxin: A, B, C1, D, E, F and G. Toxin types A and B are used to decrease muscular spasticity. Botulinum toxin prevents the formation of acetylcholine from cholinergic nerve tissues in muscles, which in the end irreversibly destroys neuromuscular synapses. It is called temporary local chemodenervation. It does not affect the synthesis of acetylcholine. As it affects neuromuscular bond it also affects one of the symptoms of cerebral palsy - spasticity Decreasing the spasticity of children with cerebral palsy leads to the improvement of conscious movements, muscles are less toned, passive mobility is improved, orthosis tolerance is also improved, and the child is enabled to perform easier and better motor functions such as crawling, standing and walking. Since the action of Botulinum toxin is limited to 2-6 months, new neural collaterals are formed and neuromuscular conductivity is reestablished which in the end once again develops a muscular spasm. This leads to a conclusion that botulinum toxin should again be applied into spastic muscles. It is very important for good effect of Botulinum toxin to set the goals of the therapy in advance. The goals include improvement of a function, prevention of contractions and deformities, ease of care and decrease of pain for children with cerebral palsy. After application of botulinum toxin, it is necessary to perform adequate and intensive physical treatment with regular monitoring of effects. This work shows a case of a boy with spastic form of cerebral palsy. After being habilitated using Vojta therapy and Bobath concept and the conduct of certain physical procedures, botulinum toxin is administered into his lower limbs’ muscles and kinezitherapy intensified. After the administration of botulinum toxin significant functional improvement is noted. PMID:18039197

Improvement of spasticity following intermittent theta burst stimulation in multiple sclerosis is associated with modulation of resting-state functional connectivity of the primary motor cortices.

PubMed

Boutière, Clémence; Rey, Caroline; Zaaraoui, Wafaa; Le Troter, Arnaud; Rico, Audrey; Crespy, Lydie; Achard, Sophie; Reuter, Françoise; Pariollaud, Fanelly; Wirsich, Jonathan; Asquinazi, Patrick; Confort-Gouny, Sylviane; Soulier, Elisabeth; Guye, Maxime; Pelletier, Jean; Ranjeva, Jean-Philippe; Audoin, Bertrand

2017-05-01

Intermittent theta burst stimulation (iTBS) of the primary motor cortex improves transiently lower limbs spasticity in multiple sclerosis (MS). However, the cerebral mechanisms underlying this effect have never been investigated. To assess whether modulation of spasticity induced by iTBS is underlined by functional reorganization of the primary motor cortices. A total of 17 patients with MS suffering from lower limbs spasticity were randomized to receive real iTBS or sham iTBS during the first half of a 5-week indoor rehabilitation programme. Spasticity was assessed using the Modified Ashworth Scale and the Visual Analogue Scale at baseline, after the stimulation session and at the end of the rehabilitation programme. Resting-state functional magnetic resonance imaging (fMRI) was performed at the three time points, and brain functional networks topology was analysed using graph-theoretical approach. At the end of stimulation, improvement of spasticity was greater in real iTBS group than in sham iTBS group ( p = 0.026). iTBS had a significant effect on the balance of the connectivity degree between the stimulated and the homologous primary motor cortex ( p = 0.005). Changes in inter-hemispheric balance were correlated with improvement of spasticity (rho = 0.56, p = 0.015). This longitudinal resting-state fMRI study evidences that functional reorganization of the primary motor cortices may underlie the effect of iTBS on spasticity in MS.

Spastic paraplegia gene 7 in patients with spasticity and/or optic neuropathy

PubMed Central

Klebe, Stephan; Depienne, Christel; Gerber, Sylvie; Challe, Georges; Anheim, Mathieu; Charles, Perrine; Fedirko, Estelle; Lejeune, Elodie; Cottineau, Julien; Brusco, Alfredo; Dollfus, Hélène; Chinnery, Patrick F.; Mancini, Cecilia; Ferrer, Xavier; Sole, Guilhem; Destée, Alain; Mayer, Jean-Michel; Fontaine, Bertrand; de Seze, Jérôme; Clanet, Michel; Ollagnon, Elisabeth; Busson, Philippe; Cazeneuve, Cécile; Stevanin, Giovanni; Kaplan, Josseline; Rozet, Jean-Michel; Brice, Alexis

2012-01-01

Mutations in the spastic paraplegia 7 (SPG7) gene encoding paraplegin are responsible for autosomal recessive hereditary spasticity. We screened 135 unrelated index cases, selected in five different settings: SPG7-positive patients detected during SPG31 analysis using SPG31/SPG7 multiplex ligation-dependent probe amplification (n = 7); previously reported ambiguous SPG7 cases (n = 5); patients carefully selected on the basis of their phenotype (spasticity of the lower limbs with cerebellar signs and/or cerebellar atrophy on magnetic resonance imaging/computer tomography scan and/or optic neuropathy and without other signs) (n = 24); patients with hereditary spastic paraparesis referred consecutively from attending neurologists and the national reference centre in a diagnostic setting (n = 98); and the index case of a four-generation family with autosomal dominant optic neuropathy but no spasticity linked to the SPG7 locus. We identified two SPG7 mutations in 23/134 spastic patients, 21% of the patients selected according to phenotype but only 8% of those referred directly. Our results confirm the pathogenicity of Ala510Val, which was the most frequent mutation in our series (65%) and segregated at the homozygous state with spastic paraparesis in a large family with autosomal recessive inheritance. All SPG7-positive patients tested had optic neuropathy or abnormalities revealed by optical coherence tomography, indicating that abnormalities in optical coherence tomography could be a clinical biomarker for SPG7 testing. In addition, the presence of late-onset very slowly progressive spastic gait (median age 39 years, range 18–52 years) associated with cerebellar ataxia (39%) or cerebellar atrophy (47%) constitute, with abnormal optical coherence tomography, key features pointing towards SPG7-testing. Interestingly, three relatives of patients with heterozygote SPG7 mutations had cerebellar signs and atrophy, or peripheral neuropathy, but no spasticity of the lower limbs, suggesting that SPG7 mutations at the heterozygous state might predispose to late-onset neurodegenerative disorders, mimicking autosomal dominant inheritance. Finally, a novel missense SPG7 mutation at the heterozygous state (Asp411Ala) was identified as the cause of autosomal dominant optic neuropathy in a large family, indicating that some SPG7 mutations can occasionally be dominantly inherited and be an uncommon cause of isolated optic neuropathy. Altogether, these results emphasize the clinical variability associated with SPG7 mutations, ranging from optic neuropathy to spastic paraplegia, and support the view that SPG7 screening should be carried out in both conditions. PMID:23065789

A pharmacokinetic-pharmacodynamic model for intrathecal baclofen in patients with severe spasticity.

PubMed

Heetla, H W; Proost, J H; Molmans, B H; Staal, M J; van Laar, T

2016-01-01

Intrathecal baclofen (ITB) has proven to be an effective and safe treatment for severe spasticity. However, although ITB is used extensively, clinical decisions are based on very scarce pharmacokinetic-pharmacodynamic (PKPD) data. The aim of this study was to measure baclofen CSF concentrations and clinical effects after administration of various ITB boluses in patients with spasticity and to create a PKPD model for ITB. Twelve patients with severe spasticity received four different bolus doses of ITB (0, 25, 50, 75 μg and an optional dose of 100 μg), administered via a catheter with the tip at thoracic level (Th) 10. After each bolus, 10 CSF samples were taken at fixed time intervals, using a catheter with the tip located at Th12. Clinical effect was assessed by measuring spasticity with the Modified Ashworth Scale (MAS). These data were used to develop a PKPD model. All patients achieved an adequate spasmolytic effect with ITB doses varying from 50 to 100 μg. No serious side effects were observed. CSF baclofen concentrations, as well as the clinical effects, correlated significantly with ITB doses. The PK model predicted a steep spinal concentration gradient of ITB along the spinal axis. The clinical effect could be predicted using a delayed-effect model. ITB is an effective and safe therapy with, however, a steep concentration gradient along the spinal axis. This means that the administered baclofen is staying mainly around the catheter tip, which stresses the importance to position the ITB catheter tip closely to the targeted spinal level. © 2015 The British Pharmacological Society.

[Medical care of patients with spasticity following stroke : Evaluation of the treatment situation in Germany with focus on the use of botulinum toxin].

PubMed

Kerkemeyer, L; Lux, G; Walendzik, A; Wasem, J; Neumann, A

2017-08-01

Upper limb spasticity is a common complication following stroke. Cohort studies found 19% of post-stroke patients had upper limb spasticity at 3 months and 38% of patients at 12 months. For focal spasticity, intramuscular injections of botulinum toxin are indicated. In Germany, it is assumed that patients with the described indication are undersupplied with botulinum toxin. The aim of the present study is to evaluate the medical care of patients with upper limb spasticity post-stroke with the focus on the use of botulinum toxin as one treatment option. A standardized questionnaire was developed and a postal survey of a representative national random sample of 800 neurologists to capture the actual medical care situation. The response rate amounted to 37% (n = 292). 59% of the neurologists surveyed had never used botulinum toxin. In total, 87% of neurologists noticed barriers regarding the use of botulinum toxin, where the amount of the doctor's remuneration in 40% and the lack of reimbursement of costs in off-label use in 60% were the most commonly used answers. The achievement of an advanced training in using botulinum toxin was also stated as a general obstacle for resident neurologists. Due to a response rate of 37% for the postal survey a selection bias cannot be excluded. Although botulinum toxin is recommended in the national treatment guidelines, many neurologists do not use botulinum toxin. The reasons can be seen from the barriers described.

Neurophysiological mechanisms and functional impact of mirror movements in children with unilateral spastic cerebral palsy.

PubMed

Kuo, Hsing-Ching; Friel, Kathleen M; Gordon, Andrew M

2018-02-01

Children with unilateral spastic cerebral palsy (CP) often have mirror movements, i.e. involuntary imitations of unilateral voluntary movements of the contralateral upper extremity. The pathophysiology of mirror movements has been investigated in small and heterogeneous cohorts in the literature. Specific pathophysiology of mirror movements and their impact on upper extremity function require systematic investigation in larger and homogeneous cohorts of children with unilateral spastic CP. Here we review two possible neurophysiological mechanisms underlying mirror movements in children with CP and those with typical development: (1) an ipsilateral corticospinal tract projecting from the contralesional motor cortex (M1) to both upper extremities; (2) insufficient interhemispheric inhibition between the two M1s. We also discuss clinical implications of mirror movements in children with unilateral CP and suggest that a thorough examination of the relationship between the pathophysiology and clinical manifestations of mirror movements is warranted. We suggest two premises: (1) the presence of mirror movements is indicative of an ipsilateral corticospinal tract reorganization; and (2) the corticospinal tract organization may affect patients' responses to certain treatment. If these premises are supported through future research, mirror movements should be clinically evaluated for patient selection to maximize benefits of therapy, hence promoting individualized medicine in this population. Mirror movements may be indicative of the underlying corticospinal tract reorganization in children with unilateral spastic cerebral palsy (CP). Future research will benefit from systematic investigations of the relationship between mirror movements and its pathophysiology. Mirror movements may be a potential biomarker for individualized medicine in children with unilateral spastic CP. © 2017 Mac Keith Press.

Paediatric vocal fold paralysis.

PubMed

Garcia-Lopez, Isabel; Peñorrocha-Teres, Julio; Perez-Ortin, Magdalena; Cerpa, Mauricio; Rabanal, Ignacio; Gavilan, Javier

2013-01-01

Vocal fold paralysis (VFP) is a relatively common cause of stridor and dysphonia in the paediatric population. This report summarises our experience with VFP in the paediatric age group. All patients presenting with vocal fold paralysis over a 12-month period were included. Medical charts were revised retrospectively. The diagnosis was performed by flexible endoscopic examination. The cases were evaluated with respect to aetiology of the paralysis, presenting symptoms, delay in diagnosis, affected side, vocal fold position, need for surgical treatment and outcome. The presenting symptoms were stridor and dysphonia. Iatrogenic causes formed the largest group, followed by idiopathic, neurological and obstetric VFP. Unilateral paralysis was found in most cases. The median value for delay in diagnosis was 1 month and it was significantly higher in the iatrogenic group. Surgical treatment was not necessary in most part of cases. The diagnosis of VFP may be suspected based on the patient's symptoms and confirmed by flexible endoscopy. Infants who develop stridor or dysphonia following a surgical procedure have to be examined without delay. The surgeon has to keep in mind that there is a possibility of late spontaneous recovery or compensation. Copyright © 2012 Elsevier España, S.L. All rights reserved.

Quality of life differences in patients with right- versus left-sided facial paralysis: Universal preference of right-sided human face recognition.

PubMed

Ryu, Nam Gyu; Lim, Byung Woo; Cho, Jae Keun; Kim, Jin

2016-09-01

We investigated whether experiencing right- or left-sided facial paralysis would affect an individual's ability to recognize one side of the human face using hybrid hemi-facial photos by preliminary study. Further investigation looked at the relationship between facial recognition ability, stress, and quality of life. To investigate predominance of one side of the human face for face recognition, 100 normal participants (right-handed: n = 97, left-handed: n = 3, right brain dominance: n = 56, left brain dominance: n = 44) answered a questionnaire that included hybrid hemi-facial photos developed to determine decide superiority of one side for human face recognition. To determine differences of stress level and quality of life between individuals experiencing right- and left-sided facial paralysis, 100 patients (right side:50, left side:50, not including traumatic facial nerve paralysis) answered a questionnaire about facial disability index test and quality of life (SF-36 Korean version). Regardless of handedness or hemispheric dominance, the proportion of predominance of the right side in human face recognition was larger than the left side (71% versus 12%, neutral: 17%). Facial distress index of the patients with right-sided facial paralysis was lower than that of left-sided patients (68.8 ± 9.42 versus 76.4 ± 8.28), and the SF-36 scores of right-sided patients were lower than left-sided patients (119.07 ± 15.24 versus 123.25 ± 16.48, total score: 166). Universal preference for the right side in human face recognition showed worse psychological mood and social interaction in patients with right-side facial paralysis than left-sided paralysis. This information is helpful to clinicians in that psychological and social factors should be considered when treating patients with facial-paralysis. Copyright © 2016 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

Multidisciplinary rehabilitation following botulinum toxin and other focal intramuscular treatment for post-stroke spasticity.

PubMed

Demetrios, Marina; Khan, Fary; Turner-Stokes, Lynne; Brand, Caroline; McSweeney, Shane

2013-06-05

Spasticity may affect stroke survivors by contributing to activity limitations, caregiver burden, pain and reduced quality of life (QoL). Spasticity management guidelines recommend multidisciplinary (MD) rehabilitation programmes following botulinum toxin (BoNT) treatment for post-stroke spasticity. However, the evidence base for the effectiveness of MD rehabilitation is unclear. To assess the effectiveness of MD rehabilitation, following BoNT and other focal intramuscular treatments such as phenol, in improving activity limitations and other outcomes in adults and children with post-stroke spasticity. To explore what settings, types and intensities of rehabilitation programmes are effective. We searched the Cochrane Stroke Group Trials Register (February 2012), the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2011, Issue 12), MEDLINE (1948 to December 2011), EMBASE (1980 to January 2012), CINAHL (1982 to January 2012), AMED (1985 to January 2012), LILACS (1982 to September 2012), PEDro, REHABDATA and OpenGrey (September 2012). In an effort to identify further published, unpublished and ongoing trials we searched trials registries and reference lists, handsearched journals and contacted authors. We included randomised controlled trials (RCTs) that compared MD rehabilitation (delivered by two or more disciplines in conjunction with medical input) following BoNT and other focal intramuscular treatments for post-stroke spasticity with placebo, routinely available local services, or lower levels of intervention; or studies that compared MD rehabilitation in different settings, of different types, or at different levels of intensity. We excluded RCTs that assessed the effectiveness of unidisciplinary therapy (for example physiotherapy only) or a single modality (for example stretching, casting, electrical stimulation or splinting only). The primary outcomes were validated measures of activity level (active and passive function) according to the World Health Organization's International Classification of Functioning, Disability and Health. Secondary outcomes included measures of symptoms, impairments, participation, QoL, impact on caregivers and adverse events. We independently selected the trials, extracted data, and assessed methodological quality using the Grades of Recommendation, Assessment, Development and Evaluation (GRADE). Due to the limited number of included studies, with clinical, methodological and statistical heterogeneity, quantitative meta-analysis was not possible. Therefore, GRADE provided qualitative synthesis of 'best evidence'. We included three RCTs involving 91 participants. All three studies scored 'low quality' on the methodological quality assessment, implying high risk of bias. All studies investigated various types and intensities of outpatient rehabilitation programmes following BoNT for upper limb spasticity in adults with chronic stroke. Rehabilitation programmes included: modified constraint-induced movement therapy (mCIMT) compared with a neurodevelopmental therapy programme; task practice therapy with cyclic functional electrical stimulation (FES) compared with task practice therapy only; and occupational, manual therapy with dynamic elbow extension splinting compared with occupational therapy only. There was 'low quality' evidence for mCIMT improving upper limb motor function and spasticity in chronic stroke survivors with residual voluntary upper limb activity, up to six months, and 'very low quality' evidence for dynamic elbow splinting and occupational therapy reducing elbow range of movement at 14 weeks. Task practice therapy with cyclic FES did not improve upper limb function more than task practice therapy alone, only at 12 weeks. No studies addressed interventions in children and those with lower limb spasticity, or after other focal intramuscular treatments for spasticity. At best there was 'low level' evidence for the effectiveness of outpatient MD rehabilitation in improving active function and impairments following BoNT for upper limb spasticity in adults with chronic stroke. No trials explored the effect of MD rehabilitation on 'passive function' (caring for the affected limb), caregiver burden, or the individual's priority goals for treatment. The optimal types (modalities, therapy approaches, settings) and intensities of therapy for improving activity (active and passive function) in adults and children with post-stroke spasticity, in the short and longer term, are unclear. Further research is required to build evidence in this area.

Guillan-Barré syndrome in heroin addiction.

PubMed

SMith, W R; Wilson, A F

1975-03-31

In two cases of Guillain-Barré syndrome (acute polyneuritis) as a complication of narcotic abuse, the onset of paralysis began approximately 4 and 12 hours after self-administration of opiates intravenously. Bulbar and respiratory paralysis developed in one patient and required prolonged ventilation therapy; the other patient had peripheral muscular and unilateral facial weakness, with notable pleocytosis was present in both.

Influence of Botulinum Toxin Therapy on Postural Control and Lower Limb Intersegmental Coordination in Children with Spastic Cerebral Palsy

PubMed Central

Degelaen, Marc; de Borre, Ludo; Kerckhofs, Eric; de Meirleir, Linda; Buyl, Ronald; Cheron, Guy; Dan, Bernard

2013-01-01

Botulinum toxin injections may significantly improve lower limb kinematics in gait of children with spastic forms of cerebral palsy. Here we aimed to analyze the effect of lower limb botulinum toxin injections on trunk postural control and lower limb intralimb (intersegmental) coordination in children with spastic diplegia or spastic hemiplegia (GMFCS I or II). We recorded tridimensional trunk kinematics and thigh, shank and foot elevation angles in fourteen 3–12 year-old children with spastic diplegia and 14 with spastic hemiplegia while walking either barefoot or with ankle-foot orthoses (AFO) before and after botulinum toxin infiltration according to a management protocol. We found significantly greater trunk excursions in the transverse plane (barefoot condition) and in the frontal plane (AFO condition). Intralimb coordination showed significant differences only in the barefoot condition, suggesting that reducing the degrees of freedom may limit the emergence of selective coordination. Minimal relative phase analysis showed differences between the groups (diplegia and hemiplegia) but there were no significant alterations unless the children wore AFO. We conclude that botulinum toxin injection in lower limb spastic muscles leads to changes in motor planning, including through interference with trunk stability, but a combination of therapies (orthoses and physical therapy) is needed in order to learn new motor strategies. PMID:23344454

Influence of post-stroke spasticity on EMG-force coupling and force steadiness in biceps brachii.

PubMed

Carlyle, Jennilee K; Mochizuki, George

2018-02-01

Individuals with spasticity after stroke experience a decrease in force steadiness which can impact function. Alterations in the strength of EMG-force coupling may contribute to the reduction in force steadiness observed in spasticity. The aim was to determine the extent to which force steadiness and EMG-force coupling is affected by post-stroke spasticity. This cross-sectional study involved individuals with upper limb spasticity after stroke. Participants were required to generate and maintain isometric contractions of the elbow flexors at varying force levels. Coefficient of variation of force, absolute force, EMG-force cross-correlation function peak and peak latency was measured from both limbs with surface electromyography and isometric dynamometry. Statistically significant differences were observed between the affected and less affected limbs for all outcome measures. Significant main effects of force level were also observed. Force steadiness was not statistically significantly correlated with EMG-force coupling; however, both force steadiness and absolute force were associated with the level of impairment as measured by the Chedoke McMaster Stroke Assessment Scale. Spasticity after stroke uncouples the relationship between EMG and force and is associated with reduced force steadiness during isometric contractions; however, these features of control are not associated in individuals with spasticity. Copyright © 2017 Elsevier Ltd. All rights reserved.

[Risk factors for interictal epileptiform discharges on electroencephalogram in children with spastic hemiplegic cerebral palsy].

PubMed

Li, Su-Yun; Qian, Xu-Guang; Zhao, Yi-Li; Fu, Wen-Jie; Tan, Xiao-Ru; Liu, Zhen-Huan

2015-12-01

To investigate the clinical symptoms and features of interictal epileptiform discharges (IED) on electroencephalogram (EEG) in children with spastic hemiplegic cerebral palsy (CP) and to analyze the risk factors for IED. Eighty-three children with spastic hemiplegic CP were recruited, and their clinical data, results of video-electroencephalogram, imaging findings, and cognitive levels were collected. The influencing factors for IED were determined by multiple logistic regression analysis. The incidence of epilepsy was 13% in children with spastic hemiplegic CP; 34% of these cases had IED. The incidence of epilepsy in children with IED (32%) was significantly higher than that in those without IED (4%) (P<0.01). The incidence of IED in children with complications and brain cortex impairment increased significantly (P<0.01). The incidence of IED varied significantly between patients with different cognitive levels (P<0.01). Brain cortex impairment (OR=11.521) and low cognitive level (OR=2.238)were risk factors for IED in children with spastic hemiplegic CP (P<0.05). Spastic hemiplegic CP is often found with IED on EEG, and the incidence of epilepsy is higher in children with IED than in those without IED. Brain cortex impairment and low cognitive level have predictive values for IED in children with spastic hemiplegic CP.

Nerve lesioning with direct current

NASA Astrophysics Data System (ADS)

Ravid, E. Natalie; Shi Gan, Liu; Todd, Kathryn; Prochazka, Arthur

2011-02-01

Spastic hypertonus (muscle over-activity due to exaggerated stretch reflexes) often develops in people with stroke, cerebral palsy, multiple sclerosis and spinal cord injury. Lesioning of nerves, e.g. with phenol or botulinum toxin is widely performed to reduce spastic hypertonus. We have explored the use of direct electrical current (DC) to lesion peripheral nerves. In a series of animal experiments, DC reduced muscle force by controlled amounts and the reduction could last several months. We conclude that in some cases controlled DC lesioning may provide an effective alternative to the less controllable molecular treatments available today.

A novel ABCD1 mutation detected by next generation sequencing in presumed hereditary spastic paraplegia: A 30-year diagnostic delay caused by misleading biochemical findings.

PubMed

Koutsis, Georgios; Lynch, David S; Tucci, Arianna; Houlden, Henry; Karadima, Georgia; Panas, Marios

2015-08-15

To present a Greek family in which 5 male and 2 female members developed progressive spastic paraplegia. Plasma very long chain fatty acids (VLCFA) were reportedly normal at first testing in an affected male and for over 30 years the presumed diagnosis was hereditary spastic paraplegia (HSP). Targeted next generation sequencing (NGS) was used as a further diagnostic tool. Targeted exome sequencing in the proband, followed by Sanger sequencing confirmation; mutation segregation testing in multiple family members and plasma VLCFA measurement in the proband. NGS of the proband revealed a novel frameshift mutation in ABCD1 (c.1174_1178del, p.Leu392Serfs*7), bringing an end to diagnostic uncertainty by establishing the diagnosis of adrenomyeloneuropathy (AMN), the myelopathic phenotype of X-linked adrenoleukodystrophy (ALD). The mutation segregated in all family members and the diagnosis of AMN/ALD was confirmed by plasma VLCFA measurement. Confounding factors that delayed the diagnosis are presented. This report highlights the diagnostic utility of NGS in patients with undiagnosed spastic paraplegia, establishing a molecular diagnosis of AMN, allowing proper genetic counseling and management, and overcoming the diagnostic delay that can be rarely caused by false negative VLCFA analysis. Copyright © 2015 Elsevier B.V. All rights reserved.

A case report of HTLV-I associated myelopathy presenting with cerebellar ataxia and nystagmus.

PubMed

Taki, Masakatsu; Nin, Fumiaki; Hasegawa, Tatsuhisa; Sakaguchi, Hirofumi; Suzuki, Toshihiro; Hisa, Yasuo; Azuma, Yumiko; Nakagawa, Masanori

2011-06-01

HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) is characterized by spastic paraparesis in the lower extremities, and urinary disturbance. HAM/TSP has also been less frequently associated with cerebellar syndromes and nystagmus. We report a case of HAM/TSP presenting with cerebellar ataxia and nystagmus. The patient was a 73-year-old woman who was born in southern Japan. At age 41, she developed pain and spasticity in the bilateral lower limbs and gradually progressive gait disturbance. At age 57, she was diagnosed with HAM/TSP based on spastic paraparesis in the lower limbs, urinary disturbance and positive anti HTLV-I antibody in serum and cerebrospinal fluid. In June 2008, she was referred to our university and hospitalized for rehabilitation. Twenty days later, she experienced rotatory vertigo sensation. Magnetic resonance imaging revealed pontocerebellar atrophy. The patient presented with cerebellar signs in the upper limbs, gaze-evoked nystagmus in the sitting position and right-beating horizontal nystagmus in the supine and head-hanging positions. Electronystagmography (ENG) showed horizontal saccadic overshoot dysmetria and horizontal saccadic pursuit. Nystagmus is rare among the literature on HAM/TSP. ENG is helpful to evaluate and confirm the cerebellar syndromes of HAM/TSP. Copyright © 2010. Published by Elsevier Ireland Ltd.

Defective human T-cell lymphotropic virus type I (HTLV-I) provirus in seronegative tropical spastic paraparesis/HTLV-I-associated myelopathy (TSP/HAM) patients.

PubMed

Ramirez, E; Fernandez, J; Cartier, L; Villota, C; Rios, M

2003-02-01

Infection with human T-cell lymphotropic virus type I (HTLV-I) have been associated with the development of the tropical spastic paraparesis/HTLV-I-associated myelopathy (TSP/HAM). We studied the presence of HTLV-I provirus in peripheral blood mononuclear cells (PBMC) from 72 Chilean patients with progressive spastic paraparesis by polymerase chain reaction: 32 seropositive and 40 seronegative cases. We amplified different genomic regions of HTLV-I using primers of 5' ltr, tax, env/tax, pX, pol and env genes. These genes were detected from all seropositive patients. The seronegative patients were negative with 5' ltr, pol, env, and pX primers. However, amplified product of tax and env/tax genes was detected from 16 and four seronegative patients, respectively. Three of them were positive with both genetic regions. The results of this study show that the complete HTLV-I provirus is found in 100% of seropositive cases. In seronegative cases, clinically very similar of seropositive cases, was found only tax gene in 42.5% (17/40) of patients. These results suggest the presence of a defective HTLV-I provirus in some seronegative patients with progressive spastic paraparesis, and suggest a pathogenic role of this truncate provirus for a group of TSP/HAM.

Bone mineral density and insulin-like growth factor-1 in children with spastic cerebral palsy.

PubMed

Nazif, H; Shatla, R; Elsayed, R; Tawfik, E; Osman, N; Korra, S; Ibrahim, A

2017-04-01

Children with cerebral palsy (CP) have significant decrease linear growth rate and low bone mineral density (BMD). This study is to evaluate BMD in children with CP and its relation to the levels of insulin-like growth factor-1 (IGF-1). This cross-sectional study was carried out on 58 children suffering from spastic CP with the age range 4-12 years compared to 19 controls. All assessed by dual energy x-ray absorptiometry (DXA) to measure BMD, serum level of IGF-1, and serum vitamin D. The patients were classified according to their GMFCS. Fractures were reported in seven (12.1%) of cases. Our study demonstrated that, IGF-1 level and BMD decrease in correlation with the severity of CP. IGF-1correlates positively with serum vitamin D, BMI, and BMD. CP children with severe GMFCS level or who use anticonvulsive drugs are at a high risk for low BMD and low levels of IGF-1. Both BMD and IGF-1 were significantly in low children with spastic CP; IGF-1 negatively correlates with the severity of osteopenia in children with spastic. Children with CP who are not independently ambulant or with severe GMFCS level or who use anticonvulsive drugs are at a high risk for developing low BMD.

A randomised controlled cross-over double-blind pilot study protocol on THC:CBD oromucosal spray efficacy as an add-on therapy for post-stroke spasticity.

PubMed

Marinelli, Lucio; Balestrino, Maurizio; Mori, Laura; Puce, Luca; Rosa, Gian Marco; Giorello, Laura; Currà, Antonio; Fattapposta, Francesco; Serrati, Carlo; Gandolfo, Carlo; Abbruzzese, Giovanni; Trompetto, Carlo

2017-09-07

Stroke is the most disabling neurological disorder and often causes spasticity. Transmucosal cannabinoids (tetrahydrocannabinol and cannabidiol (THC:CBD), Sativex) is currently available to treat spasticity-associated symptoms in patients with multiple sclerosis. Cannabinoids are being considered useful also in the treatment of pain, nausea and epilepsy, but may bear and increased risk for cardiovascular events. Spasticity is often assessed with subjective and clinical rating scales, which are unable to measure the increased excitability of the monosynaptic reflex, considered the hallmark of spasticity. The neurophysiological assessment of the stretch reflex provides a precise and objective method to measure spasticity. We propose a novel study to understand if Sativex could be useful in reducing spasticity in stroke survivors and investigating tolerability and safety by accurate cardiovascular monitoring. We will recruit 50 patients with spasticity following stroke to take THC:CBD in a double-blind placebo-controlled cross-over study. Spasticity will be assessed with a numeric rating scale for spasticity, the modified Ashworth scale and with the electromyographical recording of the stretch reflex. The cardiovascular risk will be assessed prior to inclusion. Blood pressure, heart rate, number of daily spasms, bladder function, sleep disruption and adverse events will be monitored throughout the study. A mixed-model analysis of variance will be used to compare the stretch reflex amplitude between the time points; semiquantitative measures will be compared using the Mann-Whitney test (THC:CBD vs placebo) and Wilcoxon test (baseline vs treatment). The study was registered on the EudraCT database with number 2016-001034-10 and approved by both the Italian Medicines Agency (Agenzia Italiana del Farmaco) and local Ethics Committee 'Comitato Etico Regionale della Liguria'. Data will be made anonymous and uploaded to a open access repository. Results will be disseminated by presentations at national and international conferences and by publication in journals of clinical neuroscience and neurology. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Effects of whole body vibration on muscle spasticity for people with central nervous system disorders: a systematic review.

PubMed

Huang, Meizhen; Liao, Lin-Rong; Pang, Marco Yc

2017-01-01

To examine the effects of whole-body vibration on spasticity among people with central nervous system disorders. Electronic searches were conducted using CINAHL, Cochrane Library, MEDLINE, Physiotherapy Evidence Database, PubMed, PsycINFO, SPORTDiscus and Scopus to identify randomized controlled trials that investigated the effect of whole-body vibration on spasticity among people with central nervous system disorders (last search in August 2015). The methodological quality and level of evidence were rated using the PEDro scale and guidelines set by the Oxford Centre for Evidence-Based Medicine. Nine trials with totally 266 subjects (three in cerebral palsy, one in multiple sclerosis, one in spinocerebellar ataxia, and four in stroke) fulfilled all selection criteria. One study was level 1b (PEDro⩾6 and sample size>50) and eight were level 2b (PEDro<6 or sample size ⩽50). All three cerebral palsy trials (level 2b) reported some beneficial effects of whole-body vibration on reducing leg muscle spasticity. Otherwise, the results revealed no consistent benefits on spasticity in other neurological conditions studied. There is little evidence that change in spasticity was related to change in functional performance. The optimal protocol could not be identified. Many reviewed studies were limited by weak methodological and reporting quality. Adverse events were minor and rare. Whole-body vibration may be useful in reducing leg muscle spasticity in cerebral palsy but this needs to be verified by future high quality trials. There is insufficient evidence to support or refute the notion that whole-body vibration can reduce spasticity in stroke, spinocerebellar ataxia or multiple sclerosis.

Thyrotoxic hypokalemic periodic paralysis due to dietary weight-loss supplement.

PubMed

Akinyemi, Emmanuel; Bercovici, Silvia; Niranjan, Selvanayagam; Paul, Nisha; Hemavathy, Bhakthavatsalam

2011-05-01

Herbal and dietary supplements for weight loss and in treatment of obesity are growing in popularity and acceptance in the United States. Most of these supplements can be obtained over the counter and can have serious adverse effects associated with their consumption. We describe 2 patients who developed thyrotoxic hypokalemic periodic paralysis 2-3 weeks after consuming thyroxine-containing weight-loss supplements. This is the first known case of thyrotoxic hypokalemic periodic paralysis secondary to dietary supplements. It is important that patients and physicians are aware of the severe adverse reactions associated with dietary supplements. Physicians should as a routine inquire about herbal and dietary supplement consumption during all patient encounters.

Systematic review of systematic reviews for medical cannabinoids: Pain, nausea and vomiting, spasticity, and harms.

PubMed

Allan, G Michael; Finley, Caitlin R; Ton, Joey; Perry, Danielle; Ramji, Jamil; Crawford, Karyn; Lindblad, Adrienne J; Korownyk, Christina; Kolber, Michael R

2018-02-01

To determine the effects of medical cannabinoids on pain, spasticity, and nausea and vomiting, and to identify adverse events. MEDLINE, the Cochrane Database, and the references of included studies were searched. Systematic reviews with 2 or more randomized controlled trials (RCTs) that focused on medical cannabinoids for pain, spasticity, or nausea and vomiting were included. For adverse events, any meta-analysis for the conditions listed or of adverse events of cannabinoids was included. From 1085 articles, 31 relevant systematic reviews were identified including 23 for pain, 5 for spasticity, 6 for nausea and vomiting, and 12 for adverse events. Meta-analysis of 15 RCTs found more patients taking cannabinoids attained at least a 30% pain reduction: risk ratio (RR) of 1.37 (95% CI 1.14 to 1.64), number needed to treat (NNT) of 11. Sensitivity analysis found study size and duration affected findings (subgroup differences, P ≤ .03), with larger and longer RCTs finding no benefit. Meta-analysis of 4 RCTs found a positive global impression of change in spasticity (RR = 1.45, 95% CI 1.08 to 1.95, NNT = 7). Other results were not consistently statistically significant, but when positive, a 30% or more improvement in spasticity had an NNT of 10. Meta-analysis of 7 RCTs for control of nausea and vomiting after chemotherapy found an RR of 3.60 (95% CI 2.55 to 5.09) with an NNT of 3. Adverse effects caused more patients to stop treatment (number needed to harm [NNH] of 8 to 22). Individual adverse events were very common, including dizziness (NNH = 5), sedation (NNH = 5), confusion (NNH = 15), and dissociation (NNH = 20). "Feeling high" was reported in 35% to 70% of users. The GRADE (Grading of Recommendations Assessment, Development and Evaluation) evaluation reduced evidence ratings of benefit to low or very low. There is reasonable evidence that cannabinoids improve nausea and vomiting after chemotherapy. They might improve spasticity (primarily in multiple sclerosis). There is some uncertainty about whether cannabinoids improve pain, but if they do, it is neuropathic pain and the benefit is likely small. Adverse effects are very common, meaning benefits would need to be considerable to warrant trials of therapy. Copyright© the College of Family Physicians of Canada.

[The application of high-frequency and iTBS transcranial magnetic stimulation for the treatment of spasticity in the patients presenting with secondary progressive multiple sclerosis].

PubMed

Korzhova, J E; Chervyakov, A V; Poydasheva, A G; Kochergin, I A; Peresedova, A V; Zakharova, M N; Suponeva, N A; Chernikova, L A; Piradov, M A

Spasticity is considered to be a common manifestation of multiple sclerosis. Muscle relaxants are not sufficiently effective; more than that, some of them often cause a variety of adverse reactions. Transcranial magnetic stimulation (TMS) can be a promising new tool for the treatment of spasticity. The objective of the present study was to compare the effectiveness of the two TMS protocols: rhythmic (high-frequency) TMS (rTMS) and stimulation with the theta bursts (iTBS) in terms of their ability to reduce spasticity in the patients presenting with multiple sclerosis. Twenty two patients with secondary-progressive multiple sclerosis were pseudo-randomized into two groups: those in the first (high-frequency) group received the treatment with the use of rTMS therapy at a frequency of 10 Hz; the patients of the second group, underwent stimulation with the theta bursts (iTBS). All the patients received 10 sessions of either stimulation applied to the primary motor area (M1) of both legs. The effectiveness of TMS protocols was evaluated before therapy and after 10 sessions of stimulation based on the Modified Ashworth scale (MAS), the expanded disability status scale (EDSS), and the Kurtzke functional scale (Kfs). In addition, the patients were interviewed before treatment, after 10 rTMS sessions, immediately after and within 2 and 12 weeks after the completion of the treatment using questionnaires for the evaluation of spasticity (SESS) , fatigue, and dysfunction of the pelvic organs (severity of defecation and urination disorders), fatigue. The study has demonstrated a significant reduction in spasticity in the patients of both groups at the end of the TMS protocol based on the MAS scale. There was no significant difference between the outcomes of the two protocols. Both had positive effect on the concomitant «non-motor» symptoms (fatigue, dysfunction of the pelvic organs). High-frequency transcranial magnetic stimulation (10 sessions of rTMS therapy at a frequency of 10 Hz) and stimulation with the theta-bursts applied to the M1 area in both legs can be an effective alternative treatment of spasticity in the patients with secondary-progressive multiple sclerosis. Further research is needed to detect more accurately the differences between the outcomes of the two stimulation protocols and the development of indications for their application on an individual basis.

Botulinum toxin injection techniques for the management of adult spasticity.

PubMed

Walker, Heather W; Lee, Michael Y; Bahroo, Laxman B; Hedera, Peter; Charles, David

2015-04-01

Spasticity is often experienced by individuals with injury or illness of the central nervous system from etiologies such as stroke, spinal cord injury, brain injury, multiple sclerosis, or other neurologic conditions. Although spasticity may provide benefits in some patients, it more often leads to complications negatively impacting the patient. Nonpharmacologic treatment options often do not provide long-term reduction of spasticity, and systemic interventions, such as oral medications, can have intolerable side effects. The use of botulinum neurotoxin injections is one option for management of focal spasticity. Several localization techniques are available to physicians that allow for identification of the selected target muscles. These methods include anatomic localization in isolation or in conjunction with electromyography guidance, electrical stimulation guidance, or ultrasound guidance. This article will focus on further description of each of these techniques in relation to the treatment of adult spasticity and will discuss the advantages and disadvantages of each technique, as well as review the literature comparing the techniques. Copyright © 2015 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.

Cautious Use of Intrathecal Baclofen in Walking Spastic Patients: Results on Long-term Follow-up.

PubMed

Dones, Ivano; Nazzi, Vittoria; Tringali, Giovanni; Broggi, Giovanni

2006-04-01

Intrathecal baclofen is presently the most effective treatment for diffuse spasticity whatever the cause. The fact that both spasticity is always accompanied by a degree of muscle weakness and that any antispastic treatment causes a decrease in muscle strength indicate that major attention must be paid in treating spasticity in ambulant patients. Methods.  We present here a retrospective study, approved by the insitutional ethics committee, of 22 ambulant spastic patients, selected as homogeneous for disease and disease duration, who were treated with intrathecal baclofen at the Istituto Nazionale Neurologico "C.Besta" in Milan. These patients were followed-up for to 15 years of treatment and their clinical assessment was enriched by the evaluation of their functional independence measurement (FIM) before and during treatment. Results.  There was improvement in quality of life as measured by the FIM scale; however, an increase in the patient's motor performance could not be detected. Conclusion.  Although we did not show any improvement in muscle performance, intrathecal baclofen did improve daily quality of life, even in spastic patients who were able to walk.

Hypokalemic Periodic Paralysis: a case report and review of the literature

PubMed Central

Soule, Benjamin R; Simone, Nicole L

2008-01-01

Hypokalemic Periodic Paralysis is one form of Periodic Paralysis, a rare group of disorders that can cause of sudden onset weakness. A case of a 29 year old male is presented here. The patient presented with sudden onset paralysis of his extremities. Laboratory evaluation revealed a markedly low potassium level. The patient's paralysis resolved upon repletion of his low potassium and he was discharged with no neurologic deficits. An association with thyroid disease is well established and further workup revealed Grave's disease in this patient. Although rare, Periodic Paralysis must differentiated from other causes of weakness and paralysis so that the proper treatment can be initiated quickly. PMID:18939979

Intrathecal Baclofen Therapy for the Treatment of Spasticity in Sjögren-Larsson Syndrome.

PubMed

Hidalgo, Eveline Teresa; Orillac, Cordelia; Hersh, Andrew; Harter, David H; Rizzo, William B; Weiner, Howard L

2017-01-01

Intrathecal baclofen therapy is widely accepted as a treatment option for patients with severe spasticity. The current treatment of spasticity in patients with Sjögren-Larsson syndrome is largely symptomatic, given that no effective causal therapy treatments are available. We report the outcome of 2 patients with Sjögren-Larsson syndrome who had pump implantation for intrathecal baclofen. We observed a positive response, with a decrease of spasticity, reflecting in the Modified Ashworth Scale, and parents and caregivers observed a functional improvement in both patients. One patient experienced skin irritation 15 months after surgery, necessitating pump repositioning. No infection occurred. Our report shows that intrathecal baclofen therapy can have a positive therapeutic effect on spasticity in patients with Sjögren-Larsson syndrome, and therefore may be a promising addition to current treatments.

Hindlimb spasticity after unilateral motor cortex lesion in rats is reduced by contralateral nerve root transfer.

PubMed

Zong, Haiyang; Ma, Fenfen; Zhang, Laiyin; Lu, Huiping; Gong, Jingru; Cai, Min; Lin, Haodong; Zhu, Yizhun; Hou, Chunlin

2016-12-01

Lower extremity spasticity is a common sequela among patients with acquired brain injury. The optimum treatment remains controversial. The aim of our study was to test the feasibility and effectiveness of contralateral nerve root transfer in reducing post stroke spasticity of the affected hindlimb muscles in rats. In our study, we for the first time created a novel animal hindlimb spastic hemiplegia model in rats with photothrombotic lesion of unilateral motor cortex and we established a novel surgical procedure in reducing motor cortex lesion-induced hindlimb spastic hemiplegia in rats. Thirty six rats were randomized into three groups. In group A, rats received sham operation. In group B, rats underwent unilateral hindlimb motor cortex lesion. In group C, rats underwent unilateral hindlimb cortex lesion followed by contralateral L4 ventral root transfer to L5 ventral root of the affected side. Footprint analysis, Hoffmann reflex (H-reflex), cholera toxin subunit B (CTB) retrograde tracing of gastrocnemius muscle (GM) motoneurons and immunofluorescent staining of vesicle glutamate transporter 1 (VGLUT1) on CTB-labelled motoneurons were used to assess spasticity of the affected hindlimb. Sixteen weeks postoperatively, toe spread and stride length recovered significantly in group C compared with group B (P<0.001). H max (H-wave maximum amplitude)/M max (M-wave maximum amplitude) ratio of gastrocnemius and plantaris muscles (PMs) significantly reduced in group C (P<0.01). Average VGLUT1 positive boutons per CTB-labelled motoneurons significantly reduced in group C (P<0.001). We demonstrated for the first time that contralateral L4 ventral root transfer to L5 ventral root of the affected side was effective in relieving unilateral motor cortex lesion-induced hindlimb spasticity in rats. Our data indicated that this could be an alternative treatment for unilateral lower extremity spasticity after brain injury. Therefore, contralateral neurotization may exert a potential therapeutic candidate to improve the function of lower extremity in patients with spastic hemiplegia. © 2016 The Author(s).

Percutaneous epidural neurostimulation in modulation of paraplegic spasticity. Six case reports.

PubMed

Richardson, R R; Cerullo, L J; McLone, D G; Gutierrez, F A; Lewis, V

1979-01-01

Six cases of paraplegic, post-traumatic spasticity, alleviated by percutaneous epidural neurostimulation with temporary or permanent implanted neuroelectrodes from the L1 to L4 intervertebral levels are presented. Modulation of this spasticity and secondary beneficial physiological effects were achieved, including regulation of bowel regimens, production of sweating and piloerection below the level of the lesion, and morning erections. The main advantages of percutaneous epidural neurostimulation in modulating spasticity are the avoidance of destructive neurosurgical procedures, the regulation of secondary physiological and autonomic responses, the avoidance of antispasticity medications, and the reversibility of the neurostimulation procedure.

Intra-operatively measured spastic semimembranosus forces of children with cerebral palsy.

PubMed

Yucesoy, Can A; Temelli, Yener; Ateş, Filiz

2017-10-01

The knee kept forcibly in a flexed position is typical in cerebral palsy. Using a benchmark, we investigate intra-operatively if peak spastic hamstring force is measured in flexed knee positions. This tests the assumed shift of optimal length due to adaptation of spastic muscle and a decreasing force trend towards extension. Previously we measured spastic gracilis (GRA) and semitendinosus (ST) forces. Presently, we studied spastic semimembranosus (SM) and tested the following hypotheses: spastic SM forces are (1) high in flexed and (2) low in extended positions. We compared the data to those of GRA and ST to test (3) if percentages of peak force produced in flexed positions are different. During muscle lengthening surgery of 8 CP patients (9years, 4months; GMFCS levels=II-IV; limbs tested=13) isometric SM forces were measured from flexion (120°) to full extension (0°). Spastic SM forces were low in flexed knee positions (only 4.2% (3.4%) and 10.7% (9.7%) of peak force at KA=120° and KA=90° respectively, indicating less force production compared to the GRA or ST) and high in extended knee positions (even 100% of peak force at KA=0°). This indicates an absence of strong evidence for a shift of optimal muscle length of SM towards flexion. Copyright © 2017 Elsevier Ltd. All rights reserved.

Beneficial effects of footbaths in controlling spasticity after stroke

NASA Astrophysics Data System (ADS)

Matsumoto, Shuji; Shimodozono, Megumi; Etoh, Seiji; Shimozono, Yurika; Tanaka, Nobuyuki; Kawahira, Kazumi

2010-07-01

Footbaths are considered to provide beneficial thermal therapy for post-stroke patients with spasticity, but their anti-spastic effects have not been investigated comprehensively. The present study aimed to evaluate alterations in motor-neuron excitability using F-wave parameters in post-stroke patients with spastic hemiplegia. Subjects’ legs below the knee joint were immersed in water at 41°C and F-wave recordings were made over the abductor hallucis muscle before, immediately after, and 30 min after thermal treatment. Antidromic stimulation was performed on the tibial nerve at the ankle. Measurements included F-wave amplitude, F-wave/M-response ratio, changes in modified Ashworth scale (MAS), body temperature and surface-skin temperature. The mean values of both F-wave parameters were higher on the affected side before footbath treatment. In post-stroke patients, the mean values of F-wave parameters were significantly reduced after footbath treatment ( P < 0.01). The anti-spastic effects of footbath treatment were indicated by decreased F-wave parameters, in parallel with decreases in MAS. Body temperature was significantly increased both immediately after, and 30 min following footbath treatment in both groups, which appeared to play an important role in decreased spasticity. Surface-skin temperature increased immediately after footbath treatment in both groups and returned to baseline 30 min later. These findings demonstrate that the use of footbaths is an effective nonpharmacological anti-spastic treatment that might facilitate stroke rehabilitation.

Intensive Voice Treatment (LSVT LOUD) for Children with Spastic Cerebral Palsy and Dysarthria

ERIC Educational Resources Information Center

Fox, Cynthia Marie; Boliek, Carol Ann

2012-01-01

Purpose: The purpose of this study was to examine the effects of an intensive voice treatment (Lee Silverman Voice Treatment, commonly known as LSVT LOUD) for children with spastic cerebral palsy (CP) and dysarthria. Method: A nonconcurrent multiple baseline single-subject design with replication across 5 children with spastic CP was used.…

Prevalence and Causes of Paralysis—United States, 2013

PubMed Central

Armour, Brian S.; Courtney-Long, Elizabeth A.; Fox, Michael H.; Fredine, Heidi; Cahill, Anthony

2017-01-01

Objectives To estimate the prevalence and causes of functional paralysis in the United States. Methods We used the 2013 US Paralysis Prevalence & Health Disparities Survey to estimate the prevalence of paralysis, its causes, associated sociodemographic characteristics, and health effects among this population. Results Nearly 5.4 million persons live with paralysis. Most persons with paralysis were younger than 65 years (72.1%), female (51.7%), White (71.4%), high school graduates (64.8%), married or living with a partner (47.4%), and unable to work (41.8%). Stroke is the leading cause of paralysis, affecting 33.7% of the population with paralysis, followed by spinal cord injury (27.3%), multiple sclerosis (18.6%), and cerebral palsy (8.3%). Conclusions According to the functional definition, persons living with paralysis represent a large segment of the US population, and two thirds of them are between ages 18 and 64 years. Targeted health promotion that uses inclusion strategies to account for functional limitations related to paralysis can be undertaken in partnership with state and local health departments. PMID:27552260

Cannabinoids for spasticity due to multiple sclerosis or paraplegia: A systematic review and meta-analysis of randomized clinical trials.

PubMed

da Rovare, Victoria P; Magalhães, Gabriel P A; Jardini, Guilherme D A; Beraldo, Matheus L; Gameiro, Mariel O; Agarwal, Arnav; Luvizutto, Gustavo José; Paula-Ramos, Lucas; Camargo, Samira Esteves Afonso; de Oliveira, Luciane Dias; Bazan, Rodrigo; El Dib, Regina

2017-10-01

Spasticity remains highly prevalent in patients with spinal cord injury and multiple sclerosis. To summarize the effects of cannabinoids compared with usual care, placebo for spasticity due to multiple sclerosis (MS) or paraplegia. Searches of MEDLINE, EMBASE, CENTRAL and LILACS to March 2017 were performed to identify randomized controlled trials. The primary outcomes were spasticity and spasm frequency. The criteria were any patient with MS and spasticity affecting upper or lower limbs or both, and that had a confirmed diagnosis of MS based on validated criteria, or however defined by the authors of the included studies. 16 trials including 2597 patients were eligible. Moderate-certainty evidence suggested a non-statistically significant decrease in spasticity (standardized mean difference (SMD) 0.36 [confidential interval (CI) 95% -0.17 to 0.88; p=0.18; I2=88%]), and spasm frequency (SMD 0.04 [CI 95% -0.15 to 0.22]). There was an increase in adverse events such as dizziness (risk ratio (RR) 3.45 [CI 95% 2.71-4.4; p=0.20; I2=23%]), somnolence (RR 2.9 [CI 95% 1.98-4.23; p=0.77; I2=0%]), and nausea (RR 2.25 [CI 95% 1.62-3.13; p=0.83; I2=0%]). There is moderate certainty evidence regarding the impact of cannabinoids in spasticity (average 0.36 more spasticity; 0.17 fewer to 0.88 more) due to multiple sclerosis or paraplegia, and in adverse events such as dizziness (419 more dizziness/1000 over 19 weeks), somnolence (127 more somnolence/1000 over 19 weeks), and nausea (125 more somnolence/1000 over 19 weeks). Copyright © 2017. Published by Elsevier Ltd.

Deep anterior cerebellar stimulation reduces symptoms of secondary dystonia in patients with cerebral palsy treated due to spasticity.

PubMed

Sokal, Paweł; Rudaś, Marcin; Harat, Marek; Szylberg, Łukasz; Zieliński, Piotr

2015-08-01

Deep anterior cerebellar stimulation (DACS) is a neuromodulation therapy of spasticity. Bilateral DACS is applied in young patients with cerebral palsy (CP). In these patients symptoms of spasticity coexist with symptoms of focal or segmental dystonia, which can cause chronic pain. We performed the study to investigate the therapeutic effects of DACS in spasticity, secondary dystonia and pain. We examined 10 from 13 patients with CP treated with DACS due to spasticity in years 2006-2012. We compared Ashworth scores of spasticity, VAS scale of pain and UDRS (Unified Dystonia Rating Scale) score before DACS and after it in follow-up lasting from 2 to 11 years it in these patients basing on clinical examination and evaluating forms given by the patients or parents. We received statistically significant reduction of spasticity in upper extremities (median: from 3 to 1,5 in Ashworth scale) in 8 patients (p = 0,01), in lower extremities in 7 patients (median: from 3 to 1,75) (p = 0,02). Symptoms of focal dystonia were reduced. Total score for the UDRS (median = 18,0 before surgery) after DACS decreased significantly (median = 10,3) (p = 0,043). Change in consecutive parts of UDRS before (median = 1,6) and after (median = 1,0) surgery in 7 patients had statistical significance (p = 0,0179). There were not significant changes in intensity of pain before and after surgery (p = 0,108). Chronic bilateral DACS aimed for spasticity treatment not only decreases muscular tone in quadriplegic or paraplegic patients with CP but also is associated with reduction of symptoms of focal or segmental, secondary dystonia. Copyright © 2015 Elsevier B.V. All rights reserved.

Advances in the management of multiple sclerosis spasticity: recent clinical trials.

PubMed

Fernández, Oscar

2014-01-01

Most patients with multiple sclerosis (MS) experience spasticity as the clinical course evolves. Associated symptoms include (often painful) spasms, urinary dysfunction and sleep disturbances. THC:CBD oromucosal spray (Sativex®) is approved for symptom improvement in adult patients with moderate to severe MS-related spasticity who have not responded adequately to other antispasticity medication and who demonstrate clinically significant improvement in spasticity-related symptoms during an initial trial of therapy. In pivotal clinical trials of THC:CBD oromucosal spray, a meaningful proportion of patients with treatment-resistant MS spasticity achieved clinically relevant improvement with active treatment versus placebo. The utility of a 4-week trial of therapy to identify patients who respond to treatment was demonstrated in an enriched-design study. THC:CBD oromucosal spray was well tolerated in these studies, with no evidence of effects typically associated with recreational cannabis use. In a subsequent post approval clinical trial, THC:CBD oromucosal spray had no statistically significant effect on cognition and mood compared with placebo. Moreover, after 50 weeks' treatment, approximately two-thirds of patients, physicians and caregivers reported improvement from baseline in spasticity based on global impressions of change. Key Messages: In phase III clinical trials, approximately one-third of MS patients with treatment-resistant spasticity had a clinically relevant and statistically significant response to THC:CBD oromucosal spray. In addition to a reduction in spasticity, responders experienced meaningful relief from associated symptoms. THC:CBD oromucosal spray was generally well tolerated and efficacy was maintained over the longer term. A post-approval clinical trial indicated no effect of THC:CBD oromucosal spray on cognition or mood after 50 weeks of use. © 2014 S. Karger AG, Basel.

Association Among Facial Paralysis, Depression, and Quality of Life in Facial Plastic Surgery Patients

PubMed Central

Nellis, Jason C.; Ishii, Masaru; Byrne, Patrick J.; Boahene, Kofi D. O.; Dey, Jacob K.; Ishii, Lisa E.

2017-01-01

IMPORTANCE Though anecdotally linked, few studies have investigated the impact of facial paralysis on depression and quality of life (QOL). OBJECTIVE To measure the association between depression, QOL, and facial paralysis in patients seeking treatment at a facial plastic surgery clinic. DESIGN, SETTING, PARTICIPANTS Data were prospectively collected for patients with all-cause facial paralysis and control patients initially presenting to a facial plastic surgery clinic from 2013 to 2015. The control group included a heterogeneous patient population presenting to facial plastic surgery clinic for evaluation. Patients who had prior facial reanimation surgery or missing demographic and psychometric data were excluded from analysis. MAIN OUTCOMES AND MEASURES Demographics, facial paralysis etiology, facial paralysis severity (graded on the House-Brackmann scale), Beck depression inventory, and QOL scores in both groups were examined. Potential confounders, including self-reported attractiveness and mood, were collected and analyzed. Self-reported scores were measured using a 0 to 100 visual analog scale. RESULTS There was a total of 263 patients (mean age, 48.8 years; 66.9% were female) were analyzed. There were 175 control patients and 88 patients with facial paralysis. Sex distributions were not significantly different between the facial paralysis and control groups. Patients with facial paralysis had significantly higher depression, lower self-reported attractiveness, lower mood, and lower QOL scores. Overall, 37 patients with facial paralysis (42.1%) screened positive for depression, with the greatest likelihood in patients with House-Brackmann grade 3 or greater (odds ratio, 10.8; 95% CI, 5.13–22.75) compared with 13 control patients (8.1%) (P < .001). In multivariate regression, facial paralysis and female sex were significantly associated with higher depression scores (constant, 2.08 [95% CI, 0.77–3.39]; facial paralysis effect, 5.98 [95% CI, 4.38–7.58]; female effect, 1.95 [95% CI, 0.65–3.25]). Facial paralysis was associated with lower QOL scores (constant, 81.62 [95% CI, 78.98–84.25]; facial paralysis effect, −16.06 [95% CI, −20.50 to −11.62]). CONCLUSIONS AND RELEVANCE For treatment-seeking patients, facial paralysis was significantly associated with increased depression and worse QOL scores. In addition, female sex was significantly associated with increased depression scores. Moreover, patients with a greater severity of facial paralysis were more likely to screen positive for depression. Clinicians initially evaluating patients should consider the psychological impact of facial paralysis to optimize care. LEVEL OF EVIDENCE 2. PMID:27930763

Kinematic gait deficits at the trunk and pelvis: characteristic features in children with hereditary spastic paraplegia.

PubMed

Adair, Brooke; Rodda, Jillian; McGinley, Jennifer L; Graham, H Kerr; Morris, Meg E

2016-08-01

To examine the kinematic gait deviations at the trunk and pelvis of children with hereditary spastic paraplegia (HSP). This exploratory observational study quantified gait kinematics for the trunk and pelvis from 11 children with HSP (7 males, 4 females) using the Gait Profile Score and Gait Variable Scores (GVS), and compared the kinematics to data from children with typical development using a Mann-Whitney U test. Children with HSP (median age 11y 4mo, interquartile range 4y) demonstrated large deviations in the GVS for the trunk and pelvis in the sagittal and coronal planes when compared to the gait patterns of children with typical development (p=0.010-0.020). Specific deviations included increased range of movement for the trunk in the coronal plane and increased excursion of the trunk and pelvis in the sagittal plane. In the transverse plane, children with HSP demonstrated later peaks in posterior pelvic rotation. The kinematic gait deviations identified in this study raise questions about the contribution of muscle weakness in HSP. Further research is warranted to determine contributing factors for gait dysfunction in HSP, especially the relative influence of spasticity and weakness. © 2016 Mac Keith Press.

Analysis of electromyographic activity in spastic biceps brachii muscle following neural mobilization.

PubMed

Castilho, Jéssica; Ferreira, Luiz Alfredo Braun; Pereira, Wagner Menna; Neto, Hugo Pasini; Morelli, José Geraldo da Silva; Brandalize, Danielle; Kerppers, Ivo Ilvan; Oliveira, Claudia Santos

2012-07-01

Hypertonia is prevalent in anti-gravity muscles, such as the biceps brachii. Neural mobilization is one of the techniques currently used to reduce spasticity. The aim of the present study was to assess electromyographic (EMG) activity in spastic biceps brachii muscles before and after neural mobilization of the upper limb contralateral to the hemiplegia. Repeated pre-test and post-test EMG measurements were performed on six stroke victims with grade 1 or 2 spasticity (Modified Ashworth Scale). The Upper Limb Neurodynamic Test (ULNT1) was the mobilization technique employed. After neural mobilization contralateral to the lesion, electromyographic activity in the biceps brachii decreased by 17% and 11% for 90° flexion and complete extension of the elbow, respectively. However, the results were not statistically significant (p gt; 0.05). When performed using contralateral techniques, neural mobilization alters the electrical signal of spastic muscles. Copyright © 2011 Elsevier Ltd. All rights reserved.

A patient with bilateral facial palsy associated with hypertension and chickenpox: learning points.

PubMed

Al-Abadi, Eslam; Milford, David V; Smith, Martin

2010-11-26

Bilateral facial nerve paralysis is an uncommon presentation and even more so in children. There are reports of different causes of bilateral facial nerve palsy. It is well-established that hypertension and chickenpox causes unilateral facial paralysis and the importance of checking the blood pressure in children with facial nerve paralysis cannot be stressed enough. The authors report a boy with bilateral facial nerve paralysis in association with hypertension and having recently recovered from chickenpox. The authors review aspects of bilateral facial nerve paralysis as well as hypertension and chickenpox causing facial nerve paralysis.

Effects of Age and Exposure on the Health Status of U.S. Navy Divers.

DTIC Science & Technology

1984-01-01

Neuritis/Neuralgia/ Sciatica - 1.3 6.9 7.0 0 Facial Paralysis/Cerebral Paralysis 0 - - 0 - Diseases of the Circulatory System 15.6 18.3 24.9 38.3* 120.8...10.3 Otitts Externa/Other Ear Disorders 8.9 6.8 5.8 Deafness 3.5 3.4 2.6 Neuritis/Neuralgia/ Sciatica 2.6 2.6 - racial Paralysis/Cerebral Paralysis...20 18 10 9 Deafness 10 11 9 9 5 4 Neuritis/Neuralqia/ Sciatica 5 8 7 7 5 2 Facial Paralysis/Cerebral Paralysis 2 2 2 2 1 1 Diseases of the Circulatory

A patient with bilateral facial palsy associated with hypertension and chickenpox: learning points

PubMed Central

Al-Abadi, Eslam; Milford, David V; Smith, Martin

2010-01-01

Bilateral facial nerve paralysis is an uncommon presentation and even more so in children. There are reports of different causes of bilateral facial nerve palsy. It is well-established that hypertension and chickenpox causes unilateral facial paralysis and the importance of checking the blood pressure in children with facial nerve paralysis cannot be stressed enough. The authors report a boy with bilateral facial nerve paralysis in association with hypertension and having recently recovered from chickenpox. The authors review aspects of bilateral facial nerve paralysis as well as hypertension and chickenpox causing facial nerve paralysis. PMID:22797481

Use of botulinum toxin type A and type B for spasticity in upper and lower limbs.

PubMed

Bell, Kathleen R; Williams, Faren

2003-11-01

BT is likely effective in controlling spasticity in the smaller muscles of the arm and hand, although there has been only one large controlled trial. For lower limb spasticity, the outcomes are more mixed. No large randomized, controlled trials have been done, and the larger size of the muscles results in a decreased ability to treat widespread spasticity. For more focal treatment in the legs and feet, however, and when combined with other denervating agents or physical modalities, BT is probably effective. Careful analysis is warranted before performing any chemodenervation on a limb muscle or muscles.

[The usefulness of tizanidine. A one-year follow-up of the treatment of spasticity in infantile cerebral palsy].

PubMed

Vásquez-Briceño, A; Arellano-Saldaña, M E; León-Hernández, S R; Morales-Osorio, M G

The cerebral palsy has the first place of physical handicap in children (type spastic, 88%). Tizanidine imidazole derivative is centrally acting as a a2-adrenergic agonist. To demonstrate clinically the effectiveness of tizanidine in the decrease of the spasticity. We assigned randomly in a double blind study 10 children treated with tizanidine (0.05 mg/kg/day) and 30 with placebo for a 6-month period, after which they were unified in the group of tizanidine. The dependent variables were spasticity, Ashworth scale, posture tone scale, reflex scale and liver function test. The spasticity and the reflex decreased in the group of tizanidine an 78.85% in comparison with a 7.64% in the group of placebo (p = 0.0001); in the monitoring of 6 months 35 patients reduced this to 78.2% (p= 0.0001). The duration of effectiveness of tizanidine in four patients was two months and they never returned to their appraisal basal. Without reported adverse effects, the liver function test remains normal. Tizanidine produces a significant reduction of the spasticity in children without adverse effects, having a high percentage of acceptance to the prescribe dose.

Loss-of-function mutations in the ATP13A2/PARK9 gene cause complicated hereditary spastic paraplegia (SPG78)

PubMed Central

Estrada-Cuzcano, Alejandro; Martin, Shaun; Chamova, Teodora; Synofzik, Matthis; Timmann, Dagmar; Holemans, Tine; Andreeva, Albena; Reichbauer, Jennifer; De Rycke, Riet; Chang, Dae-In; van Veen, Sarah; Samuel, Jean; Schöls, Ludger; Pöppel, Thorsten; Mollerup Sørensen, Danny; Asselbergh, Bob; Klein, Christine; Zuchner, Stephan; Jordanova, Albena; Vangheluwe, Peter; Tournev, Ivailo; Schüle, Rebecca

2017-01-01

Abstract Hereditary spastic paraplegias are heterogeneous neurodegenerative disorders characterized by progressive spasticity of the lower limbs due to degeneration of the corticospinal motor neurons. In a Bulgarian family with three siblings affected by complicated hereditary spastic paraplegia, we performed whole exome sequencing and homozygosity mapping and identified a homozygous p.Thr512Ile (c.1535C > T) mutation in ATP13A2. Molecular defects in this gene have been causally associated with Kufor-Rakeb syndrome (#606693), an autosomal recessive form of juvenile-onset parkinsonism, and neuronal ceroid lipofuscinosis (#606693), a neurodegenerative disorder characterized by the intracellular accumulation of autofluorescent lipopigments. Further analysis of 795 index cases with hereditary spastic paraplegia and related disorders revealed two additional families carrying truncating biallelic mutations in ATP13A2. ATP13A2 is a lysosomal P5-type transport ATPase, the activity of which critically depends on catalytic autophosphorylation. Our biochemical and immunocytochemical experiments in COS-1 and HeLa cells and patient-derived fibroblasts demonstrated that the hereditary spastic paraplegia-associated mutations, similarly to the ones causing Kufor-Rakeb syndrome and neuronal ceroid lipofuscinosis, cause loss of ATP13A2 function due to transcript or protein instability and abnormal intracellular localization of the mutant proteins, ultimately impairing the lysosomal and mitochondrial function. Moreover, we provide the first biochemical evidence that disease-causing mutations can affect the catalytic autophosphorylation activity of ATP13A2. Our study adds complicated hereditary spastic paraplegia (SPG78) to the clinical continuum of ATP13A2-associated neurological disorders, which are commonly hallmarked by lysosomal and mitochondrial dysfunction. The disease presentation in our patients with hereditary spastic paraplegia was dominated by an adult-onset lower-limb predominant spastic paraparesis. Cognitive impairment was present in most of the cases and ranged from very mild deficits to advanced dementia with fronto-temporal characteristics. Nerve conduction studies revealed involvement of the peripheral motor and sensory nerves. Only one of five patients with hereditary spastic paraplegia showed clinical indication of extrapyramidal involvement in the form of subtle bradykinesia and slight resting tremor. Neuroimaging cranial investigations revealed pronounced vermian and hemispheric cerebellar atrophy. Notably, reduced striatal dopamine was apparent in the brain of one of the patients, who had no clinical signs or symptoms of extrapyramidal involvement. PMID:28137957

Facial nerve paralysis secondary to occult malignant neoplasms.

PubMed

Boahene, Derek O; Olsen, Kerry D; Driscoll, Colin; Lewis, Jean E; McDonald, Thomas J

2004-04-01

This study reviewed patients with unilateral facial paralysis and normal clinical and imaging findings who underwent diagnostic facial nerve exploration. Study design and setting Fifteen patients with facial paralysis and normal findings were seen in the Mayo Clinic Department of Otorhinolaryngology. Eleven patients were misdiagnosed as having Bell palsy or idiopathic paralysis. Progressive facial paralysis with sequential involvement of adjacent facial nerve branches occurred in all 15 patients. Seven patients had a history of regional skin squamous cell carcinoma, 13 patients had surgical exploration to rule out a neoplastic process, and 2 patients had negative exploration. At last follow-up, 5 patients were alive. Patients with facial paralysis and normal clinical and imaging findings should be considered for facial nerve exploration when the patient has a history of pain or regional skin cancer, involvement of other cranial nerves, and prolonged facial paralysis. Occult malignancy of the facial nerve may cause unilateral facial paralysis in patients with normal clinical and imaging findings.

'The devil lay upon her and held her down'. Hypnagogic hallucinations and sleep paralysis described by the Dutch physician Isbrand van Diemerbroeck (1609-1674) in 1664.

PubMed

Kompanje, E J O

2008-12-01

Hypnagogic and hypnopompic hallucinations are visual, tactile, auditory or other sensory events, usually brief but sometimes prolonged, that occur at the transition from wakefulness to sleep (hypnagogic) or from sleep to wakefulness (hypnopompic). Hypnagogic and hypnopompic hallucinations are often associated with sleep paralysis. Sleep paralysis occurs immediately prior to falling asleep (hypnagogic paralysis) or upon waking (hypnopompic paralysis). In 1664, the Dutch physician Isbrand Van Diemerbroeck (1609-1674) published a collection of case histories. One history with the title 'Of the Night-Mare' describes the nightly experiences of the 50-year-old woman. This case report is subject of this article. The experiences in this case could without doubt be diagnosed as sleep paralysis accompanied by hypnagogic hallucinations. This case from 1664 should be cited as the earliest detailed account of sleep paralysis associated with hypnagogic illusions and as the first observation that sleep paralysis and hypnagogic experiences occur more often in supine position of the body.

Evaluation of diaphragmatic motion in normal and diaphragmatic paralyzed dogs using M-mode ultrasonography.

PubMed

Choi, Mihyun; Lee, Namsoon; Kim, Ahyoung; Keh, Seoyeon; Lee, Jinsoo; Kim, Hyunwook; Choi, Mincheol

2014-01-01

Diagnosis of unilateral diaphragmatic paralysis in dogs is currently based on fluoroscopic detection of unequal movement between the crura. Bilateral paralysis may be more difficult to confirm with fluoroscopy because diaphragmatic movement is sometimes produced by compensatory abdominal muscle contractions. The purpose of this study was to develop a new method to evaluate diaphragmatic movement using M-mode ultrasonography and to describe findings for normal and diaphragmatic paralyzed dogs. Fifty-five clinically normal dogs and two dogs with diaphragmatic paralysis were recruited. Thoracic radiographs were acquired for all dogs and fluoroscopy studies were also acquired for clinically affected dogs. Two observers independently measured diaphragmatic direction of motion and amplitude of excursion using M-mode ultrasonography for dogs meeting study inclusion criteria. Eight of the clinically normal dogs were excluded due to abnormal thoracic radiographic findings. For the remaining normal dogs, the lower limit values of diaphragmatic excursion were 2.85-2.98 mm during normal breathing. One dog with bilateral diaphragmatic paralysis showed paradoxical movement of both crura at the end of inspiration. One dog with unilateral diaphragmatic paralysis had diaphragmatic excursion values of 2.00 ± 0.42 mm on the left side and 4.05 ± 1.48 mm on the right side. The difference between left and right diaphragmatic excursion values was 55%. Findings indicated that M-mode ultrasonography is a relatively simple and objective method for measuring diaphragmatic movement in dogs. Future studies are needed in a larger number of dogs with diaphragmatic paralysis to determine the diagnostic sensitivity of this promising new technique. © 2013 American College of Veterinary Radiology.

Comparisons of steroid, acyclovir, lipoprostoglandin E1 and steroid + acyclovir treatments in facial paralysis: a rat study.

PubMed

Gök, Uzeyir; Alpay, Hayrettin Cengiz; Akpolat, Nusret; Yoldaş, Tahir; Kilic, Abdullah; Yilmaz, Bayram; Kabakuş, Nimet

2005-09-01

To induce experimental peripheral facial paralysis by inoculation of HSV1 and to compare the effects of steroid, acyclovir, lipoprostoglandin E2 and steroid + acyclovir treatments in terms of clinical recovery, electrophysiologically and histopathologically. A total of 132 adult female rats were used in this study. HSV type 1 strain was inoculated at the back of the left ear by using 27 gauge needle. Of all animals, 70 (53%) rats which developed facial paralysis were divided into five groups (n = 14 for each group) as control, steroid + acyclovir, lipoprostaglandin E1, steroid only and acyclovir only. At the end of the 21 days period, the rats were clinically examined and electrophysiological tests were performed, then decapitated and the nerve specimens were obtained. A modified electroneurography (ENoG) test was performed and the latencies and the amplitudes were compared. The findings of the intact side were better, but with no significant difference. Histopathologicaly edema was significantly smaller in all groups compared to the controls (p < 0.05). Similarly, no difference was seen in terms of vacuolar degeneration and Schwann cell hyperchromatisation among the groups and no significant difference in recovery period and rate of facial paralysis when all groups were compared. Facial paralysis induced by HSV1 recovered spontaneously within a week. In the treatment of facial paralysis, steroid alone, acyclovir alone, steroid + acyclovir, or lipoprostaglandin E1 all reduced edema in the infected facial nerve but there was no statistical difference in of the rate or degree of recovery.

Best Practices for Intrathecal Baclofen Therapy: Patient Selection.

PubMed

Saulino, Michael; Ivanhoe, Cindy B; McGuire, John R; Ridley, Barbara; Shilt, Jeffrey S; Boster, Aaron L

2016-08-01

When spasticity interferes with comfort, function, activities of daily living, mobility, positioning, or caregiver assistance, patients should be considered for intrathecal baclofen (ITB) therapy. An expert panel consulted on best practices. ITB can be considered for problematic spasticity involving muscles/muscle groups during all phases of diseases, including progressive neurologic diseases. ITB alone or with other treatments should not be exclusively reserved for individuals who have failed other approaches. ITB combined with rehabilitation can be effective in certain ambulatory patients. ITB is also highly effective in managing spasticity in children, who may suffer limb deformity, joint dislocation, and poor motor function from spasticity and muscle tightness on the growing musculoskeletal system. Spasticity management often allows individuals to achieve higher function. When cognition is impaired, ITB controls spasticity without the cognitive side effects of some oral medications. Goal setting addresses expectations and treatment in the framework of pathology, impairment, and disability. ITB is contraindicated in patients with hypersensitivity to baclofen, which is rare, or active infection. Some patients with an adverse reaction to oral baclofen may be mistakenly classified as having an allergic reaction and may benefit from ITB. Relative contraindications include unrealistic goals, unmanageable mental health issues, psychosocial factors affecting compliance, and financial burden. Vascular shunting for hydrocephalus is not a contraindication, but concurrent use may affect cerebrospinal fluid flow. Seizures or prior abdominal or pelvic surgery should be discussed before proceeding to an ITB screening test. ITB should be considered when spasticity interferes with comfort or function. © 2016 International Neuromodulation Society.

Long-term therapy with intrathecal baclofen improves quality of life in children with severe spastic cerebral palsy.

PubMed

Kraus, Tanja; Gegenleitner, Kathrin; Svehlik, Martin; Novak, Michael; Steinwender, Gerhardt; Singer, Georg

2017-05-01

Children with severe spastic cerebral palsy (CP) are highly limited in daily life activities causing a reduced quality of life (QoL). This is partly due to an increased muscle tone causing pain and contractures. Continuous intrathecal infusion of baclofen (ITB) reduces the spasticity of affected patients. The hypothesis of the present study was that ITB leads to a significant improvement of QoL in non-ambulant children with CP. 13 patients (10 male, 3 female, mean age 14 years) were included. Mean time between pump implantation and follow-up was 60 months (range, 12-100). QoL was assessed before and after baclofen pump implantation using standardized questionnaires (CP CHILD, KINDL). Spasticity was evaluated using the modified Ashworth Scale (MAS) at the two time points. QoL evaluated with the CPCHILD questionnaire and the KINDL improved from pre - implantation to follow-up. MAS markedly decreased from 3.8 to 1.7. All interviewed participants indicated that their expectations had been met and that they would choose ITB treatment again. Intrathecal treatment of baclofen is an excellent method for spasticity management in children with severe cerebral palsy. Quality of life sustainably improves, parents' satisfaction is high and the level of spasticity decreases. Therefore, baclofen treatment can be highly recommended in non-ambulant children with CP suffering from spasticity. Copyright © 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

A new multiple sclerosis spasticity treatment option: effect in everyday clinical practice and cost-effectiveness in Germany.

PubMed

Flachenecker, Peter

2013-02-01

Sativex® (GW Pharmaceuticals PLC, Porton Down, UK; Laboratorios Almirall, SA, Barcelona, Spain), a cannabinoid oromucosal spray containing a 1:1 ratio of 9-δ-tetrahydrocannabinol and cannabidiol, has been licensed in Germany since July 2011 as add-on therapy for moderate-to-severe multiple sclerosis (MS) treatment-resistant spasticity symptoms. The 'MOVE 2' study evaluated clinical outcomes, treatment satisfaction, quality of life (QoL) and provision of care in MS patients with spasticity receiving Sativex in everyday clinical practice. Data from 300 patients were collected from 42 specialized MS centers across Germany and were available for this analysis. Assessments, including the MS spasticity 0-10 numerical rating scale, modified Ashworth scale, patients' and physicians' clinical impressions, and QoL scales were rated at baseline and at 1 and 3 months after starting treatment with Sativex. Sativex provided relief of MS-related spasticity in the majority of patients who were previously resistant to treatment. In addition, clear improvements were noted in MS spasticity-associated symptoms (e.g., sleep quality, bladder function and mobility), activities of daily living and QoL. Sativex was generally well tolerated. The majority of patients (84%) reported no adverse events, and there was only a limited risk of serious adverse reactions. Furthermore, based on data from Sativex clinical trials, a Markov model-based analysis has shown that Sativex is a cost-effective treatment option for patients with MS spasticity in Germany.

Clinical and imaging characterization of progressive spastic dysarthria

PubMed Central

Clark, Heather M.; Duffy, Joseph R.; Whitwell, Jennifer L.; Ahlskog, J. Eric; Sorenson, Eric J.; Josephs, Keith A.

2013-01-01

Objective To describe speech, neurological and imaging characteristics of a series of patients presenting with progressive spastic dysarthria (PSD) as the first and predominant sign of a presumed neurodegenerative disease. Methods Participants were 25 patients with spastic dysarthria as the only or predominant speech disorder. Clinical features, pattern of MRI volume loss on voxel-based morphometry, and pattern of hypometabolism with F18-Fluorodeoxyglucose (FDG-PET) scan are described. Results All patients demonstrated speech characteristics consistent with spastic dysarthria, including strained voice quality, slow speaking rate, monopitch and monoloudness, and slow and regular speech alternating motion rates. Eight patients did not have additional neurological findings on examination. Pseudobulbar affect, upper motor neuron pattern limb weakness, spasticity, Hoffman sign and positive Babinski reflexes were noted in some of the remaining patients. Twenty-three patients had electromyographic assessment and none had diffuse motor neuron disease or met El Escorial criteria for ALS. Voxel-based morphometry revealed striking bilateral white matter volume loss, , affecting the motor cortex (BA 4), including the frontoparietal operculum (BA 43) with extension into the middle cerebral peduncle. FDG-PET showed subtle hypometabolism affecting the premotor and motor cortices in some patients, particularly in those who had a disease duration longer than two years. Conclusions We have characterized a neurodegenerative disorder that begins focally with spastic dysarthria due to involvement of the motor and premotor cortex and descending corticospinal and corticobulbar pathways. We propose the descriptive label “progressive spastic dysarthria” to best capture the dominant presenting feature of the syndrome. PMID:24053325

Delayed presentation of traumatic facial nerve (CN VII) paralysis.

PubMed

Napoli, Anthony M; Panagos, Peter

2005-11-01

Facial nerve paralysis (Cranial Nerve VII, CN VII) can be a disfiguring disorder with profound impact upon the patient. The etiology of facial nerve paralysis may be congenital, iatrogenic, or result from neoplasm, infection, trauma, or toxic exposure. In the emergency department, the most common cause of unilateral facial paralysis is Bell's palsy, also known as idiopathic facial paralysis (IFP). We report a case of delayed presentation of unilateral facial nerve paralysis 3 days after sustaining a traumatic head injury. Re-evaluation and imaging of this patient revealed a full facial paralysis and temporal bone fracture extending into the facial canal. Because cranial nerve injuries occur in approximately 5-10% of head-injured patients, a good history and physical examination is important to differentiate IFP from another etiology. Newer generation high-resolution computed tomography (CT) scans are commonly demonstrating these fractures. An understanding of this complication, appropriate patient follow-up, and early involvement of the Otolaryngologist is important in management of these patients. The mechanism as well as the timing of facial nerve paralysis will determine the proper evaluation, consultation, and management for the patient. Patients with total or immediate paralysis as well as those with poorly prognostic audiogram results are good candidates for surgical repair.

Thyrotoxic periodic paralysis associated with a mutation in the sodium channel gene SCN4A.

PubMed

Lane, Andrew H; Markarian, Katherine; Braziunene, Ieva

2004-12-01

Thyrotoxic hypokalemic periodic paralysis (THypoKPP) is an uncommon disorder with an unknown etiology. We describe a family in which the proband presented with paralysis and thyrotoxicosis. Because of similarities between familial hypokalemic periodic paralysis (FHypoKPP) and THypoKPP, we sequenced exon 12 of the SCN4A gene, which is known to be mutated in FHypoKPP. We identified an Arg672Ser mutation in the proband and his affected father, as well as the proband's brother. As the brother has paralysis without thyrotoxicosis, our finding suggests that the genetic spectrum of FHypoKPP and THypoKPP overlap. We speculate that thyroid hormone may exert a threshold or permissive effect in hypokalemic periodic paralysis. Non-thyrotoxic family members of individuals with THypoKPP may have an unrecognized risk for paralysis.

Supplemental Stimulation Improves Swing Phase Kinematics During Exoskeleton Assisted Gait of SCI Subjects With Severe Muscle Spasticity.

PubMed

Ekelem, Andrew; Goldfarb, Michael

2018-01-01

Spasticity is a common comorbidity associated with spinal cord injury (SCI). Robotic exoskeletons have recently emerged to facilitate legged mobility in people with motor complete SCI. Involuntary muscle activity attributed to spasticity, however, can prevent such individuals from using an exoskeleton. Specifically, although most exoskeleton technologies can accommodate low to moderate spasticity, the presence of moderate to severe spasticity can significantly impair gait kinematics when using an exoskeleton. In an effort to potentially enable individuals with moderate to severe spasticity to use exoskeletons more effectively, this study investigates the use of common peroneal stimulation in conjunction with exoskeleton gait assistance. The electrical stimulation is timed with the exoskeleton swing phase, and is intended to acutely suppress extensor spasticity through recruitment of the flexion withdrawal reflex (i.e., while the stimulation is activated) to enable improved exoskeletal walking. In order to examine the potential efficacy of this approach, two SCI subjects with severe extensor spasticity (i.e., modified Ashworth ratings of three to four) walked in an exoskeleton with and without supplemental stimulation while knee and hip motion was measured during swing phase. Stimulation was alternated on and off every ten steps to eliminate transient therapeutic effects, enabling the acute effects of stimulation to be isolated. These experiments indicated that common peroneal stimulation on average increased peak hip flexion during the swing phase of walking by 21.1° (236%) and peak knee flexion by 14.4° (56%). Additionally, use of the stimulation decreased the swing phase RMS motor current by 228 mA (15%) at the hip motors and 734 mA (38%) at the knee motors, indicating improved kinematics were achieved with reduced effort from the exoskeleton. Walking with the exoskeleton did not have a significant effect on modified Ashworth scores, indicating the common peroneal stimulation has only acute effects on suppressing extensor tone and aiding flexion. This preliminary data indicates that such supplemental stimulation may be used to improve the quality of movement provided by exoskeletons for persons with severe extensor spasticity in the lower limb.

Supplemental Stimulation Improves Swing Phase Kinematics During Exoskeleton Assisted Gait of SCI Subjects With Severe Muscle Spasticity

PubMed Central

Ekelem, Andrew; Goldfarb, Michael

2018-01-01

Spasticity is a common comorbidity associated with spinal cord injury (SCI). Robotic exoskeletons have recently emerged to facilitate legged mobility in people with motor complete SCI. Involuntary muscle activity attributed to spasticity, however, can prevent such individuals from using an exoskeleton. Specifically, although most exoskeleton technologies can accommodate low to moderate spasticity, the presence of moderate to severe spasticity can significantly impair gait kinematics when using an exoskeleton. In an effort to potentially enable individuals with moderate to severe spasticity to use exoskeletons more effectively, this study investigates the use of common peroneal stimulation in conjunction with exoskeleton gait assistance. The electrical stimulation is timed with the exoskeleton swing phase, and is intended to acutely suppress extensor spasticity through recruitment of the flexion withdrawal reflex (i.e., while the stimulation is activated) to enable improved exoskeletal walking. In order to examine the potential efficacy of this approach, two SCI subjects with severe extensor spasticity (i.e., modified Ashworth ratings of three to four) walked in an exoskeleton with and without supplemental stimulation while knee and hip motion was measured during swing phase. Stimulation was alternated on and off every ten steps to eliminate transient therapeutic effects, enabling the acute effects of stimulation to be isolated. These experiments indicated that common peroneal stimulation on average increased peak hip flexion during the swing phase of walking by 21.1° (236%) and peak knee flexion by 14.4° (56%). Additionally, use of the stimulation decreased the swing phase RMS motor current by 228 mA (15%) at the hip motors and 734 mA (38%) at the knee motors, indicating improved kinematics were achieved with reduced effort from the exoskeleton. Walking with the exoskeleton did not have a significant effect on modified Ashworth scores, indicating the common peroneal stimulation has only acute effects on suppressing extensor tone and aiding flexion. This preliminary data indicates that such supplemental stimulation may be used to improve the quality of movement provided by exoskeletons for persons with severe extensor spasticity in the lower limb. PMID:29910710

Development of future indications for BOTOX.

PubMed

Brin, Mitchell F

2009-10-01

Since the late 1970s, local injections of BoNT have provided clinical benefit for patients with inappropriately contracting muscles with or without pain or sensory disturbance. Marketing authorization for some BoNTs, depending on country, include core indications of dystonia (blepharospasm and cervical dystonia), large muscle spastic disorders (not yet approved in the United States, e.g., adult post-stroke spasticity and equinus foot deformity), hyperhidrosis and aesthetic. Subsequent development has extended to selected conditions characterized by recurrent or chronic pain (migraine headache), and urologic indications (neurogenic/idiopathic overactive bladder; prostate hyperplasia), with multiple additional opportunities available. Portfolio management requires a careful individual opportunity assessment of scientific and technical aspects (basic science foundation, potential to treat unmet medical need, product-specific risk in specific populations, therapeutic margin/safety profile, and probability of successful registration pathway). This article describes ongoing development targets for BOTOX.

The use of botulinum toxin and epidural analgesia for the treatment of spasticity and pain in a patient with maple syrup urine disease

PubMed Central

Kaki, Abdullah M.; Arab, Abeer A.

2012-01-01

A 7-year-old boy, weighing 18 kg, was diagnosed with maple syrup urine disease (MSUD). He suffered from spasticity of the lower limbs and pain that did not respond to oral medications. Injections of botulinum toxin A (BTX-A) at 10 sites and epidural analgesia with 0.125% bupivacaine were used to treat spasticity with good results. We conclude that BTX-A combined with epidural analgesia may be a useful treatment option for incapacitating, painful spasticity related to MSUD. This treatment modality allowed a comprehensive rehabilitation program to be completed and it lasted longer than 9 months. PMID:22754448

Effects of intrathecal baclofen therapy on motor and cognitive functions in a rat model of cerebral palsy.

PubMed

Nomura, Sadahiro; Kagawa, Yoshiteru; Kida, Hiroyuki; Maruta, Yuichi; Imoto, Hirochika; Fujii, Masami; Suzuki, Michiyasu

2012-02-01

Cerebral palsy (CP) arises in the early stages of brain development and manifests as spastic paresis that is often associated with cognitive dysfunction. Available CP treatments are aimed at the management of spasticity and include botulinum toxin administration, selective dorsal rhizotomy, and intrathecal baclofen (ITB). In this study, the authors investigated whether the management of spasticity with ITB therapy affected motor function and whether the release of spasticity was associated with an improvement in intellectual function. Newborn Sprague-Dawley rats were divided into the following groups: control, CP model, and CP model with ITB therapy. For the CP model, postnatal Day 7 (P7) rats were exposed to hypoxic conditions (8% O(2)) for 150 minutes after ligation of the right common carotid artery. In the groups receiving ITB therapy, a spinal catheter was connected to an osmotic pump filled with baclofen and placed in the spinal subarachnoid space on P21 in the early group and on P35 in the late group. A daily dose of 12 μg of baclofen was continuously administered until P49, resulting in 28 days of therapy in the early group and 14 days in the late group. Changes in spasticity in the CP and CP with ITB treatment groups were confirmed by assessing the motor evoked potential in the plantar muscle. In the CP group, the time required to complete a beam-walking test on P49 was significantly longer than that in the control and ITB treatment groups (4.15 ± 0.60 vs 2.10 ± 0.18 and 2.22 ± 0.22 seconds, respectively). Results of the beam-walking test are expressed as the mean ± SD. Radial arm maze performance on P49 indicated that spatial reference memory had significantly deteriorated in the CP group compared with controls (2.33 ± 0.87 vs 0.86 ± 0.90 points); moreover, working memory was also negatively affected by CP (0.78 ± 1.09 vs 0.14 ± 0.38 points). Results of the memory tests are expressed as the mean ± SE. These memory functions did not recover after ITB treatment. Management of spasticity with ITB therapy improved the walking ability in the rat CP model. Intrathecal baclofen therapy-which reduces harmful sensory and motor stimulations caused by spasticity to more optimal levels-contributed to motor function recovery; however, it had no effect on intellectual recovery as assessed by memory performance in the rat CP model.

Selective dorsal rhizotomy for the treatment of severe spastic cerebral palsy: efficacy and therapeutic durability in GMFCS grade IV and V children.

PubMed

D'Aquino, Daniel; Moussa, Ahmad A; Ammar, Amr; Ingale, Harshal; Vloeberghs, Michael

2018-04-01

Selective dorsal rhizotomy (SDR) has been established as an effective surgical treatment for spastic diplegia. The applicability of SDR to the full spectrum of spastic cerebral palsy and the durability of its therapeutic effects remain under investigation. There are currently limited data in the literature regarding efficacy and outcomes following SDR in Gross Motor Function Classification System (GMFCS) IV and V patients. Intrathecal baclofen has traditionally been the surgical treatment of choice for these patients. When utilised primarily as a treatment for the relief of spasticity, it is proposed that SDR represents a rational and effective treatment option for this patient group. We report our outcomes of SDR performed on children with severe cerebral palsy (GMFCS grade IV and V). The commensurate improvement in upper as well as lower limb spasticity is highlighted. Apparent benefit to urological function following SDR in this patient group is also discussed. A retrospective review of prospectively collected data for 54 paediatric patients with severe cerebral palsy (GMFCS IV-V) who received SDR plus specialised physiotherapy. Mean age was 10.2 years (range, 3.0-19.5). SDR guided by electrophysiological monitoring was performed by a single experienced neurosurgeon. All subjects received equivalent physiotherapy. The primary outcome measure was change to the degree of spasticity following SDR. Spasticity of upper and lower limb muscle groups were quantified and standardised using the Ashworth score. Measures were collected at baseline and at 2-, 8- and 14-month postoperative intervals. In addition, baseline and 6-month postoperative urological function was also evaluated as a secondary outcome measure. The mean lower limb Ashworth score at baseline was 3.2 (range, 0-4). Following SDR, significant reduction in lower limb spasticity scores was observed at 2 months and maintained at 8 and 14 months postoperatively (Wilcoxon rank, p < 0.001). The mean reduction at 2, 8 and 14 months was 3.0, 3.2 and 3.2 points respectively (range, 1-4), confirming a sustained improvement of spasticity over a 1-year period of follow-up. Significant reduction in upper limb spasticity scores following SDR was also observed (mean, 2.9; Wilcoxon rank, p < 0.001). Overall, the improvement to upper and lower limb tone following SDR-generally to post-treatment Ashworth scores of 0-was clinically and statistically significant in GMFCS IV and V patients. Urological assessment identified pre-existing bladder dysfunction in 70% and 90% of GMFCS IV and V patients respectively. Following SDR, improvement in urinary continence was observed in 71% of affected GMFCS IV and 42.8% of GMFCS V patients. No serious postoperative complications were identified. We conclude that SDR is safe and-in combination with physiotherapy-effectively reduces spasticity in GMFCS grade IV and V patients. Our series suggests that spastic quadriplegia is effectively managed with significant improvements in upper limb spasticity that are commensurate with those observed in lower limb muscle groups. These gains are furthermore sustained more than a year postoperatively. In light of these findings, we propose that SDR constitutes an effective treatment option for GMFCS IV and V patients and a rational alternative to intrathecal baclofen.

Availability of latissimus dorsi minigraft in smile reconstruction for incomplete facial paralysis: quantitative assessment based on the optical flow method.

PubMed

Takushima, Akihiko; Harii, Kiyonori; Okazaki, Mutsumi; Ohura, Norihiko; Asato, Hirotaka

2009-04-01

Acute unilateral facial paralysis, such as occurs in Bell palsy and Hunt syndrome, is mostly a benign neurologic morbidity that resolves within a few months. However, incomplete or misdirected return of the affected nerve results in unfavorable cosmetic sequelae in some patients. Although functional problems such as lagophthalmos are rare, facial asymmetry on smiling resulting from a lack of mimetic muscle strength in the cheek is often psychologically annoying to patients. To obtain a more natural smile, the authors transfer latissimus dorsi muscle to assist in cheek movement. A small, thinned muscle (mini-latissimus dorsi) is sufficient for transplant in this situation. In this study, 96 patients with incomplete facial paralysis who underwent mini-latissimus dorsi transfer were examined. In this series, along with evaluation using the grading scale used in previous reports, preoperative and postoperative videos of 30 patients were analyzed for quantitative assessment using newly developed computer software. Temporary deterioration of paralysis was recognized in three cases but did not last more than a few months. Signs of transferred muscle contraction were recorded after 4 to 12 months among 91 patients. No apparent clinical signs of contraction were recognized in one patient, and four patients could not be followed postoperatively. The synchronized ratio of vertical movement and the symmetrical ratio of horizontal movement both in the cheek and in the lower lip between healthy and paralyzed sides among 30 patients were statistically improved. Statistical analysis using newly developed computer software revealed that a more symmetrical smile can be achieved by muscle transfer among patients with incomplete facial paralysis. Mini-latissimus dorsi transfer can avoid postoperative muscle bulkiness of the cheek and can achieve more natural cheek movement.

Prognosis of West Nile virus associated acute flaccid paralysis: a case series.

PubMed

Johnstone, Jennie; Hanna, Steven E; Nicolle, Lindsay E; Drebot, Michael A; Neupane, Binod; Mahony, James B; Loeb, Mark B

2011-08-19

Little is known about the long-term health related quality of life outcomes in patients with West Nile virus associated acute flaccid paralysis. We describe the quality of life scores of seven patients with acute flaccid paralysis who presented to hospital between 2003 and 2006, and were followed for up to two years. Between 2003 and 2006, 157 symptomatic patients with West Nile virus were enrolled in a longitudinal cohort study of West Nile virus in Canada. Seven patients (4%) had acute flaccid paralysis. The first patient was a 55-year-old man who presented with left upper extremity weakness. The second patient was a 54-year-old man who presented with bilateral upper extremity weakness. The third patient was a 66-year-old woman who developed bilateral upper and lower extremity weakness. The fourth patient was a 67-year-old man who presented with right lower extremity weakness. The fifth patient was a 60-year-old woman who developed bilateral lower extremity weakness. The sixth patient was a 71-year-old man with a history of Parkinson's disease and acute onset bilateral lower extremity weakness. The seventh patient was a 52-year-old man who presented with right lower extremity weakness. All were Caucasian. Patients were followed for a mean of 1.1 years. At the end of follow-up the mean score on the Physical Component Summary of the Short-Form 36 scale had only slightly increased to 39. In contrast, mean score on the Mental Component Summary of the Short-Form 36 scale at the end of follow-up had normalized to 50. Despite the poor physical prognosis for patients with acute flaccid paralysis, the mental health outcomes are generally favorable.

Facial Nerve Paralysis due to a Pleomorphic Adenoma with the Imaging Characteristics of a Facial Nerve Schwannoma

PubMed Central

Nader, Marc-Elie; Bell, Diana; Sturgis, Erich M.; Ginsberg, Lawrence E.; Gidley, Paul W.

2014-01-01

Background Facial nerve paralysis in a patient with a salivary gland mass usually denotes malignancy. However, facial paralysis can also be caused by benign salivary gland tumors. Methods We present a case of facial nerve paralysis due to a benign salivary gland tumor that had the imaging characteristics of an intraparotid facial nerve schwannoma. Results The patient presented to our clinic 4 years after the onset of facial nerve paralysis initially diagnosed as Bell palsy. Computed tomography demonstrated filling and erosion of the stylomastoid foramen with a mass on the facial nerve. Postoperative histopathology showed the presence of a pleomorphic adenoma. Facial paralysis was thought to be caused by extrinsic nerve compression. Conclusions This case illustrates the difficulty of accurate preoperative diagnosis of a parotid gland mass and reinforces the concept that facial nerve paralysis in the context of salivary gland tumors may not always indicate malignancy. PMID:25083397

Facial Nerve Paralysis due to a Pleomorphic Adenoma with the Imaging Characteristics of a Facial Nerve Schwannoma.

PubMed

Nader, Marc-Elie; Bell, Diana; Sturgis, Erich M; Ginsberg, Lawrence E; Gidley, Paul W

2014-08-01

Background Facial nerve paralysis in a patient with a salivary gland mass usually denotes malignancy. However, facial paralysis can also be caused by benign salivary gland tumors. Methods We present a case of facial nerve paralysis due to a benign salivary gland tumor that had the imaging characteristics of an intraparotid facial nerve schwannoma. Results The patient presented to our clinic 4 years after the onset of facial nerve paralysis initially diagnosed as Bell palsy. Computed tomography demonstrated filling and erosion of the stylomastoid foramen with a mass on the facial nerve. Postoperative histopathology showed the presence of a pleomorphic adenoma. Facial paralysis was thought to be caused by extrinsic nerve compression. Conclusions This case illustrates the difficulty of accurate preoperative diagnosis of a parotid gland mass and reinforces the concept that facial nerve paralysis in the context of salivary gland tumors may not always indicate malignancy.

Radiofrequency cordotomy for the relief of spasticity in decerebrate cats1

PubMed Central

Soriano, Daniel; Herman, Richard

1971-01-01

The effectiveness of radiofrequency (RF) cordotomy of segmental motoneurone pools of the lumbosacral cord in reducing spasticity of decerebrate cats is evaluated. The need for a new form of therapy for clinical spasticity is based upon the limitations of contemporary methods, including surgical and pharmacological techniques. In man, spasticity of spinal origin may be treated effectively by intrathecal administration of hyperbaric phenol solutions. The advantages and disadvantages are described. Difficulty in controlling the lesion is emphasized. Tension and EMG-length curves of the spastic triceps surae muscle in acute and chronic animals show that RF lesions (fixed amperage and duration) of the segmental motoneurone pools reduces myotatic reflex activity in accordance with the number of segments cordotomized. Clinical examination including cinematography and electromyography complement the physiological interpretation. RF lesions of the internuncial pool induce spontaneous EMG discharges. This finding is related to similar observations of EMG discharges and alterations in muscle tone after asphyxiation of the spinal cord. Images PMID:5287593

[Peripheral nerves phenolisation in spasticity management - forgotten art or interesting perspective?

PubMed

Kiwerski, Jerzy; Turżańska, Karolina; Pluta, Ryszard; Drelich, Małgorzata; Zarębska, Aneta; Jabłoński, Mirosław

Spasticity is one of the most common obstacles in the process of rehabilitation after permanent central nervous system injury. Statistics show that the problem affects more than 12 billion people all over the world. Treatment of spasticity is necessary for proper rehabilitation, prevention of secondary disability, pain management and also strongly influences quality of life. Nowadays the most common formula used for the treatment of local spasticity is injection of botulinum toxin. Years ago and also nowadays physicians all over the world successfully used phenol as the chemodenervating factor. This technique today almost forgot in Poland but still used in other countries. Peripheral nerves phenolisation had been used in STOCER (Konstancin) since 1965. The procedure was performed once in 59 patients, twice in 13 patients and three times in 7 patients. Evaluation was based on clinical presentation completed with patient self evaluation form. Good - 36 cases (45%), moderate - 34 cases (43%), bad - 9 cases (12%). Peripheral nerve phenolisation seems to be an interesting alternative to botulinum toxin in the management of spasticity.

Minimally invasive brow suspension for facial paralysis.

PubMed

Costantino, Peter D; Hiltzik, David H; Moche, Jason; Preminger, Aviva

2003-01-01

To report a new technique for unilateral brow suspension for facial paralysis that is minimally invasive, limits supraciliary scar formation, does not require specialized endoscopic equipment or expertise, and has proved to be equal to direct brow suspension in durability and symmetry. Retrospective survey of a case series of 23 patients between January 1997 and December 2000. Metropolitan tertiary care center. Patients with head and neck tumors and brow ptosis caused by facial nerve paralysis. The results of the procedure were determined using the following 3-tier rating system: outstanding (excellent elevation and symmetry); acceptable (good elevation and fair symmetry); and unacceptable (loss of elevation). The results were considered outstanding in 12 patients, acceptable in 9 patients, and unacceptable in only 1 patient. One patient developed a hematoma, and 1 patient required a secondary adjustment. The technique has proved to be superior to standard brow suspension procedures with regard to scar formation and equal with respect to facial symmetry and suspension. These results have caused us to abandon direct brow suspension and to use this minimally invasive method in all cases of brow ptosis due to facial paralysis.

Hyperthyroidism with Periodic Paralysis

PubMed Central

Feldman, David L.; Goldberg, W. M.

1969-01-01

Hyperthyroidism may be associated with hypokalemic periodic paralysis. Two cases are presented demonstrating intermittent attacks of flaccid paralysis associated with clinical symptoms, signs and laboratory findings of hyperthyroidism. During an attack, one patient had a serum potassium of 2.1 mEq. per litre. Various factors such as trauma, exposure to cold, excessive carbohydrate ingestion and certain medications have been stated to precipitate an episode of paralysis. Attacks may range from mild weakness to generalized flaccid paralysis with loss of deep tendon reflexes. Several reported patients have died owing to cardiac arrest or respiratory paralysis. During attacks, the serum potassium is usually in the range of 2.2 to 3.2 mEq. per litre. It is postulated that a metabolic abnormality affecting the muscle-cell membrane can occur in the hyperthyroid state resulting in a shift of potassium to the intracellular position, thus producing a situation of hyperpolarization of the muscle-cell membrane which in turn alters the muscle contractibility. The importance of recognizing the unusual association of hypokalemic periodic paralysis with hyperthyroidism is stressed because, with successful treatment of the hyperthyroidism, the episodes of paralysis disappear. PMID:5353150

[Vocal cord paralysis associated with tracheal intubation: incidence, risk analysis, and classification of severity].

PubMed

Kikura, Mutsuhito; Suzuki, Yuji; Itagaki, Taiga; Sato, Tsunehisa; Nishino, Junko

2015-01-01

Vocal cord paralysis after tracheal intubation is rare. It causes severe hoarseness and aspiration, and delays recovery and discharge. Arytenoid cartilage dislocation and recurrent nerve paralysis are main causes of vocal cord paralysis. Physical stimulation of the tracheal tube as well as patient and surgical characteristics also contribute. Vocal cord paralysis occurs in 1 (0.07%) of 1,500 general surgery patients and on the left side in 70% of cases. It is associated with surgery/anesthesia time (two-fold, 3-6 hours; 15-fold, over 6 hours), age (three-fold, over 50 years), and diabetes mellitus or hypertension (two-fold). Symptoms resolve in 2-3 months. In adult cardiovascular surgery, vocal cord paralysis occurs in 1 (0.7-2%) of 50-100 cardiac surgery patients and 1 (8.6-32%) of 3-10 thoracic aortic surgery patients. In pediatric cardiac surgery, vocal cord paralysis occurs in 1 (0.1-0.5%) of 200-1,000 patients. We classified the severity of vocal cord paralysis as I, severe hoarseness; II, aspiration or dysphagia; and III, bilateral vocal cord paralysis, aspiration pneumonia, or the need for tracheal re-intubation or tracheotomy. We discuss the importance of informed consent for the patient and family.

[Aetiology and treatment of vocal fold paralysis: retrospective study of 108 patients].

PubMed

Bothe, Carolina; López, Montserrat; Quer, Miquel; León, Xavier; García, Jacinto; Lop, Joan

2014-01-01

To review the aetiology and treatment of laryngeal paralysis diagnosed at our hospital and to describe the available therapeutic options. Retrospective review of medical records of 108 patients diagnosed with unilateral and bilateral vocal fold paralysis between 2000 and 2012, identifying the cause of paralysis and its treatment. Of the 108 cases analysed, 70% had unilateral vocal fold immobility and 30% bilateral immobility. The most frequent aetiology in both cases was trauma (represented mainly by surgical injury), followed by tumours in unilateral paralysis and medical causes in bilateral paralysis. Half of the patients with unilateral paralysis (38) were treated surgically, with medialization thyroplasty. In bilateral vocal fold immobility, the treatment consisted of tracheostomy in patients with threatened airway (40%). We planned to widen the air passage in 9 patients (27%), performing cordectomy in most of them. The aetiology observed in our patients is similar to that described in the literature. In cases of unilateral vocal fold paralysis, we believe thyroplasty is the procedure of choice. In bilateral paralysis, it is possible to perform cordectomy in selected patients once the airway has been secured. Copyright © 2013 Elsevier España, S.L. y Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

Inter-Rater Reliability of the Modified Ashworth Scale and Modified Modified Ashworth Scale in Assessing Poststroke Elbow Flexor Spasticity

ERIC Educational Resources Information Center

Kaya, Taciser; Goksel Karatepe, Altinay; Gunaydin, Rezzan; Koc, Aysegul; Altundal Ercan, Ulku

2011-01-01

The Modified Ashworth Scale (MAS) is commonly used in clinical practice for grading spasticity. However, it was modified recently by omitting grade "1+" of the MAS and redefining grade "2". The aim of this study was to investigate the inter-rater reliability of MAS and modified MAS (MMAS) for the assessment of poststroke elbow flexor spasticity.…

Effects of Prolonged Standing on Gait in Children with Spastic Cerebral Palsy

ERIC Educational Resources Information Center

Salem, Yasser; Lovelace-Chandler, Venita; Zabel, Reta J.; McMillan, Amy Gross

2010-01-01

The purpose of this study was to determine the effects of prolonged standing on gait characteristics in children with spastic cerebral palsy. Six children with spastic cerebral palsy participated in this study with an average age of 6.5 years (SD = 2.5, range = 4.0-9.8 years). A reverse baseline design (A-B-A) was used over a 9-week period. During…

Alu elements mediate large SPG11 gene rearrangements: further spatacsin mutations.

PubMed

Conceição Pereira, Maria; Loureiro, José Leal; Pinto-Basto, Jorge; Brandão, Eva; Margarida Lopes, Ana; Neves, Georgina; Dias, Pureza; Geraldes, Ruth; Martins, Isabel Pavão; Cruz, Vitor Tedim; Kamsteeg, Erik-Jan; Brunner, Han G; Coutinho, Paula; Sequeiros, Jorge; Alonso, Isabel

2012-01-01

Hereditary spastic paraplegias compose a group of neurodegenerative disorders with a large clinical and genetic heterogeneity. Among the autosomal recessive forms, spastic paraplegia type 11 is the most common. To better understand the spastic paraplegia type 11 mutation spectrum, we studied a group of 54 patients with hereditary spastic paraplegia. Mutation screening was performed by PCR amplification of SPG11 coding regions and intron boundaries, followed by sequencing. For the detection of large gene rearrangements, we performed multiplex ligation-dependent probe amplification. We report 13 families with spastic paraplegia type 11 carrying either novel or previously identified mutations. We describe a complex entire SPG11 rearrangement and show that large gene rearrangements are frequent among patients with spastic paraplegia type 11. Moreover, we mapped the deletion breakpoints of three different large SPG11 deletions and provide evidence for Alu microhomology-mediated exon deletion. Our analysis shows that the high number of repeated elements in SPG11 together with the presence of recombination hotspots and the high intrinsic instability of the 15q locus all contribute toward making this genomic region more prone to large gene rearrangements. These findings enlarge the amount of data relating repeated elements with neurodegenerative disorders and highlight their importance in human disease and genome evolution.

Clinical Usefulness of the Pendulum Test Using a NK Table to Measure the Spasticity of Patients with Brain Lesions

PubMed Central

Kim, Yong-Wook

2013-01-01

. [Purpose] The purpose of the present study was to investigate the clinical usefulness (reliability and validity) of the pendulum test using a Noland-Kuckhoff (NK) table with an attached electrogoniometer to measure the spasticity of patients with brain lesions. [Subjects] The subjects were 31 patients with stroke or traumatic brain injury. [Methods] The intraclass correlation coefficient (ICC) was used to verify the test–retest reliability of spasticity measures obtained using the pendulum test. Pearson's product correlation coefficient was used to examine the validity of the pendulum test using the amplitude of the patellar tendon reflex (PTR) test, an objective and quantitative measure of spasticity. [Results] The test–retest reliability was high, reflecting a significant correlation between the test and the retest (ICCs = 0.95–0.97). A significant negative correlation was found between the amplitude of the PTR test and the four variables measured in the pendulum test (r = −0.77– −0.85). [Conclusion] The pendulum test using a NK table is an objective measure of spasticity and can be used in the clinical setting in place of more expensive and complicated equipment. Further studies are needed to investigate the therapeutic effect of this method on spasticity. PMID:24259775

Clinical usefulness of the pendulum test using a NK table to measure the spasticity of patients with brain lesions.

PubMed

Kim, Yong-Wook

2013-10-01

. [Purpose] The purpose of the present study was to investigate the clinical usefulness (reliability and validity) of the pendulum test using a Noland-Kuckhoff (NK) table with an attached electrogoniometer to measure the spasticity of patients with brain lesions. [Subjects] The subjects were 31 patients with stroke or traumatic brain injury. [Methods] The intraclass correlation coefficient (ICC) was used to verify the test-retest reliability of spasticity measures obtained using the pendulum test. Pearson's product correlation coefficient was used to examine the validity of the pendulum test using the amplitude of the patellar tendon reflex (PTR) test, an objective and quantitative measure of spasticity. [Results] The test-retest reliability was high, reflecting a significant correlation between the test and the retest (ICCs = 0.95-0.97). A significant negative correlation was found between the amplitude of the PTR test and the four variables measured in the pendulum test (r = -0.77- -0.85). [Conclusion] The pendulum test using a NK table is an objective measure of spasticity and can be used in the clinical setting in place of more expensive and complicated equipment. Further studies are needed to investigate the therapeutic effect of this method on spasticity.

[Experience in using xeomin in the treatment of arm and hand spasticity in the early rehabilitation phase of stroke].

PubMed

Kostenko, E V; Petrova, L V; Ganzhula, P A; Lisenker, L N; Otcheskaia, O V; Khozova, A A; Boĭko, A N

2012-01-01

To reduce arm and hand spasticity, 28 patients in the early rehabilitation phase of ischemic hemisphere stroke received injections of the botulinum toxin A preparation xeomin in the content of complex rehabilitation programs. The following muscles: m. biceps brachii, m. flexor digitorum profundus, m. flexor digitorum superficialis, m. flexor carpi ulnaris, m. flexor carpi radialis were injected according to standard scheme. The total dose of drug was 200U in moderate (2-3 scores on the Ashworth scale) and 300U in marked (3-4 scores on the Ashworth scale) spasticity. Efficacy and safety of treatment was assessed at baseline and 2, 4, 8, 12, 16 weeks after injections. Xeomin significantly (p<0.05) reduced muscle tonus in patients with post-stroke spasticity of different severity. Clinical effect was seen 2 weeks after injection, it reached maximum at week 4 and then slowly decreased to week 16. The improved functional activity of the paretic arm (due to patient's and caregiver's reports) remained for to 12 weeks. The treatment was most effective in the group of patients with moderate spasticity. The correlation analysis confirmed that the severity of spasticity increased with the disease duration that reduced rehabilitation efficiency. The treatment with xeomin was safe, no serious side-effects were found.

Who benefits most from THC:CBD spray? Learning from clinical experience.

PubMed

Koehler, Jürgen

2014-01-01

Patients with multiple sclerosis (MS) represent a diverse and heterogeneous population varying in terms of disease type, its severity and variable progression/time-course, and with regard to the wide range of presenting symptoms. Consequently, detailed experience with individual patients is important to provide examples of therapy to specific patient types. In this article, real-life data from clinical practice showing specific aspects relating to use of 9-delta-tetrahydocannabinol and cannabidiol (THC:CBD) oromucosal spray (Sativex®) in patients with moderate to severe spasticity resistant to usual therapy will be presented. Three common clinical scenarios will be considered: MS patients with resistance to usual spasticity therapies; patients with impairment in MS spasticity symptoms; MS patients with relevant impairment in quality of life/activities of daily living (QoL/ADL). These case reports highlight the diverse nature of the MS spasticity population and they show the possible usefulness of THC:CBD oromucosal spray in individual patients with moderate to severe spasticity resistant to existing therapies, within the frame of use approved after large clinical trial results. Perhaps the most important finding is the possibility of obtaining relevant improvements in QoL/ADL in some patients with resistant MS spasticity, allowing them to engage back in physical and social activities. © 2014 S. Karger AG, Basel.

Facial Paralysis in Patients With Hemifacial Microsomia: Frequency, Distribution, and Association With Other OMENS Abnormalities.

PubMed

Li, Qiang; Zhou, Xu; Wang, Yue; Qian, Jin; Zhang, Qingguo

2018-05-15

Although facial paralysis is a fundamental feature of hemifacial microsomia, the frequency and distribution of nerve abnormalities in patients with hemifacial microsomia remain unclear. In this study, the authors classified 1125 cases with microtia (including 339 patients with hemifacial microsomia and 786 with isolated microtia) according to Orbital Distortion Mandibular Hypoplasia Ear Anomaly Nerve Involvement Soft Tissue Dependency (OMENS) scheme. Then, the authors performed an independent analysis to describe the distribution feature of nerve abnormalities and reveal the possible relationships between facial paralysis and the other 4 fundamental features in the OMENS system. Results revealed that facial paralysis is present 23.9% of patients with hemifacial microsomia. The frontal-temporal branch is the most vulnerable branch in the total 1125 cases with microtia. The occurrence of facial paralysis is positively correlated with mandibular hypoplasia and soft tissue deficiency both in the total 1125 cases and the hemifacial microsomia patients. Orbital asymmetry is related to facial paralysis only in the total microtia cases, and ear deformity is related to facial paralysis only in hemifacial microsomia patients. No significant association was found between the severity of facial paralysis and any of the other 4 OMENS anomalies. These data suggest that the occurrence of facial paralysis may be associated with other OMENS abnormalities. The presence of serious mandibular hypoplasia or soft tissue deficiency should alert the clinician to a high possibility but not a high severity of facial paralysis.

Guillain Barre syndrome: the leading cause of acute flaccid paralysis in Hazara division.

PubMed

Anis-ur-Rehman; Idris, Muhammad; Elahi, Manzoor; Jamshed; Arif, Adeel

2007-01-01

Acute flaccid paralysis (AFP) can be caused by a number of conditions. A common preventable cause is poliomyelitis which is still being reported in Pakistan, Guillain Barre Syndrome (GBS), also known as Acute Inflammatory Demyelinating Polyneuropathy, is another common cause of acute flaccid paralysis. It is important to recognize GBS in childhood as parents consider all acute flaccid paralysis to be due to poliomyelitis. The present study was designed to know the frequency of different causes of acute flaccid paralysis in Hazara division. This is a retrospective analysis of cases of acute flaccid paralysis reported from various districts of Hazara division during the period January 2003 to December 2004. Acute flaccid paralysis was diagnosed clinically through history and clinical examination. The underlying cause of acute flaccid paralysis was investigated by appropriate laboratory tests, such as serum electrolytes, cerebrospinal fluid analysis, electromyogram, nerve conduction study and stool culture for polio virus and other enteroviruses. Diagnosis of Poliomyelitis was confirmed by stool testing for poliovirus. 74 patients presented with AFP during the study period. 36 were male and 38 were female. Guillain Barre syndrome and enteroviral encephalopathy were the two leading causes of acute flaccid paralysis. Majority of the cases were reported from Mansehra district. Children of age groups 12 to 24 months and > 96 months constituted the majority (20% each). Guillian Barre syndrome was the leading cause of acute flaccid paralysis reported from various parts of Hazara division.

Intrathecal baclofen for treating spasticity in children with cerebral palsy.

PubMed

Hasnat, Monika J; Rice, James E

2015-11-13

Cerebral palsy is a disorder of movement and posture arising from a non-progressive lesion in the developing brain. Spasticity, a disorder of increased muscle tone, is the most common motor difficulty and is associated with activity limitation to varying degrees in mobility and self care.Oral baclofen, a gamma-aminobutyric acid (GABA) agonist, has been used in oral form to treat spasticity for some time, but it has a variable effect on spasticity and the dose is limited by the unwanted effect of excessive sedation. Intrathecal baclofen produces higher local concentrations in cerebrospinal fluid at a fraction of the equivalent oral dose and avoids this excessive sedation. To determine whether intrathecal baclofen is an effective treatment for spasticity in children with cerebral palsy. We searched the CENTRAL, MEDLINE, EMBASE and CINAHL databases, handsearched recent conference proceedings, and communicated with researchers in the field and pharmaceutical and drug delivery system companies. We included studies which compared the effect of intrathecal baclofen treatment on spasticity, gross motor function or other areas of function with controls. Two authors selected studies, two authors extracted data and two authors assessed the methodological quality of included studies. Six studies met the inclusion criteria. The data obtained were unsuitable for the conduct of a meta-analysis; we have completed a qualitative summary.All studies were found to have high or unclear risk of bias in some aspects of their methodology.Five of the six studies reported data collected in the randomised controlled phase of the study. A sixth study did not report sufficient results to determine the effect of intrathecal baclofen versus placebo. Of these five studies, four were conducted using lumbar puncture or other short-term means of delivering intrathecal baclofen. One study assessed the effectiveness of implantable intrathecal baclofen pumps over six months.The four short-term studies demonstrated that intrathecal baclofen therapy reduces spasticity in children with cerebral palsy. However, two of these studies utilised inappropriate techniques for statistical analysis of results. The single longer-term study demonstrated minimal reduction in spasticity with the use of intrathecal baclofen therapy.One of the short-term studies and the longer term study showed improvement in comfort and ease of care. The longer term study found a small improvement in gross motor function and also in some domains of health-related quality of life.Some caution is required in interpreting the findings of the all the studies in the review due to methodological issues. In particular, there was a high risk of bias in the methodology of the longer term study due to the lack of placebo use in the control group and the absence of blinding to the intervention after randomisation for both participants and investigators. There is some limited short-term evidence that intrathecal baclofen is an effective therapy for reducing spasticity in children with cerebral palsy. The effect of intrathecal baclofen on long-term spasticity outcomes is less certain.The validity of the evidence for the effectiveness of intrathecal baclofen in treating spasticity in children with cerebral palsy from the studies in the review is constrained by the small sample sizes of the studies and methodological issues in some studies.Spasticity is a impairment in the domain of body structure and function. Consideration must also be given to the broader context in determining whether intrathecal baclofen therapy is effective. The aim of therapy may be, for example, to improve gross motor function, to increase participation at a social role level, to improve comfort, to improve the ease of care by others or to improve the overall quality of life of the individual. Intrathecal baclofen may improve gross motor function in children with cerebral palsy, but more reliable evidence is needed to determine this.There is some evidence that intrathecal baclofen improves ease of care and the comfort and quality of life of the individuals receiving it, but again small sample sizes and methodological issues in the studies mean that these results should be interpreted with caution.Further evidence of the effectiveness of intrathecal baclofen for treating spasticity, increasing gross motor function and improving comfort, ease of care and quality of life is needed from other investigators in order to validate these results.The short duration of the controlled studies included in this review did not allow for the exploration of questions regarding whether the subsequent need for orthopaedic surgery in children receiving intrathecal baclofen therapy is altered, or the safety and the economic implications of intrathecal baclofen treatment when long-term therapy is administered via an implanted device. Controlled studies are not the most appropriate study design to address these questions, cohort studies may be more appropriate.

Vocal cord paralysis after aortic surgery.

PubMed

DiLisio, Ralph P; Mazzeffi, Michael A; Bodian, Carol A; Fischer, Gregory W

2013-06-01

The purpose of this study was to investigate variables associated with vocal cord paralysis during complex aortic procedures. A retrospective review. A tertiary care center. Four hundred ninety-eight patients who underwent aortic surgery between 2002 and 2007. Two groups were studied. Group A patients had procedures only involving their aortic root and/or ascending aorta. Group B patients had procedures only involving their aortic arch and/or descending aorta. The incidence of vocal cord paralysis was higher (7.26% v 0.8%) in group B patients (p < 0.0001). Increasing the duration of cardiopulmonary bypass time was associated with an increased risk of vocal cord paralysis and death in both groups A and B (p = 0.0002 and 0.002, respectively). Additionally, within group B, descending aneurysms emerged as an independent risk factor associated with vocal cord paralysis (p = 0.03). Length of stay was statistically significantly longer among group A patients who suffered vocal cord paralysis (p = 0.017) and trended toward significance in group B patients who suffered vocal cord paralysis (p = 0.059). The association between tracheostomy and vocal cord paralysis among group A patients reached statistical significance (p = 0.007) and trended toward significance in group B patients (p = 0.057). Increasing duration of cardiopulmonary bypass time was associated with a higher risk of vocal cord paralysis in patients undergoing aortic surgery. Additionally, within group B patients, descending aortic aneurysm was an independent risk factor associated with vocal cord paralysis. Most importantly, vocal cord paralysis appeared to have an association between an increased length of stay and tracheostomy among a select group of patients undergoing aortic surgery. Copyright © 2013 Elsevier Inc. All rights reserved.

The relationship between the Southern Oscillation Index, rainfall and the occurrence of canine tick paralysis, feline tick paralysis and canine parvovirus in Australia.

PubMed

Rika-Heke, Tamara; Kelman, Mark; Ward, Michael P

2015-07-01

The aim of this study was to describe the association between climate, weather and the occurrence of canine tick paralysis, feline tick paralysis and canine parvovirus in Australia. The Southern Oscillation Index (SOI) and monthly average rainfall (mm) data were used as indices for climate and weather, respectively. Case data were extracted from a voluntary national companion animal disease surveillance resource. Climate and weather data were obtained from the Australian Government Bureau of Meteorology. During the 4-year study period (January 2010-December 2013), a total of 4742 canine parvovirus cases and 8417 tick paralysis cases were reported. No significant (P ≥ 0.05) correlations were found between the SOI and parvovirus, canine tick paralysis or feline tick paralysis. A significant (P < 0.05) positive cross-correlation was found between parvovirus occurrence and rainfall in the same month (0.28), and significant negative cross-correlations (-0.26 to -0.36) between parvovirus occurrence and rainfall 4-6 months previously. Significant (P < 0.05) negative cross-correlations (-0.34 to -0.39) were found between canine tick paralysis occurrence and rainfall 1-3 months previously, and significant positive cross-correlations (0.29-0.47) between canine tick paralysis occurrence and rainfall 7-10 months previously. Significant positive cross-correlations (0.37-0.68) were found between cases of feline tick paralysis and rainfall 6-10 months previously. These findings may offer a useful tool for the management and prevention of tick paralysis and canine parvovirus, by providing an evidence base supporting the recommendations of veterinarians to clients thus reducing the impact of these diseases. Copyright © 2015 Elsevier Ltd. All rights reserved.

Endoscopic laterofixation in bilateral vocal cords paralysis in children.

PubMed

Lidia, Zawadzka-Glos; Magdalena, Frackiewicz; Mieczyslaw, Chmielik

2010-06-01

Vocal cords paralysis is the second most frequent cause of laryngeal stridor in children. Symptoms of congenital vocal cords paralysis can occur shortly after birth or later. Vocal cords paralysis can be unilateral or bilateral. Symptoms of unilateral paralysis include hoarse weeping or stridor during a deep inhalation. In children unilateral vocal cords paralysis often retreats spontaneously or can be completely compensated. Children with bilateral vocal cords paralysis present mainly breathing disorders while phonation is normal. Symptoms are different, starting from complete occlusion of respiratory tracts and ending on small symptoms connected with the lack of effort tolerance. When symptoms are severe, patients from this group require a tracheotomy. The lack of restoration of normal function of vocal cords or lack of complete compensation and maintenance of symptoms are an indication for surgical treatment. The aim of this study is to present results of the treatment of bilateral vocal cords paralysis in children using the endoscopic method of laterofixation of vocal cords. In the Pediatric ENT Department between 1998 and 2009 sixty four children with dyspnoea and/or phonation disorders caused by vocal cords paralysis were treated. In ten cases laterofixation of vocal cords was performed, in most cases with good result. In this article the authors present the method of endoscopic laterofixation and achieved results. Endoscopic laterofixation of vocal cords in children is a safe and an easy method of surgical treatment of bilateral vocal cords paralysis. This method can be used as a first and often as a one stage treatment of vocal cords paralysis. In some cases this procedure is insufficient and has to be completed with other methods. Copyright (c) 2010 Elsevier Ireland Ltd. All rights reserved.

Review of devices used in neuromuscular electrical stimulation for stroke rehabilitation.

PubMed

Takeda, Kotaro; Tanino, Genichi; Miyasaka, Hiroyuki

2017-01-01

Neuromuscular electrical stimulation (NMES), specifically functional electrical stimulation (FES) that compensates for voluntary motion, and therapeutic electrical stimulation (TES) aimed at muscle strengthening and recovery from paralysis are widely used in stroke rehabilitation. The electrical stimulation of muscle contraction should be synchronized with intended motion to restore paralysis. Therefore, NMES devices, which monitor electromyogram (EMG) or electroencephalogram (EEG) changes with motor intention and use them as a trigger, have been developed. Devices that modify the current intensity of NMES, based on EMG or EEG, have also been proposed. Given the diversity in devices and stimulation methods of NMES, the aim of the current review was to introduce some commercial FES and TES devices and application methods, which depend on the condition of the patient with stroke, including the degree of paralysis.

A single trial of transcutaneous electrical nerve stimulation (TENS) improves spasticity and balance in patients with chronic stroke.

PubMed

Cho, Hwi-young; In, Tae Sung; Cho, Ki Hun; Song, Chang Ho

2013-03-01

Spasticity management is pivotal for achieving functional recovery of stroke patients. The purpose of this study was to investigate the effects of a single trial of transcutaneous electrical nerve stimulation (TENS) on spasticity and balance in chronic stroke patients. Forty-two chronic stroke patients were randomly allocated into the TENS (n = 22) or the placebo-TENS (n = 20) group. TENS stimulation was applied to the gastrocnemius for 60 min at 100 Hz, 200 µs with 2 to 3 times the sensory threshold (the minimal threshold in detecting electrical stimulation for subjects) after received physical therapy for 30 min. In the placebo-TENS group, electrodes were placed but no electrical stimulation was administered. For measuring spasticity, the resistance encountered during passive muscle stretching of ankle joint was assessed using the Modified Ashworth Scale, and the Hand held dynamometer was used to assess the resistive force caused by spasticity. Balance ability was measured using a force platform that measures postural sway generated by postural imbalance. The TENS group showed a significantly greater reduction in spasticity of the gastrocnemius, compared to the placebo-TENS group (p < 0.05). TENS resulted in greater balance ability improvements, especially during the eyes closed condition (p < 0.05). However, these effects returned to baseline values within one day. This study shows that TENS provides an immediately effective means of reducing spasticity and of improving balance in chronic stroke patients. The present data may be useful to establish the standard parameters for TENS application in the clinical setting of stroke.

Long-Term Results of Microsurgical Selective Tibial Neurotomy for Spastic Foot : Comparison of Adult and Child

PubMed Central

Kim, Jong-Hoon; Lee, Jae-Il; Kim, Min-Su

2010-01-01

Objective Selective neurotomy is generally a safe, effective, and long-lasting treatment for patients with spastic equinovarus foot deformity. We retrospectively analyzed the results of microsurgical selective tibial neurotomy (STN) for spastic feet in adults and children. Methods A neurosurgeon selected 32 patients with 45 spastic feet (adults : 13, children : 32) to undergo microsurgical STN between October 1998 and September 2007. A physician of rehabilitation assessed spasticity pre- and postoperatively, that was based on the Ashworth scale, ankle clonus, and the amplitude of ankle dorsiflexion. The mean postoperative follow-up period was 36.7 months in adults and 42.5 months in children. Results Spastic components of the feet were corrected immediately after surgery in both the adult and child groups. The mean Ashworth's grade changed from 3.6 ± 0.40 to 1.6 ± 0.70 in adults and from 3.7 ± 0.69 to 1.4 ± 0.49 in children. Mean ankle clonus decreased markedly, from 1.6 ± 0.79 to 0.3 ± 0.42 in adults and from 1.7 ± 0.65 to 0.3 ± 0.56 in children. The mean amplitude of ankle dorsiflexion was improved, but eight (adults: 4, children: 4) contracted feet needed complementary orthopedic correction for acceptable results. Conclusion STN can be effective in the long-term for improving lower limb function and reduction of equinovarus deformity. Our results demonstrate that STN might be an effective procedure for treating localized harmful spastic feet in adults and children. PMID:20461163

Brief report: Preliminary study on evaluation of spasticity in patients with brain lesions using mechanomyography.

PubMed

Jun, Sang Woo; Yong, Suk Joong; Jo, Min; Kim, Young Ho; Kim, Sung Hoon

2018-05-01

Electromyography and the modified Ashworth scale (MAS) are among the most effective methods for evaluating spasticity; however, these are often inappropriate for clinical use, owing to the complicated procedure and subjective evaluation outcomes. A passive stretch reflex test was performed on 10 subjects with brain lesions. Furthermore, mechanomyography and electromyography were conducted on the vastus lateralis muscle (agonist) and semitendinosus muscle (antagonist) of the subjects with brain lesions. A new equation to define the normalized hull area; that is, the mechanomyography (MMG) ratio, was applied to quantify the triaxial motion of the agonist muscle versus antagonist muscles, reflecting the electromyographic firing point of the spastic muscle. The MMG ratio was proposed, which statistically distinguishes the spastic and normal muscles (p = 0.01) and exhibits a concordance with the conventional mean MAS (r = 0.69, p = 0.01). Patients suspected to have spasticity of 0 to 1+ grade can be quantitatively evaluated using the normalized hull area ratio, which can be used as an additional clinical indicator for spasticity evaluation. The study was conducted in conformity with the Helsinki declaration principles and performed in the Korea Centers for Disease Control and Prevention, Ministry of Health and Welfare (Republic of Korea); 2010; KCT0002385; A new approach of spasticity measurement using mechanomyography in patients with brain lesions: A randomized pilot study for a parallel randomized controlled trial; October 8, 2015 [Cited on July 21, 2017]; [1 screen]. Available from: https://cris.nih.go.kr/cris/search/search_result_st01_en.jsp?seq=7805<ype=&rtype=. Copyright © 2018. Published by Elsevier Ltd.

Measurement properties of a new wireless electrogoniometer for quantifying spasticity during the pendulum test in ARSACS patients.

PubMed

Bui, Hung Tien; Gagnon, Cynthia; Audet, Olivier; Mathieu, Jean; Leone, Mario

2017-04-15

Autosomal recessive spastic ataxia of Charlevoix/Saguenay (ARSACS) is a neuromuscular disorder that induces spasticity in lower limbs. The Wartenberg pendulum test is a classical method of assessing lower limb spasticity based on the dynamics of the pendular leg motion. However, in its original form, this test only provides subjective results and do not allow accurate assessment of spasticity. Thirteen ARSACS patients were assessed using a new wireless electrogoniometer to measure spasticity by quantifying oscillation amplitudes and relaxation indices during the Wartenburg pendulum test. The validity of the instrument was evaluated by comparing its measurements to a known precise goniometer whereas discriminant validity was evaluated by comparing healthy participants and ARSACS patients. Reliability was measured using intraclass correlation (ICC) between pendulum test scores obtained at different moments in time. Data from different tests show that the proposed device is accurate (standard error of measurement of 0.0005°), discriminates healthy and ARSACS patients (most variables have p=0.00) and provides repeatable results (significant ICC usually higher than 0.64 and p<0.05). The proposed tool allows the clinician to analyze pendulum oscillation amplitudes and ratios and thus, provide an index of spasticity for the patients affected by ARSACS. This is important as the original procedure is only evaluated visually and the progression cannot be detected until the condition changes drastically. Thus, the system proposed meets the requirements of being useful, precise and user-friendly in the evaluation of patients in a research as well as a clinical environment. Copyright © 2017 Elsevier B.V. All rights reserved.

Increased lower limb muscle coactivation reduces gait performance and increases metabolic cost in patients with hereditary spastic paraparesis.

PubMed

Rinaldi, Martina; Ranavolo, Alberto; Conforto, Silvia; Martino, Giovanni; Draicchio, Francesco; Conte, Carmela; Varrecchia, Tiwana; Bini, Fabiano; Casali, Carlo; Pierelli, Francesco; Serrao, Mariano

2017-10-01

The aim of this study was to investigate the lower limb muscle coactivation and its relationship with muscles spasticity, gait performance, and metabolic cost in patients with hereditary spastic paraparesis. Kinematic, kinetic, electromyographic and energetic parameters of 23 patients and 23 controls were evaluated by computerized gait analysis system. We computed ankle and knee antagonist muscle coactivation indexes throughout the gait cycle and during the subphases of gait. Energy consumption and energy recovery were measured as well. In addition to the correlation analysis between coactivation indexes and clinical variables, correlations between coactivation indexes and time-distance, kinematic, kinetic, and energetic parameters were estimated. Increased coactivity indexes of both knee and ankle muscles throughout the gait cycle and during the subphases of gait were observed in patients compared with controls. Energetic parameters were significantly higher in patients than in controls. Both knee and ankle muscle coactivation indexes were positively correlated with knee and ankle spasticity (Ashworth score), respectively. Knee and ankle muscle coactivation indexes were both positively correlated with energy consumption and both negatively correlated with energy recovery. Positive correlations between the Ashworth score and lower limb muscle coactivation suggest that abnormal lower limb muscle coactivation in patients with hereditary spastic paraparesis reflects a primary deficit linked to lower limb spasticity. Furthermore, these abnormalities influence the energetic mechanisms during walking. Identifying excessive muscle coactivation may be helpful in individuating the rehabilitative treatments and designing specific orthosis to restrain spasticity. Copyright © 2017 Elsevier Ltd. All rights reserved.

Comprehensive quantification of the spastic catch in children with cerebral palsy.

PubMed

Lynn, Bar-On; Erwin, Aertbeliën; Guy, Molenaers; Herman, Bruyninckx; Davide, Monari; Ellen, Jaspers; Anne, Cazaerck; Kaat, Desloovere

2013-01-01

In clinical settings, the spastic catch is judged subjectively. This study assessed the psychometric properties of objective parameters that define and quantify the severity of the spastic catch in children with cerebral palsy (CP). A convenience sample of children with spastic CP (N=46; age range: 4-16 years) underwent objective spasticity assessments. High velocity, passive stretches were applied to the gastrocnemius (GAS) and medial hamstrings (MEH). Muscle activity was measured with surface electromyography (sEMG), joint angle characteristics using inertial sensors and reactive torque using a force sensor. To test reliability, a group of 12 children were retested after an average of 13 ± 9 days. The angle of spastic catch (AOC) was estimated by three biomechanical definitions: joint angle at (1) maximum angular deceleration; (2) maximum change in torque; and (3) minimum power. Each definition was checked for reliability and validity. Construct and clinical validity were evaluated by correlating each AOC definition to the averaged root mean square envelope of EMG (RMS-EMG) and the Modified Tardieu Scale (MTS). Severity categories were created based on selected parameters to establish face validity. All definitions showed moderate to high reliability. Significant correlations were found between AOC3 and the MTS of both muscles and the RMS-EMG of the MEH, though coefficients were only weak. AOC3 further distinguished between mild, moderate and severe catches. Objective parameters can define and quantify the severity of the spastic catch in children with CP. However, a comprehensive understanding requires the integration of both biomechanical and RMS-EMG data. Copyright © 2012 Elsevier Ltd. All rights reserved.

Effect of repetitive transcranial magnetic stimulation on reducing spasticity in patients suffering from HTLV-1-associated myelopathy.

PubMed

Amiri, Mostafa; Nafissi, Shahriar; Jamal-Omidi, Shirin; Amiri, Motahareh; Fatehi, Farzad

2014-12-01

Human T-lymphotropic virus type 1 has been implicated in human T-lymphotropic virus type 1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). Regarding its endemicity in Iran and the role of repetitive transcranial magnetic stimulation in reducing spasticity, we decided to evaluate the efficacy of repetitive transcranial magnetic stimulation in reducing spasticity (as primary outcome) and pain, muscle power, and quality of life (as secondary outcomes) in patients suffering from HAM/TSP. In this pretest-posttest study, nine definite patients with HAM/TSP (according to WHO guidelines) were recruited. All patients underwent five consecutive daily sessions of active repetitive transcranial magnetic stimulation (each session consisting of 20 trains of 10 pulses at 5 Hz and an intensity of 90% of resting motor threshold for the biceps brachii muscle). Main outcome measures including spasticity (by modified Ashworth scale), pain (by visual analog scale), muscle power, and quality of life (by SF 36) were measured before the study and days 5, 7, 30 after the termination of the sessions. Seven (77.8%) females and 2 (22.2%) males were recruited with the mean age of 52 ± 12.67 years, and the mean duration of the disease was 5 ± 3.94. Comparison of the repeated measures showed a statistically significant decrease in pain and spasticity in lower limbs. The decrement in spasticity was persistent even 30 days after the intervention; however, the pain reduction was seen only 5 days after the procedure. No change in quality of life, and muscle power was detected. It seems that repetitive transcranial magnetic stimulation could decrease spasticity and pain in patients with HAM/TSP, and this effect could persistently continue by 1 month, but it did not influence patients' muscle power and quality of life, and it could be used as an adjuvant therapy in patients suffering from human T-lymphotropic virus type 1-associated HAM/TSP.

Effects of low-level laser therapy (LLLT 808 nm) on lower limb spastic muscle activity in chronic stroke patients.

PubMed

das Neves, Marcele Florêncio; Dos Reis, Mariana César Ribeiro; de Andrade, Eliana Aparecida Fonseca; Lima, Fernanda Pupio Silva; Nicolau, Renata Amadei; Arisawa, Emília Ângela Loschiavo; Andrade, Adriano Oliveira; Lima, Mário Oliveira

2016-09-01

A cerebrovascular accident (CVA) may affect basic motor functions, including spasticity that may be present in the upper extremity and/or the lower extremity, post-stroke. Spasticity causes pain, muscle force reduction, and decreases the time to onset of muscle fatigue. Several therapeutic resources have been employed to treat CVA to promote functional recovery. The clinical use of low-level laser therapy (LLLT) for rehabilitation of muscular disorders has provided better muscle responses. Thus, the aim of this study was to evaluate the effect of the application of LLLT in spastic muscles in patients with spasticity post-CVA. A double-blind clinical trial was conducted with 15 volunteer stroke patients who presented with post-stroke spasticity. Both males and females were treated; the average age was 51.5 ± 11.8 years old; the participants entered the study ranging from 11 to 48 months post-stroke onset. The patients participated in three consecutive phases (control, placebo, and real LLLT), in which all tests of isometric endurance of their hemiparetic lower limb were performed. LLLT (diode laser, 100 mW 808 nm, beam spot area 0.0314 cm(2), 127.39 J/cm(2)/point, 40 s) was applied before isometric endurance. After the real LLLT intervention, we observed significant reduction in the visual analogue scale for pain intensity (p = 0.0038), increased time to onset of muscle fatigue (p = 0.0063), and increased torque peak (p = 0.0076), but no significant change in the root mean square (RMS) value (electric signal in the motor unit during contraction, as obtained with surface electromyography). Our results suggest that the application of LLLT may contribute to increased recruitment of muscle fibers and, hence, to increase the onset time of the spastic muscle fatigue, reducing pain intensity in stroke patients with spasticity, as has been observed in healthy subjects and athletes.

Goals Set by Patients Using the ICF Model before Receiving Botulinum Injections and Their Relation to Spasticity Distribution

PubMed Central

Choi, Kevin; Peters, Jaclyn; Tri, Andrew; Chapman, Elizabeth; Sasaki, Ayako; Ismail, Farooq; Boulias, Chris; Reid, Shannon

2017-01-01

Purpose: Goal Attainment Scaling (GAS) is used to assess functional gains in response to treatment. Specific characteristics of the functional goals set by individuals receiving botulinum toxin type A (BoNTA) injections for spasticity management are unknown. The primary objectives of this study were to describe the characteristics of the goals set by patients before receiving BoNTA injections using the International Classification of Functioning, Disability and Health (ICF) and to determine whether the pattern of spasticity distribution affected the goals set. Methods: A cross-sectional retrospective chart review was carried out in an outpatient spasticity-management clinic in Toronto. A total of 176 patients with a variety of neurological lesions attended the clinic to receive BoNTA injections and completed GAS from December 2012 to December 2013. The main outcome measures were the characteristics of the goals set by the participants on the basis of ICF categories (body functions and structures, activity and participation) and the spasticity distribution using Modified Ashworth Scale scores. Results: Of the patients, 73% set activity and participation goals, and 27% set body functions and structures goals (p<0.05). In the activity and participation category, 30% of patients set moving and walking goals, 28% set self-care and dressing goals, and 12% set changing and maintaining body position goals. In the body functions and structures category, 18% set neuromuscular and movement-related goals, and 8% set pain goals. The ICF goal categories were not related to the patterns of spasticity (upper limb vs. lower limb or unilateral vs. bilateral spasticity) or type of upper motor neuron (UMN) lesion (p>0.05). Conclusion: Our results show that patients receiving BoNTA treatment set a higher percentage of activity and participation goals than body functions and structures goals. Goal classification was not affected by type of spasticity distribution or type of UMN disorder. PMID:28539691

Genetic and phenotypic characterization of complex hereditary spastic paraplegia

PubMed Central

Kara, Eleanna; Tucci, Arianna; Manzoni, Claudia; Lynch, David S.; Elpidorou, Marilena; Bettencourt, Conceicao; Chelban, Viorica; Manole, Andreea; Hamed, Sherifa A.; Haridy, Nourelhoda A.; Federoff, Monica; Preza, Elisavet; Hughes, Deborah; Pittman, Alan; Jaunmuktane, Zane; Brandner, Sebastian; Xiromerisiou, Georgia; Wiethoff, Sarah; Schottlaender, Lucia; Proukakis, Christos; Morris, Huw; Warner, Tom; Bhatia, Kailash P.; Korlipara, L.V. Prasad; Singleton, Andrew B.; Hardy, John; Wood, Nicholas W.; Lewis, Patrick A.

2016-01-01

Abstract The hereditary spastic paraplegias are a heterogeneous group of degenerative disorders that are clinically classified as either pure with predominant lower limb spasticity, or complex where spastic paraplegia is complicated with additional neurological features, and are inherited in autosomal dominant, autosomal recessive or X-linked patterns. Genetic defects have been identified in over 40 different genes, with more than 70 loci in total. Complex recessive spastic paraplegias have in the past been frequently associated with mutations in SPG11 (spatacsin), ZFYVE26/SPG15 , SPG7 (paraplegin) and a handful of other rare genes, but many cases remain genetically undefined. The overlap with other neurodegenerative disorders has been implied in a small number of reports, but not in larger disease series. This deficiency has been largely due to the lack of suitable high throughput techniques to investigate the genetic basis of disease, but the recent availability of next generation sequencing can facilitate the identification of disease-causing mutations even in extremely heterogeneous disorders. We investigated a series of 97 index cases with complex spastic paraplegia referred to a tertiary referral neurology centre in London for diagnosis or management. The mean age of onset was 16 years (range 3 to 39). The SPG11 gene was first analysed, revealing homozygous or compound heterozygous mutations in 30/97 (30.9%) of probands, the largest SPG11 series reported to date, and by far the most common cause of complex spastic paraplegia in the UK, with severe and progressive clinical features and other neurological manifestations, linked with magnetic resonance imaging defects. Given the high frequency of SPG11 mutations, we studied the autophagic response to starvation in eight affected SPG11 cases and control fibroblast cell lines, but in our restricted study we did not observe correlations between disease status and autophagic or lysosomal markers. In the remaining cases, next generation sequencing was carried out revealing variants in a number of other known complex spastic paraplegia genes, including five in SPG7 (5/97), four in FA2H (also known as SPG35 ) (4/97) and two in ZFYVE26 / SPG15 . Variants were identified in genes usually associated with pure spastic paraplegia and also in the Parkinson’s disease-associated gene ATP13A2 , neuronal ceroid lipofuscinosis gene TPP1 and the hereditary motor and sensory neuropathy DNMT1 gene, highlighting the genetic heterogeneity of spastic paraplegia. No plausible genetic cause was identified in 51% of probands, likely indicating the existence of as yet unidentified genes. PMID:27217339

The pharmacological management of post-stroke muscle spasticity.

PubMed

Bakheit, Abdel Magid O

2012-12-01

Muscle hypertonia following upper motor neurone lesions (referred to here as 'spasticity') is a common problem in patients with neurological disease, and its management is one of the major challenges in clinical practice. Understanding the pathogenesis and clinical course of spasticity is essential for the effective management of this condition. The hypertonia initially results from increased excitability of the alpha motor neurones due to an imbalance between the excitatory and inhibitory influences of the vestibulospinal and reticulospinal tracts. This is the 'neural component' of muscle hypertonia. However, usually within 3-4 weeks, changes in the structure and mechanical properties of the paralysed muscles and the effect of thixotropy also contribute to the hypertonia. The selection of the optimal treatment option is often influenced by whether the neural or the non-neural component is more pronounced. Muscle spasticity often interferes with motor function or causes distressing symptoms, such as painful muscle spasms. If untreated, spasticity may also lead to soft tissue shortening (fixed contractures). However, spasticity can also be beneficial to patients. For example, despite severe leg muscle weakness, most hemiplegic patients are able to walk because the spasticity of the extensor muscles braces the lower limb in a rigid pillar. Other reported benefits of spasticity include the maintenance of muscle bulk and bone mineral density and possibly a reduced risk of lower limb deep vein thrombosis. Several factors, such as skin pressure sores, faecal impaction, urinary tract infections and stones in the urinary bladder, can aggravate muscle spasticity. These factors should always be looked for as their adequate treatment is often sufficient to reduce muscle tone without the need for specific antispasticity medication. Therefore, a careful evaluation of the patient's symptoms and their impact on function, and the setting of clear and realistic therapy goals are important prerequisites to treatment. The best treatment outcomes are usually achieved when pharmacological and non-pharmacological treatment modalities are used in tandem. Different drugs are available for the management of spasticity, including oral muscle relaxants, anticonvulsant drugs, intrathecal baclofen, cannabis extract, phenol and alcohol (for peripheral nerve blocks) and botulinum toxin injections. Similarly, there is a range of non-pharmacological methods of treatment, e.g. regular muscle stretching, the use of splints and orthoses, electrical stimulation, etc. Although these are not discussed here, this should not detract from the importance of combining them with antispasticity drugs in order to maximize the clinical benefit of treatment.

SPG11 Presenting with Tremor

PubMed Central

Schneider, Susanne A.; Mummery, Catherine J.; Mehrabian, Mohadeseh; Houlden, Henry; Bain, Peter G.

2012-01-01

Background Hereditary spastic paraplegias (HSPs) are a clinically and genetically heterogeneous group of neurological diseases, which typically present with progressive lower extremity weakness and spasticity causing progressive walking difficulties. Complicating neurological or extraneurological features may be present. Case Report We describe a 19-year-old male who was referred because of an action tremor of the hands; he later developed walking difficulties. Callosal atrophy was present on his cerebral magnetic resonance imaging scan, prompting genetic testing for SPG11, which revealed homozygous mutations. Discussion The clinical features, differential diagnosis and management of SPG11, the most common form of autosomal recessive complicated HSP with a thin corpus callosum are discussed. PMID:23439843

Epidemiology of Vocal Fold Paralyses After Total Thyroidectomy for Well-Differentiated Thyroid Cancer in Medicare Population

PubMed Central

Francis, David O.; Pearce, Elizabeth C.; Ni, Shenghua; Garrett, C. Gaelyn; Penson, David F.

2014-01-01

Objectives Population-level incidence of vocal fold paralysis after thyroidectomy for well-differentiated thyroid carcinoma (WDTC) is not known. This study aimed to measure longitudinal incidence of post-operative vocal fold paralyses and need for directed interventions in the Medicare population undergoing total thyroidectomy for WDTC. Study Design Retrospective Cohort Study Setting United States Population Subjects Medicare Beneficiaries Methods SEER-Medicare data (1991 – 2009) were used to identify beneficiaries who underwent total thyroidectomy for WDTC. Incident vocal fold paralyses and directed interventions were identified. Multivariate analyses were used to determine factors associated with odds of developing these surgical complications. Results Of 5,670 total thyroidectomies for WDTC, 9.5% were complicated by vocal fold paralysis [8.2% unilateral vocal fold paralysis (UVFP); 1.3% bilateral vocal fold paralysis (BVFP)]. Rate of paralyses decreased 5% annually from 1991 to 2009 (OR 0.95, 95% CI 0.93 – 0.97; p<0.001). Overall, 22% of patients with vocal fold paralysis required surgical intervention (UVFP 21%, BVFP 28%). Multivariate logistic regression revealed odds of post-thyroidectomy paralysis increased with each additional year of age, with non-Caucasian race, particular histologic types, advanced stage, and in particular registry regions. Conclusions Annual rates of post-thyroidectomy vocal fold paralyses are decreasing among Medicare beneficiaries with WDTC. High incidence in this aged population is likely due to a preponderance of temporary paralyses, which is supported by the need for directed intervention in less than a quarter of affected patients. Further population-based studies are needed to refine the population incidence and risk factors for paralyses in the aging population. PMID:24482349

The developing shoulder has a limited capacity to recover after a short duration of neonatal paralysis

PubMed Central

Potter, Ryan; Havlioglu, Necat; Thomopoulos, Stavros

2014-01-01

Mechanical stimuli are required for the proper development of the musculoskeletal system. Removal of muscle forces during fetal or early post-natal timepoints impairs the formation of bone, tendon, and their attachment (the enthesis). The goal of the current study was to examine the capacity of the shoulder to recover after a short duration of neonatal rotator cuff paralysis, a condition mimicking the clinical condition neonatal brachial plexus palsy. We asked if reapplication of muscle load to a transiently paralyzed muscle would allow for full recovery of tissue properties. CD-1 mice were injected with botulinum toxin A to paralyze the supraspinatus muscle from birth through 2 weeks and subsequently allowed to recover. The biomechanics of the enthesis was determined using tensile testing and the morphology of the shoulder joint was determined using micro computed tomography and histology. A recovery period of at least 10 weeks was required to achieve control properties, demonstrating a limited capacity of the shoulder to recover after only two weeks of muscle paralysis. Although care must be taken when extrapolating results from an animal model to the human condition, the results of the current study imply that treatment of neonatal brachial plexus palsy should be aggressive, as even short periods of paralysis could lead to long-term deficiencies in enthesis biomechanics and shoulder morphology. PMID:24831237

SPASTICITY—Its Nature and Treatment

PubMed Central

Levine, Milton G.; Kabat, Herman

1954-01-01

There are a number of physiological means of relaxing spasticity, including active resistive exercise, cold hydrotherapy, heat, electrical stimulation of antagonistic muscles, passive stretch in diagonal movement patterns, and the Von Bechterew reflex. Although none of them will cure spasticity, temporary relaxation may permit a patient to achieve better functioning of an affected joint. The choice of procedure will depend on the nature of the lesion and the muscular distribution of the spasticity. PMID:13150200

De novo REEP2 missense mutation in pure hereditary spastic paraplegia.

PubMed

Roda, Ricardo H; Schindler, Alice B; Blackstone, Craig

2017-05-01

Alterations in proteins that regulate endoplasmic reticulum morphology are common causes of hereditary spastic paraplegia (SPG1-78, plus others). Mutations in the REEP1 gene that encodes an endoplasmic reticulum-shaping protein are well-known causes of SPG31, a common autosomal dominant spastic paraplegia. A closely-related gene, REEP2, is mutated in SPG72, with both autosomal and recessive inheritances. Here, we report a patient with a pure hereditary spastic paraplegia due to a de novo missense mutation (c.119T > G, p.Met40Arg) in REEP2 at a highly-conserved residue very close to another known pathogenic missense change. This represents only the second autosomal dominant SPG72 missense mutation reported.

Paralysis

MedlinePlus

Paralysis is the loss of muscle function in part of your body. It happens when something goes ... way messages pass between your brain and muscles. Paralysis can be complete or partial. It can occur ...

Bilateral vocal cord paralysis secondary to head and neck surgery.

PubMed

Tekin, Muhammet; Acar, Gul Ozbilen; Kaytaz, Asim; Savrun, Feray Karaali; Çelik, Melek; Cam, Osman Halit

2012-01-01

Even endotracheal intubation could be considered safe in operations under general anesthesia; rarely, it could cause recurrent laryngeal nerve paralysis as a complication. As mentioned in the literature, as a possible reason for this, anterior branches of the recurrent laryngeal nerve in the larynx could suffer from compression between the posteromedial part of the thyroid cartilage and the cuff of the tube. In the literature, unilateral vocal cord paralysis due to endotracheal intubation occurs more frequently in comparison to bilateral vocal cord paralysis. These types of palsies usually totally improve in approximately 6 months. A patient who experienced bilateral vocal cord paralysis in the early postoperative period after undergoing an endotracheal intubation process for general anesthesia and primary partial lip resection and supraomohyoid neck dissection due to lower lip carcinoma is presented in our article. Although vocal cord paralysis occurring after head and neck surgery is first thought as a complication of the surgery, endotracheal intubation should be considered as a possible cause of this paralysis. In relation with this patient, causes, clinical symptoms, and treatment procedures of vocal cord paralysis due to endotracheal intubation are discussed under guidance of the literature.

[Effects of virtual reality training on limb movement in children with spastic diplegia cerebral palsy].

PubMed

Ren, Kai; Gong, Xiao-Ming; Zhang, Rong; Chen, Xiu-Hui

2016-10-01

To study the effects of virtual reality (VR) training on the gross motor function of the lower limb and the fine motor function of the upper limb in children with spastic diplegia cerebral palsy. Thirty-five children with spastic diplegia cerebral palsy were randomly assigned to VR training group (n=19) and conventional training group (n=16). The conventional training group received conventional physical therapy and occupational therapy for three months. The VR training group received VR training and occupational therapy for three months. Grip and visual-motor integration subtests in Peabody Developmental Motor Scales-2 were used to evaluate the fine movement in patients before and after treatment. The D and E domains of the 88-item version of the Gross Motor Function Measure (GMFM-88), Modified Ashworth Scale (MAS), and Berg Balance Scale (BBS) were used to evaluate the gross movement in patients before and after treatment. Before treatment, there were no significant differences in grip, visual-motor integration, fine motor development quotient, scores of D and E domains of GMFM-88, MAS score, or BBS score between the two groups (P>0.05). After treatment, all the indices were significantly improved in the VR training group compared with the conventional training group (P<0.05). VR training can effectively improve the gross motor function of the lower limb and the fine motor function of the upper limb in children with spastic diplegia cerebral palsy.

Brain magnetic resonance imaging findings and auditory brainstem response in a child with spastic paraplegia 2 due to a PLP1 splice site mutation.

PubMed

Kubota, Kazuo; Saito, Yoshiaki; Ohba, Chihiro; Saitsu, Hirotomo; Fukuyama, Tetsuhiro; Ishiyama, Akihiko; Saito, Takashi; Komaki, Hirofumi; Nakagawa, Eiji; Sugai, Kenji; Sasaki, Masayuki; Matsumoto, Naomichi

2015-01-01

A boy with spastic paraplegia type 2 (SPG2) due to a novel splice site mutation of PLP1 presented with progressive spasticity of lower limbs, which was first observed during late infancy, when he gained the ability to walk with support. His speech was slow and he had dysarthria. The patient showed mildly delayed intellectual development. Subtotal dysmyelination in the central nervous system was revealed, which was especially prominent in structures known to be myelinated during earlier period, whereas structures that are myelinated later were better myelinated. These findings on the brain magnetic resonance imaging were unusual for subjects with PLP1 mutations. Peaks I and II of the auditory brainstem response (ABR) were normally provoked, but peaks III-V were not clearly demarcated, similarly to the findings in Pelizaeus-Merzbacher disease. These findings of brain MRI and ABR may be characteristic for a subtype of SPG2 patients. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Plantarflexor weakness negatively impacts walking in persons with multiple sclerosis more than plantarflexor spasticity

PubMed Central

Wagner, Joanne M.; Kremer, Theodore R.; Van Dillen, Linda R.; Naismith, Robert T.

2014-01-01

Objective To determine if plantarflexor (PF) spasticity or ankle strength best predicts variance in walking capacity or self-perceived limitations in walking in persons with multiple sclerosis (pwMS), and if pwMS with PF spasticity are weaker and have greater walking dysfunction than pwMS without PF spasticity. Design Cross-sectional study. Setting University research laboratory. Participants Forty-two pwMS (age: 42.9 ± 10.1 years; Expanded Disability Status Scale (EDSS): median = 3.0, range = 0–6) and 14 adults without disability (WD) (age: 41.9 ± 10.1 years). Intervention Not applicable. Main Outcome Measures PF spasticity and dorsiflexion (DF) and PF maximum voluntary isometric torque (MVIT) were assessed using the Modified Ashworth Scale (MAS) and a computerized dynamometer, respectively. The Timed 25-Foot Walk Test (T25FWT) was the primary outcome measure of walking capacity. Secondary measures included the Six Minute Walk Test (6MWT) and 12-item Multiple Sclerosis Walking Scale (MSWS-12). Results PF strength was the most consistent predictor of the variance in walking capacity (T25FWT: R2 change = 0.23 to 0.29, p ≤ 0.001; 6MWT: R2 change = 0.12 to 0.29, p ≤ 0.012), and self-perceived limitations of walking (MSWS-12: R2 change = 0.04 to 0.14, p < 0.18). There were no significant differences (p > 0.05) between the pwMS with PF spasticity and pwMS without PF spasticity for any of the outcome measures. Conclusions Our study suggests a unique contribution of PF weakness to walking dysfunction in pwMS, and highlights the importance of evaluating PF strength in this clinical population. PMID:24582617

Delta-9-tetrahydrocannabinol/cannabidiol (Sativex®): a review of its use in patients with moderate to severe spasticity due to multiple sclerosis.

PubMed

Syed, Yahiya Y; McKeage, Kate; Scott, Lesley J

2014-04-01

Delta-9-tetrahydrocannabinol (THC)/cannabidiol (CBD) [Sativex®] is an oromucosal spray formulation that contains principally THC and CBD at an approximately 1:1 fixed ratio, derived from cloned Cannabis sativa L. plants. The main active substance, THC, acts as a partial agonist at human cannabinoid receptors (CB1 and CB2), and thus, may modulate the effects of excitatory (glutamate) and inhibitory (gamma-aminobutyric acid) neurotransmitters. THC/CBD is approved in a number of countries, including Germany and the UK, as an add-on treatment for symptom improvement in adult patients with moderate to severe spasticity due to multiple sclerosis who have not responded adequately to other anti-spasticity medication and who demonstrate clinically significant improvement in spasticity-related symptoms during an initial trial of therapy. In the largest multinational clinical trial that evaluated the approved THC/CBD regimen in this population, 12 weeks' double-blind treatment with THC/CBD significantly reduced spasticity severity (primary endpoint) compared with placebo in patients who achieved a clinically significant improvement in spasticity after 4 weeks' single-blind THC/CBD treatment, as assessed by a patient-rated numerical rating scale. A significantly greater proportion of THC/CBD than placebo recipients achieved a ≥ 30% reduction (a clinically relevant reduction) in spasticity severity. The efficacy of THC/CBD has been also shown in at least one everyday clinical practice study (MOVE 2). THC/CBD was generally well tolerated in clinical trials. Dizziness and fatigue were reported most frequently during the first 4 weeks of treatment and resolved within a few days even with continued treatment. Thus, add-on THC/CBD is a useful symptomatic treatment option for its approved indication.

Effects of repetitive peripheral magnetic stimulation on upper-limb spasticity and impairment in patients with spastic hemiparesis: a randomized, double-blind, sham-controlled study.

PubMed

Krewer, Carmen; Hartl, Sandra; Müller, Friedemann; Koenig, Eberhard

2014-06-01

To investigate short-term and long-term effects of repetitive peripheral magnetic stimulation (rpMS) on spasticity and motor function. Monocentric, randomized, double-blind, sham-controlled trial. Neurologic rehabilitation hospital. Patients (N=66) with severe hemiparesis and mild to moderate spasticity resulting from a stroke or a traumatic brain injury. The average time ± SD since injury for the intervention groups was 26 ± 71 weeks or 37 ± 82 weeks. rpMS for 20 minutes or sham stimulation with subsequent occupational therapy for 20 minutes, 2 times a day, over a 2-week period. Modified Tardieu Scale and Fugl-Meyer Assessment (arm score), assessed before therapy, at the end of the 2-week treatment period, and 2 weeks after study treatment. Additionally, the Tardieu Scale was assessed after the first and before the third therapy session to determine any short-term effects. Spasticity (Tardieu >0) was present in 83% of wrist flexors, 62% of elbow flexors, 44% of elbow extensors, and 10% of wrist extensors. Compared with the sham stimulation group, the rpMS group showed short-term effects on spasticity for wrist flexors (P=.048), and long-term effects for elbow extensors (P<.045). Arm motor function (rpMS group: median 5 [4-27]; sham group: median 4 [4-9]) did not significantly change over the study period in either group, whereas rpMS had a positive effect on sensory function. Therapy with rpMS increases sensory function in patients with severe limb paresis. The magnetic stimulation, however, has limited effect on spasticity and no effect on motor function. Copyright © 2014 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

Watsu approach for improving spasticity and ambulatory function in hemiparetic patients with stroke.

PubMed

Chon, Seung Chul; Oh, Duck Won; Shim, Jae Hun

2009-06-01

This study reports the effect of Watsu as rehabilitation method for hemiparetic patients with stroke. Watsu consisted of 40 treatment sessions for 8 weeks, delivered underwater or at water surface level, it applied in three patients. Outcome measures included tools for assessing spasticity and ambulatory function. All patients showed decreased scores in the TAS and RVGA after Watsu application. Watsu was helpful in controlling spasticity and improving ambulatory function of the patients with hemiparesis.

Tetraplegia Management Update.

PubMed

Fridén, Jan; Gohritz, Andreas

2015-12-01

Tetraplegia is a profound impairment of mobility manifesting as a paralysis of all 4 extremities owing to cervical spinal cord injury. The purpose of this article is to provide an update and analyze current management, treatment options, and outcomes of surgical reconstruction of arm and hand function. Surgical restoration of elbow and wrist extension or handgrip has tremendous potential to improve autonomy, mobility, and critical abilities, for example, eating, personal care, and self-catheterization and productive work in at least 70% of tetraplegic patients. Tendon and nerve transfers, tenodeses, and joint stabilizations reliably enable improved arm and hand usability, reduce muscle imbalance and pain in spasticity, and prevent joint contractures. One-stage combined procedures have proven considerable advantages over traditional multistage approaches. Immediate activation of transferred muscles reduces the risk of adhesions, facilitates relearning, avoids adverse effects of immobilization, and enhances functional recovery. Transfer of axillary, musculocutaneous, and radial nerve fascicles from above the spinal cord injury are effective and promising options to enhance motor outcome and sensory protection, especially in groups with limited resources. Improved communication between medical disciplines, therapists, patients, and their relatives should help that more individuals can benefit from these advances and could empower many thousands tetraplegic individuals "to take life into their own hands" and live more independently. Copyright © 2015 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

Therapeutics for multiple sclerosis symptoms.

PubMed

Ben-Zacharia, Aliza Bitton

2011-01-01

Symptoms management in multiple sclerosis is an integral part of its care. Accurate assessment and addressing the different symptoms provides increased quality of life among patients with multiple sclerosis. Multiple sclerosis symptoms may be identified as primary, secondary, or tertiary symptoms. Primary symptoms, such as weakness, sensory loss, and ataxia, are directly related to demyelination and axonal loss. Secondary symptoms, such as urinary tract infections as a result of urinary retention, are a result of the primary symptoms. Tertiary symptoms, such as reactive depression or social isolation, are a result of the social and psychological consequences of the disease. Common multiple sclerosis symptoms include fatigue and weakness; decreased balance, spasticity and gait problems; depression and cognitive issues; bladder, bowel, and sexual deficits; visual and sensory loss; and neuropathic pain. Less-common symptoms include dysarthria and dysphagia, vertigo, and tremors. Rare symptoms in multiple sclerosis include seizures, hearing loss, and paralysis. Symptom management includes nonpharmacological methods, such as rehabilitation and psychosocial support, and pharmacological methods, ie, medications and surgical procedures. The keys to symptom management are awareness, knowledge, and coordination of care. Symptoms have to be recognized and management needs to be individualized. Multiple sclerosis therapeutics include nonpharmacological strategies that consist of lifestyle modifications, rehabilitation, social support, counseling, and pharmacological agents or surgical procedures. The goal is vigilant management to improve quality of life and promote realistic expectations and hope. © 2011 Mount Sinai School of Medicine.

Effects of Dietary Vitamin E Supplementation in Bladder Function and Spasticity during Spinal Cord Injury

PubMed Central

Cordero, Kathia; Coronel, Gemma G.; Serrano-Illán, Miguel; Cruz-Bracero, Jennifer

2018-01-01

Traumatic spinal cord injury (SCI) results in debilitating autonomic dysfunctions, paralysis and significant sensorimotor impairments. A key component of SCI is the generation of free radicals that contributes to the high levels of oxidative stress observed. This study investigates whether dietary supplementation with the antioxidant vitamin E (alpha-tocopherol) improves functional recovery after SCI. Female adult Sprague-Dawley rats were fed either with a normal diet or a dietary regiment supplemented with vitamin E (51 IU/g) for eight weeks. The rats were subsequently exposed either to a contusive SCI or sham operation, and evaluated using standard functional behavior analysis. We report that the rats that consumed the vitamin E-enriched diet showed an accelerated bladder recovery and significant improvements in locomotor function relative to controls, as determined by residual volumes and Basso, Beatie, and Bresnaham BBB scores, respectively. Interestingly, the prophylactic dietary intervention did not preserve neurons in the ventral horn of injured rats, but it significantly increased the numbers of oligodendrocytes. Vitamin E supplementation attenuated the depression of the H-reflex (a typical functional consequence of SCI) while increasing the levels of supraspinal serotonin immunoreactivity. Our findings support the potential complementary use of vitamin E to ameliorate sensory and autonomic dysfunctions associated with spinal cord injury, and identified promising new cellular and functional targets of its neuroprotective effects. PMID:29495419

Use of Hybrid Assistive Limb (HAL®) for a postoperative patient with cerebral palsy: a case report.

PubMed

Mataki, Yuki; Kamada, Hiroshi; Mutsuzaki, Hirotaka; Shimizu, Yukiyo; Takeuchi, Ryoko; Mizukami, Masafumi; Yoshikawa, Kenichi; Takahashi, Kazushi; Matsuda, Mayumi; Iwasaki, Nobuaki; Kawamoto, Hiroaki; Wadano, Yasuyoshi; Sankai, Yoshiyuki; Yamazaki, Masashi

2018-03-27

The Hybrid Assistive Limb (HAL ® ) is an exoskeleton wearable robot suit that assists in voluntary control of knee and hip joint motion. There have been several studies on HAL intervention effects in stroke, spinal cord injury, and cerebral palsy. However, no study has investigated HAL intervention for patients with cerebral palsy after surgery. We report a case of using HAL in a postoperative patient with cerebral palsy. A 15-year-old boy was diagnosed with spastic diplegia cerebral palsy Gross Motor Function Classification System level IV, with knee flection contracture, equinus foot, and paralysis of the right upper extremity with adduction contracture. He underwent tendon lengthening of the bilateral hamstrings and Achilles tendons. Although the flexion contractures of the bilateral knees and equinus foot improved, muscle strength decreased after the soft tissue surgery. HAL intervention was performed twice during postoperative months 10 and 11. Walking speed, stride, and cadence were increased after HAL intervention. Post HAL intervention, extension angles of the knee in stance phase and hip in the pre-swing phase were improved. In the gait cycle, the proportion of terminal stance in the stance and swing phase was increased. Hybrid Assistive Limb intervention for postoperative patients with cerebral palsy whose muscle strength decreases can enhance improvement in walking ability. Further studies are needed to examine the safety and potential application of HAL in this setting.

Effects of severe spasticity treatment with intrathecal Baclofen in multiple sclerosis patients: Long term follow-up.

PubMed

Stampacchia, Giulia; Gerini, Adriana; Mazzoleni, Stefano

2016-04-06

Intrathecal Baclofen is available to treat severe generalized spasticity in Multiple Sclerosis (MS) unresponsive to oral drug delivery. The aims of this study were to investigate the effects and the drug dosage of intrathecal Baclofen in a selected population of MS patients, affected by severe spasticity at long term follow-up. A prospective cohort study of 14 MS patients is presented. Spasticity and pain were periodically assessed and the Baclofen dosage was adjusted. The initial Baclofen dosage was 136.2 ± 109.3 μg, then it was increased at 12 months to 228.6 ± 179.2 μg (p < 0.05). The subsequent dose adjustments did not result in significant changes up to 76 months. Spasticity on the lower limbs decreased significantly from pre-implantation assessment (median: 3.5, IQR: 3.0-4.0) to 12 months evaluation (median: 0.5, IQR: 0.0-2.0) (p < 0.001); no further decrease was observed after 24 months (median: 0.5, IQR: 0.0-1.5); when pain was present, it decreased. Some effects on cerebellar symptoms were observed. Botulinum toxin injections were used with intrathecal Baclofen therapy. A reduced spasticity and pain was observed after the intrathecal Baclofen infusion for at least 76 months. To obtain these results a dosage adjustment was needed only in the first year after the implantation.

A review of the properties and limitations of the Ashworth and modified Ashworth Scales as measures of spasticity.

PubMed

Pandyan, A D; Johnson, G R; Price, C I; Curless, R H; Barnes, M P; Rodgers, H

1999-10-01

The Ashworth Scale and the modified Ashworth Scale are the primary clinical measures of spast city. A prerequisite for using any scale is a knowledge of its characteristics and limitations, as these will play a part in analysing and interpreting the data. Despite the current emphasis on treating spasticity, clinicians rarely measure it. To determine the validity and the reliability of the Ashworth and modified Ashworth Scales. A theoretical analysis following a structured literature review (key words: Ashworth; Spasticity; Measurement) of 40 papers selected from the BIDS-EMBASE, First Search and Medline databases. The application of both scales would suggest that confusion exists on their characteristics and limitations as measures of spasticity. Resistance to passive movement is a complex measure that will be influenced by many factors, only one of which could be spasticity. The Ashworth Scale (AS) can be used as an ordinal level measure of resistance to passive movement, but not spasticity. The modified Ashworth Scale (MAS) will need to be treated as a nominal level measure of resistance to passive movement until the ambiguity between the '1' and '1+' grades is resolved. The reliability of the scales is better in the upper limb. The AS may be more reliable than the MAS. There is a need to standardize methods to apply these scales in clinical practice and research.

Enhancing patient-provider communication for long-term post-stroke spasticity management.

PubMed

Sunnerhagen, K S; Francisco, G E

2013-11-01

Stroke is a major public health concern, with estimated 16 million people worldwide experiencing first-time strokes each year, a number that is expected to rise. Two-thirds of those experiencing a stroke are younger than 70 years of age. Stroke is a leading cause of disability in adults as a result of major sequelae that include spasticity, cognitive impairment, paresis, and depression. Disabling spasticity, defined as spasticity severe enough to require intervention, occurs in 4% of stroke survivors within 1 year of first-time stroke. The aim of this report is to focus instead on a discussion of patient-provider communication, and its role in post-stroke spasticity (PSS) rehabilitation within the context of patient-centered health care. A discussion based on a review of the literature, mainly since 2000. Problems within communication are identified and suggestion to enhance communication are proposed thus improving patient-centered goal setting/goal achievement for the effective management of spasticity rehabilitation. These are as follows: (i) involving family members, (ii) educating patients and family members on stroke and rehabilitation, and (iii) establishing a common definition for long-term goals. Increased communication among physicians, patients, and payers may bridge some of the gaps and increase the effectiveness of PSS rehabilitation and management. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Transcutaneous electrical nerve stimulation for spasticity: A systematic review.

PubMed

Fernández-Tenorio, E; Serrano-Muñoz, D; Avendaño-Coy, J; Gómez-Soriano, J

2016-07-26

Although transcutaneous electrical nerve stimulation (TENS) has traditionally been used to treat pain, some studies have observed decreased spasticity after use of this technique. However, its use in clinical practice is still limited. Our purpose was twofold: to determine whether TENS is effective for treating spasticity or associated symptoms in patients with neurological involvement, and to determine which stimulation parameters exert the greatest effect on variables associated with spasticity. Two independent reviewers used PubMed, PEDro, and Cochrane databases to search for randomised clinical trials addressing TENS and spasticity published before 12 May 2015, and selected the articles that met the inclusion criteria. Of the initial 96 articles, 86 were excluded. The remaining 10 articles present results from 207 patients with a cerebrovascular accident, 84 with multiple sclerosis, and 39 with spinal cord lesions. In light of our results, we recommend TENS as a treatment for spasticity due to its low cost, ease of use, and absence of adverse reactions. However, the great variability in the types of stimulation used in the studies, and the differences in parameters and variables, make it difficult to assess and compare any results that might objectively determine the effectiveness of this technique and show how to optimise parameters. Copyright © 2016 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

The nature of arm movement in children with cerebral palsy when using computer-generated exercise games.

PubMed

Weightman, Andrew; Preston, Nick; Levesley, Martin; Bhakta, Bipin; Holt, Raymond; Mon-Williams, Mark

2014-05-01

To compare upper limb kinematics of children with spastic cerebral palsy (CP) using a passive rehabilitation joystick with those of adults and able-bodied children, to better understand the design requirements of computer-based rehabilitation devices. A blocked comparative study involving seven children with spastic CP, nine able-bodied adults and nine able-bodied children, using a joystick system to play a computer game whilst the kinematics of their upper limb were recorded. The translational kinematics of the joystick's end point and the participant's shoulder movement (protraction/retraction) and elbow rotational kinematics (flexion/extension) were analysed for each group. Children with spastic CP matched their able-bodied peers in the time taken to complete the computer task, but this was due to a failure to adhere to the task instructions of travelling along a prescribed straight line when moving between targets. The spastic CP group took longer to initiate the first movement, which showed jerkier trajectories and demonstrated qualitatively different movement patterns when using the joystick, with shoulder movements that were significantly of greater magnitude than the able-bodied participants. Children with spastic CP generate large shoulder and hence trunk movements when using a joystick to undertake computer-generated arm exercises. This finding has implications for the development and use of assistive technologies to encourage exercise and the instructions given to users of such systems. A kinematic analysis of upper limb function of children with CP when using joystick devices is presented. Children with CP may use upper body movements to compensate for limitations in voluntary shoulder and elbow movements when undertaking computer games designed to encourage the practice of arm movement. The design of rehabilitative computer exercise systems should consider movement of the torso/shoulder as it may have implications for the quality of therapy in the rehabilitation of the upper limb in children with CP.

Clinical practice guideline: Bell's palsy.

PubMed

Baugh, Reginald F; Basura, Gregory J; Ishii, Lisa E; Schwartz, Seth R; Drumheller, Caitlin Murray; Burkholder, Rebecca; Deckard, Nathan A; Dawson, Cindy; Driscoll, Colin; Gillespie, M Boyd; Gurgel, Richard K; Halperin, John; Khalid, Ayesha N; Kumar, Kaparaboyna Ashok; Micco, Alan; Munsell, Debra; Rosenbaum, Steven; Vaughan, William

2013-11-01

Bell's palsy, named after the Scottish anatomist, Sir Charles Bell, is the most common acute mono-neuropathy, or disorder affecting a single nerve, and is the most common diagnosis associated with facial nerve weakness/paralysis. Bell's palsy is a rapid unilateral facial nerve paresis (weakness) or paralysis (complete loss of movement) of unknown cause. The condition leads to the partial or complete inability to voluntarily move facial muscles on the affected side of the face. Although typically self-limited, the facial paresis/paralysis that occurs in Bell's palsy may cause significant temporary oral incompetence and an inability to close the eyelid, leading to potential eye injury. Additional long-term poor outcomes do occur and can be devastating to the patient. Treatments are generally designed to improve facial function and facilitate recovery. There are myriad treatment options for Bell's palsy, and some controversy exists regarding the effectiveness of several of these options, and there are consequent variations in care. In addition, numerous diagnostic tests available are used in the evaluation of patients with Bell's palsy. Many of these tests are of questionable benefit in Bell's palsy. Furthermore, while patients with Bell's palsy enter the health care system with facial paresis/paralysis as a primary complaint, not all patients with facial paresis/paralysis have Bell's palsy. It is a concern that patients with alternative underlying etiologies may be misdiagnosed or have unnecessary delay in diagnosis. All of these quality concerns provide an important opportunity for improvement in the diagnosis and management of patients with Bell's palsy. The primary purpose of this guideline is to improve the accuracy of diagnosis for Bell's palsy, to improve the quality of care and outcomes for patients with Bell's palsy, and to decrease harmful variations in the evaluation and management of Bell's palsy. This guideline addresses these needs by encouraging accurate and efficient diagnosis and treatment and, when applicable, facilitating patient follow-up to address the management of long-term sequelae or evaluation of new or worsening symptoms not indicative of Bell's palsy. The guideline is intended for all clinicians in any setting who are likely to diagnose and manage patients with Bell's palsy. The target population is inclusive of both adults and children presenting with Bell's palsy. ACTION STATEMENTS: The development group made a strong recommendation that (a) clinicians should assess the patient using history and physical examination to exclude identifiable causes of facial paresis or paralysis in patients presenting with acute-onset unilateral facial paresis or paralysis, (b) clinicians should prescribe oral steroids within 72 hours of symptom onset for Bell's palsy patients 16 years and older, (c) clinicians should not prescribe oral antiviral therapy alone for patients with new-onset Bell's palsy, and (d) clinicians should implement eye protection for Bell's palsy patients with impaired eye closure. The panel made recommendations that (a) clinicians should not obtain routine laboratory testing in patients with new-onset Bell's palsy, (b) clinicians should not routinely perform diagnostic imaging for patients with new-onset Bell's palsy, (c) clinicians should not perform electrodiagnostic testing in Bell's palsy patients with incomplete facial paralysis, and (d) clinicians should reassess or refer to a facial nerve specialist those Bell's palsy patients with (1) new or worsening neurologic findings at any point, (2) ocular symptoms developing at any point, or (3) incomplete facial recovery 3 months after initial symptom onset. The development group provided the following options: (a) clinicians may offer oral antiviral therapy in addition to oral steroids within 72 hours of symptom onset for patients with Bell's palsy, and (b) clinicians may offer electrodiagnostic testing to Bell's palsy patients with complete facial paralysis. The development group offered the following no recommendations: (a) no recommendation can be made regarding surgical decompression for patients with Bell's palsy, (b) no recommendation can be made regarding the effect of acupuncture in patients with Bell's palsy, and (c) no recommendation can be made regarding the effect of physical therapy in patients with Bell's palsy.

Review of devices used in neuromuscular electrical stimulation for stroke rehabilitation

PubMed Central

Takeda, Kotaro; Tanino, Genichi; Miyasaka, Hiroyuki

2017-01-01

Neuromuscular electrical stimulation (NMES), specifically functional electrical stimulation (FES) that compensates for voluntary motion, and therapeutic electrical stimulation (TES) aimed at muscle strengthening and recovery from paralysis are widely used in stroke rehabilitation. The electrical stimulation of muscle contraction should be synchronized with intended motion to restore paralysis. Therefore, NMES devices, which monitor electromyogram (EMG) or electroencephalogram (EEG) changes with motor intention and use them as a trigger, have been developed. Devices that modify the current intensity of NMES, based on EMG or EEG, have also been proposed. Given the diversity in devices and stimulation methods of NMES, the aim of the current review was to introduce some commercial FES and TES devices and application methods, which depend on the condition of the patient with stroke, including the degree of paralysis. PMID:28883745

Postoperative Paralysis From Thoracic Ossification of Posterior Longitudinal Ligament Surgery Risk Factor of Neurologic Injury: Nationwide Multiinstitution Survey.

PubMed

Ito, Zenya; Matsuyama, Yukihiro; Ando, Muneharu; Kawabata, Shigenori; Kanchiku, Tsukasa; Kida, Kazunobu; Fujiwara, Yasushi; Yamada, Kei; Yamamoto, Naoya; Kobayashi, Sho; Saito, Takanori; Wada, Kanichiro; Tadokoro, Nobuaki; Takahashi, Masato; Satomi, Kazuhiko; Shinomiya, Kenichi; Tani, Toshikazu

2016-10-01

Retrospective case-control study. The purpose of this study was to examine the factors of postoperative paralysis in patients who have undergone thoracic ossification of posterior longitudinal ligament (OPLL) surgery. A higher percentage of thoracic OPLL patients experience postoperative aggravation of paralysis than cervical OPLL patients, including patients that presented great difficulties in treatment. However, there were a few reports to prevent paralysis thoracic OPLL. The 156 patients who had received thoracic OPLL surgery were selected as the subjects of this study. The items for review were the duration of disease; the preoperative muscle strength (Muscle Manual Testing); OPLL levels (T1/2-4/5: high, T5/6-8/9: middle, and T9/10-11/12: low); the spinal canal occupancy ratio; the ratio of yellow ligament ossification as a complication; the ratio of transcranial-motor evoked potential (Tc-MEP) derivation; the preoperative/postoperative kyphotic angles in the thoracic vertebrae; the correction angle of kyphosis; the duration of surgery; and the amount of bleeding. The subjects were divided into two groups based on the absence or presence of postoperative paralysis to determine the factors of postoperative paralysis. Twenty-three patients (14.7%) exhibited postoperative paralysis. Multivariate analysis identified factors associated with postoperative paralysis: the duration of disease (odds ratio, OR = 3.3); the correction angle of kyphosis (OR = 2.4); and the ratio of Tc-MEP derivation (OR = 2.2). The risk factors of postoperative paralysis are a short duration of disease and a small correction angle of kyphosis. In addition, ratios of Tc-MEP derivation below 50% may anticipate paralysis. 4.

Experience with developmental facial paralysis: part II. Outcomes of reconstruction.

PubMed

Terzis, Julia K; Anesti, Katerina

2012-01-01

The purpose of this study was to document the 30-year experience of the authors' center in the management of developmental facial paralysis and to analyze the outcomes of microsurgical reconstruction. Forty-two cases of developmental facial paralysis were identified in a retrospective clinical review (1980 to 2010); 34 (80.95 percent) were children (age, 8 ± 6 years) and eight (19.05 percent) were adults (age, 27 ± 12 years). Comparisons between preoperative and postoperative results were performed with electrophysiologic studies and video evaluations by three independent observers. Mean follow-up was 8 ± 6.3 years (range, 1 to 23 years). Overall, outcome scores improved in all of the patients, as was evident from the observers' mean scores (preoperatively, 2.44; 2 years postoperatively, 3.66; final, 4.11; p < 0.001, Kruskal-Wallis test) and the electrophysiologic data (p < 0.0001). The improvement in eye closure, smile, and depressor function was greater in children as compared with adults (p < 0.005, Mann-Whitney test). Early targeted screening and diagnosis, with prompt specialized treatment, improves the physical and emotional development of children with developmental facial paralysis and reduces the prevalence of aesthetic and functional sequelae of the condition, thus facilitating reintegration among their peers. The experience of this center should serve as a framework for the establishment of accurate and reliable guidelines that will facilitate early diagnosis and management of developmental facial paralysis and provide support and counseling to the family.

“Finding a Voice”: Imaging Features after Phonosurgical Procedures for Vocal Fold Paralysis

PubMed Central

Vachha, B.A.; Ginat, D.T.; Mallur, P.; Cunnane, M.; Moonis, G.

2017-01-01

SUMMARY Altered communication (hoarseness, dysphonia, and breathy voice) that can result from vocal fold paralysis, secondary to numerous etiologies, may be amenable to surgical restoration. In this article, both traditional and cutting-edge phonosurgical procedures targeting the symptoms resulting from vocal fold paralysis are reviewed, with emphasis on the characteristic imaging appearances of various injectable materials, implants, and augmentation procedures used in the treatment of vocal fold paralysis. In addition, complications of injection laryngoplasty and medialization laryngoplasty are illustrated. Familiarity with the expected imaging changes following treatment of vocal fold paralysis may prevent the misinterpretation of posttreatment changes as pathology. Identifying common complications related to injection laryngoplasty and localization of displaced implants is crucial in determining specific management in patients who have undergone phonosurgical procedures for the management of vocal fold paralysis. PMID:27173367

Hereditary spastic paraplegia: clinical principles and genetic advances.

PubMed

Fink, John K

2014-07-01

Hereditary spastic paraplegia (HSP) refers to inherited disorders in which spastic gait is either the only feature or is a major syndrome feature. There are more than 70 genetic types of HSP. Neuropathological studies, albeit limited to only a few genetic types of HSP, have identified axon degeneration involving the distal ends of the corticospinal tracts and fasciculus gracilis fibers. In this review, the author highlights the clinical and genetic features of HSP. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Terror and bliss? Commonalities and distinctions between sleep paralysis, lucid dreaming, and their associations with waking life experiences.

PubMed

Denis, Dan; Poerio, Giulia L

2017-02-01

Sleep paralysis and lucid dreaming are both dissociated experiences related to rapid eye movement (REM) sleep. Anecdotal evidence suggests that episodes of sleep paralysis and lucid dreaming are related but different experiences. In this study we test this claim systematically for the first time in an online survey with 1928 participants (age range: 18-82 years; 53% female). Confirming anecdotal evidence, sleep paralysis and lucid dreaming frequency were related positively and this association was most apparent between lucid dreaming and sleep paralysis episodes featuring vestibular-motor hallucinations. Dissociative experiences were the only common (positive) predictor of both sleep paralysis and lucid dreaming. Both experiences showed different associations with other key variables of interest: sleep paralysis was predicted by sleep quality, anxiety and life stress, whereas lucid dreaming was predicted by a positive constructive daydreaming style and vividness of sensory imagery. Overall, results suggest that dissociative experiences during wakefulness are reflected in dissociative experiences during REM sleep; while sleep paralysis is related primarily to issues of sleep quality and wellbeing, lucid dreaming may reflect a continuation of greater imaginative capacity and positive imagery in waking states. © 2016 The Authors. Journal of Sleep Research published by John Wiley & Sons Ltd on behalf of European Sleep Research Society.

Experience of isolated sleep paralysis in clinical practice in Nigeria.

PubMed

Ohaeri, J U

1992-06-01

The supernatural fears associated with the experience of isolated sleep paralysis in the culture of developing countries is sometimes associated with the evolution of somatic symptoms of psychological origin in patients predisposed to neurotic illness. Patients rarely spontaneously volunteer these fears and doctors pay them scant attention. Illustrative case histories that demonstrate the dynamics of the clinical presentation, as well as the treatment approach, are highlighted. It is hoped that doctors in general medical practice and in psychological medicine in developing countries where belief in supernatural causation of illness is rife will consider these factors in order to provide more effective treatment.

Early effects of embryonic movement: ‘a shot out of the dark’

PubMed Central

Pitsillides, Andrew A

2006-01-01

It has long been appreciated that studying the embryonic chick in ovo provides a variety of advantages, including the potential to control the embryo's environment and its movement independently of maternal influences. This allowed early workers to identify movement as a pivotal factor in the development of the locomotor apparatus. With an increasing focus on the earliest detectable movements, we have exploited this system by developing novel models and schemes to examine the influence of defined periods of movement during musculoskeletal development. Utilizing drugs with known neuromuscular actions to provoke hyperactivity (4-aminopyridine, AP) and either rigid (decamethonium bromide, DMB) or flaccid (pancuronium bromide, PB) paralysis, we have examined the role of movement in joint, osteochondral and muscle development. Our initial studies focusing on the joint showed that AP-induced hyperactivity had little, if any, effect on the timing or scope of joint cavity elaboration, suggesting that endogenous activity levels provide sufficient stimulus, and additional mobilization is without effect. By contrast, imposition of either rigid or flaccid paralysis prior to cavity formation completely blocked this process and, with time, produced fusion of cartilaginous elements and formation of continuous single cartilaginous rods across locations where joints would ordinarily form. The effect of these distinct forms of paralysis differed, however, when treatment was initiated after formation of an overt cavity; rigid, but not flaccid, paralysis partly conserved precavitated joints. This observation suggests that ‘static’ loading derived from ‘spastic’ rigidity can act to preserve joint cavities. Another facet of these studies was the observation that DMB-induced rigid paralysis produces a uniform and specific pattern of limb deformity whereas PB generated a diverse range of fixed positional deformities. Both also reduced limb growth, with different developmental periods preferentially modifying specific osteochondral components. Changes in cartilage and bone growth induced by 3-day periods of flaccid immobilization, imposed at distinct developmental phases, provides support for a diminution in cartilage elaboration at an early phase and for a relatively delayed influence of movement on osteogenesis, invoking critical periods during which the developing skeleton becomes receptive to the impact of movement. Immobilization also exerts differential impact along the proximo-distal axis of the limb. Finally, our preliminary results support the possibility that embryonic hyperactivity influences the potential for postnatal muscle growth. PMID:16637868

Impact of integrated upper limb spasticity management including botulinum toxin A on patient-centred goal attainment: rationale and protocol for an international prospective, longitudinal cohort study (ULIS-III)

PubMed Central

Turner-Stokes, Lynne; Ashford, Stephen; Jacinto, Jorge; Maisonobe, Pascal; Balcaitiene, Jovita; Fheodoroff, Klemens

2016-01-01

Objectives Describe the rationale and protocol for the Upper Limb International Spasticity (ULIS)-III study, which aims to evaluate the impact of integrated spasticity management, involving multiple botulinum toxin A (BoNT-A) injection cycles and concomitant therapies, on patient-centred goal attainment. Outline novel outcome assessment methods for ULIS-III and report initial evaluation data from goal setting in early stages of the study. Design Large international longitudinal cohort study of integrated upper limb spasticity management, including BoNT-A. Participants and setting ULIS-III is a 2-year study expected to enrol >1000 participants at 58 study centres across 14 countries. Interventions The study design is non-interventional and intended to reflect real-life clinical practice. It will describe injection practices and additional treatment strategies, and record clinical decision-making in a serial approach to long-term spasticity management. Outcome measures ULIS-III will use a goal-directed approach to selection of targeted standardised measures to capture the diversity of presentation, goals and outcomes. ULIS-III will implement the Upper Limb Spasticity Index, a battery of assessments including a structured approach to goal attainment scaling (Goal Attainment Scaling—Evaluation of Outcomes for Upper Limb Spasticity tool), alongside a limited set of standardised measures, chosen according to patients' selected goal areas. Concomitant therapy inputs, patient satisfaction with engagement in goal setting, health economic end points and health-related quality of life data will also be captured. Results of initial evaluation of goal quality Recruitment started in January 2015. By June 2015, 58 sites had been identified and initial data collected for 79 patients across 13 sites in 3 countries. Goal setting data were quality-checked and centres rated on the basis of function-related and Specific, Measurable, Achievable, Realistic, Timed (SMART) characteristics of goal statements. Overall, 11/13 centres achieved the highest rating (A++). Conclusions ULIS-III will provide valuable information regarding treatment of and outcomes from real-life upper limb spasticity management worldwide. Trial registration number NCT02454803; Pre-results. PMID:27315835

Practice Parameter: Pharmacologic treatment of spasticity in children and adolescents with cerebral palsy (an evidence-based review)

PubMed Central

Delgado, M R.; Hirtz, D; Aisen, M; Ashwal, S; Fehlings, D L.; McLaughlin, J; Morrison, L A.; Shrader, M W.; Tilton, A; Vargus-Adams, J

2010-01-01

Objective: To evaluate published evidence of efficacy and safety of pharmacologic treatments for childhood spasticity due to cerebral palsy. Methods: A multidisciplinary panel systematically reviewed relevant literature from 1966 to July 2008. Results: For localized/segmental spasticity, botulinum toxin type A is established as an effective treatment to reduce spasticity in the upper and lower extremities. There is conflicting evidence regarding functional improvement. Botulinum toxin type A was found to be generally safe in children with cerebral palsy; however, the Food and Drug Administration is presently investigating isolated cases of generalized weakness resulting in poor outcomes. No studies that met criteria are available on the use of phenol, alcohol, or botulinum toxin type B injections. For generalized spasticity, diazepam is probably effective in reducing spasticity, but there are insufficient data on its effect on motor function and its side-effect profile. Tizanidine is possibly effective, but there are insufficient data on its effect on function and its side-effect profile. There were insufficient data on the use of dantrolene, oral baclofen, and intrathecal baclofen, and toxicity was frequently reported. Recommendations: For localized/segmental spasticity that warrants treatment, botulinum toxin type A should be offered as an effective and generally safe treatment (Level A). There are insufficient data to support or refute the use of phenol, alcohol, or botulinum toxin type B (Level U). For generalized spasticity that warrants treatment, diazepam should be considered for short-term treatment, with caution regarding toxicity (Level B), and tizanidine may be considered (Level C). There are insufficient data to support or refute use of dantrolene, oral baclofen, or continuous intrathecal baclofen (Level U). GLOSSARY AAN = American Academy of Neurology; AE = adverse event; AS = Ashworth scale; BoNT-A = botulinum toxin type A; BoNT-B = botulinum toxin type B; CP = cerebral palsy; FDA = Food and Drug Administration; GAS = Goal Attainment Scale; GMFM = Gross Motor Function Measure; ITB = intrathecal baclofen; MAS = Modified Ashworth scale; OT = occupational therapy; PT = physiotherapy; QUEST = Quality of Upper Extremity Skills Test; TS = Tardieu scale. PMID:20101040

Lumbosacral Dorsal Rhizotomy for Spastic Cerebral Palsy: A Health Technology Assessment

PubMed Central

Pron, Gaylene; Chan, Brian; Tu, Hong Anh; Xie, Xuanqian; Weir, Mark; Wells, David; Higgins, Caroline

2017-01-01

Background Cerebral palsy, a spectrum of neuromuscular conditions caused by abnormal brain development or early damage to the brain, is the most common cause of childhood physical disability. Lumbosacral dorsal rhizotomy is a neurosurgical procedure that permanently decreases spasticity and is always followed by physical therapy. The objectives of this health technology assessment were to evaluate the clinical effectiveness, safety, cost effectiveness, and family perspectives of dorsal rhizotomy. Methods We performed a systematic literature search until December 2015 with auto-alerts until December 2016. Search strategies were developed by medical librarians, and a single reviewer reviewed the abstracts. The health technology assessment included a clinical review based on functional outcomes, safety, and treatment satisfaction; an economic study reviewing cost-effective literature; a budget impact analysis; and interviews with families evaluating the intervention. Results Eighty-four studies (1 meta-analysis, 5 randomized controlled studies [RCTs], 75 observational pre-post studies, and 3 case reports) were reviewed. A meta-analysis of RCTs involving dorsal rhizotomy and physical therapy versus physical therapy confirmed reduced lower-limb spasticity and increased gross motor function (4.5%, P = .002). Observational studies reported statistically significant improvements in gross motor function over 2 years or less (12 studies, GRADE moderate) and over more than 2 years (10 studies, GRADE moderate) as well as improvements in functional independence in the short term (10 studies, GRADE moderate) and long term (4 studies, GRADE low). Major operative complications, were infrequently reported (4 studies). Bony abnormalities and instabilities monitored radiologically in the spine (15 studies) and hip (8 studies) involved minimal or clinically insignificant changes after surgery. No studies evaluated the cost effectiveness of dorsal rhizotomy. The budget impact of funding dorsal rhizotomy for treatment of Ontario children with cerebral palsy was $1.3 million per year. Families reported perceived improvements in their children and expressed satisfaction with treatment. Ontario families reported inadequate medical information on benefits or risk to make an informed decision, enormous financial burdens, and lack rehabilitation support after surgery. Conclusions Lumbrosacral dorsal rhizotomy and physical therapy effectively reduces lower-limb spasticity in children with spastic cerebral palsy and significantly improves their gross motor function and functional independence. Major peri-operative complications were infrequently reported. Families reported perceived improvements with dorsal rhizotomy, and surgery and post-operative rehabilitation were intensive and demanding. PMID:28757906

Lumbosacral Dorsal Rhizotomy for Spastic Cerebral Palsy: A Health Technology Assessment.

PubMed

2017-01-01

Cerebral palsy, a spectrum of neuromuscular conditions caused by abnormal brain development or early damage to the brain, is the most common cause of childhood physical disability. Lumbosacral dorsal rhizotomy is a neurosurgical procedure that permanently decreases spasticity and is always followed by physical therapy. The objectives of this health technology assessment were to evaluate the clinical effectiveness, safety, cost effectiveness, and family perspectives of dorsal rhizotomy. We performed a systematic literature search until December 2015 with auto-alerts until December 2016. Search strategies were developed by medical librarians, and a single reviewer reviewed the abstracts. The health technology assessment included a clinical review based on functional outcomes, safety, and treatment satisfaction; an economic study reviewing cost-effective literature; a budget impact analysis; and interviews with families evaluating the intervention. Eighty-four studies (1 meta-analysis, 5 randomized controlled studies [RCTs], 75 observational pre-post studies, and 3 case reports) were reviewed. A meta-analysis of RCTs involving dorsal rhizotomy and physical therapy versus physical therapy confirmed reduced lower-limb spasticity and increased gross motor function (4.5%, P = .002). Observational studies reported statistically significant improvements in gross motor function over 2 years or less (12 studies, GRADE moderate) and over more than 2 years (10 studies, GRADE moderate) as well as improvements in functional independence in the short term (10 studies, GRADE moderate) and long term (4 studies, GRADE low). Major operative complications, were infrequently reported (4 studies). Bony abnormalities and instabilities monitored radiologically in the spine (15 studies) and hip (8 studies) involved minimal or clinically insignificant changes after surgery. No studies evaluated the cost effectiveness of dorsal rhizotomy. The budget impact of funding dorsal rhizotomy for treatment of Ontario children with cerebral palsy was $1.3 million per year. Families reported perceived improvements in their children and expressed satisfaction with treatment. Ontario families reported inadequate medical information on benefits or risk to make an informed decision, enormous financial burdens, and lack rehabilitation support after surgery. Lumbrosacral dorsal rhizotomy and physical therapy effectively reduces lower-limb spasticity in children with spastic cerebral palsy and significantly improves their gross motor function and functional independence. Major peri-operative complications were infrequently reported. Families reported perceived improvements with dorsal rhizotomy, and surgery and post-operative rehabilitation were intensive and demanding.

Bell's palsy before Bell: Evert Jan Thomassen à Thuessink and idiopathic peripheral facial paralysis.

PubMed

van de Graaf, R C; IJpma, F F A; Nicolai, J-P A; Werker, P M N

2009-11-01

Bell's palsy is the eponym for idiopathic peripheral facial paralysis. It is named after Sir Charles Bell (1774-1842), who, in the first half of the nineteenth century, discovered the function of the facial nerve and attracted the attention of the medical world to facial paralysis. Our knowledge of this condition before Bell's landmark publications is very limited and is based on just a few documents. In 1804 and 1805, Evert Jan Thomassen à Thuessink (1762-1832) published what appears to be the first known extensive study on idiopathic peripheral facial paralysis. His description of this condition was quite accurate. He located several other early descriptions and concluded from this literature that, previously, the condition had usually been confused with other afflictions (such as 'spasmus cynicus', central facial paralysis and trigeminal neuralgia). According to Thomassen à Thuessink, idiopathic peripheral facial paralysis and trigeminal neuralgia were related, being different expressions of the same condition. Thomassen à Thuessink believed that idiopathic peripheral facial paralysis was caused by 'rheumatism' or exposure to cold. Many aetiological theories have since been proposed. Despite this, the cold hypothesis persists even today.

A 63-year-old man with peripheral facial nerve paralysis and a pulmonary lesion.

PubMed

Yserbyt, J; Wilms, G; Lievens, Y; Nackaerts, K

2009-01-01

Occasionally, malignant neoplasms may cause peripheral facial nerve paralysis as a presenting symptom. A 63-year-old man was referred to the Emergency Department because of a peripheral facial nerve paralysis, lasting for 10 days. Initial diagnostic examinations revealed no apparent cause for this facial nerve paralysis. Chest X-ray, however, showed a suspicious tumoural mass, located in the right hilar region, as confirmed by CAT scan. The diagnosis of an advanced stage lung adenocarcinoma was finally confirmed by bronchial biopsy. MRI scanning showed diffuse brain metastases and revealed a pontine lesion as the most probable underlying cause of this case of peripheral facial nerve paralysis. Platin-based palliative chemotherapy was given, after an initial pancranial irradiation. According to the MRI findings, the pontine lesion was responsible for the peripheral facial nerve paralysis, as an initial presenting symptom in this case of lung adenocarcinoma. This clinical case of a peripheral facial nerve paralysis was caused by a pontine brain metastasis and illustrates a rather rare presenting symptom of metastatic lung cancer.

A novel mutation in KIF5A in a Malian family with spastic paraplegia and sensory loss.

PubMed

Guinto, Cheick O; Diarra, Salimata; Diallo, Salimata; Cissé, Lassana; Coulibaly, Thomas; Diallo, Seybou H; Taméga, Abdoulaye; Chen, Ke-Lian; Schindler, Alice B; Bagayoko, Koumba; Simaga, Assiatou; Blackstone, Craig; Fischbeck, Kenneth H; Landouré, Guida

2017-04-01

Hereditary spastic paraplegias (HSPs) are well-characterized disorders but rarely reported in Africa. We evaluated a Malian family in which three individuals had HSP and distal muscle atrophy and sensory loss. HSP panel testing identified a novel heterozygous missense mutation in KIF5A (c.1086G>C, p.Lys362Asn) that segregated with the disease (SPG10). Lys362 is highly conserved across species and Lys362Asn is predicted to be damaging. This study shows that HSPs are present in sub-Saharan Africa, although likely underdiagnosed. Increasing efficiency and decreasing costs of DNA sequencing will make it more feasible to diagnose HSPs in developing countries.

Client-centred development of an infrared thermal access switch for a young adult with severe spastic quadriplegic cerebral palsy.

PubMed

Memarian, Negar; Venetsanopoulos, Anastasios N; Chau, Tom

2011-01-01

This study reports a client-centred development of a non-contact access switch based on an infrared thermal imaging of mouth opening-closing activity of an individual with severe spastic quadriplegic cerebral palsy. Over a 6-month period, the client participated in five test sessions to inform the development of an infrared thermal switch. The client completed eight stimulus-response trials (switch test) and eight word-matching trials (scan test) using the infrared thermal switch and provided subjective feedback throughout. For the switch test, the client achieved an average correct activation rate of 90% and average response time of 2.4 s. His mean correct activation rate on the scan test improved from 65 to 80% over the course of system development, with an average response time of 11.7 s. An infrared thermography switch tuned to a client's extant orofacial gestures is a practical non-invasive access solution and warrants further research in clients with severe physical disability.

Facial paralysis caused by malignant skull base neoplasms.

PubMed

Marzo, Sam J; Leonetti, John P; Petruzzelli, Guy

2002-12-01

Bell palsy remains the most common cause of facial paralysis. Unfortunately, this term is often erroneously applied to all cases of facial paralysis. The authors performed a retrospective review of data obtained in 11 patients who were treated at a university-based referral practice between July 1988 and September 2001 and who presented with acute facial nerve paralysis mimicking Bell palsy. All patients were subsequently found to harbor an occult skull base neoplasm. A delay in diagnosis was demonstrated in all cases. Seven patients died of their disease, and four patients are currently free of disease. Although Bell palsy remains the most common cause of peripheral facial nerve paralysis, patients in whom neoplasms invade the facial nerve may present with acute paralysis mimicking Bell palsy that fails to resolve. Delays in diagnosis and treatment in such cases may result in increased rates of mortality and morbidity.

Facial paralysis caused by malignant skull base neoplasms.

PubMed

Marzo, Sam J; Leonetti, John P; Petruzzelli, Guy

2002-05-15

Bell palsy remains the most common cause of facial paralysis. Unfortunately, this term is often erroneously applied to all cases of facial paralysis. The authors performed a retrospective review of data obtained in 11 patients who were treated at a university-based referral practice between July 1988 and September 2001 and who presented with acute facial nerve paralysis mimicking Bell palsy. All patients were subsequently found to harbor an occult skull base neoplasm. A delay in diagnosis was demonstrated in all cases. Seven patients died of their disease, and four patients are currently free of disease. Although Bell palsy remains the most common cause of peripheral facial nerve paralysis, patients in whom neoplasms invade of the facial nerve may present with acute paralysis mimicking Bell palsy that fails to resolve. Delays in diagnosis and treatment in such cases may result in increased rates of mortality and morbidity.

Toward A Simulation-Based Tool for the Treatment of Vocal Fold Paralysis

PubMed Central

Mittal, Rajat; Zheng, Xudong; Bhardwaj, Rajneesh; Seo, Jung Hee; Xue, Qian; Bielamowicz, Steven

2011-01-01

Advances in high-performance computing are enabling a new generation of software tools that employ computational modeling for surgical planning. Surgical management of laryngeal paralysis is one area where such computational tools could have a significant impact. The current paper describes a comprehensive effort to develop a software tool for planning medialization laryngoplasty where a prosthetic implant is inserted into the larynx in order to medialize the paralyzed vocal fold (VF). While this is one of the most common procedures used to restore voice in patients with VF paralysis, it has a relatively high revision rate, and the tool being developed is expected to improve surgical outcomes. This software tool models the biomechanics of airflow-induced vibration in the human larynx and incorporates sophisticated approaches for modeling the turbulent laryngeal flow, the complex dynamics of the VFs, as well as the production of voiced sound. The current paper describes the key elements of the modeling approach, presents computational results that demonstrate the utility of the approach and also describes some of the limitations and challenges. PMID:21556320

Serial casting for neuromuscular flatfoot and vertical talus in an adolescent with hereditary spastic paraplegia.

PubMed

Sweet, Laurene A; OʼNeill, Lindsey M; Dobbs, Matthew B

2014-01-01

The purpose of this report is to explore assessment and serial casting intervention for painful rigid flatfoot deformities with vertical talus in an adolescent girl with hereditary spastic paraplegia who was nonambulatory. The participant's right foot underwent 2 phases of casting with correction first toward hindfoot inversion and then dorsiflexion. Because of a vertical talus, her left foot required an intermediate casting toward plantar flexion, inversion, and forefoot adduction prior to casting toward dorsiflexion. The patient improved despite the underlying progressive neuromuscular disorder. Pain ameliorated and she returned to supported standing and transfers. Spasticity decreased bilaterally and the flexibility of her foot deformities improved to allow orthotic fabrication in subtalar neutral. Results were maintained at 12 and 16 months. Individualized multiphase serial casting requires further investigation with patients such as those with hereditary spastic paraplegia.

[Aquatic exercise in the treatment of children with cerebral palsy].

PubMed

Dimitrijević, Lidija; Bjelaković, Bojko; Lazović, Milica; Stanković, Ivona; Čolović, Hristina; Kocić, Mirjana; Zlatanović, Dragan

2012-01-01

Aquatic exercise is one of the most popular supplementary treatments for children with neuro-motor impairment, especially for cerebral palsy (CP). As water reduces gravity force which increases postural stability, a child with CP exercises more easily in water than on land. The aim of the study was to examine aquatic exercise effects on gross motor functioning, muscle tone and cardiorespiratory endurance in children with spastic CP. The study included 19 children of both sexes, aged 6 to 12 years, with spastic CP. They were included in a 12-week aquatic exercise program, twice a week. Measurements of GMFM (gross motor function measurement), spasticity (MAS-Modified Ashworth Scale), heart rate (HR) and maximal oxygen consumption (VO2max) were carried out before and after treatment. The measurement results were compared before and after treatment. GMFM mean value before therapy was 80.2% and statistically it was significantly lower in comparison to the same value after therapy, which was 86.2% (p < 0.05). The level of spasticity was considerably decreased after therapy; the mean value before treatment was 3.21 according to MAS, and after treatment it was 1.95 (p < 0.001). After treatment there was a statistically significant improvement of cardiorespiratory indurance, i.e., there was a significant decrease in the mean value of HR and a significant increase of VO2max (p < 0.001). Aquatic exercise program can be useful in improving gross motor functioning, reducing spasticity and increasing cardiorespiratory endurance in children with spastic CP.

Spasticity Management in Disorders of Consciousness

PubMed Central

Laureys, Steven

2017-01-01

Background: Spasticity is a motor disorder frequently encountered after a lesion involving the central nervous system. It is hypothesized to arise from an anarchic reorganization of the pyramidal and parapyramidal fibers and leads to hypertonia and hyperreflexia of the affected muscular groups. While this symptom and its management is well-known in patients suffering from stroke, multiple sclerosis or spinal cord lesion, little is known regarding its appropriate management in patients presenting disorders of consciousness after brain damage. Objectives: Our aim was to review the occurrence of spasticity in patients with disorders of consciousness and the therapeutic interventions used to treat it. Methods: We conducted a systematic review using the PubMed online database. It returned 157 articles. After applying our inclusion criteria (i.e., studies about patients in coma, unresponsive wakefulness syndrome or minimally conscious state, with spasticity objectively reported as a primary or secondary outcome), 18 studies were fully reviewed. Results: The prevalence of spasticity in patients with disorders of consciousness ranged from 59% to 89%. Current treatment options include intrathecal baclofen and soft splints. Several treatment options still need further investigation; including acupuncture, botulin toxin or cortical activation by thalamic stimulation. Conclusion: The small number of articles available in the current literature highlights that spasticity is poorly studied in patients with disorders of consciousness although it is one of the most common motor disorders. While treatments such as intrathecal baclofen and soft splints seem effective, large randomized controlled trials have to be done and new therapeutic options should be explored. PMID:29232836

Influence of Previous Failed Antispasticity Therapy on the Efficacy and Tolerability of THC:CBD Oromucosal Spray for Multiple Sclerosis Spasticity.

PubMed

Haupts, Michael; Vila, Carlos; Jonas, Anna; Witte, Kerstin; Álvarez-Ossorio, Lourdes

2016-01-01

Sativex® (THC:CBD oromucosal spray) is indicated as add-on treatment for patients with moderate to severe multiple sclerosis (MS) spasticity. We aimed to determine whether antispasticity treatment history influenced the efficacy and safety of add-on THC:CBD oromucosal spray in MS spasticity patients. Post hoc analysis of an enriched-design clinical trial of THC:CBD oromucosal spray versus placebo, using records of patients under previous and current ineffective antispasticity therapies. Subgroups were patients with at least 1 failed therapy attempt with either baclofen or tizanidine (Group 1) or at least 2 failed therapy attempts with both baclofen and tizanidine (Group 2). Of 241 patients in the intent-to-treat population, 162 and 57 patients met the criteria for Groups 1 and 2, respectively. In all groups, response on the spasticity 0-10 Numerical Rating Scale was significantly greater with THC:CBD oromucosal spray versus placebo, for minimal clinically important difference (MCID ≥18% improvement vs. baseline) and clinically important difference (CID, ≥30% improvement vs. baseline). THC:CBD oromucosal spray improved spasticity-related symptoms such as sleep quality and timed 10-meter walk independent of the number of prior failed therapy attempts. Tolerability was not influenced by pre-treatment history. THC:CBD oromucosal spray provided consistent relief with good tolerability in MS spasticity patients irrespective of their antispasticity pre-treatment history. © 2016 S. Karger AG, Basel.

Spasticity Measurement Based on Tonic Stretch Reflex Threshold in Children with Cerebral Palsy Using the PediAnklebot.

PubMed

Germanotta, Marco; Taborri, Juri; Rossi, Stefano; Frascarelli, Flaminia; Palermo, Eduardo; Cappa, Paolo; Castelli, Enrico; Petrarca, Maurizio

2017-01-01

Nowadays, objective measures are becoming prominent in spasticity assessment, to overcome limitations of clinical scales. Among others, Tonic Stretch Reflex Threshold (TSRT) showed promising results. Previous studies demonstrated the validity and reliability of TSRT in spasticity assessment at elbow and ankle joints in adults. Purposes of the present study were to assess: (i) the feasibility of measuring TSRT to evaluate spasticity at the ankle joint in children with Cerebral Palsy (CP), and (ii) the correlation between objective measures and clinical scores. A mechatronic device, the pediAnklebot, was used to impose 50 passive stretches to the ankle of 10 children with CP and 3 healthy children, to elicit muscles response at 5 different velocities. Surface electromyography, angles, and angular velocities were recorded to compute dynamic stretch reflex threshold; TSRT was computed with a linear regression through angles and angular velocities. TSRTs for the most affected side of children with CP resulted into the biomechanical range (95.7 ± 12.9° and 86.7 ± 17.4° for Medial and Lateral Gastrocnemius, and 75.9 ± 12.5° for Tibialis Anterior). In three patients, the stretch reflex was not elicited in the less affected side. TSRTs were outside the biomechanical range in healthy children. However, no correlation was found between clinical scores and TSRT values. Here, we demonstrated the capability of TSRT to discriminate between spastic and non-spastic muscles, while no significant outcomes were found for the dorsiflexor muscle.

MRI findings in an infant with vaccine-associated paralytic poliomyelitis.

PubMed

Ferraz-Filho, José Roberto Lopes; dos Santos Torres, Ulysses; de Oliveira, Eduardo Portela; Souza, Antonio Soares

2010-12-01

Although acute flaccid paralysis is a manifestation observed in several neurologic and muscular disorders, vaccine-associated paralytic poliomyelitis (VAPP) is an exceedingly rare etiology. In the clinical setting of acute flaccid paralysis, MRI is useful in differentiating between VAPP and other conditions. Additionally, MRI can assess the extent of lesions. However, reports on MRI findings in VAPP are scarce in the pediatric radiology literature. We report a Brazilian infant who developed VAPP 40 days after receiving the first dose of oral polio vaccine (OPV). MR images of the cervical and thoracic spinal cord showed lesions involving the anterior horn cell, with increased signal intensity on T2-weighted sequences. We would like to emphasize the importance of considering VAPP as a differential diagnosis in patients with acute flaccid paralysis and an MRI showing involvement of medulla oblongata or spinal cord, particularly in countries where OPV is extensively administered.

Psychological wellbeing, physical impairments and rural aging in a developing country setting.

PubMed

Abas, Melanie A; Punpuing, Sureeporn; Jirapramupitak, Tawanchai; Tangchonlatip, Kanchana; Leese, Morven

2009-07-16

There has been very little research on wellbeing, physical impairments and disability in older people in developing countries. A community survey of 1147 older parents, one per household, aged sixty and over in rural Thailand. We used the Burvill scale of physical impairment, the Thai Psychological Wellbeing Scale and the brief WHO Disability Assessment Schedule. We rated received and perceived social support separately from children and from others and rated support to children. We used weighted analyses to take account of the sampling design. Impairments due to arthritis, pain, paralysis, vision, stomach problems or breathing were all associated with lower wellbeing. After adjusting for disability, only impairment due to paralysis was independently associated with lowered wellbeing. The effect of having two or more impairments compared to none was associated with lowered wellbeing after adjusting for demographic factors and social support (adjusted difference -2.37 on the well-being scale with SD = 7.9, p < 0.001) but after adjusting for disability the coefficient fell and was non-significant. The parsimonious model for wellbeing included age, wealth, social support, disability and impairment due to paralysis (the effect of paralysis was -2.97, p = 0.001). In this Thai setting, received support from children and from others and perceived good support from and to children were all independently associated with greater wellbeing whereas actual support to children was associated with lower wellbeing. Low received support from children interacted with paralysis in being especially associated with low wellbeing. In this Thai setting, as found in western settings, most of the association between physical impairments and lower wellbeing is explained by disability. Disability is potentially mediating the association between impairment and low wellbeing. Received support may buffer the impact of some impairments on wellbeing in this setting. Giving actual support to children is associated with less wellbeing unless the support being given to children is perceived as good, perhaps reflecting parental obligation to support adult children in need. Improving community disability services for older people and optimizing received social support will be vital in rural areas in developing countries.

Genetics Home Reference: hyperkalemic periodic paralysis

MedlinePlus

... reduce the ability of skeletal muscles to contract, leading to episodes of muscle weakness or paralysis. In 30 to 40 percent of cases, the cause of hyperkalemic periodic paralysis is unknown. Changes in other genes, which have not been identified, ...

Changes in body composition after spasticity treatment with intrathecal baclofen.

PubMed

Skogberg, Olle; Samuelsson, Kersti; Ertzgaard, Per; Levi, Richard

2017-01-19

To assess changes in body composition, body weight and resting metabolic rate in patients who received intrathecal baclofen therapy for spasticity. Prospective, longitudinal, quasi-experimental, with a pre/post design. Twelve patients with spasticity, fulfilling study criteria, and due for pump implantation for intrathecal baclofen therapy, completed the study. Data were obtained before, 6 months and 12 months after commencement of intrathecal baclofen therapy as regards body composition (by skinfold calliper), body weight, and resting metabolic rate (by resting oxygen consumption). Spasticity was assessed according to the Modified Ashworth Scale (MAS) and Penn Spasm Frequency Scale (PSFS). A reduction in spasticity according to MAS occurred. Mean fat body mass increased and mean lean body mass decreased. Mean body weight showed a non-significant increase and resting metabolic rate a non-significant decrease. This explorative study indicates that unfavourable changes in body composition might occur after intrathecal baclofen therapy. Since obesity and increased fat body mass contribute to an increased cardiovascular risk, these findings may indicate a need for initiation of countermeasures, e.g. increased physical activity and/or dietary measures, in conjunction with intrathecal baclofen therapy. Further studies, including larger study samples and control groups, are needed to corroborate these findings.

Caregiver burden, productivity loss, and indirect costs associated with caring for patients with poststroke spasticity

PubMed Central

Ganapathy, Vaidyanathan; Graham, Glenn D; DiBonaventura, Marco D; Gillard, Patrick J; Goren, Amir; Zorowitz, Richard D

2015-01-01

Objective Many stroke survivors experience poststroke spasticity and the related inability to perform basic activities, which necessitates patient management and treatment, and exerts a considerable burden on the informal caregiver. The current study aims to estimate burden, productivity loss, and indirect costs for caregivers of stroke survivors with spasticity. Methods Internet survey data were collected from 153 caregivers of stroke survivors with spasticity including caregiving time and difficulty (Oberst Caregiver Burden Scale), Work Productivity and Activity Impairment measures, and caregiver and patient characteristics. Fractional logit models examined predictors of work-related restriction, and work losses were monetized (2012 median US wages). Results Mean Oberst Caregiver Burden Scale time and difficulty scores were 46.1 and 32.4, respectively. Employed caregivers (n=71) had overall work restriction (32%), absenteeism (9%), and presenteeism (27%). Caregiver characteristics, lack of nursing home coverage, and stroke survivors’ disability predicted all work restriction outcomes. The mean total lost-productivity cost per employed caregiver was US$835 per month (>$10,000 per year; 72% attributable to presenteeism). Conclusion These findings demonstrate the substantial burden of caring for stroke survivors with spasticity illustrating the societal and economic impact of stroke that extends beyond the stroke survivor. PMID:26609225

[Consensus document on spasticity in patients with multiple sclerosis. Grupo de Enfermedades Desmielinizantes de la Sociedad Española de Neurología].

PubMed

Oreja-Guevara, Celia; Montalban, Xavier; de Andrés, Clara; Casanova-Estruch, Bonaventura; Muñoz-García, Delicias; García, Inmaculada; Fernández, Óscar

2013-10-16

Multiple sclerosis is a chronic neurological inflammatory demyelinating disease. Specialists involved in the symptomatic treatment of this disease tend to apply heterogeneous diagnostic and treatment criteria. To establish homogeneous criteria for treating spasticity based on available scientific knowledge, facilitating decision-making in regular clinical practice. A group of multiple sclerosis specialists from the Spanish Neurological Society demyelinating diseases working group met to review aspects related to spasticity in this disease and draw up the consensus. After an exhaustive bibliographic search and following a metaplan technique, a number of preliminary recommendations were established to incorporate into the document. Finally, each argument was classified depending on the degree of recommendation according to the SIGN (Scottish Intercollegiate Guidelines Network) system. The resulting text was submitted for review by the demyelinating disease group. An experts' consensus was reached regarding spasticity triggering factors, related symptoms, diagnostic criteria, assessment methods, quality of life and therapeutic management (drug and non-drug) criteria. The recommendations included in this consensus can be a useful tool for improving the quality of life of multiple sclerosis patients, as they enable improved diagnosis and treatment of spasticity.

Physical Therapy for an Adult with Chronic Stroke after Botulinum Toxin Injection for Spasticity: A Case Report

PubMed Central

Phadke, Chetan P.; Ismail, Farooq; Boulias, Chris

2015-01-01

ABSTRACT Purpose: In this case report, we describe the type and duration of a physical therapy and botulinum toxin type A (BoNTA) intervention directed at lower limb spasticity and the gait and balance improvement in a patient post-stroke. Treatment of focal spasticity with BoNTA intramuscular injections combined with physical therapy is recommended by rehabilitation experts. However, the optimal type and duration of physical therapy intervention to optimize any functional gains that follow chemodenervation induced by BoNTA has not been established. Method: One individual with chronic stroke who received BoNTA injections for upper and lower extremity spasticity was included. Physical therapy intervention consisted of 45- to 60-min sessions twice weekly for 12 weeks, based on the Bobath–neurodevelopmental therapy approach, and an activity-based home program. Results: After BoNTA injections and physical therapy, the patient made clinically significant improvements in balance and gait speed and became more independent with his ambulation. Conclusions: This case report demonstrates that physical therapy after BoNTA injections can result in significant functional improvements for individuals with spasticity after chronic stroke that may not be possible with BoNTA injections alone. PMID:25931655

Ultrasound-guided injection of botulinum toxin A in the treatment of iliopsoas spasticity

PubMed Central

Sconfienza, L.M.; Perrone, N.; Lacelli, F.; Lentino, C.; Serafini, G.

2008-01-01

Purpose Intramuscular injection of botulinum toxin A (BTX-A) is a common treatment for iliopsoas muscle spasticity, but it is not easy to position the needle in this muscle without guidance. In this paper we describe an ultrasound-guided technique for the intramuscular injection of BTX-A to treat spasticity of the iliopsoas muscle. Its effectiveness was assessed in 10 patients. Method and materials The ultrasound-guided technique for BTX-A injection was used on 10 patients. The needle was inserted into the muscle belly at an angle of 45° along the longitudinal axis of the muscle when allowed by patient's condition. Results In all cases, the iliopsoas muscle was easily identified and both the iliac and psoas components were assessed. Introduction of the needle and drug injection were entirely carried out under ultrasonographic guidance. The procedure was successful in all patients, even in those with a high-grade spasticity, and general anesthesia was not required. Conclusions This ultrasound-guided technique allows accurate guidance for the injection of BTX-A, and it can be considered as an alternate supportive therapy in patients with spasticity and dystonia. PMID:23396653

Combined treatment of botulinumtoxin and robot-assisted rehabilitation therapy on poststroke, upper limb spasticity

PubMed Central

Lee, So Young; Jeon, Young Tae; Kim, Bo Ryun; Han, Eun Young

2017-01-01

Abstract Rationale: Spasticity is a major complication after stroke, and botulinumtoxin A (BoNT-A) injection is commonly used to manage focal spasticity. However, it is uncertain whether BoNT-A can improve voluntary motor control or activities of daily living function of paretic upper limbs. This study investigated whether BoNT-A injection combined with robot-assisted upper limb therapy improves voluntary motor control or functions of upper limbs after stroke. Patient concerns: Two subacute stroke patients were transferred to the Department of Rehabilitation. Diagnoses: Patients demonstrated spasticity in the upper extremity on the affected side. Interventions: BoNT-A was injected into the paretic muscles of the shoulder, arm, and forearm of the 2 patients at the subacute stage. Conventional rehabilitation therapy and robot-assisted upper limb training were performed during the rehabilitation period. Outcomes: Manual dexterity, grip strength, muscle tone, and activities of daily living function were improved after multidisciplinary rehabilitation treatment. Lessons: BoNT-A injection in combination with multidisciplinary rehabilitation treatment, including robot-assisted arm training, should be recommended for subacute spastic stroke patients to enhance appropriate motor recovery. PMID:29390585

What's new in multiple sclerosis spasticity research? Poster session highlights.

PubMed

Linker, Ralf

2017-11-01

Each year at the Multiple Sclerosis Experts Summit, relevant research in the field of multiple sclerosis spasticity is featured in poster sessions. The main studies presented at this year's meeting are summarized herein.

Spastic Colon: What Does It Mean?

MedlinePlus

... M.D. Spastic colon is another term for irritable bowel syndrome (IBS), a common disorder characterized by abdominal cramping, abdominal ... in the small and large intestines associated with IBS. These contractions are sometimes called spasms. However, because ...

Effect of Auditory Constraints on Motor Performance Depends on Stage of Recovery Post-Stroke

PubMed Central

Aluru, Viswanath; Lu, Ying; Leung, Alan; Verghese, Joe; Raghavan, Preeti

2014-01-01

In order to develop evidence-based rehabilitation protocols post-stroke, one must first reconcile the vast heterogeneity in the post-stroke population and develop protocols to facilitate motor learning in the various subgroups. The main purpose of this study is to show that auditory constraints interact with the stage of recovery post-stroke to influence motor learning. We characterized the stages of upper limb recovery using task-based kinematic measures in 20 subjects with chronic hemiparesis. We used a bimanual wrist extension task, performed with a custom-made wrist trainer, to facilitate learning of wrist extension in the paretic hand under four auditory conditions: (1) without auditory cueing; (2) to non-musical happy sounds; (3) to self-selected music; and (4) to a metronome beat set at a comfortable tempo. Two bimanual trials (15 s each) were followed by one unimanual trial with the paretic hand over six cycles under each condition. Clinical metrics, wrist and arm kinematics, and electromyographic activity were recorded. Hierarchical cluster analysis with the Mahalanobis metric based on baseline speed and extent of wrist movement stratified subjects into three distinct groups, which reflected their stage of recovery: spastic paresis, spastic co-contraction, and minimal paresis. In spastic paresis, the metronome beat increased wrist extension, but also increased muscle co-activation across the wrist. In contrast, in spastic co-contraction, no auditory stimulation increased wrist extension and reduced co-activation. In minimal paresis, wrist extension did not improve under any condition. The results suggest that auditory task constraints interact with stage of recovery during motor learning after stroke, perhaps due to recruitment of distinct neural substrates over the course of recovery. The findings advance our understanding of the mechanisms of progression of motor recovery and lay the foundation for personalized treatment algorithms post-stroke. PMID:25002859

A mouse model of paralytic myelitis caused by enterovirus D68

PubMed Central

Yu, Guixia; Leser, J. Smith; Yagi, Shigeo; Tyler, Kenneth L.

2017-01-01

In 2014, the United States experienced an epidemic of acute flaccid myelitis (AFM) cases in children coincident with a nationwide outbreak of enterovirus D68 (EV-D68) respiratory disease. Up to half of the 2014 AFM patients had EV-D68 RNA detected by RT-PCR in their respiratory secretions, although EV-D68 was only detected in cerebrospinal fluid (CSF) from one 2014 AFM patient. Given previously described molecular and epidemiologic associations between EV-D68 and AFM, we sought to develop an animal model by screening seven EV-D68 strains for the ability to induce neurological disease in neonatal mice. We found that four EV-D68 strains from the 2014 outbreak (out of five tested) produced a paralytic disease in mice resembling human AFM. The remaining 2014 strain, as well as 1962 prototype EV-D68 strains Fermon and Rhyne, did not produce, or rarely produced, paralysis in mice. In-depth examination of the paralysis caused by a representative 2014 strain, MO/14-18947, revealed infectious virus, virion particles, and viral genome in the spinal cords of paralyzed mice. Paralysis was elicited in mice following intramuscular, intracerebral, intraperitoneal, and intranasal infection, in descending frequency, and was associated with infection and loss of motor neurons in the anterior horns of spinal cord segments corresponding to paralyzed limbs. Virus isolated from spinal cords of infected mice transmitted disease when injected into naïve mice, fulfilling Koch’s postulates in this model. Finally, we found that EV-D68 immune sera, but not normal mouse sera, protected mice from development of paralysis and death when administered prior to viral challenge. These studies establish an experimental model to study EV-D68-induced myelitis and to better understand disease pathogenesis and develop potential therapies. PMID:28231269

Converging cellular themes for the hereditary spastic paraplegias.

PubMed

Blackstone, Craig

2018-05-10

Hereditary spastic paraplegias (HSPs) are neurologic disorders characterized by prominent lower-extremity spasticity, resulting from a length-dependent axonopathy of corticospinal upper motor neurons. They are among the most genetically-diverse neurologic disorders, with >80 distinct genetic loci and over 60 identified genes. Studies investigating the molecular pathogenesis underlying HSPs have emphasized the importance of converging cellular pathogenic themes in the most common forms of HSP, providing compelling targets for therapy. Most notably, these include organelle shaping and biogenesis as well as membrane and cargo trafficking. Published by Elsevier Ltd.

Cellular pathways of hereditary spastic paraplegia.

PubMed

Blackstone, Craig

2012-01-01

Human voluntary movement is controlled by the pyramidal motor system, a long CNS pathway comprising corticospinal and lower motor neurons. Hereditary spastic paraplegias (HSPs) are a large, genetically diverse group of inherited neurologic disorders characterized by a length-dependent distal axonopathy of the corticospinal tracts, resulting in lower limb spasticity and weakness. A range of studies are converging on alterations in the shaping of organelles, particularly the endoplasmic reticulum, as well as intracellular membrane trafficking and distribution as primary defects underlying the HSPs, with clear relevance for other long axonopathies affecting peripheral nerves and lower motor neurons.

Equine hyperkalemic periodic paralysis: review and implications.

PubMed Central

Naylor, J M

1994-01-01

The purpose of this review is to present an up-to-date summary of the signs, diagnosis, treatment, and implications of equine hyperkalemic periodic paralysis. The review encompasses all original articles published between 1986 and early 1993. Hyperkalemic periodic paralysis is the result of a genetic mutation in the skeletal muscle sodium channel gene. It is inherited as an autosomal dominant trait; most affected horses are heterozygotes. The classical signs are muscle fasciculation, spasm, and weakness associated with hyperkalemia. However, these signs are only rarely observed in affected horses. Potential sequelae to attacks are abrasions and involuntary recumbency; these problems are not specific for hyperkalemic periodic paralysis, but they occur more frequently in hyperkalemic periodic paralysis-affected horses. It is also likely that hyperkalemic periodic paralysis results in greater muscle mass. There are suggestions that homozygotes may be more severely affected and show signs of upper respiratory obstruction as foals. The practitioner needs to be aware of the tests for hyperkalemic periodic paralysis, and their limitations, so that he can properly diagnose this condition. The industry has the difficult problem of deciding whether or not testing should be mandatory and the fate of positive horses. Images Figure 2. PMID:8050073

Cisplatin-induced hypokalemic paralysis.

PubMed

Mohammadianpanah, Mohammad; Omidvari, Shapour; Mosalaei, Ahmad; Ahmadloo, Niloofar

2004-08-01

Profound hypokalemic conditions resulting from cisplatin therapy have been known to produce hypokalemic paralysis in rare cases. We describe such a case of cisplatin-induced hypokalemic paralysis. A 15-year-old Persian girl with ovarian dysgerminoma presented with severe generalized weakness and paraplegia 1 week after the fourth course of cisplatin-based chemotherapy. On physical examination, there was symmetric flaccid paralysis and areflexia in all of the extremities and particularly in the lower limbs. Her serum potassium concentration was 1.7 mmol/L. Metastatic disease was excluded by a comprehensive systemic evaluation. Complete clinical and paraclinical recovery was achieved after short-term administration of potassium supplement. Adverse drug reactions are common with cisplatin, but the drug is only rarely associated with hypokalemic paralysis. Based on the Naranjo causality algorithm, an objective assessment revealed cisplatin to be a probable cause of hypokalemic paralysis in this case. This adverse drug event--whether isolated or secondary to hypomagnesemia--may be deceptive, leading to a fatal mistake in the oncology setting, and should therefore be precisely differentiated from cancer-related complications. This case suggests that cisplatin should be added to the list of agents causing hypokalemic paralysis. Regular serum electrolyte measurement, the early detection of cation deficiency, and appropriate replacement of cations are all recommended.

[A case of medulla oblongata compression by tortuous vertebral arteries presenting with spastic quadriplegia].

PubMed

Kamada, Takashi; Tateishi, Takahisa; Yamashita, Tamayo; Nagata, Shinji; Ohyagi, Yasumasa; Kira, Jun-Ichi

2013-01-01

We report a 58-year-old man showing spastic paraparesis due to medulla oblongata compression by tortuous vertebral arteries. He noticed weakness of both legs and gait disturbance at the age of 58 years and his symptoms progressively worsened during the following several months. General physical findings were normal. Blood pressure was normal and there were no signs of arteriosclerosis. Neurological examination on admission revealed lower-limb-dominant spasticity in all four extremities, lower-limb weakness, hyperreflexia in all extremities with positive Wartenberg's, Babinski's and Chaddock's signs, mild hypesthesia and hypopallesthesia in both lower limbs, and spastic gait. Cranial nerves were all normal. Serum was negative for antibodies against human T-cell lymphotropic virus-1 antibody. Nerve conduction and needle electromyographic studies of all four limbs revealed normal findings. Cervical, thoracic and lumbo-sacral magnetic resonance imaging (MRI) findings were all normal. Brain MRI and magnetic resonance angiography demonstrated bilateral tortuous vertebral arteries compressing the medulla oblongata. Neurovascular decompression of the right vertebral artery was performed because compression of the right side was more severe than that of the left side. Post-operative MRI revealed outward translocation of the right vertebral artery and relieved compression of the medulla oblongata on the right side. The patient's symptoms and neurological findings improved gradually after the operation. Bilateral pyramidal tract signs without cranial nerve dysfunction due to compression of the medulla oblongata by tortuous vertebral arteries are extremely rare and clinically indistinguishable from hereditary spastic paraplegia (HSP). Although we did not perform a genetic test for HSP, we consider that the spastic paraparesis and mild lower-limb hypesthesia were caused by compression of the medulla oblongata by bilateral tortuous vertebral arteries based on the post-operative improvement in symptoms. Given the favorable effects of surgery, tortuous vertebral arteries should be considered in the differential diagnosis of patients presenting with progressive spastic paraparesis.

The Relationship of Isolated Sleep Paralysis and Panic Disorder to Hypertension

PubMed Central

Bell, Carl C.; Hildreth, Carolyn J.; Jenkins, Esther J.; Carter, Cynthia

1988-01-01

An hypothesis is proposed that there exists a subgroup of African-American hypertensive patients whose hypertension could have been prevented by the early detection and treatment of easily recognizable symptoms that signal the initiation of the pathophysiologic processes that lead to essential hypertension. A pilot study of 31 patients with elevated blood pressure revealed that 41.9 percent had isolated sleep paralysis, 35.5 percent had panic attacks, and 9.7 percent had panic disorder. These proposed hyperadrenergic phenomena may be related to the development of hypertension in certain individuals. PMID:3351970

Facial paralysis

MedlinePlus

... a physical, speech, or occupational therapist. If facial paralysis from Bell palsy lasts for more than 6 to 12 months, plastic surgery may be recommended to help the eye close and improve the appearance of the face. Alternative Names Paralysis of the face Images Ptosis, drooping of the ...

Thyrotoxic hypokalemic periodic paralysis in a Hispanic male.

PubMed Central

Zumo, Lawrence A.; Terzian, Christian; Brannan, Timothy

2002-01-01

We report a case of a Hispanic male presenting with acute onset of bilateral lower extremity weakness, without any antecedent viral or bacterial illness, dietary changes, infiltrative orbitopathy, diffuse goiter, infiltrative dermopathy, and family history of periodic paralysis, who was later found to have Graves' disease. This demonstrates a rare case of periodic paralysis as the initial presentation of hyperthyroidism. Thyrotoxic hypokalemic periodic paralysis is common in Asian and Hispanic individuals and uncommon in whites and African Americans. PMID:12069220

Tri-service Disability Evaluation Systems Database Analysis and Research Annual Report 2013

DTIC Science & Technology

2013-05-29

and Marine Corps; paralysis was most common type of neurological condition in the Navy and Air Force. The most common disposition assigned following...five years while Army cases of traumatic brain injury remained stable in 2012 as compared to previous years. Paralysis was the most common...brain injury 2,842 27.3 Residuals of traumatic brain injury 1,005 28.3 Paralysis 2,786 26.8 Migraine 988 27.1 Migraine 1,892 18.2 Paralysis 961 27.8

Genetics Home Reference: autosomal recessive spastic ataxia of Charlevoix-Saguenay

MedlinePlus

... with ARSACS have also been identified in Japan, Turkey, Tunisia, Spain, Italy, and Belgium. The signs and ... spastic ataxia of Charlevoix-Saguenay (ARSACS) families from Turkey. Neurogenetics. 2004 Sep;5(3):165-70. Epub ...

Impact of Sativex(®) on quality of life and activities of daily living in patients with multiple sclerosis spasticity.

PubMed

Arroyo, Rafael; Vila, Carlos; Dechant, Kerry L

2014-07-01

In individuals with multiple sclerosis (MS) spasticity, associated symptoms such as spasms, pain, mobility restrictions and sleep disturbances can interfere with the ability to perform activities of daily living and reduce quality of life (QoL). Recent cross-sectional studies from Europe have confirmed that advancing severity of MS spasticity correlates directly with worsening QoL. The treatment effect of Sativex(®) (GW Pharmaceuticals PLC, Porton Down, UK; Laboratorios Almirall, SA, Barcelona, Spain) on QoL has been evaluated in randomized controlled trials, observational studies conducted under everyday clinical practice conditions and a survey in long-term users. Symptomatic relief of MS spasticity in responders to Sativex was associated with quantifiable improvements in QoL and activities of daily living that were maintained over time. Benefits were perceived by both patients and caregivers.

Recovery of neuronal and network excitability after spinal cord injury and implications for spasticity

PubMed Central

D'Amico, Jessica M.; Condliffe, Elizabeth G.; Martins, Karen J. B.; Bennett, David J.; Gorassini, Monica A.

2014-01-01

The state of areflexia and muscle weakness that immediately follows a spinal cord injury (SCI) is gradually replaced by the recovery of neuronal and network excitability, leading to both improvements in residual motor function and the development of spasticity. In this review we summarize recent animal and human studies that describe how motoneurons and their activation by sensory pathways become hyperexcitable to compensate for the reduction of functional activation of the spinal cord and the eventual impact on the muscle. Specifically, decreases in the inhibitory control of sensory transmission and increases in intrinsic motoneuron excitability are described. We present the idea that replacing lost patterned activation of the spinal cord by activating synaptic inputs via assisted movements, pharmacology or electrical stimulation may help to recover lost spinal inhibition. This may lead to a reduction of uncontrolled activation of the spinal cord and thus, improve its controlled activation by synaptic inputs to ultimately normalize circuit function. Increasing the excitation of the spinal cord with spared descending and/or peripheral inputs by facilitating movement, instead of suppressing it pharmacologically, may provide the best avenue to improve residual motor function and manage spasticity after SCI. PMID:24860447

Paralysis recovery in humans and model systems

NASA Technical Reports Server (NTRS)

Edgerton, V. Reggie; Roy, Roland R.

2002-01-01

Considerable evidence now demonstrates that extensive functional and anatomical reorganization following spinal cord injury occurs in centers of the brain that have some input into spinal motor pools. This is very encouraging, given the accumulating evidence that new connections formed across spinal lesions may not be initially functionally useful. The second area of advancement in the field of paralysis recovery is in the development of effective interventions to counter axonal growth inhibition. A third area of significant progress is the development of robotic devices to quantify the performance level of motor tasks following spinal cord injury and to 'teach' the spinal cord to step and stand. Advances are being made with robotic devices for mice, rats and humans.

Subdural hematoma caused by intracranial hypotension after syringosubarachnoid shunting--case report.

PubMed

Yamazaki, Tomosato; Yanaka, Kiyoyuki; Uemura, Kazuya; Tsukada, Atsuro

2004-09-01

A 32-year-old man developed an extremely rare subdural hematoma after syringosubarachnoid shunting for syringomyelia. He presented with a 4-year history of neck pain and spastic paraparesis resulting from T-7 and T-8 vertebral body fracture suffered in a traffic accident at age 22 years. Magnetic resonance imaging revealed syringomyelia between the craniocervical junction and the T-10 level. The symptoms were slowly progressive, and a syringosubarachnoid shunting was performed. His spasticity improved after surgery, but he developed orthostatic headache 7 days after surgery. Magnetic resonance imaging of the brain demonstrated a thin subdural hematoma over the right cerebral convexity. The subdural hematoma resolved spontaneously within a week with conservative treatment. Vigorous cerebrospinal fluid outflow observed during surgery presumably lowered the pressure in the syrinx cavity, leading to significant but transient intracranial hypotension and consequently the formation of subdural hematoma.

Sleep paralysis in medieval Persia – the Hidayat of Akhawayni (?–983 AD)

PubMed Central

Golzari, Samad EJ; Khodadoust, Kazem; Alakbarli, Farid; Ghabili, Kamyar; Islambulchilar, Ziba; Shoja, Mohammadali M; Khalili, Majid; Abbasnejad, Feridoon; Sheikholeslamzadeh, Niloufar; Shahabi, Nasrollah Moghaddam; Hosseini, Seyed Fazel; Ansarin, Khalil

2012-01-01

Among the first three manuscripts written in Persian, Akhawayni’s Hidayat al-muta’allemin fi al-tibb was the most significant work compiled in the 10th century. Along with the hundreds of chapters on hygiene, anatomy, physiology, symptoms and treatments of the diseases of various organs, there is a chapter on sleep paralysis (night-mare) prior to description and treatment of epilepsy. The present article is a review of the Akhawayni’s teachings on sleep paralysis and of descriptions and treatments of sleep paralysis by the Greek, medieval, and Renaissance scholars. Akhawayni’s descriptions along with other early writings provide insight into sleep paralysis during the Middle Ages in general and in Persia in particular. PMID:22701323

A young man presenting with paralysis after vigorous exercise.

PubMed

Gubran, Christopher; Narain, Rajay; Malik, Luqmaan; Saeed, Saad Aldeen

2012-08-27

Thyrotoxic periodic paralysis (TPP) is a rare metabolic disorder characterised by muscular weakness and paralysis in predisposed thyrotoxic patients. Although patients with TPP are almost uniformly men of Asian descent, cases have been reported in Caucasian and other ethnic populations. The rapid increase in ethnic diversity in Western and European nations has led to increase in TPP reports, where it was once considered exceedingly rare. Correcting the hypokalaemic and hyperthyroid state tends to reverse the paralysis. However, failure to recognise the condition may lead to delay in diagnosis and serious consequences including respiratory failure and death. We describe a young man who was diagnosed with hyperthyroidism who presented with acute paralysis. The clinical characteristics, pathophysiology and management of TTP are reviewed.

Measurement of plantarflexor spasticity in traumatic brain injury: correlational study of resistance torque compared with the modified Ashworth scale.

PubMed

Annaswamy, Thiru; Mallempati, Srinivas; Allison, Stephen C; Abraham, Lawrence D

2007-05-01

To examine the usefulness of a biomechanical measure, resistance torque (RT), in quantifying spasticity by comparing its use with a clinical scale, the modified Ashworth scale (MAS), and quantitative electrophysiological measures. This is a correlational study of spasticity measurements in 34 adults with traumatic brain injury and plantarflexor spasticity. Plantarflexor spasticity was measured in the seated position before and after cryotherapy using the MAS and also by strapping each subject's foot and ankle to an apparatus that provided a ramp and hold stretch. The quantitative measures were (1) reflex threshold angle (RTA) calculated through electromyographic signals and joint angle traces, (2) Hdorsiflexion (Hdf)/Hcontrol (Hctrl) amplitude ratio obtained through reciprocal inhibition of the soleus H-reflex, (3) Hvibration (Hvib)/Hctrl ratio obtained through vibratory inhibition of the soleus H-reflex, and (4) RT calculated as the time integral of the torque graph between the starting and ending pulses of the stretch. Correlation coefficients between RT and MAS scores in both pre-ice (0.41) and post-ice trials (0.42) were fair (P = 0.001). The correlation coefficients between RT scores and RTA scores in both the pre-ice (0.66) and post-ice trials (0.75) were moderate (P

Surgical correction of scoliosis in children with spastic quadriplegia: benefits, adverse effects, and patient selection.

PubMed

Legg, Julian; Davies, Evan; Raich, Annie L; Dettori, Joseph R; Sherry, Ned

2014-04-01

Cerebral palsy (CP) is a group of nonprogressive syndromes of posture and motor impairment associated with lesions of the immature brain. Spastic quadriplegia is the most severe form with a high incidence of scoliosis, back pain, respiratory compromise, pelvic obliquity, and poor sitting balance. Surgical stabilization of the spine is an effective technique for correcting deformity and restoring sitting posture. The decision to operate in this group of patients is challenging. The aim of this study is to determine the benefits of surgical correction of scoliosis in children with spastic quadriplegia, the adverse effects of this treatment, and what preoperative factors affect patient outcome after surgical correction. A systematic review was undertaken to identify studies describing benefits and adverse effects of surgery in spastic quadriplegia. Factors affecting patient outcome following surgical correction of scoliosis were assessed. Studies involving adults and nonspastic quadriplegia were excluded. A total of 10 case series and 1 prospective and 3 retrospective cohort studies met inclusion criteria. There was significant variation in the overall risk of complications (range, 10.9-70.9%), mortality (range, 2.8-19%), respiratory/pulmonary complications (range, 26.9-57.1%), and infection (range, 2.5-56.8%). Factors associated with a worse outcome were a significant degree of thoracic kyphosis, days in the intensive care unit, and poor nutritional status. Caregivers report a high degree of satisfaction with scoliosis surgery for children with spastic quadriplegia. There is limited evidence of preoperative factors that can predict patient outcome after scoliosis. There is a need for well-designed prospective studies of scoliosis surgery in spastic quadriplegia.

Neurotomy of the rectus femoris nerve: Short-term effectiveness for spastic stiff knee gait: Clinical assessment and quantitative gait analysis.

PubMed

Gross, R; Robertson, J; Leboeuf, F; Hamel, O; Brochard, S; Perrouin-Verbe, B

2017-02-01

Stiff knee gait is a troublesome gait disturbance related to spastic paresis, frequently associated with overactivity of the rectus femoris muscle in the swing phase of gait. The aim of this study was to assess the short-term effects of rectus femoris neurotomy for the treatment of spastic stiff-knee gait in patients with hemiparesis. An Intervention study (before-after trial) with an observational design was carried out in a university hospital. Seven ambulatory patients with hemiparesis of spinal or cerebral origin and spastic stiff-knee gait, which had previously been improved by botulinum toxin injections, were proposed a selective neurotomy of the rectus femoris muscle. A functional evaluation (Functional Ambulation Classification and maximal walking distance), clinical evaluation (spasticity - Ashworth scale and Duncan-Ely test, muscle strength - Medical Research Council scale), and quantitative gait analysis (spatiotemporal parameters, stiff knee gait-related kinematic and kinetic parameters, and dynamic electromyography of rectus femoris) were performed as outcome measures, before and 3 months after rectus femoris neurotomy. Compared with preoperative values, there was a significant increase in maximal walking distance, gait speed, and stride length at 3 months. All kinematic parameters improved, and the average early swing phase knee extension moment decreased. The duration of the rectus femoris burst decreased post-op. This study is the first to show that rectus femoris neurotomy helps to normalise muscle activity during gait, and results in improvements in kinetic, kinematic, and functional parameters in patients with spastic stiff knee gait. Copyright © 2016 Elsevier B.V. All rights reserved.

Positive Effects of Extracorporeal Shock Wave Therapy on Spasticity in Poststroke Patients: A Meta-Analysis.

PubMed

Guo, Peipei; Gao, Fuqiang; Zhao, Tingting; Sun, Wei; Wang, Bailiang; Li, Zirong

2017-11-01

Spasticity is a common and serious complication following a stroke, and many clinical research have been conducted to evaluate the effect of extracorporeal shock wave therapy (ESWT) on muscle spasticity in poststroke patients. This meta-analysis aimed to evaluate the therapeutic effect on decreasing spasticity caused by a stroke immediately and 4 weeks after the application of shock wave therapy. We searched PubMed, Embase, Web of Science, and Cochrane Library databases for relevant studies through November 2016 using the following item: (Hypertonia OR Spasticity) and (Shock Wave or ESWT) and (Stroke). The outcomes were evaluated by Modified Ashworth Scale (MAS) grades and pooled by Stata 12.0 (Stata Corp, College Station, TX, USA). Six studies consisting of 9 groups were included in this meta-analysis. The MAS grades immediately after ESWT were significantly improved compared with the baseline values (standardized mean difference [SMD], -1.57; 95% confidence intervals [CIs], -2.20, -.94). Similarly, the MAS grades judged at 4 weeks after ESWT were also showed to be significantly lower than the baseline values (SMD, -1.93; 95% CIs, -2.71, -1.15). ESWT for the spasticity of patients after a stroke is effective, as measured by MAS grades. Moreover, no serious side effects were observed in any patients after shock wave therapy. Nevertheless, our current study with some limitations such as the limited sample size only provided limited quality of evidence; confirmation from a further systematic review or meta-analysis with large-scale, well-designed randomized control trials is required. Copyright © 2017. Published by Elsevier Inc.

Continuous intrathecal baclofen infusion by a programmable pump in 131 consecutive patients with severe spasticity of spinal origin.

PubMed

Ordia, Joe I; Fischer, Edward; Adamski, Ellen; Chagnon, Kimberly G; Spatz, Edward L

2002-01-01

We began this study to determine the efficacy and safety of intrathecal baclofen (ITB) delivered by a programmable pump for the treatment of severe spasticity of spinal cord origin. One hundred fifty two patients with severe spasticity of spinal origin, refractory to oral baclofen, or who experienced intolerable side-effects were given a test dose of ITB. Only those who had a satisfactory response were considered to be appropriate for pump implantation. All but one of the 152 patients had a satisfactory response, and the pump was implanted in 131 patients. Pre- and postoperative spasticity scores were compared and analyzed. The mean Ashworth score for rigidity decreased from 4.2 preoperatively to 1.3 (p < 0.0005) on ITB. The spasm score decreased from a mean of 3.4 to 0.6 (p < 0.0005). Reduction of spasticity resulted in improved levels of physical activity, decreased pain, and augmentation of sleep. Drug-related complications included constipation, muscular hypotonia, urinary retention, erectile dysfunction, nausea, dizziness, drowsiness, hypotension and bradycardia as well as tolerance to baclofen. Some patients experienced post-spinal puncture headaches. Catheter-related problems included occlusions, breaks, punctures, and dislodgments. Superficial pump pocket infection, pocket erosion, cerebrospinal fluid (CSF) leak, post-spinal puncture headache, and meningitis were some of the procedure-related complications. Two pumps flipped and another pump valve was stuck. We conclude that long-term intrathecal baclofen by an implanted programmable pump is a safe and effective method of treating severe intractable spinal spasticity.

Cost-minimization analysis in the treatment of spasticity in children with cerebral palsy with botulinum toxin type A: an observational, longitudinal, retrospective study.

PubMed

Tapias, Gloria; García-Romero, Mar; Crespo, Carlos; Cuesta, Maribel; Forné, Carles; Pascual-Pascual, Samuel Ignacio

2016-09-01

Cost-minimization analysis of onabotulinumtoxinA and abobotulinumtoxinA, taking into account the real dose administered to children with spasticity associated with dynamic equinus foot deformity due to cerebral palsy. A single centre, observational, longitudinal, and retrospective study which included spastic paediatric patients aged 2-to-18-years and treated with onabotulinumtoxinA or abobotulinumtoxinA from December 1995 to October 2012, in the Paediatric Neurology Unit of a first-level Spanish hospital. A longitudinal analysis of spasticity severity was made to confirm the similar efficacy of both treatments. Cost minimization was analyzed using the dose administered and the direct costs (pharmacological and medical visits costs) from the perspective of the National Health System (in euros from 2016). We analyzed 895 patients with paediatric spasticity: 543 were treated only with onabotulinumtoxinA, 292 only with abobotulinumtoxinA, and 60 with both treatments. The mean doses administered were 5.44 U/kg (SD = 2.17) for onabotulinumtoxinA, and 14.73 U/kg (5.26) for abobotulinumto xinA. The total annual direct cost (pharmacological and medical visits) was € 839.56 for onabotulinumtoxinA and € 631.23 for abobotulinumtoxinA, which represents a difference of € 208.34 per year in favour of treatment with abobotulinumtoxinA. It has been demonstrated that in real clinical practice, the cost per patient and year for treatment of paediatric spasticity was lower when abobotulinumtoxinA was used. Copyright AULA MEDICA EDICIONES 2014. Published by AULA MEDICA. All rights reserved.

The Snake with the Scorpion's Sting: Novel Three-Finger Toxin Sodium Channel Activators from the Venom of the Long-Glanded Blue Coral Snake (Calliophis bivirgatus).

PubMed

Yang, Daryl C; Deuis, Jennifer R; Dashevsky, Daniel; Dobson, James; Jackson, Timothy N W; Brust, Andreas; Xie, Bing; Koludarov, Ivan; Debono, Jordan; Hendrikx, Iwan; Hodgson, Wayne C; Josh, Peter; Nouwens, Amanda; Baillie, Gregory J; Bruxner, Timothy J C; Alewood, Paul F; Lim, Kelvin Kok Peng; Frank, Nathaniel; Vetter, Irina; Fry, Bryan G

2016-10-18

Millions of years of evolution have fine-tuned the ability of venom peptides to rapidly incapacitate both prey and potential predators. Toxicofera reptiles are characterized by serous-secreting mandibular or maxillary glands with heightened levels of protein expression. These glands are the core anatomical components of the toxicoferan venom system, which exists in myriad points along an evolutionary continuum. Neofunctionalisation of toxins is facilitated by positive selection at functional hotspots on the ancestral protein and venom proteins have undergone dynamic diversification in helodermatid and varanid lizards as well as advanced snakes. A spectacular point on the venom system continuum is the long-glanded blue coral snake ( Calliophis bivirgatus ), a specialist feeder that preys on fast moving, venomous snakes which have both a high likelihood of prey escape but also represent significant danger to the predator itself. The maxillary venom glands of C. bivirgatus extend one quarter of the snake's body length and nestle within the rib cavity. Despite the snake's notoriety its venom has remained largely unstudied. Here we show that the venom uniquely produces spastic paralysis, in contrast to the flaccid paralysis typically produced by neurotoxic snake venoms. The toxin responsible, which we have called calliotoxin (δ-elapitoxin-Cb1a), is a three-finger toxin (3FTx). Calliotoxin shifts the voltage-dependence of Na V 1.4 activation to more hyperpolarised potentials, inhibits inactivation, and produces large ramp currents, consistent with its profound effects on contractile force in an isolated skeletal muscle preparation. Voltage-gated sodium channels (Na V ) are a particularly attractive pharmacological target as they are involved in almost all physiological processes including action potential generation and conduction. Accordingly, venom peptides that interfere with Na V function provide a key defensive and predatory advantage to a range of invertebrate venomous species including cone snails, scorpions, spiders, and anemones. Enhanced activation or delayed inactivation of sodium channels by toxins is associated with the extremely rapid onset of tetanic/excitatory paralysis in envenomed prey animals. A strong selection pressure exists for the evolution of such toxins where there is a high chance of prey escape. However, despite their prevalence in other venomous species, toxins causing delay of sodium channel inhibition have never previously been described in vertebrate venoms. Here we show that Na V modulators, convergent with those of invertebrates, have evolved in the venom of the long-glanded coral snake. Calliotoxin represents a functionally novel class of 3FTx and a structurally novel class of Na V toxins that will provide significant insights into the pharmacology and physiology of Na V . The toxin represents a remarkable case of functional convergence between invertebrate and vertebrate venom systems in response to similar selection pressures. These results underscore the dynamic evolution of the Toxicofera reptile system and reinforces the value of using evolution as a roadmap for biodiscovery.

The Snake with the Scorpion’s Sting: Novel Three-Finger Toxin Sodium Channel Activators from the Venom of the Long-Glanded Blue Coral Snake (Calliophis bivirgatus)

PubMed Central

Yang, Daryl C.; Deuis, Jennifer R.; Dashevsky, Daniel; Dobson, James; Jackson, Timothy N. W.; Brust, Andreas; Xie, Bing; Koludarov, Ivan; Debono, Jordan; Hendrikx, Iwan; Hodgson, Wayne C.; Josh, Peter; Nouwens, Amanda; Baillie, Gregory J.; Bruxner, Timothy J. C.; Alewood, Paul F.; Lim, Kelvin Kok Peng; Frank, Nathaniel; Vetter, Irina; Fry, Bryan G.

2016-01-01

Millions of years of evolution have fine-tuned the ability of venom peptides to rapidly incapacitate both prey and potential predators. Toxicofera reptiles are characterized by serous-secreting mandibular or maxillary glands with heightened levels of protein expression. These glands are the core anatomical components of the toxicoferan venom system, which exists in myriad points along an evolutionary continuum. Neofunctionalisation of toxins is facilitated by positive selection at functional hotspots on the ancestral protein and venom proteins have undergone dynamic diversification in helodermatid and varanid lizards as well as advanced snakes. A spectacular point on the venom system continuum is the long-glanded blue coral snake (Calliophis bivirgatus), a specialist feeder that preys on fast moving, venomous snakes which have both a high likelihood of prey escape but also represent significant danger to the predator itself. The maxillary venom glands of C. bivirgatus extend one quarter of the snake’s body length and nestle within the rib cavity. Despite the snake’s notoriety its venom has remained largely unstudied. Here we show that the venom uniquely produces spastic paralysis, in contrast to the flaccid paralysis typically produced by neurotoxic snake venoms. The toxin responsible, which we have called calliotoxin (δ-elapitoxin-Cb1a), is a three-finger toxin (3FTx). Calliotoxin shifts the voltage-dependence of NaV1.4 activation to more hyperpolarised potentials, inhibits inactivation, and produces large ramp currents, consistent with its profound effects on contractile force in an isolated skeletal muscle preparation. Voltage-gated sodium channels (NaV) are a particularly attractive pharmacological target as they are involved in almost all physiological processes including action potential generation and conduction. Accordingly, venom peptides that interfere with NaV function provide a key defensive and predatory advantage to a range of invertebrate venomous species including cone snails, scorpions, spiders, and anemones. Enhanced activation or delayed inactivation of sodium channels by toxins is associated with the extremely rapid onset of tetanic/excitatory paralysis in envenomed prey animals. A strong selection pressure exists for the evolution of such toxins where there is a high chance of prey escape. However, despite their prevalence in other venomous species, toxins causing delay of sodium channel inhibition have never previously been described in vertebrate venoms. Here we show that NaV modulators, convergent with those of invertebrates, have evolved in the venom of the long-glanded coral snake. Calliotoxin represents a functionally novel class of 3FTx and a structurally novel class of NaV toxins that will provide significant insights into the pharmacology and physiology of NaV. The toxin represents a remarkable case of functional convergence between invertebrate and vertebrate venom systems in response to similar selection pressures. These results underscore the dynamic evolution of the Toxicofera reptile system and reinforces the value of using evolution as a roadmap for biodiscovery. PMID:27763551

Genetics Home Reference: spastic paraplegia type 3A

MedlinePlus

... on PubMed or Free article on PubMed Central Blackstone C. Cellular pathways of hereditary spastic paraplegia. Annu ... PP, Soderblom C, Tao-Cheng JH, Stadler J, Blackstone C. SPG3A protein atlastin-1 is enriched in ...

Risk Factors for Developing Scoliosis in Cerebral Palsy: A Cross-Sectional Descriptive Study.

PubMed

Bertoncelli, Carlo M; Solla, Federico; Loughenbury, Peter R; Tsirikos, Athanasios I; Bertoncelli, Domenico; Rampal, Virginie

2017-06-01

This study aims to identify the risk factors leading to the development of severe scoliosis among children with cerebral palsy. A cross-sectional descriptive study of 70 children (aged 12-18 years) with severe spastic and/or dystonic cerebral palsy treated in a single specialist unit is described. Statistical analysis included Fisher exact test and logistic regression analysis to identify risk factors. Severe scoliosis is more likely to occur in patients with intractable epilepsy ( P = .008), poor gross motor functional assessment scores ( P = .018), limb spasticity ( P = .045), a history of previous hip surgery ( P = .048), and nonambulatory patients ( P = .013). Logistic regression model confirms the major risk factors are previous hip surgery ( P = .001), moderate to severe epilepsy ( P = .007), and female gender ( P = .03). History of previous hip surgery, intractable epilepsy, and female gender are predictors of developing severe scoliosis in children with cerebral palsy. This knowledge should aid in the early diagnosis of scoliosis and timely referral to specialist services.

The functional anatomy of suggested limb paralysis.

PubMed

Deeley, Quinton; Oakley, David A; Toone, Brian; Bell, Vaughan; Walsh, Eamonn; Marquand, Andre F; Giampietro, Vincent; Brammer, Michael J; Williams, Steven C R; Mehta, Mitul A; Halligan, Peter W

2013-02-01

Suggestions of limb paralysis in highly hypnotically suggestible subjects have been employed to successfully model conversion disorders, revealing similar patterns of brain activation associated with attempted movement of the affected limb. However, previous studies differ with regard to the executive regions involved during involuntary inhibition of the affected limb. This difference may have arisen as previous studies did not control for differences in hypnosis depth between conditions and/or include subjective measures to explore the experience of suggested paralysis. In the current study we employed functional magnetic resonance imaging (fMRI) to examine the functional anatomy of left and right upper limb movements in eight healthy subjects selected for high hypnotic suggestibility during (i) hypnosis (NORMAL) and (ii) attempted movement following additional left upper limb paralysis suggestions (PARALYSIS). Contrast of left upper limb motor function during NORMAL relative to PARALYSIS conditions revealed greater activation of contralateral M1/S1 and ipsilateral cerebellum, consistent with the engagement of these regions in the completion of movements. By contrast, two significant observations were noted in PARALYSIS relative to NORMAL conditions. In conjunction with reports of attempts to move the paralysed limb, greater supplementary motor area (SMA) activation was observed, a finding consistent with the role of SMA in motor intention and planning. The anterior cingulate cortex (ACC, BA 24) was also significantly more active in PARALYSIS relative to NORMAL conditions - suggesting that ACC (BA 24) may be implicated in involuntary, as well as voluntary inhibition of prepotent motor responses. Copyright © 2012 Elsevier Ltd. All rights reserved.

[Efficacy on hemiplegic spasticity treated with plum blossom needle tapping therapy at the key points and Bobath therapy: a randomized controlled trial].

PubMed

Wang, Fei; Zhang, Lijuan; Wang, Jianhua; Shi, Yan; Zheng, Liya

2015-08-01

To evaluate the efficacy on hemiplegic spasticity after cerebral infarction treated with plum blossom needle tapping therapy at the key points and Bobath therapy. Eighty patients were collected, in compliance with the inclusive criteria of hemiplegic spasticity after cerebral infarction, and randomized into an observation group and a control group, 40 cases in each one. In the control group, Bobath manipulation therapy was adopted to relieve spasticity and the treatment of 8 weeks was required. In the observation group, on the basis of the treatment as the control group, the tapping therapy with plum blossom needle was applied to the key points, named Jianyu (LI 15), Jianliao (LI 14), Jianzhen (SI 9), Hegu (LI 4), Chengfu (BL 36), Zusanli (ST 36), Xiyangguan (GB 33), etc. The treatment was given for 15 min each time, once a day. Before treatment, after 4 and 8 weeks of treatment, the Fugl-Meyer assessment (FMA) and Barthel index (BI) were adopted to evaluate the motor function of the extremity and the activity of daily life in the patients of the two groups separately. The modified Ashworth scale was used to evaluate the effect of anti-spasticity. In 4 and 8 weeks of treatment, FMA: scores and BI scores were all significantly increased as compared with those before treatment in the two groups: (both P<0. 05). The results in 8 weeks of treatment in the observation group were significantly better than those in the control group (all P<0. 05). In 4 and 8 weeks of treatment, the scores of spasticity state were improved as compared with those before treatment in the patients of the two groups (all P<0. 05). The result in 8 weeks of treatment in the observation group was significantly better than that in the control group (P<0. 05). In 8 weeks of treatment, the total effective rate of anti-spasticity was 90. 0% (36/40) in the observation group, better than 75. 0% (30/40) in the control group (P<0. 05). The tapping therapy with plum blossom needle at the key points combined with Bobath therapy effectively relieves hemiplegic spasticity in the patients of cerebral infarction and improves the motor function of extremity and the activity of daily life.

Glottic airway gain after 'suture arytenoid laterofixation' in bilateral vocal cord paralysis.

PubMed

Korkmaz, Mehmet Hakan; Bayır, Ömer; Tatar, Emel Çadallı; Saylam, Güleser; Öcal, Bülent; Keseroğlu, Kemal; Özdek, Ali

2015-09-01

This method is an easy, non-expensive, and effective technique in bilateral vocal cord paralysis to improve glottic airway and clinical performance. To evaluate the effectiveness of 'suture arytenoid laterofixation' surgery in bilateral vocal cord paralysis. A retrospective analysis of patients' medical history undergoing 'suture arytenoid laterofixation' surgery for bilateral vocal cord paralysis. This technique was applied under general anesthesia with both microlaryngoscopy and video-monitoring. Two 16 g needles and one 1/0 nylon thread were used for the procedure with 1 cm skin incision; no tracheotomy or tissue excision was required. Pre-post-operative photographs of the glottic region were taken from the endoscopic records, and the areas of rima glottis openings were calculated with the Image-J programme. Forty-seven patients were analyzed. The mean pre-post-operative rima glottis areas were 1.11 ± 0.56 and 2.24 ± 0.93 mm(2), respectively (p < 0.001). Five patients with previous tracheotomy were decannulated within a few days after the operation. In three patients, mild complications developed in the early post-operative period (two laryngeal edemas, one submucosal hematoma). Tracheotomy was performed to only one pregnant patient in the post-operative first day. None of the patients had granulation formation or synechia.

Periodic paralysis: An unusual presentation of drug-induced hyperkalemia.

PubMed

Agrawal, Poonam; Chopra, Deepti; Patra, Surajeet K; Madaan, Himanshu

2014-01-01

Hyperkalemia is a life-threatening electrolyte abnormality. The most common cause of hyperkalemia includes renal disease and ingestion of medications. Drug-induced hyperkalemia may develop in patients with underlying renal impairment, disturbed cellular uptake of potassium load, excessive ingestion or infusion of potassium-containing substances. We report a case of "drug-induced severe hyperkalemia" presenting as periodic paralysis. A 67-year-old diabetic and hypertensive woman presented to emergency department with the complaint of intermittent episode of inability to walk for the past 5 days. Each episode lasted for 15-20 minutes and was associated with breathlessness and restlessness. There was no family history of periodic paralysis and drug history revealed that the patient was onolmesartan 20 mg per day (for past 2 years), perindopril 4 mg per day (for past 16 months), and torsemide 10 mg/day. On examination patient was found to be conscious, alert, and afebrile. Vitals were normal. Examination of cardiovascular and respiratory system did not reveal any significant finding. Blood report of the patient showed serum K+ level 8.6 mmol/l. All other investigations were within normal limits. A diagnosis of drug-induced hyperkalemia was made. Patient responded well to the symptomatic treatment. To the best of the author's knowledge, this is the first case report of drug-induced hyperkalemia presenting as periodic paralysis.

Bilateral Vocal Fold Paralysis After Surgery Immediately in Adult Patient With Chiari Malformation.

PubMed

Chen, Yan; Yue, Jianhong; Yuan, Weixiu

2016-06-01

The authors report the case of a 50-year-old woman with a bilateral vocal fold paralysis after foramen magnum decompression and resection of partial cerebellar tonsil for Chiari malformation. The possible mechanisms of postoperative bilateral vocal fold paralysis are discussed.

Societal Value of Surgery for Facial Reanimation.

PubMed

Su, Peiyi; Ishii, Lisa E; Joseph, Andrew; Nellis, Jason; Dey, Jacob; Bater, Kristin; Byrne, Patrick J; Boahene, Kofi D O; Ishii, Masaru

2017-03-01

Patients with facial paralysis are perceived negatively by society in a number of domains. Society's perception of the health utility of varying degrees of facial paralysis and the value society places on reconstructive surgery for facial reanimation need to be quantified. To measure health state utility of varying degrees of facial paralysis, willingness to pay (WTP) for a repair, and the subsequent value of facial reanimation surgery as perceived by society. This prospective observational study conducted in an academic tertiary referral center evaluated a group of 348 casual observers who viewed images of faces with unilateral facial paralysis of 3 severity levels (low, medium, and high) categorized by House-Brackmann grade. Structural equation modeling was performed to understand associations among health utility metrics, WTP, and facial perception domains. Data were collected from July 16 to September 26, 2015. Observer-rated (1) quality of life (QOL) using established health utility metrics (standard gamble, time trade-off, and a visual analog scale) and (2) their WTP for surgical repair. Among the 348 observers (248 women [71.3%]; 100 men [28.7%]; mean [SD] age, 29.3 [11.6] years), mixed-effects linear regression showed that WTP increased nonlinearly with increasing severity of paralysis. Participants were willing to pay $3487 (95% CI, $2362-$4961) to repair low-grade paralysis, $8571 (95% CI, $6401-$11 234) for medium-grade paralysis, and $20 431 (95% CI, $16 273-$25 317) for high-grade paralysis. The dominant factor affecting the participants' WTP was perceived QOL. Modeling showed that perceived QOL decreased with paralysis severity (regression coefficient, -0.004; 95% CI, -0.005 to -0.004; P < .001) and increased with attractiveness (regression coefficient, 0.002; 95% CI, 0.002 to 0.003; P < .001). Mean (SD) health utility scores calculated by the standard gamble metric for low- and high-grade paralysis were 0.98 (0.09) and 0.77 (0.25), respectively. Time trade-off and visual analog scale measures were highly correlated. We calculated mean (SD) WTP per quality-adjusted life-year, which ranged from $10 167 ($14 565) to $17 008 ($38 288) for low- to high-grade paralysis, respectively. Society perceives the repair of facial paralysis to be a high-value intervention. Societal WTP increases and perceived health state utility decreases with increasing House-Brackmann grade. This study demonstrates the usefulness of WTP as an objective measure to inform dimensions of disease severity and signal the value society places on proper facial function. NA.

A single group, pretest-posttest clinical trial for the effects of dry needling on wrist flexors spasticity after stroke.

PubMed

Fakhari, Zahra; Ansari, Noureddin Nakhostin; Naghdi, Soofia; Mansouri, Korosh; Radinmehr, Hojjat

2017-01-01

Spasticity is a common complication after stroke. Dry needling (DN) is suggested as a novel method for treatment of muscle spasticity. To explore the effects of DN on wrist flexors spasticity poststroke. A single group, pretest-posttest clinical trial was used. Twenty nine patients with stroke (16 male; mean age 54.3 years) were tested at baseline (T0), immediately after DN (T1), and one hour after DN (T2). DN was applied for flexor carpi radialis (FCR) and flexor carpi ulnaris on the affected arm for single session, one minute per muscle. The Modified Modified Ashworth Scale (MMAS), passive resistance force, wrist active and passive range of motion, Box and Block Test, and FCR H-reflex were outcome measures. Significant reductions in MMAS scores were seen both immediately after DN and at 1-hour follow-up (median 2 at T0 to 1 at T1 and T2). There were significant improvements in other measures between the baseline values at T0 and those recorded immediately after the DN at T1 or one hour later at T2. This study suggests that DN reduced wrist flexors spasticity and alpha motor neuron excitability in patients with stroke, and improvements persisted for one hour after DN.

Co-segregation of Huntington disease and hereditary spastic paraplegia in 4 generations.

PubMed

Panas, Marios; Karadima, Georgia; Kalfakis, Nikolaos; Vassilopoulos, Dimitris

2011-07-01

Huntington disease (HD) is an autosomal dominant neurodegenerative disease characterized by choreic hyperkinesias, cognitive decline, and psychiatric manifestations, caused by an increased number of CAG repeats in the IT15 gene on chromosome 4p16.3. Silver syndrome is a rare autosomal dominant form of complicated hereditary spastic paraplegia, characterized by lower limb spasticity in addition to amyotrophy of the small muscles of the hands. In addition to the previously identified locus SPG17 on chromosome 11q12-q14, a new locus (SPG38) on chromosome 4p16-p15 has been recently identified, a region that includes the HD gene. We present a Greek family with 5 members diagnosed with HD in 4 generations. All affected members also presented with clinical features of Silver syndrome showing severe spastic paraplegia and prominent atrophy of all small hand muscles bilaterally. None of the other family members showed features of either HD or spastic paraplegia. The reported coexistence of Silver syndrome with HD in 4 generations is not fortuitous, suggesting that these 2 distinct genetic disorders are in linkage disequilibrium. Although rare, it is reasonable to expect additional similar cases. Clinical neurologists should perhaps investigate this possibility in cases combining features of HD and involvement of the upper and lower motor neurons.

Spastic quadriplegia in Down syndrome with congenital duodenal stenosis/atresia.

PubMed

Kurosawa, Kenji; Enomoto, Keisuke; Tominaga, Makiko; Furuya, Noritaka; Sameshima, Kiyoko; Iai, Mizue; Take, Hiroshi; Shinkai, Masato; Ishikawa, Hiroshi; Yamanaka, Michiko; Matsui, Kiyoshi; Masuno, Mitsuo

2012-06-01

Down syndrome is an autosomal chromosome disorder, characterized by intellectual disability and muscle hypotonia. Muscle hypotonia is observed from neonates to adulthood in Down syndrome patients, but muscle hypertonicity is extremely unusual in this syndrome. During a study period of nine years, we found three patients with severe spastic quadriplegia among 20 cases with Down syndrome and congenital duodenal stenosis/atresia (3/20). However, we could find no patient with spastic quadriplegia among 644 cases with Down syndrome without congenital duodenal stenosis/atresia during the same period (0/644, P < 0.05). Further, we did not find any cases with spastic quadriplegia among 17 patients with congenital duodenal stenosis/atresia without Down syndrome admitted during the same period to use as a control group (0/17, P < 0.05). Our results suggest that congenital duodenal stenosis/atresia is a potential risk factor for spastic quadriplegia in patients with Down syndrome. Long-term survival is improving, and the large majority of people with Down syndrome are expected to live well into adult life. Management and further study for the various problems, representing a low prevalence but serious and specific to patients with Down syndrome, are required to improve their quality of life. © 2012 The Authors. Congenital Anomalies © 2012 Japanese Teratology Society.

Effects of intermittent theta burst stimulation on spasticity after spinal cord injury.

PubMed

Nardone, Raffaele; Langthaler, Patrick B; Orioli, Andrea; Höller, Peter; Höller, Yvonne; Frey, Vanessa N; Brigo, Francesco; Trinka, Eugen

2017-01-01

Spasticity is a common disorder in patients with spinal cord injury (SCI). The aim of this study was to investigate whether intermittent theta burst stimulation (iTBS), a safe, non-invasive and well-tolerated protocol of excitatory repetitive transcranial magnetic stimulation (rTMS), is effective in modulating spasticity in SCI patients. In this randomized, double-blind, crossover, sham-controlled study, ten subjects with incomplete cervical or thoracic SCI received 10 days of daily sessions of real or sham iTBS. The H/M amplitude ratio of the Soleus H reflex, the amplitude of the motor evoked potentials (MEPs) at rest and during background contraction, as well as Modified Ashworth Scale (MAS) and the Spinal Cord Injury Assessment Tool for Spasticity (SCAT) were compared before and after the stimulation protocols. Patients receiving real iTBS showed significant increased resting and active MEPs amplitude and a significant reduction of the H/M amplitude ratio. In these patients also the MAS and SCAT scores were significantly reduced after treatment. These changes persisted up to 1 week after the end of the iTBS treatment, and were not observed under the sham-TBS condition. These findings suggest that iTBS may be a promising therapeutic tool for the spasticity in SCI patients.

Skeletal Maturation and Mineralisation of Children with Moderate to Severe Spastic Quadriplegia.

PubMed

Sharawat, Indar Kumar; Sitaraman, Sadasivan

2016-06-01

Diminished bone mineral density and delayed skeletal maturation are common in children with spastic quadriplegia. The purpose of our study was to evaluate the Bone Mineral Density (BMD) of children with moderate to severe spastic quadriplegia and its relationship with other variables like nutrition and growth. This was a hospital based, cross- sectional, case-control study. Forty-two (28 males, 14 females) children with spastic quadriplegia and 42 (24 males, 18 females) healthy children were included in the study. BMD of cases and control were measured by Dual Energy X-ray Absorptiometry (DEXA). Radiographs of left hand and wrist of cases and controls were taken and bone age was determined. BMD values of upper extremity, lower extremity, thoraco-lumbar spine and pelvis in cases were lower than those of controls (p <0.0001). In children with non severe malnutrition, 75% of the cases had lower bone age than chronological age, whereas all cases with severe malnutrition had lower bone age than chronological age. Step wise regression analysis showed that nutritional status independently contributed to lower BMD values but the BMD values did not correlate significantly with the use of anticonvulsant drugs and presence of physical therapy. Decreased BMD and delayed bone age is prevalent in children with spastic quadriplegia and nutritional status is an important contributing factor.

Bilateral vocal cord paralysis in children.

PubMed

Chen, Eunice Y; Inglis, Andrew F

2008-10-01

Bilateral vocal cord paralysis in children with its many causes presents a challenging problem to the pediatric otolaryngologist. Traditionally, management of bilateral vocal cord paralysis includes securing the airway with a tracheotomy and waiting for spontaneous recovery. Surgeons have tried a variety of surgical procedures in lieu of or in addition to tracheotomy, but none are perfect solutions to the problem. This article reviews the current surgical procedures for bilateral vocal cord paralysis in the pediatric population with a particular focus on the senior author's experience with the endoscopic posterior costal cartilage grafting procedure.

Cricothyroid Muscle Botulinum Toxin Injection to Improve Airway for Bilateral Recurrent Laryngeal Nerve Paralysis, A Case Series.

PubMed

Benninger, Michael S; Hanick, Andrea; Hicks, Douglas M

2016-01-01

Bilateral vocal fold paralysis most commonly results from iatrogenic trauma to the recurrent laryngeal nerve during surgical procedures in the anterior neck. Patients may require tracheostomy because of acute or gradual onset of dyspnea and airway compromise. The intralaryngeal injection of Botox has been considered as a possible therapy for these airway symptoms of bilateral vocal fold paralysis. Chronic unopposed activity of intact cricothyroid muscles could potentially result in gradual medialization of the vocal folds in patients with bilateral recurrent laryngeal nerve paralysis. This case series describes three patients who successfully underwent injections of botulinum toxin into the bilateral cricothyroid muscles to offer sustained relief of dyspnea resulting from bilateral vocal fold paralysis. Copyright © 2016 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

Relationship of vocal cord paralysis to the coil diameter of vagus nerve stimulator leads.

PubMed

Robinson, Leslie C; Winston, Ken R

2015-03-01

This investigation was done to examine, following implantation of vagus nerve stimulators, the relationship of vocal cord paralysis to the inner diameter of the coils used to attach the stimulator lead to the nerve. All data in this investigation were collected, as mandated by the FDA, by the manufacturer of vagus nerve stimulators and were made available without restrictions for analysis by the authors. The data reflect all initial device implantations in the United States for the period from 1997 through 2012. Vocal cord paralysis was reported in 193 of 51,882 implantations. In patients aged 18 years and older, the incidence of paralysis was 0.26% when the stimulator leads had coil diameters of 3 mm and 0.51% when the leads had 2-mm-diameter coils (p < 0.05). Across all age groups, the incidence of vocal cord paralysis increased with age at implantation for leads having 2-mm-diameter coils. In patients aged 18 years and older, vocal cord paralysis occurred at almost twice the rate with the implantation of vagus nerve stimulator leads having 2-mm-diameter coils than with leads having 3-mm-diameter coils. The incidence of vocal cord paralysis increases with patient age at implantation.

Is there an accurate method to measure metabolic requirement of institutionalized children with spastic cerebral palsy?

PubMed

Lee, Siu Pik Peggy; Cheung, Ka Ming; Ko, Chun Hung; Chiu, Heung Chin

2011-07-01

This study hypothesized that there is no difference between energy expenditure measured by indirect calorimetry (IC) and that estimated by predicted formulas compared with the actual intake of children with spastic cerebral palsy (CP). Fifteen children aged 3 to 18 years with spastic CP and associated complications were recruited. IC was used to measure mean energy expenditure (MEE) compared with 3 predicted equations for energy expenditure (PEE), including body surface area (BSA), the recommended daily allowance (RDA), and an equation designed specifically for patients with CP. Friedman and paired t tests were used to examine the variance between PEE and MEE. Intraclass correlation coefficient (ICC) was used to explore the correlation between MEE and PEE. The pretest and posttest core temperatures were compared using the Wilcoxon signed rank test. Mean ± standard deviation MEE was 800.5 ± 295.7 kcal/d; BSA was 1,213.4 ± 171.2 kcal/d; RDA was 1,928.1 ± 341.0 kcal/d; and CP was 1,603.1 ± 215.8 kcal/d. The actual diet intake provided 935.3 ± 222.9 kcal/d. Post hoc analysis revealed a significant difference between mean MEE and PEE (P < .001) but not mean actual intake (P = .128). In addition, the ICC of MEE vs PEE was 0.635 at a 95% confidence interval, indicating a weak correlation. In addition, mean pretest body temperature was 36.4°C ± 1°C, and mean posttest body temperature was 35.8°C ± 2°C. The study showed that MEE was significantly different from PEE, but not from actual intake. This warrants further exploration to develop a population-specific PEE for children with spastic CP.

Autosomal dominant familial spastic paraplegia: Tight linkage to chromosome 15q

DOE Office of Scientific and Technical Information (OSTI.GOV)

Fink, J.K.; Wu, C.T.B.; Jones, S.M.

1994-09-01

Familial spastic paraplegia (FSP) (MIM No.18260) constitutes a clinically and genetically diverse group of disorders that share the primary feature of progressive, severe, lower extremity spasticity. FSP is classified according to the mode of inheritance and whether progressive spasticity occurs in isolation ({open_quotes}uncomplicated FSP{close_quotes}) or with other neurologic abnormalities ({open_quotes}complicated FSP{close_quotes}), including optic neuropathy, retinopathy, extrapyramidal disturbance, dementia, ataxia, ichthyosis, mental retardation, or deafness. Recently, autosomal dominant, uncomplicated FSP was shown to be genetically heterogeneous and tightly linked to a group of microsatellite markers on chromosome 14q in one large kindred. We examined 126 members of a non-consanguineous North Americanmore » kindred of Irish descent. FSP was diagnosed in 31 living subjects who developed insidiously progressive gait disturbance between ages 12 and 35 years. Using genetic linkage analysis to microsatellite DNA polymorphisms, we showed that the FSP locus on chromosome 14q was exluded from linkage with the disorder in our family. Subsequently, we searched for genetic linkage between the disorder and microsatellite DNA polymorphisms spanning approximately 50% of the genome. We observed significantly positive, two-point maximum lod scores (Z) for markers on chromosome 15q: D15S128 (Z=9.70, {theta}=0.05), D15S165 (Z=3.30, {theta}=0.10), and UT511 (Z=3.86, {theta}=0.10). Our data clearly establishes that one locus for autosomal dominant, uncomplicated FSP is mapped to the pericentric region of chromosome 15q. Identifying genes responsible for chromosome 15q-linked and chromosome 14q-linked FSP will greatly advance our understanding of this condition and hopefully other inherited and degenerative brain and spinal cord disorders that are also characterized by axonal degeneration.« less

Artificial Walking Technologies to Improve Gait in Cerebral Palsy: Multichannel Neuromuscular Stimulation.

PubMed

Rose, Jessica; Cahill-Rowley, Katelyn; Butler, Erin E

2017-11-01

Cerebral palsy (CP) is the most common childhood motor disability and often results in debilitating walking abnormalities, such as flexed-knee and stiff-knee gait. Current medical and surgical treatments are only partially effective in improving gait abnormalities and may cause significant muscle weakness. However, emerging artificial walking technologies, such as step-initiated, multichannel neuromuscular electrical stimulation (NMES), can substantially improve gait patterns and promote muscle strength in children with spastic CP. NMES may also be applied to specific lumbar-sacral sensory roots to reduce spasticity. Development of tablet computer-based multichannel NMES can leverage lightweight, wearable wireless stimulators, advanced control design, and surface electrodes to activate lower-limb muscles. Musculoskeletal models have been used to characterize muscle contributions to unimpaired gait and identify high muscle demands, which can help guide multichannel NMES-assisted gait protocols. In addition, patient-specific NMES-assisted gait protocols based on 3D gait analysis can facilitate the appropriate activation of lower-limb muscles to achieve a more functional gait: stance-phase hip and knee extension and swing-phase sequence of hip and knee flexion followed by rapid knee extension. NMES-assisted gait treatment can be conducted as either clinic-based or home-based programs. Rigorous testing of multichannel NMES-assisted gait training protocols will determine optimal treatment dosage for future clinical trials. Evidence-based outcome evaluation using 3D kinematics or temporal-spatial gait parameters will help determine immediate neuroprosthetic effects and longer term neurotherapeutic effects of step-initiated, multichannel NMES-assisted gait in children with spastic CP. Multichannel NMES is a promising assistive technology to help children with spastic CP achieve a more upright, functional gait. © 2017 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.

Correlations between risk factors and functional evolution in patients with spastic quadriplegia

PubMed Central

Rogoveanu, OC; Tuțescu, NC; Kamal, D; Alexandru, DO; Kamal, C; Streba, L; Trăistaru, MR

2016-01-01

Cerebral palsy is the most common cause of developing neuro-motor disability in children, in many cases, the triggering cause remaining unknown. Quadriplegia is the most severe spastic cerebral palsy, characterized by severe mental retardation and bi-pyramidal syndrome. The purpose of this paper was to demonstrate the importance of knowing the risk factors and the psychosomatic ones, determining to what extent they influence the functional evolution in patients diagnosed with spastic quadriplegia. 23 children diagnosed with spastic quadriplegia were included in the study, being aged between 1 year and half and 12 years. Patients were assessed at baseline (T1), at one year (T2) and after two years at the end of the study (T3). Patients received a comprehensive rehabilitation program for the motor and sensory deficits throughout the study. Initially, a comprehensive evaluation (etiopathogenic, clinical and functional) that started from a thorough medical history of children (the older ones), was conducted but chose parents to identify the risk factors, and a complete physical exam. At each assessment, joint and muscle balance was conducted. To assess functionality, the gross motor function classification systems (GMFCS) and manual ability (MACS) were used. Many risk factors that were classified according to the timeline in prenatal factors, perinatal and postnatal, were identified from a thorough history. A direct correlation was noticed between the decrease of coarse functionality and manual ability, both initially and in dynamic and low APGAR scores, low gestational age, low birth weight and a higher body mass index of the mother. A direct link was observed between the gross motor function and the manual ability. A significant improvement in the MACS score was noticed in patients with a better GMFCS score. PMID:27453749

Correlations between risk factors and functional evolution in patients with spastic quadriplegia.

PubMed

Rogoveanu, O C; Tuțescu, N C; Kamal, D; Alexandru, D O; Kamal, C; Streba, L; Trăistaru, M R

2016-01-01

Cerebral palsy is the most common cause of developing neuro-motor disability in children, in many cases, the triggering cause remaining unknown. Quadriplegia is the most severe spastic cerebral palsy, characterized by severe mental retardation and bi-pyramidal syndrome. The purpose of this paper was to demonstrate the importance of knowing the risk factors and the psychosomatic ones, determining to what extent they influence the functional evolution in patients diagnosed with spastic quadriplegia. 23 children diagnosed with spastic quadriplegia were included in the study, being aged between 1 year and half and 12 years. Patients were assessed at baseline (T1), at one year (T2) and after two years at the end of the study (T3). Patients received a comprehensive rehabilitation program for the motor and sensory deficits throughout the study. Initially, a comprehensive evaluation (etiopathogenic, clinical and functional) that started from a thorough medical history of children (the older ones), was conducted but chose parents to identify the risk factors, and a complete physical exam. At each assessment, joint and muscle balance was conducted. To assess functionality, the gross motor function classification systems (GMFCS) and manual ability (MACS) were used. Many risk factors that were classified according to the timeline in prenatal factors, perinatal and postnatal, were identified from a thorough history. A direct correlation was noticed between the decrease of coarse functionality and manual ability, both initially and in dynamic and low APGAR scores, low gestational age, low birth weight and a higher body mass index of the mother. A direct link was observed between the gross motor function and the manual ability. A significant improvement in the MACS score was noticed in patients with a better GMFCS score.

A Hereditary Spastic Paraplegia Mouse Model Supports a Role of ZFYVE26/SPASTIZIN for the Endolysosomal System

PubMed Central

Khundadze, Mukhran; Kollmann, Katrin; Koch, Nicole; Biskup, Christoph; Nietzsche, Sandor; Zimmer, Geraldine; Hennings, J. Christopher; Huebner, Antje K.; Symmank, Judit; Jahic, Amir; Ilina, Elena I.; Karle, Kathrin; Schöls, Ludger; Kessels, Michael; Braulke, Thomas; Qualmann, Britta; Kurth, Ingo; Beetz, Christian; Hübner, Christian A.

2013-01-01

Hereditary spastic paraplegias (HSPs) are characterized by progressive weakness and spasticity of the legs because of the degeneration of cortical motoneuron axons. SPG15 is a recessively inherited HSP variant caused by mutations in the ZFYVE26 gene and is additionally characterized by cerebellar ataxia, mental decline, and progressive thinning of the corpus callosum. ZFYVE26 encodes the FYVE domain-containing protein ZFYVE26/SPASTIZIN, which has been suggested to be associated with the newly discovered adaptor protein 5 (AP5) complex. We show that Zfyve26 is broadly expressed in neurons, associates with intracellular vesicles immunopositive for the early endosomal marker EEA1, and co-fractionates with a component of the AP5 complex. As the function of ZFYVE26 in neurons was largely unknown, we disrupted Zfyve26 in mice. Zfyve26 knockout mice do not show developmental defects but develop late-onset spastic paraplegia with cerebellar ataxia confirming that SPG15 is caused by ZFYVE26 deficiency. The morphological analysis reveals axon degeneration and progressive loss of both cortical motoneurons and Purkinje cells in the cerebellum. Importantly, neuron loss is preceded by accumulation of large intraneuronal deposits of membrane-surrounded material, which co-stains with the lysosomal marker Lamp1. A density gradient analysis of brain lysates shows an increase of Lamp1-positive membrane compartments with higher densities in Zfyve26 knockout mice. Increased levels of lysosomal enzymes in brains of aged knockout mice further support an alteration of the lysosomal compartment upon disruption of Zfyve26. We propose that SPG15 is caused by an endolysosomal membrane trafficking defect, which results in endolysosomal dysfunction. This appears to be particularly relevant in neurons with highly specialized neurites such as cortical motoneurons and Purkinje cells. PMID:24367272

A hereditary spastic paraplegia mouse model supports a role of ZFYVE26/SPASTIZIN for the endolysosomal system.

PubMed

Khundadze, Mukhran; Kollmann, Katrin; Koch, Nicole; Biskup, Christoph; Nietzsche, Sandor; Zimmer, Geraldine; Hennings, J Christopher; Huebner, Antje K; Symmank, Judit; Jahic, Amir; Ilina, Elena I; Karle, Kathrin; Schöls, Ludger; Kessels, Michael; Braulke, Thomas; Qualmann, Britta; Kurth, Ingo; Beetz, Christian; Hübner, Christian A

2013-01-01

Hereditary spastic paraplegias (HSPs) are characterized by progressive weakness and spasticity of the legs because of the degeneration of cortical motoneuron axons. SPG15 is a recessively inherited HSP variant caused by mutations in the ZFYVE26 gene and is additionally characterized by cerebellar ataxia, mental decline, and progressive thinning of the corpus callosum. ZFYVE26 encodes the FYVE domain-containing protein ZFYVE26/SPASTIZIN, which has been suggested to be associated with the newly discovered adaptor protein 5 (AP5) complex. We show that Zfyve26 is broadly expressed in neurons, associates with intracellular vesicles immunopositive for the early endosomal marker EEA1, and co-fractionates with a component of the AP5 complex. As the function of ZFYVE26 in neurons was largely unknown, we disrupted Zfyve26 in mice. Zfyve26 knockout mice do not show developmental defects but develop late-onset spastic paraplegia with cerebellar ataxia confirming that SPG15 is caused by ZFYVE26 deficiency. The morphological analysis reveals axon degeneration and progressive loss of both cortical motoneurons and Purkinje cells in the cerebellum. Importantly, neuron loss is preceded by accumulation of large intraneuronal deposits of membrane-surrounded material, which co-stains with the lysosomal marker Lamp1. A density gradient analysis of brain lysates shows an increase of Lamp1-positive membrane compartments with higher densities in Zfyve26 knockout mice. Increased levels of lysosomal enzymes in brains of aged knockout mice further support an alteration of the lysosomal compartment upon disruption of Zfyve26. We propose that SPG15 is caused by an endolysosomal membrane trafficking defect, which results in endolysosomal dysfunction. This appears to be particularly relevant in neurons with highly specialized neurites such as cortical motoneurons and Purkinje cells.

Laryngeal Manifestations of Neurofibromatosis.

PubMed

Naunheim, Matthew R; Plotkin, Scott R; Franco, Ramon A; Song, Phillip C

2016-03-01

To describe the range of findings in patients with neurofibromatosis (NF) presenting to a laryngology clinic and to analyze the etiologic factors of vocal fold dysfunction in this cohort. Case series with chart review. Tertiary laryngology practice. All cases of NF presenting to an academic laryngology practice were retrospectively reviewed (August 2005 to May 2014), with a total of 34 cases. Demographic data, symptoms, and endoscopic examination findings were reviewed. Etiologic factors of laryngeal complaints were analyzed with reference to NF-associated pathologies and surgical history. Thirty-four patients with NF-1 or NF-2 were evaluated, and 28 of these patients (6 NF-1 and 22 NF-2) had laryngeal pathology. The most common presenting symptoms were vocal weakness (n = 21), dysphagia (n = 5), and globus (n = 4). Three patients had NF-related vocal fold masses on examination, including 2 neurofibromas and 1 schwannoma. Unilateral vocal cord paralysis was seen in 17 patients; bilateral paralysis was observed in 5 patients. Of patients with unilateral or bilateral paralysis, 20 had intracranial masses (vestibular schwannoma, meningioma, or skull base tumors), and 16 had previously undergone surgery for these lesions. Of the patients with NF-associated intracranial tumors, 87.0% presented with vocal cord paralysis, whereas only 40.0% of those without intracranial masses had paralysis (P = .0560). Seven patients underwent medialization procedures. Neurofibromatosis patients may present to laryngology clinic with primary laryngeal tumors or, more commonly, unilateral or bilateral paralysis. Otolaryngologists should be keenly aware of vocal fold paralysis caused by the NF-associated tumors, with particular attention to bilateral paralysis in NF-2. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2016.

Full-movement neuromuscular electrical stimulation improves plantar flexor spasticity and ankle active dorsiflexion in stroke patients: a randomized controlled study.

PubMed

Wang, Yong-Hui; Meng, Fei; Zhang, Yang; Xu, Mao-Yu; Yue, Shou-Wei

2016-06-01

To investigate whether full-movement neuromuscular electrical stimulation, which can generate full range of movement, reduces spasticity and/or improves motor function more effectively than control, sensory threshold-neuromuscular electrical stimulation, and motor threshold-neuromuscular electrical stimulation in sub-acute stroke patients. A randomized, single-blind, controlled study. Physical therapy room and functional assessment room. A total of 72 adult patients with sub-acute post-stroke hemiplegia and plantar flexor spasticity. Patients received 30-minute sessions of neuromuscular electrical stimulation on the motor points of the extensor hallucis and digitorum longus twice a day, five days per week for four weeks. Composite Spasticity Scale, Ankle Active Dorsiflexion Score, and walking time in the Timed Up and Go Test were assessed at pretreatment, posttreatment, and at two-week follow-up. After four weeks of treatment, when comparing interclass pretreatment and posttreatment, only the full-movement neuromuscular electrical stimulation group had a significant reduction in the Composite Spasticity Scale (mean % reduction = 19.91(4.96)%, F = 3.878, p < 0.05) and improvement in the Ankle Active Dorsiflexion Score (mean scores = 3.29(0.91), F = 3.140, p < 0.05). Furthermore, these improvements were maintained two weeks after the treatment ended. However, there were no significant differences in the walking time after four weeks of treatment among the four groups (F = 1.861, p > 0.05). Full-movement neuromuscular electrical stimulation with a stimulus intensity capable of generating full movement can significantly reduce plantar flexor spasticity and improve ankle active dorsiflexion, but cannot decrease walking time in the Timed Up and Go Test in sub-acute stroke patients. © The Author(s) 2015.

Short-term effects of thermotherapy for spasticity on tibial nerve F-waves in post-stroke patients.

PubMed

Matsumoto, Shuji; Kawahira, Kazumi; Etoh, Seiji; Ikeda, Satoshi; Tanaka, Nobuyuki

2006-03-01

Thermotherapy is generally considered appropriate for post-stroke patients with spasticity, yet its acute antispastic effects have not been comprehensively investigated. F-wave parameters have been used to demonstrate changes in motor neuron excitability in spasticity and pharmacological antispastic therapy. The present study aimed to confirm the efficacy of thermotherapy for spasticity by evaluating alterations in F-wave parameters in ten male post-stroke patients with spastic hemiparesis (mean age: 49.0+/-15.0 years) and ten healthy male controls (mean age: 48.7+/-4.4 years). The subjects were immersed in water at 41 degrees C for 10 min. Recordings were made over the abductor hallucis muscle, and antidromic stimulation was performed on the tibial nerve at the ankle. Twenty F-waves were recorded before, immediately after, and 30 min following thermotherapy for each subject. F-wave amplitude and F-wave/M-response ratio were determined. Changes in body temperature and surface-skin temperature were monitored simultaneously. The mean and maximum values of both F-wave parameters were higher on the affected side before thermotherapy. In the post-stroke patients, the mean and maximum values of both parameters were significantly reduced after thermotherapy (P<0.01). Hence, the antispastic effects of thermotherapy were indicated by decreased F-wave parameters. Body temperature was significantly increased both immediately after and 30 min after thermotherapy in all subjects. This appeared to play an important role in decreased spasticity. Surface-skin temperature increased immediately after thermotherapy in both groups and returned to baseline 30 min later. These findings demonstrate that thermotherapy is an effective nonpharmacological antispastic treatment that might facilitate stroke rehabilitation.

Soleus neurotomy for treatment of the spastic equinus foot. Groupe d'Evaluation et de Traitement de la Spasticité et de la Dystonie.

PubMed

Decq, P; Filipetti, P; Cubillos, A; Slavov, V; Lefaucheur, J P; Nguyen, J P

2000-11-01

This prospective, nonrandomized, noncontrolled study was performed to evaluate the results of a new type of neurotomy, namely the soleus neurotomy, for treatment of the spastic equinus foot. Between May 1996 and March 1998, 46 patients were treated for a spastic equinus foot. Clinical status, spasticity (Ashworth Scale score), and kinematic parameters of the gait were determined before and after surgery. The neurotomy was performed on the upper nerve of the soleus in all cases and was associated with other neurotomies (lower nerve of the soleus, 21 patients; gastrocnemius, 9 patients, tibialis posterior, 18 patients; flexor hallucis longus, 16 patients; and flexor digitorum longus, 17 patients). The mean follow-up period was 15 months (range, 8-28 mo). The equinus deformity disappeared clinically in all patients. Before the operation, all patients had an Ashworth Scale score of 2, with an inexhaustible clonus present on knee extension and persisting with knee flexion (Tardieu Scale score, 4), which was abolished in 95% of the patients after surgery. Two patients still had some clonus on knee extension; this did not interfere with their clinical improvement. Knee recurvatum disappeared in eight patients. Analysis of kinematic parameters demonstrated a statistically significant increase in joint motion of the second rocker (P = 0.0026) of the ankle during stance. The duration of the stance or swing phase, length of the walking cycle, and velocity or rate of spontaneous walking were not significantly modified. The study demonstrated that soleus neurotomy is effective for the treatment of spastic equinus foot, leading to abolition of spasticity and improvement in the range of ankle motion during the stance phase of gait.

Linguistic and psychometric validation of the MSSS-88 questionnaire for patients with multiple sclerosis and spasticity in Germany.

PubMed

Henze, Thomas; von Mackensen, Sylvia; Lehrieder, Gerald; Zettl, Uwe K; Pfiffner, Carmen; Flachenecker, Peter

2014-08-01

Multiple sclerosis (MS) is an inflammatory disease where many of the patients suffer from spasticity impacting their quality-of-life. The purpose of this paper was to linguistically validate and psychometrically test the Multiple Sclerosis Spasticity Scale (MSSS-88) in German speaking MS patients. The study had two stages: 1) forward/backward translations of the original MSSS-88 scale into German, discussions with MS-experts and cognitive debriefings with MS patients; 2) psychometric evaluation of the German version. Data collection took part in an observational multi-centre study in Germany (MOVE2). The German translation of the MSSS-88 scale was discussed with three MS-experts; followed by two cognitive debriefing sessions with 12 MS patients. For psychometric evaluation the MSSS-88 was filled in by 87 MS patients with a mean age of 50.2 ± 10.4 years; 26.4% of them had severe spasticity. Data quality was acceptable. Missing data for items of the MSSS-88 were low (range 0-5.75%). Psychometric testing of the MSSS-88 revealed excellent values for reliability and validity. Significant differences between groups regarding severity, grading, type and self-ratings of MS-spasticity and sleep disturbances were found. Sensitivity to change could be demonstrated for the MSSS-88 in the group of MS patients treated with cannabinoid oromucosal spray vs. non-treated patients. In the treated group significant changes with a moderate effect size were found for 'muscle spasms', 'emotional health' and 'pain/discomfort'. No significant changes could be detected in the non-treated group. Preliminary evidence from this small study supports reliability, validity, and responsiveness of the German version of the MSSS-88 for measuring the impact of spasticity in MS.

Effects of Electrical Stimulation in Spastic Muscles After Stroke: Systematic Review and Meta-Analysis of Randomized Controlled Trials.

PubMed

Stein, Cinara; Fritsch, Carolina Gassen; Robinson, Caroline; Sbruzzi, Graciele; Plentz, Rodrigo Della Méa

2015-08-01

Neuromuscular electric stimulation (NMES) has been used to reduce spasticity and improve range of motion in patients with stroke. However, contradictory results have been reported by clinical trials. A systematic review of randomized clinical trials was conducted to assess the effect of treatment with NMES with or without association to another therapy on spastic muscles after stroke compared with placebo or another intervention. We searched the following electronic databases (from inception to February 2015): Medline (PubMed), EMBASE, Cochrane Central Register of Controlled Trials and Physiotherapy Evidence Database (PEDro). Two independent reviewers assessed the eligibility of studies based on predefined inclusion criteria (application of electric stimulation on the lower or upper extremities, regardless of NMES dosage, and comparison with a control group which was not exposed to electric stimulation), excluding studies with <3 days of intervention. The primary outcome extracted was spasticity, assessed by the Modified Ashworth Scale, and the secondary outcome extracted was range of motion, assessed by Goniometer. Of the total of 5066 titles, 29 randomized clinical trials were included with 940 subjects. NMES provided reductions in spasticity (-0.30 [95% confidence interval, -0.58 to -0.03], n=14 randomized clinical trials) and increase in range of motion when compared with control group (2.87 [95% confidence interval, 1.18-4.56], n=13 randomized clinical trials) after stroke. NMES combined with other intervention modalities can be considered as a treatment option that provides improvements in spasticity and range of motion in patients after stroke. URL: http://www.crd.york.ac.uk/PROSPERO. Unique identifier: CRD42014008946. © 2015 American Heart Association, Inc.

Early botulinum toxin treatment for spastic pes equinovarus--a randomized double-blind placebo-controlled study.

PubMed

Fietzek, U M; Kossmehl, P; Schelosky, L; Ebersbach, G; Wissel, J

2014-08-01

Spastic pes equinovarus is a frequent pathological posture of the lower extremity. Botulinum toxin (BoNT/A) has been successfully applied to treat lower limb spasticity. However, the best time to initiate treatment remains unclear. A beneficial effect of an early treatment has been suggested in previous studies. A single-centre double-blind randomized placebo-controlled trial was performed to investigate the efficacy of BoNT/A to reduce muscle hypertonicity at the ankle. Fifty-two patients with unilateral or bilateral spastic pes equinovarus with a modified Ashworth score (mAS) of at least 1+ after stroke, traumatic brain injury or hypoxic encephalopathy were allocated to receive either BoNT/A or placebo treatment. A second, open injection was optional at week 12. Patients received unilateral or bilateral injections with 230 or 460 U onabotulinumtoxinA, respectively. The course of the mAS was explored during the open study phase. Patients who had received BoNT/A treatment had lower mAS compared with placebo at week 12 (P < 0.01). During the open label phase, patients from the placebo group showed further deterioration of muscle tone despite starting from a similar baseline and receiving BoNT treatment. Spastic feet that had received BoNT/A in the first cycle had comparatively lower mAS scores over all follow-up data and at week 24 (P < 0.01). The study demonstrates a reduction of muscular hypertonicity in spastic pes equines with BoNT/A treatment given during the first 3 months after the lesion. Exploratory analyses of the course of muscular hypertonicity during the open phase favour earlier to later treatment. © 2014 The Author(s) European Journal of Neurology © 2014 EAN.

Intramuscular Connective Tissue Differences in Spastic and Control Muscle: A Mechanical and Histological Study

PubMed Central

de Bruin, Marije; Smeulders, Mark J.; Kreulen, Michiel; Huijing, Peter A.; Jaspers, Richard T

2014-01-01

Cerebral palsy (CP) of the spastic type is a neurological disorder characterized by a velocity-dependent increase in tonic stretch reflexes with exaggerated tendon jerks. Secondary to the spasticity, muscle adaptation is presumed to contribute to limitations in the passive range of joint motion. However, the mechanisms underlying these limitations are unknown. Using biopsies, we compared mechanical as well as histological properties of flexor carpi ulnaris muscle (FCU) from CP patients (n = 29) and healthy controls (n = 10). The sarcomere slack length (mean 2.5 µm, SEM 0.05) and slope of the normalized sarcomere length-tension characteristics of spastic fascicle segments and single myofibre segments were not different from those of control muscle. Fibre type distribution also showed no significant differences. Fibre size was significantly smaller (1933 µm2, SEM 190) in spastic muscle than in controls (2572 µm2, SEM 322). However, our statistical analyses indicate that the latter difference is likely to be explained by age, rather than by the affliction. Quantities of endomysial and perimysial networks within biopsies of control and spastic muscle were unchanged with one exception: a significant thickening of the tertiary perimysium (3-fold), i.e. the connective tissue reinforcement of neurovascular tissues penetrating the muscle. Note that this thickening in tertiary perimysium was shown in the majority of CP patients, however a small number of patients (n = 4 out of 23) did not have this feature. These results are taken as indications that enhanced myofascial loads on FCU is one among several factors contributing in a major way to the aetiology of limitation of movement at the wrist in CP and the characteristic wrist position of such patients. PMID:24977410

Disturbances of motor unit rate modulation are prevalent in muscles of spastic-paretic stroke survivors

PubMed Central

Heckman, C. J.; Powers, R. K.; Rymer, W. Z.; Suresh, N. L.

2014-01-01

Stroke survivors often exhibit abnormally low motor unit firing rates during voluntary muscle activation. Our purpose was to assess the prevalence of saturation in motor unit firing rates in the spastic-paretic biceps brachii muscle of stroke survivors. To achieve this objective, we recorded the incidence and duration of impaired lower- and higher-threshold motor unit firing rate modulation in spastic-paretic, contralateral, and healthy control muscle during increases in isometric force generated by the elbow flexor muscles. Impaired firing was considered to have occurred when firing rate became constant (i.e., saturated), despite increasing force. The duration of impaired firing rate modulation in the lower-threshold unit was longer for spastic-paretic (3.9 ± 2.2 s) than for contralateral (1.4 ± 0.9 s; P < 0.001) and control (1.1 ± 1.0 s; P = 0.005) muscles. The duration of impaired firing rate modulation in the higher-threshold unit was also longer for the spastic-paretic (1.7 ± 1.6 s) than contralateral (0.3 ± 0.3 s; P = 0.007) and control (0.1 ± 0.2 s; P = 0.009) muscles. This impaired firing rate of the lower-threshold unit arose, despite an increase in the overall descending command, as shown by the recruitment of the higher-threshold unit during the time that the lower-threshold unit was saturating, and by the continuous increase in averages of the rectified EMG of the biceps brachii muscle throughout the rising phase of the contraction. These results suggest that impairments in firing rate modulation are prevalent in motor units of spastic-paretic muscle, even when the overall descending command to the muscle is increasing. PMID:24572092

Short-term effects of thermotherapy for spasticity on tibial nerve F-waves in post-stroke patients

NASA Astrophysics Data System (ADS)

Matsumoto, Shuji; Kawahira, Kazumi; Etoh, Seiji; Ikeda, Satoshi; Tanaka, Nobuyuki

2006-03-01

Thermotherapy is generally considered appropriate for post-stroke patients with spasticity, yet its acute antispastic effects have not been comprehensively investigated. F-wave parameters have been used to demonstrate changes in motor neuron excitability in spasticity and pharmacological antispastic therapy. The present study aimed to confirm the efficacy of thermotherapy for spasticity by evaluating alterations in F-wave parameters in ten male post-stroke patients with spastic hemiparesis (mean age: 49.0±15.0 years) and ten healthy male controls (mean age: 48.7±4.4 years). The subjects were immersed in water at 41°C for 10 min. Recordings were made over the abductor hallucis muscle, and antidromic stimulation was performed on the tibial nerve at the ankle. Twenty F-waves were recorded before, immediately after, and 30 min following thermotherapy for each subject. F-wave amplitude and F-wave/M-response ratio were determined. Changes in body temperature and surface-skin temperature were monitored simultaneously. The mean and maximum values of both F-wave parameters were higher on the affected side before thermotherapy. In the post-stroke patients, the mean and maximum values of both parameters were significantly reduced after thermotherapy ( P<0.01). Hence, the antispastic effects of thermotherapy were indicated by decreased F-wave parameters. Body temperature was significantly increased both immediately after and 30 min after thermotherapy in all subjects. This appeared to play an important role in decreased spasticity. Surface-skin temperature increased immediately after thermotherapy in both groups and returned to baseline 30 min later. These findings demonstrate that thermotherapy is an effective nonpharmacological antispastic treatment that might facilitate stroke rehabilitation.

15 minute consultation: a structured approach to the management of facial paralysis in a child.

PubMed

Malik, Vikas; Joshi, Vineeta; Green, Kevin M J; Bruce, Iain A

2012-06-01

To present a structured approach for an outpatient consultation of a child with facial paralysis. Review of literature and description of approach followed in our unit. A focused history and examination is key to establish the cause and draw a management plan for paediatric facial paralysis.

When the bell tolls on Bell's palsy: finding occult malignancy in acute-onset facial paralysis.

PubMed

Quesnel, Alicia M; Lindsay, Robin W; Hadlock, Tessa A

2010-01-01

This study reports 4 cases of occult parotid malignancy presenting with sudden-onset facial paralysis to demonstrate that failure to regain tone 6 months after onset distinguishes these patients from Bell's palsy patients with delayed recovery and to propose a diagnostic algorithm for this subset of patients. A case series of 4 patients with occult parotid malignancies presenting with acute-onset unilateral facial paralysis is reported. Initial imaging on all 4 patients did not demonstrate a parotid mass. Diagnostic delays ranged from 7 to 36 months from time of onset of facial paralysis to time of diagnosis of parotid malignancy. Additional physical examination findings, especially failure to regain tone, as well as properly protocolled radiologic studies reviewed with dedicated head and neck radiologists, were helpful in arriving at the diagnosis. An algorithm to minimize diagnostic delays in this subset of acute facial paralysis patients is presented. Careful attention to facial tone, in addition to movement, is important in the diagnostic evaluation of acute-onset facial paralysis. Copyright 2010 Elsevier Inc. All rights reserved.

Features extraction of EMG signal using time domain analysis for arm rehabilitation device

NASA Astrophysics Data System (ADS)

Jali, Mohd Hafiz; Ibrahim, Iffah Masturah; Sulaima, Mohamad Fani; Bukhari, W. M.; Izzuddin, Tarmizi Ahmad; Nasir, Mohamad Na'im

2015-05-01

Rehabilitation device is used as an exoskeleton for people who had failure of their limb. Arm rehabilitation device may help the rehab program whom suffers from arm disability. The device that is used to facilitate the tasks of the program should improve the electrical activity in the motor unit and minimize the mental effort of the user. Electromyography (EMG) is the techniques to analyze the presence of electrical activity in musculoskeletal systems. The electrical activity in muscles of disable person is failed to contract the muscle for movements. In order to prevent the muscles from paralysis becomes spasticity, the force of movements should minimize the mental efforts. Therefore, the rehabilitation device should analyze the surface EMG signal of normal people that can be implemented to the device. The signal is collected according to procedure of surface electromyography for non-invasive assessment of muscles (SENIAM). The EMG signal is implemented to set the movements' pattern of the arm rehabilitation device. The filtered EMG signal was extracted for features of Standard Deviation (STD), Mean Absolute Value (MAV) and Root Mean Square (RMS) in time-domain. The extraction of EMG data is important to have the reduced vector in the signal features with less of error. In order to determine the best features for any movements, several trials of extraction methods are used by determining the features with less of errors. The accurate features can be use for future works of rehabilitation control in real-time.

Postanesthetic recumbency associated with hyperkalemic periodic paralysis in a quarter horse.

PubMed

Robertson, S A; Green, S L; Carter, S W; Bolon, B N; Brown, M P; Shields, R P

1992-10-15

Anesthesia and surgery in a Quarter Horse affected with hyperkalemic periodic paralysis resulted in euthanasia after 7 days of postoperative recumbency. Initial recovery was uneventful after extensive sinus surgery, but within 2 hours, the horse had severe muscle weakness. Plasma electrolyte concentrations were within the normal range during the period of recumbency. There was no clinical or laboratory evidence of severe muscle damage. Despite treatment with acetazolamide, isoproterenol, and intensive nursing, the horse was unable to stand for more than a few seconds and developed severe decubital ulcers. Ultrastructural examination revealed nemaline rods and swollen mitochondria in disrupted myofibers.

Useful surgical techniques for facial nerve preservation in tumorous intra-temporal lesions.

PubMed

Kim, Jin; Moon, In Seok; Lee, Jong Dae; Shim, Dae Bo; Lee, Won-Sang

2010-02-01

The management of the facial nerve in tumorous temporal lesions is particularly challenging due to its complex anatomic location and potential postoperative complications, including permanent facial paralysis. The most important concern regarding surgical treatment of a tumorous temporal lesion is the inevitable facial paralysis caused by nerve injury during the tumor removal, especially in patients with minimal to no preoperative facial nerve dysfunction. We describe successful four cases in which various surgical techniques were developed for the preservation of the facial nerve in treatment of intratemporal tumorous lesions. Copyright (c) 2009 Elsevier Ireland Ltd. All rights reserved.

Teeth grinding, oral motor performance and maximal bite force in cerebral palsy children.

PubMed

Botti Rodrigues Santos, Maria Teresa; Duarte Ferreira, Maria Cristina; de Oliveira Guaré, Renata; Guimarães, Antonio Sergio; Lira Ortega, Adriana

2015-01-01

Identify whether the degree of oral motor performance is related to the presence of teeth grinding and maximal bite force values in children with spastic cerebral palsy. Ninety-five spastic cerebral palsy children with and without teeth grinding, according to caregivers' reports, were submitted to a comprehensive oral motor performance evaluation during the feeding process using the Oral Motor Assessment Scale. Maximal bite force was measured using an electronic gnathodynamometer. The teeth grinding group (n = 42) was younger, used anticonvulsant drugs, and was more frequently classified within the subfunctional oral motor performance category. Teeth grinding subfunctional spastic cerebral palsy children presented lower values of maximal bite force. The functional groups showing the presence or absence of teeth grinding presented higher values of maximal bite force compared with the subfunctional groups. In spastic cerebral palsy children, teeth grinding is associated with the worse oral motor performance. © 2015 Special Care Dentistry Association and Wiley Periodicals, Inc.

The role of kinesiotaping combined with botulinum toxin to reduce plantar flexors spasticity after stroke.

PubMed

Karadag-Saygi, Evrim; Cubukcu-Aydoseli, Koza; Kablan, Nilufer; Ofluoglu, Demet

2010-01-01

To evaluate the effect of kinesiotaping as an adjuvant therapy to botulinum toxin A (BTX-A) injection in lower extremity spasticity. This is a single-center, randomized, and double-blind study. Twenty hemiplegic patients with spastic equinus foot were enrolled into the study and randomized into 2 groups. The first group (n=10) received BTX-A injection and kinesiotaping, and the second group (n=10) received BTX-A injection and sham-taping. Clinical assessment was done before injection and at 2 weeks and 1, 3, and 6 months. Outcome measures were modified Ashworth scale (MAS), passive ankle dorsiflexion, gait velocity, and step length. Improvement was recorded in both kinesiotaping and sham groups for all outcome variables. No significant difference was found between groups other than passive range of motion (ROM), which was found to have increased more in the kinesiotaping group at 2 weeks. There is no clear benefit in adjuvant kinesiotaping application with botulinum toxin for correction of spastic equinus in stroke.

A Locus for Autosomal Dominant Hereditary Spastic Ataxia, SAX1, Maps to Chromosome 12p13

PubMed Central

Meijer, I. A.; Hand, C. K.; Grewal, K. K.; Stefanelli, M. G.; Ives, E. J.; Rouleau, G. A.

2002-01-01

The hereditary spastic ataxias (HSA) are a group of clinically heterogeneous neurodegenerative disorders characterized by lower-limb spasticity and generalized ataxia. HSA was diagnosed in three unrelated autosomal dominant families from Newfoundland, who presented mainly with severe leg spasticity, dysarthria, dysphagia, and ocular-movement abnormalities. A genomewide scan was performed on one family, and linkage to a novel locus for HSA on chromosome 12p13, which contains the as-yet-unidentified gene locus SAX1, was identified. Fine mapping confirmed linkage in the two large families, and the third, smaller family showed LOD scores suggestive of linkage. Haplotype construction by use of 13 polymorphic markers revealed that all three families share a disease haplotype, which key recombinants and overlapping haplotypes refine to ∼5 cM, flanked by markers D12S93 and GATA151H05. SAX1 is the first locus mapped for autosomal dominant HSA. PMID:11774073

Hyperargininemia: 7-month follow-up under sodium benzoate therapy in an Italian child presenting progressive spastic paraparesis, cognitive decline, and novel mutation in ARG1 gene.

PubMed

Baranello, Giovanni; Alfei, Enrico; Martinelli, Diego; Rizzetto, Manuela; Cazzaniga, Fabiana; Dionisi-Vici, Carlo; Gellera, Cinzia; Castellotti, Barbara

2014-09-01

Hyperargininemia due to mutations in ARG1 gene is an autosomal recessive inborn error of metabolism caused by a defect in the final step of the urea cycle. Common clinical presentation is a variable association of progressive spastic paraparesis, epilepsy, and cognitive deficits. We describe the clinical history of an Italian child presenting progressive spastic paraparesis, carrying a new homozygous missense mutation in the ARG1 gene. A detailed clinical, biochemical, and neurophysiological follow-up after 7 months of sodium benzoate therapy is reported. Laboratory findings, gait abnormalities, spastic paraparesis, and electroencephalographic and neurophysiological abnormalities remained quite stable over the follow-up. Conversely, a mild cognitive deterioration has been detected by means of the neuropsychologic assessment. Further longitudinal studies by means of longer follow-up and using clinical, biochemical, radiological, and neurophysiological assessments are needed in such patients to describe natural history and monitor the effects of treatments. Copyright © 2014 Elsevier Inc. All rights reserved.

Effect of Knee Orthoses on Hamstring Contracture in Children With Cerebral Palsy: Multiple Single-Subject Study.

PubMed

Laessker-Alkema, Kristina; Eek, Meta Nyström

2016-01-01

To examine the effect of knee orthoses on extensibility of the hamstrings in children with spastic cerebral palsy (CP). The short-term effects of knee orthoses on passive range of motion (ROM), spasticity, and gross motor function of the hamstrings. Ten children with spastic CP, aged 5 to 14 years, at Gross Motor Function Classification System levels I to V, were followed. The orthoses were worn for a minimum of 30 minutes day, 5 days per week, during the intervention period of 8 weeks. Visual analysis using the Two Standard Deviation Band Method supported improvements in passive ROM for all 20 hamstring muscles and in 12 of 14 knee extension measurements. Analyses with the Wilcoxon signed rank test confirm the individual results and support a significant increase in hamstring muscles (P = .005) and knee extension (right: P =.028; left: P =.018) compared with baseline. In children with spastic CP, 8 weeks of treatment with knee orthoses can improve extensibility of the hamstrings.

Genetic mapping to 10q23.3-q24.2, in a large Italian pedigree, of a new syndrome showing bilateral cataracts, gastroesophageal reflux, and spastic paraparesis with amyotrophy.

PubMed Central

Seri, M; Cusano, R; Forabosco, P; Cinti, R; Caroli, F; Picco, P; Bini, R; Morra, V B; De Michele, G; Lerone, M; Silengo, M; Pela, I; Borrone, C; Romeo, G; Devoto, M

1999-01-01

We have recently observed a large pedigree with a new rare autosomal dominant spastic paraparesis. In three subsequent generations, 13 affected individuals presented with bilateral cataracts, gastroesophageal reflux with persistent vomiting, and spastic paraparesis with amyotrophy. Bilateral cataracts occurred in all affected individuals, with the exception of one patient who presented with a chorioretinal dystrophy, whereas clinical signs of spastic paraparesis showed a variable expressivity. Using a genomewide mapping approach, we mapped the disorder to the long arm of chromosome 10 on band q23.3-q24.2, in a 12-cM chromosomal region where additional neurologic disorders have been localized. The spectrum of phenotypic manifestations in this family is reminiscent of a smaller pedigree, reported recently, confirming the possibility of a new syndrome. Finally, the anticipation of symptoms suggests that an unstable trinucleotide repeat may be responsible for the condition. PMID:9973297

Mechanistic basis of an epistatic interaction reducing age at onset in hereditary spastic paraplegia.

PubMed

Newton, Timothy; Allison, Rachel; Edgar, James R; Lumb, Jennifer H; Rodger, Catherine E; Manna, Paul T; Rizo, Tania; Kohl, Zacharias; Nygren, Anders O H; Arning, Larissa; Schüle, Rebecca; Depienne, Christel; Goldberg, Lisa; Frahm, Christiane; Stevanin, Giovanni; Durr, Alexandra; Schöls, Ludger; Winner, Beate; Beetz, Christian; Reid, Evan

2018-05-01

Many genetic neurological disorders exhibit variable expression within affected families, often exemplified by variations in disease age at onset. Epistatic effects (i.e. effects of modifier genes on the disease gene) may underlie this variation, but the mechanistic basis for such epistatic interactions is rarely understood. Here we report a novel epistatic interaction between SPAST and the contiguous gene DPY30, which modifies age at onset in hereditary spastic paraplegia, a genetic axonopathy. We found that patients with hereditary spastic paraplegia caused by genomic deletions of SPAST that extended into DPY30 had a significantly younger age at onset. We show that, like spastin, the protein encoded by SPAST, the DPY30 protein controls endosomal tubule fission, traffic of mannose 6-phosphate receptors from endosomes to the Golgi, and lysosomal ultrastructural morphology. We propose that additive effects on this pathway explain the reduced age at onset of hereditary spastic paraplegia in patients who are haploinsufficient for both genes.

Progressive paralysis associated with diffuse astrocyte anaplasia in delta 202 mice homozygous for a transgene encoding the SV40 T antigen.

PubMed

López-Revilla, Rubén; Soto-Zárate, Carlos; Ridaura, Cecilia; Chávez-Dueñas, Lucía; Paul, Dieter

2004-03-01

A convenient transgenic astrocytoma model in delta202 mice, homozygous for a construct encoding the early region of the SV40 virus genome, is described. In the offspring of crosses between delta202 mice heterozygous for the transgene nearly 60% were transgenic; one third of these developed progressive paralysis starting in the hindlimbs at approximately 35 days of age and died at 90 +/- 30 days of age. In affected mice proliferating-non-neuronal cells immunostained with antibodies to the GFAP, an astrocyte marker, whose number increased with age were found in the white matter of the brain, cerebellum and spinal cord, and progressive degeneration and necrosis of spinal motoneurons was observed that-may explain the paralysis. The early onset and reproducible time course of the neurological disease suggest that homozygous delta202 mice, whose proliferating astrocytes appear to damage spinal motoneurons, are a useful model to study astrocyte differentiation, function and tumorigenesis.

Virus Infections of Honeybees Apis Mellifera

PubMed Central

Tantillo, Giuseppina; Bottaro, Marilisa; Di Pinto, Angela; Martella, Vito; Di Pinto, Pietro

2015-01-01

The health and vigour of honeybee colonies are threatened by numerous parasites (such as Varroa destructor and Nosema spp.) and pathogens, including viruses, bacteria, protozoa. Among honeybee pathogens, viruses are one of the major threats to the health and well-being of honeybees and cause serious concern for researchers and beekeepers. To tone down the threats posed by these invasive organisms, a better understanding of bee viral infections will be of crucial importance in developing effective and environmentally benign disease control strategies. Here we summarize recent progress in the understanding of the morphology, genome organization, transmission, epidemiology and pathogenesis of eight honeybee viruses: Deformed wing virus (DWV) and Kakugo virus (KV); Sacbrood virus (SBV); Black Queen cell virus (BQCV); Acute bee paralysis virus (ABPV); Kashmir bee virus (KBV); Israeli Acute Paralysis Virus (IAPV); Chronic bee paralysis virus (CBPV). The review has been designed to provide researchers in the field with updated information about honeybee viruses and to serve as a starting point for future research. PMID:27800411

[Thyrotoxic periodic paralysis--an unusual complication of hyperthyroidism].

PubMed

Mellgren, Gunnar; Holm, Pål Ivar; Lien, Ernst Asbjørn; Bleskestad, Inger H; Aanderud, Sylvi; Bindoff, Laurence

2002-04-20

Thyrotoxic periodic paralysis (TPP) is a complication of hyperthyroidism. We describe two patients with TPP. A 26-year-old man from Vietnam had weight loss, tachycardia, palpitations and heat intolerance for five months. Episodic leg and arm weakness developed three months after debut of symptoms. The second patient, a 23-year old woman from the Philippines, had had episodic leg weakness in the evenings after dinner for three weeks. Her attacks resolved spontaneously overnight. Physical examination of both patients revealed tachycardia and symmetrical proximal weakness involving both arms and legs. ECG and electrolyte analysis indicated a severe hypokalaemia; thyroid function tests showed hyperthyroidism. Both patients were diagnosed as having Graves' thyrotoxicosis and TPP. They were initially treated with propranolol and subsequently with carbimazole. The first patient had recurrence of thyrotoxicosis and paralysis after 16 months, whereas the second patient has remained symptom-free. TPP is most common in Asian males, very few cases are reported in females. In Western countries TPP is rare, but with increasing immigration, TPP is likely to occur more frequently.

[Hypoglycaemic periodic paralysis in hyperthyroidism patients].

PubMed

Kratochvíl, J; Masopust, J; Martínková, V; Charvát, J

2008-11-01

Hypokalemic periodic paralysis (HPP) is a rare disorder characterised by acute, potentially fatal atacks of muscle weakness or paralysis. Massive shift of potassium into cells is caused by elevated levels of insulin and catecholamines in the blood. Hypophosphatemia and hypomagnesemia may be also present. Acidobasic status usually is not impaired. HPP occurs as familiar (caused by ion channels inherited defects) or acquired (in patients with hyperthyroidism). On the basis of two clinical cases we present a review of hypokalemic periodic paralysis in hyperthyroid patients. We discuss patogenesis, clinical and laboratory findings as well as the principles of prevention and treatment of this rare disorder.

Societal Value of Surgery for Facial Reanimation

PubMed Central

Su, Peiyi; Ishii, Lisa E.; Joseph, Andrew; Nellis, Jason; Dey, Jacob; Bater, Kristin; Byrne, Patrick J.; Boahene, Kofi D. O.; Ishii, Masaru

2017-01-01

IMPORTANCE Patients with facial paralysis are perceived negatively by society in a number of domains. Society’s perception of the health utility of varying degrees of facial paralysis and the value society places on reconstructive surgery for facial reanimation need to be quantified. OBJECTIVE To measure health state utility of varying degrees of facial paralysis, willingness to pay (WTP) for a repair, and the subsequent value of facial reanimation surgery as perceived by society. DESIGN, SETTING, AND PARTICIPANTS This prospective observational study conducted in an academic tertiary referral center evaluated a group of 348 casual observers who viewed images of faces with unilateral facial paralysis of 3 severity levels (low, medium, and high) categorized by House-Brackmann grade. Structural equation modeling was performed to understand associations among health utility metrics, WTP, and facial perception domains. Data were collected from July 16 to September 26, 2015. MAIN OUTCOMES AND MEASURES Observer-rated (1) quality of life (QOL) using established health utility metrics (standard gamble, time trade-off, and a visual analog scale) and (2) their WTP for surgical repair. RESULTS Among the 348 observers (248 women [71.3%]; 100 men [28.7%]; mean [SD] age, 29.3 [11.6] years), mixed-effects linear regression showed that WTP increased nonlinearly with increasing severity of paralysis. Participants were willing to pay $3487 (95% CI, $2362–$4961) to repair low-grade paralysis, $8571 (95% CI, $6401–$11 234) for medium-grade paralysis, and $20 431 (95% CI, $16 273–$25 317) for high-grade paralysis. The dominant factor affecting the participants’ WTP was perceived QOL. Modeling showed that perceived QOL decreased with paralysis severity (regression coefficient, −0.004; 95% CI, −0.005 to −0.004; P < .001) and increased with attractiveness (regression coefficient, 0.002; 95% CI, 0.002 to 0.003; P < .001). Mean (SD) health utility scores calculated by the standard gamble metric for low- and high-grade paralysis were 0.98 (0.09) and 0.77 (0.25), respectively. Time trade-off and visual analog scale measures were highly correlated. We calculated mean (SD) WTP per quality-adjusted life-year, which ranged from $10 167 ($14 565) to $17 008 ($38 288) for low- to high-grade paralysis, respectively. CONCLUSIONS AND RELEVANCE Society perceives the repair of facial paralysis to be a high-value intervention. Societal WTP increases and perceived health state utility decreases with increasing House-Brackmann grade. This study demonstrates the usefulness of WTP as an objective measure to inform dimensions of disease severity and signal the value society places on proper facial function. LEVEL OF EVIDENCE NA. PMID:27892977

Literature study on clinical treatment of facial paralysis in the last 20 years using Web of Science: Comparison between rehabilitation, physiotherapy and acupuncture.

PubMed

Zhang, Xiaoge; Feng, Ling; Du, Liang; Zhang, Anxiang; Tang, Tian

2012-01-15

Facial paralysis is defined as severe or complete loss of facial muscle motor function. The study was undertaken to explore a bibliometric approach to quantitatively assess the research on clinical treatment of facial paralysis using rehabilitation, physiotherapy and acupuncture using Web of Science from 1992 to 2011. Bibliometric approach. A bibliometric analysis based on the publications on Web of Science was performed using key words such as "facial paralysis", "rehabilitation", "physiotherapy" and "acupuncture". (1) Research articles on the clinical treatment of facial paralysis using acupuncture or physiotherapy (e.g. exercise, electro-stimulation) and other rehabilitation methods; (2) researches on human and animal fundamentals, clinical trials and case reports; (3) Article types: article, review, proceedings paper, note, letter, editorial material, discussion, book chapter. (4) Publication year: 1992-2011 inclusive. (1) Articles on the causes and diagnosis on facial paralysis; (2) Type of articles: correction; (3) Articles from following databases: all databases related to social science and chemical databases in Web of Science. (1) Overall number of publications; (2) number of publications annually; (3) number of citations received annually; (4) top cited paper; (5) subject categories of publication; (6) the number of countries in which the article is published; (7) distribution of output in journals. Overall population stands at 3 543 research articles addressing the clinical treatment of facial paralysis in Web of Science during the study period. There is also a markedly increase in the number of publications on the subject "facial paralysis treatments using rehabilitation" during the first decade of the 21(st) century, except in 2004 and 2006 when there are perceptible drops in the number of articles published. The only other year during the study period saw such a drop is 1993. Specifically, there are 192 published articles on facial paralysis treated by rehabilitation in the past two decades, far more than the output of physiotherapy treatment. Physiotherapy treatment scored only 25 articles including acupuncture treatment, with over 80% of these written by Chinese researchers and clinicians. Ranked by regions, USA is by far the most productive country in terms of the number of publications on facial paralysis rehabilitation and physiotherapy research. Seeing from another angle, the journals that focus on otolaryngology published the most number of articles in rehabilitation and physiotherapy studies, whereas most acupuncture studies on facial paralysis were published in the alternative and complementary medicine journals. Study of facial paralysis remains an area of active investigation and innovation. Further clinical studies in humans addressing the use of growth factors or stem cells continue to successful facial nerve regeneration.

Adhesive taping vs. daily manual muscle stretching and splinting after botulinum toxin type A injection for wrist and fingers spastic overactivity in stroke patients: a randomized controlled trial.

PubMed

Santamato, Andrea; Micello, Maria Francesca; Panza, Francesco; Fortunato, Francesca; Picelli, Alessandro; Smania, Nicola; Logroscino, Giancarlo; Fiore, Pietro; Ranieri, Maurizio

2015-01-01

To compare the effectiveness of two procedures increasing the botulinum toxin type A effect for wrist and finger flexor spasticity after stroke. A single-blind randomized trial. Seventy patients with upper limb post-stroke spasticity. Adults with wrist and finger flexor muscles spasticity after stroke were submitted to botulinum toxin type A therapy. After the treatment, the subjects injected were randomly divided into two groups and submitted to adhesive taping (Group A) or daily muscle manual stretching, passive articular mobilization of wrist and fingers, and palmar splint (Group B) for 10 days. We measured spasticity with Modified Ashworth Scale, related disability with Disability Assessment Scale, and fingers position at rest. The measurements were done at baseline, after two weeks, and after one month from the treatment session. After two weeks, subjects in Group A reported a significantly greater decrease in spasticity scores (Modified Ashworth Scale fingers: mean (standard deviation) 1.3±0.6 vs. 2.1±0.6; Modified Ashworth Scale wrist: 1.7 ±0.6 vs. 2.3 ±0.8), and after one month in spasticity and disability scores (Modified Ashworth Scale fingers: mean (standard deviation) 1.9 ±0.7 vs. 2.5 ±0.6; Modified Ashworth Scale wrist: 2.0 ±0.7 vs. 2.6 ±0.6; Disability Assessment Scale: 1.6 ±0.7 vs. 2.1 ±0.7) compared with Group B subjects. Subjects in Group A reported also a significantly improved fingers position at rest compared with Group B subjects after two weeks (2.8 ±0.9 vs. 2.1 ±0.7) and one month (2.3 ±0.7 vs. 1.5 ±0.6). Adhesive taping of wrist and finger flexor muscles appeared to enhance the effect of botulinum toxin type A therapy more than daily manual muscle stretching combined with passive articular mobilization and palmar splint. © The Author(s) 2014.

Facial paralysis for the plastic surgeon.

PubMed

Kosins, Aaron M; Hurvitz, Keith A; Evans, Gregory Rd; Wirth, Garrett A

2007-01-01

Facial paralysis presents a significant and challenging reconstructive problem for plastic surgeons. An aesthetically pleasing and acceptable outcome requires not only good surgical skills and techniques, but also knowledge of facial nerve anatomy and an understanding of the causes of facial paralysis.The loss of the ability to move the face has both social and functional consequences for the patient. At the Facial Palsy Clinic in Edinburgh, Scotland, 22,954 patients were surveyed, and over 50% were found to have a considerable degree of psychological distress and social withdrawal as a consequence of their facial paralysis. Functionally, patients present with unilateral or bilateral loss of voluntary and nonvoluntary facial muscle movements. Signs and symptoms can include an asymmetric smile, synkinesis, epiphora or dry eye, abnormal blink, problems with speech articulation, drooling, hyperacusis, change in taste and facial pain.With respect to facial paralysis, surgeons tend to focus on the surgical, or 'hands-on', aspect. However, it is believed that an understanding of the disease process is equally (if not more) important to a successful surgical outcome. The purpose of the present review is to describe the anatomy and diagnostic patterns of the facial nerve, and the epidemiology and common causes of facial paralysis, including clinical features and diagnosis. Treatment options for paralysis are vast, and may include nerve decompression, facial reanimation surgery and botulinum toxin injection, but these are beyond the scope of the present paper.

Phonatory Effects of Type I Thyroplasty Implant Shape and Depth of Medialization in Unilateral Vocal Fold Paralysis

PubMed Central

Orestes, Michael I.; Neubauer, Juergen; Sofer, Elazar; Salinas, Jonathon; Chhetri, Dinesh K.

2015-01-01

Objectives/Hypothesis Medialization thyroplasty (MT) is commonly used to treat glottic insufficiency. In this study, we investigated the phonatory effects of MT implant medialization depth and medial surface shape. Methods Recurrent laryngeal nerve (RLN) and vagal paralysis were simulated in an in vivo canine. A type 1 MT was performed using a silicone elastomer implant with variable medialization depths and medial surface shapes: rectangular, V-shaped, divergent, and convergent. The effects on phonation onset flow/pressure relationships and acoustics were measured. Results Increasing depth of medialization led to improvements in fundamental frequency (F0) range and normalization of the slope of pressure/flow relationship toward baseline activation conditions. The effects of implant medial shape also depended on depth of medialization. Outcome measures were similar among the implants at smaller medialization depths. With large medialization depths and vagal paralysis conditions, the divergent implant maintained pressure/flow relationship closer to baseline. The vagal paralysis conditions also demonstrated decreased fundamental frequency range and worse flow/pressure relationship compared to RLN paralysis. Conclusions The depth and medial shape of a medialization laryngoplasty (ML) implant significantly affect both the F0 range and aerodynamic power required for phonation. These effects become more notable with increasing depth of medialization. The study also illustrates that ML is less effective in vagal paralysis compared to RLN paralysis. PMID:25046146

Facial paralysis for the plastic surgeon

PubMed Central

Kosins, Aaron M; Hurvitz, Keith A; Evans, Gregory RD; Wirth, Garrett A

2007-01-01

Facial paralysis presents a significant and challenging reconstructive problem for plastic surgeons. An aesthetically pleasing and acceptable outcome requires not only good surgical skills and techniques, but also knowledge of facial nerve anatomy and an understanding of the causes of facial paralysis. The loss of the ability to move the face has both social and functional consequences for the patient. At the Facial Palsy Clinic in Edinburgh, Scotland, 22,954 patients were surveyed, and over 50% were found to have a considerable degree of psychological distress and social withdrawal as a consequence of their facial paralysis. Functionally, patients present with unilateral or bilateral loss of voluntary and nonvoluntary facial muscle movements. Signs and symptoms can include an asymmetric smile, synkinesis, epiphora or dry eye, abnormal blink, problems with speech articulation, drooling, hyperacusis, change in taste and facial pain. With respect to facial paralysis, surgeons tend to focus on the surgical, or ‘hands-on’, aspect. However, it is believed that an understanding of the disease process is equally (if not more) important to a successful surgical outcome. The purpose of the present review is to describe the anatomy and diagnostic patterns of the facial nerve, and the epidemiology and common causes of facial paralysis, including clinical features and diagnosis. Treatment options for paralysis are vast, and may include nerve decompression, facial reanimation surgery and botulinum toxin injection, but these are beyond the scope of the present paper. PMID:19554190

[Amyotrophic lateral sclerosis--diagnosis and treatment].

PubMed

Jung, H H; Neumann, M; Bloch, K E

2012-07-04

Amyotrophic lateral sclerosis (ALS) represents the most common motoneuron disorder in adulthood. It is characterized by selective degeneration of the motoneurons. About 10% of patients have a genetically determined ALS. Clinically, ALS is characterized by coexistence of signs of the first motoneuron, such as spasticity and hyperreflexia, as well as the second motoneuron, such as muscular atrophy and fasciculations. If such signs are present in at least three regions and if other possible causes have been excluded, a definite diagnosis of ALS can be made based on the revised El-Escorial criteria. Initial manifestations are often focalized and generalization develops during the course. The glutamate antagonist riluzole is worldwide the only approved ALS treatment. However, symptomatic treatments to ameliorate spasticity, drooling, speech and swallowing problems, and assisted ventilation to treat respiratory failure are essential.

Hereditary spastic paraplegias: membrane traffic and the motor pathway

PubMed Central

Blackstone, Craig; O’Kane, Cahir J.; Reid, Evan

2017-01-01

Voluntary movement is a fundamental way in which animals respond to, and interact with, their environment. In mammals, the main CNS pathway controlling voluntary movement is the corticospinal tract, which encompasses connections between the cerebral motor cortex and the spinal cord. Hereditary spastic paraplegias (HSPs) are a group of genetic disorders that lead to a length-dependent, distal axonopathy of fibres of the corticospinal tract, causing lower limb spasticity and weakness. Recent work aimed at elucidating the molecular cell biology underlying the HSPs has revealed the importance of basic cellular processes — especially membrane trafficking and organelle morphogenesis and distribution — in axonal maintenance and degeneration. PMID:21139634

Hereditary spastic paraplegias: membrane traffic and the motor pathway.

PubMed

Blackstone, Craig; O'Kane, Cahir J; Reid, Evan

2011-01-01

Voluntary movement is a fundamental way in which animals respond to, and interact with, their environment. In mammals, the main CNS pathway controlling voluntary movement is the corticospinal tract, which encompasses connections between the cerebral motor cortex and the spinal cord. Hereditary spastic paraplegias (HSPs) are a group of genetic disorders that lead to a length-dependent, distal axonopathy of fibres of the corticospinal tract, causing lower limb spasticity and weakness. Recent work aimed at elucidating the molecular cell biology underlying the HSPs has revealed the importance of basic cellular processes — especially membrane trafficking and organelle morphogenesis and distribution— in axonal maintenance and degeneration.

Acute barium intoxication following ingestion of ceramic glaze.

PubMed Central

Thomas, M.; Bowie, D.; Walker, R.

1998-01-01

A case of deliberate overdose of barium sulphide in a psychiatric setting is presented, with resulting flaccid paralysis, malignant arrhythmia, respiratory arrest and severe hypokalaemia, but ultimately with complete recovery. The degree of paralysis appears to be related directly to serum barium levels. The value of early haemodialysis, particularly with respiratory paralysis and hypokalaemia, is emphasised. PMID:10211330

The Effect of Alpinia zerumbet Essential Oil on Post-Stroke Muscle Spasticity.

PubMed

Maia, Maurício Oliva Nascimento; Dantas, Camila Gomes; Xavier Filho, Lauro; Cândido, Edna Aragão Farias; Gomes, Margarete Zanardo

2016-01-01

The essential oil of Alpinia zerumbet (EOAz) presents myorelaxant and antispasmodic actions on cardiac and smooth muscles. The aim of this study was to investigate the effect of EOAz on the skeletal muscle contraction in post-stroke spasticity. Fifteen adults with unilateral hemiparesis and spasticity resulting from stroke were submitted to surface electromyography readings of the gastrocnemius muscle, before and after 10 daily applications (dermal 0.05 mL per muscle belly) of EOAz. The healthy contralateral muscles without applying the oil were used as controls. The analysis showed that, in both lateral and medial gastrocnemius, the values of all studied variables (root mean square, maximum amplitude and median power frequency) were significantly decreased in pathological legs during muscle contraction (Wilcoxon test, p < 0.05). Moreover, spastic muscles presented different results before and after dermal application of EOAz: The mean values of root mean square and median power frequency were significantly increased in lateral and medial gastrocnemius, and also, the maximum amplitude increased in medial gastrocnemius (Mann-Whitney test, p < 0.05). The results suggest that EOAz acts in the skeletal spastic muscle contraction by promoting relaxation and improvement of the muscular performance. Thus, the EOAz can be useful for the clinical management of secondary effects in patients with cerebral vascular disease. © 2015 Nordic Association for the Publication of BCPT (former Nordic Pharmacological Society).

Do flexible inter-injection intervals improve the effects of botulinum toxin A treatment in reducing impairment and disability in patients with spasticity?

PubMed

Trompetto, Carlo; Marinelli, Lucio; Mori, Laura; Puce, Luca; Pelosin, Elisa; Serrati, Carlo; Fattapposta, Francesco; Rinalduzzi, Steno; Abbruzzese, Giovanni; Currà, Antonio

2017-05-01

In patients treated with botulinum toxin-A (BoNT-A), toxin-directed antibody formation was related to the dosage and frequency of injections, leading to the empirical adoption of minimum time intervals between injections of 3months or longer. However, recent data suggest that low immunogenicity of current BoNT-A preparations could allow more frequent injections. Our hypothesis is that a short time interval between injections may be safe and effective in reducing upper limb spasticity and related disability. IncobotulinumtoxinA was injected under ultrasound guidance in spastic muscles of 11 subjects, who were evaluated just before BoNT-A injection (T0), and 1month (T1), 2months (T2) and 4months (T3) after injecting. At T1, in the case of persistent disability related to spasticity interfering with normal activities, patients received an additional toxin dose. Seven subjects received the additional dose at T1 because of persistent disability; 4 of them had a decrease of disability 1month later (T2). Rethinking the injection scheme for BoNT-A treatment may have a major impact in the management of spasticity and related disability. Future studies with larger sample sizes are warranted to confirm that injection schedules with short time intervals should no longer be discouraged in clinical practice. Copyright © 2017 Elsevier Ltd. All rights reserved.

Skeletal Maturation and Mineralisation of Children with Moderate to Severe Spastic Quadriplegia

PubMed Central

Sitaraman, Sadasivan

2016-01-01

Introduction Diminished bone mineral density and delayed skeletal maturation are common in children with spastic quadriplegia. Aim The purpose of our study was to evaluate the Bone Mineral Density (BMD) of children with moderate to severe spastic quadriplegia and its relationship with other variables like nutrition and growth. Materials and Methods This was a hospital based, cross- sectional, case-control study. Forty-two (28 males, 14 females) children with spastic quadriplegia and 42 (24 males, 18 females) healthy children were included in the study. BMD of cases and control were measured by Dual Energy X-ray Absorptiometry (DEXA). Radiographs of left hand and wrist of cases and controls were taken and bone age was determined. Results BMD values of upper extremity, lower extremity, thoraco-lumbar spine and pelvis in cases were lower than those of controls (p <0.0001). In children with non severe malnutrition, 75% of the cases had lower bone age than chronological age, whereas all cases with severe malnutrition had lower bone age than chronological age. Step wise regression analysis showed that nutritional status independently contributed to lower BMD values but the BMD values did not correlate significantly with the use of anticonvulsant drugs and presence of physical therapy. Conclusion Decreased BMD and delayed bone age is prevalent in children with spastic quadriplegia and nutritional status is an important contributing factor. PMID:27504366

Contributing Factors Analysis for the Changes of the Gross Motor Function in Children With Spastic Cerebral Palsy After Physical Therapy

PubMed Central

Yi, Tae Im; Kim, Sung Heon; Han, Kyung Hee

2013-01-01

Objective To investigate the factors which contribute to the improvements of the gross motor function in children with spastic cerebral palsy after physical therapy. Methods The subjects were 45 children with spastic cerebral palsy with no previous botulinum toxin injection or operation history within 6 months. They consisted of 24 males (53.3%) and 21 females (46.7%), and the age of the subjects ranged from 2 to 6 years, with the mean age being 41±18 months. The gross motor function was evaluated by Gross Motor Function Measure (GMFM)-88 at the time of admission and discharge, and then, the subtractions were correlated with associated factors. Results The GMFM-88 was increased by 7.17±3.10 through 52±16 days of physical therapy. The more days of admission, the more improvements of GMFM-88 were attained. The children with initial GMFM-88 values in the middle range showed more improvements in GMFM-88 (p<0.05). The children without dysphagia and children with less spasticity of lower extremities also showed more improvements in GMFM-88 (p<0.05). Conclusion We can predict the improvements of the gross motor function after physical therapy according to the days of admission, initial GMFM-88, dysphagia, and spasticity of lower extremities. Further controlled studies including larger group are necessary. PMID:24236252

Systematic review of whole body vibration exercises in the treatment of cerebral palsy: Brief report.

PubMed

Sá-Caputo, Danúbia C; Costa-Cavalcanti, Rebeca; Carvalho-Lima, Rafaelle P; Arnóbio, Adriano; Bernardo, Raquel M; Ronikeile-Costa, Pedro; Kutter, Cristiane; Giehl, Paula M; Asad, Nasser R; Paiva, Dulciane N; Pereira, Heloisa V F S; Unger, Marianne; Marin, Pedro J; Bernardo-Filho, Mario

2016-10-01

Whole body vibration (WBV) is increasingly being used to improve balance and motor function and reduce the secondary complications associated with cerebral palsy (CP). The purpose of this study was to systematically appraise published research regarding the effects of static and/or dynamic exercise performed on a vibrating platform on gait, strength, spasticity and bone mineral density (BMD) within this population. Systematic searches of six electronic databases identified five studies that met our inclusion criteria (2 at Level II and 3 at Level III-2). Studies were analysed to determine: (a) participant characteristics; (b) optimal exercise and WBV treatment protocol; (c) effect on gait, strength, spasticity and BMD; and (d) the outcome measures used to evaluate effect. As data was not homogenous a meta-analysis was not possible. Several design limitations were identified and intervention protocols are poorly described. The effects on strength, gait, spasticity and BMD in persons with CP remain inconclusive with weak evidence that WBV may improve selected muscle strength and gait parameters and that prolonged exposure may improve BMD; there is currently no evidence that WBV can reduce spasticity. The evidence for exercise performed on a vibrating platform on mobility, strength, spasticity and BMD in CP remains scant and further larger scale investigations with controlled parameters to better understand the effects of WBV exercises in this population is recommended.

THC and CBD oromucosal spray (Sativex®) in the management of spasticity associated with multiple sclerosis.

PubMed

Sastre-Garriga, Jaume; Vila, Carlos; Clissold, Stephen; Montalban, Xavier

2011-05-01

People with multiple sclerosis may present with a wide range of disease symptoms during the evolution of the disease; among these, spasticity can have a marked impact on their well-being and quality of life. Symptom control, including spasticity, remains a key management strategy to improve the patient's well-being and functional status. However, available drug therapies for spasticity sometimes have limited benefit and they are often associated with poor tolerability. Sativex is a 1:1 mix of 9-delta-tetrahydrocannabinol and cannabidiol extracted from cloned Cannabis sativa chemovars, which is available as an oromucosal spray. Clinical experience with Sativex in patients with multiple sclerosis is accumulating steadily. Results from randomized, controlled trials have reported a reduction in the severity of symptoms associated with spasticity, leading to a better ability to perform daily activities and an improved perception of patients and their carers regarding functional status when Sativex was added to the current treatment regimen. Adverse events such as dizziness, diarrhea, fatigue, nausea, headache and somnolence occur quite frequently with Sativex, but they are generally of mild-to-moderate intensity and their incidence can be markedly reduced by gradual 'uptitration'. In summary, initial well-controlled studies with Sativex oromucosal spray administered as an add-on to usual therapy have produced promising results and highlight encouraging avenues for future research.

Development of an Intelligent Stretching Device for Ankle Joints With Contracture/Spasticity

DTIC Science & Technology

2001-10-25

percentage corresponded to background dorsi-flexion muscle contraction and 0% was the relaxed state. Next, tendon reflexes were evaluated...the representative cases, joint stiffness was reduced markedly after stretching across the range of muscle contraction (Fig. 5), including both

Masseteric nerve for reanimation of the smile in short-term facial paralysis.

PubMed

Hontanilla, Bernardo; Marre, Diego; Cabello, Alvaro

2014-02-01

Our aim was to describe our experience with the masseteric nerve in the reanimation of short term facial paralysis. We present our outcomes using a quantitative measurement system and discuss its advantages and disadvantages. Between 2000 and 2012, 23 patients had their facial paralysis reanimated by masseteric-facial coaptation. All patients are presented with complete unilateral paralysis. Their background, the aetiology of the paralysis, and the surgical details were recorded. A retrospective study of movement analysis was made using an automatic optical system (Facial Clima). Commissural excursion and commissural contraction velocity were also recorded. The mean age at reanimation was 43(8) years. The aetiology of the facial paralysis included acoustic neurinoma, fracture of the skull base, schwannoma of the facial nerve, resection of a cholesteatoma, and varicella zoster infection. The mean time duration of facial paralysis was 16(5) months. Follow-up was more than 2 years in all patients except 1 in whom it was 12 months. The mean duration to recovery of tone (as reported by the patient) was 67(11) days. Postoperative commissural excursion was 8(4)mm for the reanimated side and 8(3)mm for the healthy side (p=0.4). Likewise, commissural contraction velocity was 38(10)mm/s for the reanimated side and 43(12)mm/s for the healthy side (p=0.23). Mean percentage of recovery was 92(5)mm for commissural excursion and 79(15)mm/s for commissural contraction velocity. Masseteric nerve transposition is a reliable and reproducible option for the reanimation of short term facial paralysis with reduced donor site morbidity and good symmetry with the opposite healthy side. Crown Copyright © 2013. Published by Elsevier Ltd. All rights reserved.

Quantitative analysis of poliomyelitis-like paralysis in mice induced by a poliovirus replicon.

PubMed

Arita, Minetaro; Nagata, Noriyo; Sata, Tetsutaro; Miyamura, Tatsuo; Shimizu, Hiroyuki

2006-11-01

Poliovirus (PV) infection causes severe paralysis, typically of the legs, by destruction of the motor neurons in the spinal cord. In this study, the relationship between PV replication in the spinal cord, damage in the motor neurons and poliomyelitis-like paralysis was analysed in transgenic mice expressing the human PV receptor (TgPVR21). First, a PV replicon encoding firefly luciferase in place of the capsid genes (PV-Fluc mc) was trans-encapsidated in 293T cells and the trans-encapsidated PV-Fluc mc (TE-PV-Fluc mc) was then inoculated into the spinal cords of TgPVR21 mice. TE-PV-Fluc mc was recovered with a titre of 6.3 x 10(7) infectious units ml(-1), which was comparable to those of PV1 strains. TgPVR21 mice inoculated with TE-PV-Fluc mc showed non-lethal paralysis of the hindlimbs, with severity ranging from a decline in grip strength to complete flaccid paralysis. The replication of TE-PV-Fluc mc in the spinal cord reached peak levels at 10 h post-inoculation (p.i.), followed by the appearance of paralysis at as early as 12 h p.i., reaching a plateau at 16 h p.i. Histological analysis showed a correlation between the lesion and the severity of the clinical symptoms in most mice. However, severe paralysis could also be observed with an apparently low lesion score, where as few as 5.3 x 10(2) motor neurons (1.4 % of the susceptible cells in the lumbar cord) were infected by TE-PV-Fluc mc. These results indicate that PV replication in a small population of the motor neurons was critical for severe residual poliomyelitis-like paralysis in TgPVR21 mice.

Innovative strength training-induced neuroplasticity and increased muscle size and strength in children with spastic cerebral palsy: an experimenter-blind case study--three-month follow-up.

PubMed

Lee, Dong Ryul; Kim, Yun Hee; Kim, Dong A; Lee, Jung Ah; Hwang, Pil Woo; Lee, Min Jin; You, Sung Hyun

2014-01-01

In children with cerebral palsy (CP), the never-learned-to-use (NLTU) effect and underutilization suppress the normal development of cortical plasticity in the paretic limb, which further inhibits its functional use and increases associated muscle weakness. To highlight the effects of a novel comprehensive hand repetitive intensive strengthening training system on neuroplastic changes associated with upper extremity (UE) muscle strength and motor performance in children with spastic hemiplegic CP. Two children with spastic hemiplegic CP were recruited. Intervention with the comprehensive hand repetitive intensive strengthening training system was provided for 60 min a day, three times a week, for 10 weeks. Neuroplastic changes, muscle size, strength, and associated motor function were measured using functional magnetic resonance imaging (MRI), ultrasound imaging, and standardized motor tests, respectively. The functional MRI data showed that the comprehensive hand repetitive intensive strengthening training intervention produced measurable neuroplastic changes in the neural substrates associated with motor control and learning. These neuroplastic changes were associated with increased muscle size, strength and motor function. These results provide compelling evidence of neuroplastic changes and associated improvements in muscle size and motor function following innovative upper extremity strengthening exercise.

Heart Rate Variability (HRV) modifications in adult hemiplegic patients after botulinum toxin type A (nt-201) injection.

PubMed

Invernizzi, M; Carda, S; Molinari, C; Stagno, D; Cisari, C; Baricich, A

2015-08-01

The most important adverse effect of BoNT-A is the systemic diffusion of the toxin. There is some evidence that the administration of high doses can increase the risk of systemic diffusion and the development of clinically evident adverse effects, however an international consensus does not exist about its maximum dose. The aim of this study was to evaluate changes in autonomic heart drive induced by high doses (higher than 600 units) of incobotulinumtoxinA injection in spastic stroke patients. Moreover, the treatment safety by monitoring adverse events occurrence was assessed. Case control study. Eleven stroke survivors with spastic hemiplegia. Patients were treated with intramuscular focal injections of IncobotulinumtoxinA (NT 201; Xeomin®, Merz Pharmaceuticals GmbH, Frankfurt, Germany). Doses were below 12 units/Kg. Each patient underwent an ECG recording before injection and 10 days after treatment. Linear and non-linear Heart Rate variability (HRV) measures were derived from ECGs with a dedicated software. None of the variable considered showed statistically significant changes after BoNT-A injection. The use of incobotulinumtoxinA in adult patients at doses up to 12 units/kg seems to be safe regarding autonomic heart drive. The use of IncobotulinumtoxinA up to 600 units could be a safe therapeutic option in spastic hemiplegic stroke survivors.

Consensus statement: Using laryngeal electromyography for the diagnosis and treatment of vocal cord paralysis.

PubMed

Munin, Michael C; Heman-Ackah, Yolanda D; Rosen, Clark A; Sulica, Lucian; Maronian, Nicole; Mandel, Steven; Carey, Bridget T; Craig, Earl; Gronseth, Gary

2016-06-01

The purpose of this study was to develop an evidence-based consensus statement regarding use of laryngeal electromyography (LEMG) for diagnosis and treatment of vocal fold paralysis after recurrent laryngeal neuropathy (RLN). Two questions regarding LEMG were analyzed: (1) Does LEMG predict recovery in patients with acute unilateral or bilateral vocal fold paralysis? (2) Do LEMG findings change clinical management in these individuals? A systematic review was performed using American Academy of Neurology criteria for rating of diagnostic accuracy. Active voluntary motor unit potential recruitment and presence of polyphasic motor unit potentials within the first 6 months after lesion onset predicted recovery. Positive sharp waves and/or fibrillation potentials did not predict outcome. The presence of electrical synkinesis may decrease the likelihood of recovery, based on 1 published study. LEMG altered clinical management by changing the initial diagnosis from RLN in 48% of cases. Cricoarytenoid fixation and superior laryngeal neuropathy were the most common other diagnoses observed. If prognostic information is required in a patient with vocal fold paralysis that is more than 4 weeks and less than 6 months in duration, then LEMG should be performed. LEMG may be performed to clarify treatment decisions for vocal fold immobility that is presumed to be caused by RLN. Muscle Nerve 53: 850-855, 2016. © 2016 Wiley Periodicals, Inc.

Laryngeal paralysis in dogs: an update on recent knowledge.

PubMed

Kitshoff, Adriaan M; Van Goethem, Bart; Stegen, Ludo; Vandekerckhov, Peter; de Rooster, Hilde

2013-04-05

Laryngeal paralysis is the effect of an inability to abduct the arytenoid cartilages during inspiration, resulting in respiratory signs consistent with partial airway obstruction. The aetiology of the disease can be congenital (hereditary laryngeal paralysis or congenital polyneuropathy), or acquired (trauma, neoplasia, polyneuropathy, endocrinopathy). The most common form of acquired laryngeal paralysis (LP) is typically seen in old, large breed dogs and is a clinical manifestation of a generalised peripheral polyneuropathy recently referred to as geriatric onset laryngeal paralysis polyneuropathy. Diagnosing LP based on clinical signs, breed and history has a very high sensitivity (90%) and can be confirmed bylaryngeal inspection. Prognosis after surgical correction depends on the aetiology: traumatic cases have a good prognosis, whereas tumour-induced or polyneuropathy-induced LP has a guarded prognosis. Acquired idiopathic LP is a slow progressive disease, with dogs reaching median survival times of 3-5 years after surgical correction.

Overview of pediatric peripheral facial nerve paralysis: analysis of 40 patients.

PubMed

Özkale, Yasemin; Erol, İlknur; Saygı, Semra; Yılmaz, İsmail

2015-02-01

Peripheral facial nerve paralysis in children might be an alarming sign of serious disease such as malignancy, systemic disease, congenital anomalies, trauma, infection, middle ear surgery, and hypertension. The cases of 40 consecutive children and adolescents who were diagnosed with peripheral facial nerve paralysis at Baskent University Adana Hospital Pediatrics and Pediatric Neurology Unit between January 2010 and January 2013 were retrospectively evaluated. We determined that the most common cause was Bell palsy, followed by infection, tumor lesion, and suspected chemotherapy toxicity. We noted that younger patients had generally poorer outcome than older patients regardless of disease etiology. Peripheral facial nerve paralysis has been reported in many countries in America and Europe; however, knowledge about its clinical features, microbiology, neuroimaging, and treatment in Turkey is incomplete. The present study demonstrated that Bell palsy and infection were the most common etiologies of peripheral facial nerve paralysis. © The Author(s) 2014.

Clinical features and management of facial nerve paralysis in children: analysis of 24 cases.

PubMed

Cha, H E; Baek, M K; Yoon, J H; Yoon, B K; Kim, M J; Lee, J H

2010-04-01

To evaluate the causes, treatment modalities and recovery rate of paediatric facial nerve paralysis. We analysed 24 cases of paediatric facial nerve paralysis diagnosed in the otolaryngology department of Gachon University Gil Medical Center between January 2001 and June 2006. The most common cause was idiopathic palsy (16 cases, 66.7 per cent). The most common degree of facial nerve paralysis on first presentation was House-Brackmann grade IV (15 of 24 cases). All cases were treated with steroids. One of the 24 cases was also treated surgically with facial nerve decompression. Twenty-two cases (91.6 per cent) recovered to House-Brackmann grade I or II over the six-month follow-up period. Facial nerve paralysis in children can generally be successfully treated with conservative measures. However, in cases associated with trauma, radiological investigation is required for further evaluation and treatment.

An fMRI investigation of racial paralysis.

PubMed

Norton, Michael I; Mason, Malia F; Vandello, Joseph A; Biga, Andrew; Dyer, Rebecca

2013-04-01

We explore the existence and underlying neural mechanism of a new norm endorsed by both black and white Americans for managing interracial interactions: "racial paralysis', the tendency to opt out of decisions involving members of different races. We show that people are more willing to make choices--such as who is more intelligent, or who is more polite-between two white individuals (same-race decisions) than between a white and a black individual (cross-race decisions), a tendency which was evident more when judgments involved traits related to black stereotypes. We use functional magnetic resonance imaging to examine the mechanisms underlying racial paralysis, to examine the mechanisms underlying racial paralysis, revealing greater recruitment of brain regions implicated in socially appropriate behavior (ventromedial prefrontal cortex), conflict detection (anterior cingulate cortex), deliberative processing (dorsolateral prefrontal cortex), and inhibition (ventrolateral prefrontal cortex). We also discuss the impact of racial paralysis on the quality of interracial relations.

Causes and imaging manifestations of paralysis of the recurrent laryngeal nerve.

PubMed

Méndez Garrido, S; Ocete Pérez, R F

2016-01-01

The vocal cords play a key role in the functions of the larynx. Their motor innervation depends on the recurrent laryngeal nerve (a branch of the tenth cranial nerve), which follows a long trajectory comprising intracranial, cervical, and mediastinal segments. Vocal cord paralysis usually manifests as dysphonia, the main symptom calling for CT study, the first-line imaging test to investigate the cause of the lesion. Patients are asymptomatic in a third of cases, so the incidental detection of signs of vocal cord paralysis in a CT study done for other reasons should prompt a search for a potentially severe occult lesion. This article aims to familiarize readers with the anatomy of the motor innervation of the glottis, the radiological presentation and most common causes of vocal cord paralysis, and conditions that can simulate vocal cord paralysis. Copyright © 2016 SERAM. Published by Elsevier España, S.L.U. All rights reserved.

Review of Intrathecal Baclofen Therapy for Spastic and Rigidity Disorders

ERIC Educational Resources Information Center

Obringer, S. John; Coffey, Kenneth M.

2002-01-01

Intrathecal baclofen therapy, a treatment for cerebral palsy and other spastic and rigidity disorders, is showing promise as an effective intervention. This article synthesizes both the medical and rehabilitation conceptual literature to update educators and related service providers as to the efficacy of this intervention. Implications for…

Neurologists' Views of Current Medications: Spasticity and Athetosis

ERIC Educational Resources Information Center

Obringer, S. John; Coffey, Kenneth

2008-01-01

Medications to treat individuals with cerebral palsy have increased significantly over the last few decades. The purpose of this article was to randomly survey practicing neurologists on a national level to determine prescribing patterns for both spasticity and athetosis. The results indicated that the most frequently prescribed medication for…

Classification of Topographical Pattern of Spasticity in Cerebral Palsy: A Registry Perspective

ERIC Educational Resources Information Center

Reid, Susan M.; Carlin, John B.; Reddihough, Dinah S.

2011-01-01

This study used data from a population-based cerebral palsy (CP) registry and systematic review to assess the amount of heterogeneity between registries in topographical patterns when dichotomised into unilateral (USCP) and bilateral spastic CP (BSCP), and whether the terms diplegia and quadriplegia provide useful additional epidemiological…

Operant Control of Pathological Tongue Thrust in Spastic Cerebral Palsy.

ERIC Educational Resources Information Center

Thompson, George A., Jr.

1979-01-01

The behavior modification procedure, carried out at mealtime with a ten-year-old retarded boy who had spastic cerebral palsy, consisted of differential reinforcement and punishment, and resulted in substantial decreases in tongue thrust (reverse swallowing) and food expulsion, and a large increase in observed chewing. (Author/DLS)

Thyrotoxic periodic paralysis: a case report and literature review.

PubMed

Barahona, M J; Vinagre, I; Sojo, L; Cubero, J M; Pérez, Antonio

2009-09-01

We describe a 37-year-old man with a 4-month history of episodic muscular weakness, involving mainly lower-limbs. Hypokalemia was documented in one episode and managed with intravenous potassium chloride. Hyperthyroidism was diagnosed 4 months after onset of attacks because of mild symptoms. The patient was subsequently diagnosed as having thyrotoxic periodic paralysis associated with Graves' disease. Treatment with propranolol and methimazol was initiated and one year later he remains euthyroid and symptom free. Thyrotoxic periodic paralysis is a rare disorder, especially among Caucasians, but it should always be considered in patients with acute paralysis and hypokalemia, and thyroid function should be evaluated.

Laryngeal and pharyngeal dysfunction in horses homozygous for hyperkalemic periodic paralysis.

PubMed

Carr, E A; Spier, S J; Kortz, G D; Hoffman, E P

1996-08-15

Evaluate histories, clinical signs, and laboratory data of 69 horses homozygous by DNA testing for hyperkalemic periodic paralysis (HPP). Cohort study. 69 of 189 horses testing homozygous for HPP between October 1992 and November 1994. Questionnaires addressing signalment, training regimes, medical history, and current status of affected horses were sent to owners, trainers, or attending veterinarians. Data from completed questionnaires were tabulated and evaluated, using descriptive statistics. Sixty-nine (37%) of 189 questionnaires were completed and returned. Clinical episodes of muscle weakness or paralysis varied in severity and frequency from mild muscle fasciculations to recumbency and death. Sixty-three of 68 HPP-affected horses were reported to have had stridor associated with exercise, excitement, stress, or episodes of muscle paralysis. Common endoscopic findings in affected horses included pharyngeal collapse, pharyngeal edema, laryngopalatal dislocation, and laryngeal paralysis. Twelve of 27 horses receiving acetazolamide had decreases in stridor while receiving medication. Most horses testing homozygous for HPP had clinical signs associated with pharyngeal and laryngeal dysfunction. Hyperkalemic periodic paralysis should be included on a differential list for horses examined for signs of laryngeal or pharyngeal dysfunction or stridor. Treatment with acetazolamide may help to control respiratory tract signs associated with this disease.

The effect of width of facial canal in patients with idiopathic peripheral facial paralysis on the development of paralysis.

PubMed

Eksi, Guldem; Akbay, Ercan; Bayarogullari, Hanifi; Cevik, Cengiz; Yengil, Erhan; Ozler, Gul Soylu

2015-09-01

The aim of this prospective study is to investigate whether the possible stenosis due to anatomic variations of labyrinthine segment (LS), tympanic segment (TS) and mastoid segment (MS) of the facial canal in the temporal bone is a predisposing factor in the development of paralysis. 22 patients with idiopathic peripheral facial paralysis (IPFP) were included in the study. Multi-slice computed tomography (MSCT) with 64 detectors was used for temporal bone imaging of the patients. Reconstruction images in axial, coronal and sagittal planes were created in workstation computers from the captured images. The diameters and lengths of LS, TS and MS of the facial canal were measured. The mean values of LD, ND and SL of LS were 1.31 ± 0.39, 0.91 ± 0.27, 4.17 ± 0.48 in patient group and 1.26 ± 0.29, 0.95 ± 0.21, 4.60 ± 1.36 in control group, respectively. The mean values of LD, ND and SL of TS were 1.11 ± 0.22, 0.90 ± 0.14, 12.63 ± 1.47 in patient group and 1.17 ± 0.23, 0.85 ± 0.24, 12.10 ± 1.79 in control group, respectively. The mean values of LD, ND and SL of MS were 1.80 ± 0.30, 1.44 ± 0.29 vs. 14.3 ± 1.90 in patient group 1.74 ± 0.38, 1.40 ± 0.29, 14.15 ± 2.16 in control group, respectively. The measurements of the parameters of all three segments in patient group and control group were similar. Similar results between patient and control group were obtained in this study investigating the effect of stenosis in facial canal in the development of IPFP.

Joint Angular Velocity in Spastic Gait and the Influence of Muscle-Tendon Lengthening*

PubMed Central

GRANATA, KEVIN P.; ABEL, MARK F.; DAMIANO, DIANE L.

2006-01-01

Background Joint angular velocity (the rate of flexion and extension of a joint) is related to the dynamics of muscle activation and force generation during walking. Therefore, the goal of this research was to examine the joint angular velocity in normal and spastic gait and changes resulting from muscle-tendon lengthening (recession and tenotomy) in patients who have spastic cerebral palsy. Methods The gait patterns of forty patients who had been diagnosed with spastic cerebral palsy (mean age, 8.3 years; range, 3.7 to 14.8 years) and of seventy-three age-matched, normally developing subjects were evaluated with three-dimensional motion analysis and electromyography. The patients who had cerebral palsy were evaluated before muscle-tendon lengthening and nine months after treatment. Results The gait patterns of the patients who had cerebral palsy were characterized by increased flexion of the knee in the stance phase, premature plantar flexion of the ankle, and reduced joint angular velocities compared with the patterns of the normally developing subjects. Even though muscle-tendon lengthening altered sagittal joint angles in gait, the joint angular velocities were generally unchanged at the hip and knee. Only the ankle demonstrated modified angular velocities, including reduced dorsiflexion velocity at foot-strike and improved dorsiflexion velocity through midstance, after treatment. Electromyographic changes included reduced amplitude of the gastrocnemius-soleus during the loading phase and decreased knee coactivity (the ratio of quadriceps and hamstring activation) at toe-off. Principal component analyses showed that, compared with joint-angle data, joint angular velocity was better able to discriminate between the gait patterns of the normal and cerebral palsy groups. Conclusions This study showed that muscle-tendon lengthening corrects biomechanical alignment as reflected by changes in sagittal joint angles. However, joint angular velocity and electromyographic data suggest that the underlying neural input remains largely unchanged at the hip and knee. Conversely, electromyographic changes and changes in velocity in the ankle indicate that the activation pattern of the gastrocnemius-soleus complex in response to stretch was altered by recession of the complex. PMID:10682726

Familial congenital bilateral vocal fold paralysis: a novel gene translocation.

PubMed

Hsu, Amy K; Rosow, David E; Wallerstein, Robert J; April, Max M

2015-03-01

True vocal fold (TVF) paralysis is a common cause of neonatal stridor and airway obstruction, though bilateral TVF paralysis is seen less frequently. Rare cases of familial congenital TVF paralysis have been described with implied genetic origin, but few genetic abnormalities have been discovered to date. The purpose of this study is to describe a novel chromosomal translocation responsible for congenital bilateral TVF immobility. The charts of three patients were retrospectively reviewed: a 35 year-old woman and her two children. The mother had bilateral TVF paralysis at birth requiring tracheotomy. Her oldest child had a similar presentation at birth and also required tracheotomy, while the younger child had laryngomalacia without TVF paralysis. Standard karyotype analysis was done using samples from all three patients and the parents of the mother, to assess whether a chromosomal abnormality was responsible. Karyotype analysis revealed the same balanced translocation between chromosomes 5 and 14, t(5;14) (p15.3, q11.2) in the mother and her two daughters. No other genetic abnormalities were identified. Neither maternal grandparent had the translocation, which appeared to be a spontaneous mutation in the mother with autosomal dominant inheritance and variable penetrance. A novel chromosomal translocation was identified that appears to be responsible for familial congenital bilateral TVF paralysis. While there are other reports of genetic abnormalities responsible for this condition, we believe this is the first describing this particular translocation. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Proximal femoral resection and articulated hip distraction with an external fixator for the treatment of painful spastic hip dislocations in pediatric patients with spastic quadriplegia.

PubMed

Lampropulos, Mario; Puigdevall, Miguel H; Zapozko, Daniel; Malvárez, Héctor R

2008-01-01

We describe the results obtained with an alternative method of treatment for spastic painful hip dislocations in nonambulatory patients, which consists of a proximal femoral resection with capsular interposition arthroplasty, and the addition of a hinged external fixator for postoperative articulated hip distraction to allow for an immediate upright position and the ability to sit in a wheelchair. We performed this technique in three patients (four hips) with a mean age at the time of surgery of 15 years. Postoperatively, clinical improvement was observed in all four hips, with respect to pain relief, sitting tolerance, perineal care and functional range of motion.

NIPA1 Gene Mutations Cause Autosomal Dominant Hereditary Spastic Paraplegia (SPG6)

PubMed Central

Rainier, Shirley; Chai, Jing-Hua; Tokarz, Debra; Nicholls, Robert D.; Fink, John K.

2003-01-01

The hereditary spastic paraplegias (HSPs) are genetically heterogeneous disorders characterized by progressive lower-extremity weakness and spasticity. The molecular pathogenesis is poorly understood. We report discovery of a dominant negative mutation in the NIPA1 gene in a kindred with autosomal dominant HSP (ADHSP), linked to chromosome 15q11-q13 (SPG6 locus); and precisely the same mutation in an unrelated kindred with ADHSP that was too small for meaningful linkage analysis. NIPA1 is highly expressed in neuronal tissues and encodes a putative membrane transporter or receptor. Identification of the NIPA1 function and ligand will aid an understanding of axonal neurodegeneration in HSP and may have important therapeutic implications. PMID:14508710

Severe exacerbation of Andersen-Tawil syndrome secondary to thyrotoxicosis.

PubMed

Díaz-Manera, Jordi; Querol, Luis; Alejaldre, Aída; Rojas-García, Ricard; Ramos-Fransi, Alba; Gallardo, Eduard; Illa, Isabel

2014-08-01

Thyrotoxic periodic paralysis (TPP) is a rare complication of hyperthyroidism characterized by episodes of weakness. Although TPP has been described in patients all over the world, it is especially frequent in Asiatic patients. Recently, two genomewide association studies have found a susceptibility locus on chromosome 17q24.3 near the KCNJ2 gene, which is responsible for another cause of periodic paralysis, the Andersen-Tawil syndrome (ATS). We report the first patient diagnosed with ATS with a de novo c.G899C mutation in the KCNJ2 gene in 2010 who developed an autoimmune hyperthyroidism and TPP in 2013. At the time of the ATS diagnosis other causes of periodic paralysis, including thyroid dysfunction, were ruled out. The condition of the patient, who had mild episodes of proximal weakness at follow-up, deteriorated dramatically in 2013, presenting continuous episodes of severe generalized weakness associated with low levels of potassium requiring frequent admissions to the hospital. After a few months, he also presented signs of hyperthyroidism, and a diagnosis of Grave's disease was made. In our opinion, this case clearly demonstrates that a dysfunction of the Kir2.1 potassium channel encoded by the KCNJ2 gene is a risk factor to develop TPP, and can be a useful tool to identify patients at risk in daily clinics.

Sjögren syndrome presenting with hypopotassemic periodic paralysis due to renal tubular acidosis

PubMed Central

Ataoglu, Esra Hayriye; Demir, Betul; Tuna, Mazhar; Çavus, Bilger; Cetin, Faik; Temiz, Levent Umit; Ozturk, Savas; Yenigun, Mustafa

2012-01-01

Summary Background: Sjögren syndrome (SS) is an autoimmune-lymphoproliferative disorder characterized by mononuclear cell infiltration of exocrine glands. Clinically, Sjögren syndrome (SS) has a wide spectrum, varying from autoimmune exocrinopathy to systemic involvement. There have been few cases reporting that primary SS developed with distal renal tubular acidosis clinically. Case Report: Here, we present a case with primary Sjögren syndrome accompanied by hypopotassemic paralysis due to renal tubular acidosis. Severe hypopotassemia, hyperchloremic metabolic acidosis, alkaline urine and disorder in urinary acidification test were observed in the biochemical examination of the 16-year-old female patient, who had applied to our clinic for extreme loss of muscle force. After the examinations it was determined that the patient had developed Type 1 RTA (distal RTA) due to primary Sjögren syndrome. Potassium and alkaline replacement was made and an immediate total recovery was achieved. Conclusions: Hypopotassemic paralysis due to primary Sjögren syndrome is a rare but severe disorder that could lead to death if not detected early and cured appropriately. Thus, effective treatment should be immediately initiated in cases where severe hypopotassemia is accompanied by metabolic acidosis, and the cases should also be examined for extraglandular involvement of SS. PMID:23569525

Gait Trainer for Children with Spastic Cerebral Palsy

DTIC Science & Technology

2001-10-25

same person. Spastic CP is the most common type of Cerebral Palsy. It can be subdivided into 5 types : Quadriplegia : A type of CP when occurs in...all four of their limbs-both arms and both legs it is called quadriplegia . Due to the problems of controlling the muscles in their face and upper

Quantitative and Functional Analyses of Spastin in the Nervous System: Implications for Hereditary Spastic Paraplegia

DTIC Science & Technology

2008-02-27

equivalent levels of expression of the spastin constructs, as assessed by fluorescence intensity of the Sp/AAA antibody staining. Microtu- bule levels...Errico A, Ballabio A, Rugarli EI (2002) Spastin, the protein mutated in au- tosomal dominant hereditary spastic paraplegia, is involved in microtu- bule

Mental Imagery Abilities in Adolescents with Spastic Diplegic Cerebral Palsy

ERIC Educational Resources Information Center

Courbois, Yanick; Coello, Yann; Bouchart, Isabelle

2004-01-01

Four visual imagery tasks were presented to three groups of adolescents with or without spastic diplegic cerebral palsy. The first group was composed of six adolescents with cerebral palsy who had associated visual-perceptual deficits (CP-PD), the second group was composed of five adolescents with cerebral palsy and no associated visual-perceptual…

Autosomal recessive mutilating sensory neuropathy with spastic paraplegia maps to chromosome 5p15.31-14.1.

PubMed

Bouhouche, Ahmed; Benomar, Ali; Bouslam, Naima; Ouazzani, Reda; Chkili, Taïeb; Yahyaoui, Mohamed

2006-02-01

Autosomal recessive ulcero-mutilating neuropathy with spastic paraplegia is a very rare disease since only few cases were described up to date. We report in this study a consanguineous Moroccan family with four affected males with this syndrome. The disease onset was in early infancy, with spastic paraplegia and sensory loss leading to mutilating acropathy. Electrophysiological studies revealed a severe axonal sensory neuropathy, magnetic resonance imaging ruled out compression of spinal cord and biological investigations showed decreased levels of Apo B, total cholesterol and triglycerides. A genomewide search was conducted in this family and linkage was found to chromosome 5p. Analysis of recombination events and LOD score calculation map the responsible gene in a 25 cM genetic interval between markers D5S2054 and D5S648. A maximum LOD score value of 3.92 was obtained for all markers located in this candidate interval. This study establishes the presence of a locus for autosomal recessive mutilating sensory neuropathy with spastic paraplegia on chromosome 5p15.31-14.1.

Neurological manifestations in individuals with HTLV-1-associated myelopathy/tropical spastic paraparesis in the Amazon.

PubMed

Dias, G A S; Yoshikawa, G T; Koyama, R V L; Fujihara, S; Martins, L C S; Medeiros, R; Quaresma, J A S; Fuzii, H T

2016-02-01

A cross-sectional observational study was conducted. The aim was to analyze the clinical-functional profile of patients diagnosed with HTLV-1 (human T-lymphotropic virus type 1)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) in the Amazon region. Reference center for HTLV in the city of Belém, state of Pará, Brazil. Muscle strength, muscle tone, balance and the need for gait assistance among patients with HAM/TSP were evaluated. Among the 82 patients infected with HTLV-1, 27 (10 men and 17 women) were diagnosed with HAM/TSP. No statistically significant difference in muscle tone or strength was found between the lower limbs. Muscle weakness and spasticity were predominant in the proximal lower limbs. Patients with HAM/TSP are at a high risk of falls (P=0.03), and predominantly use either a cane or a crutch on one side as a gait-assistance device (P=0.02). Patients with HAM/TSP exhibit a similar clinical pattern of muscle weakness and spasticity, with a high risk of falls, requiring gait-assistance devices.

Efficacy and safety of oral baclofen in the management of spasticity: A rationale for intrathecal baclofen.

PubMed

Ertzgaard, Per; Campo, Claudia; Calabrese, Alessandra

2017-03-06

Oral baclofen has long been a mainstay in the management of spasticity. This review looks at the clinical evidence for the efficacy and safety of oral baclofen in patients with spasticity of any origin or severity, to determine whether there is a rationale for the use of intrathecal baclofen. Results suggest that oral baclofen may be effective in many patients with spasticity, regardless of the underlying disease or severity, and that it is at least comparable with other antispasmodic agents. However, adverse effects, such as muscle weakness, nausea, somnolence and paraesthesia, are common with oral baclofen, affecting between 25% and 75% of patients, and limiting its usefulness. Intrathecal baclofen may be an effective alternative as the drug is delivered directly into the cerebrospinal fluid, thus bypassing the blood-brain barrier and thereby optimizing the efficacy of baclofen while minimizing drug-related side-effects. Intrathecal baclofen is a viable option in patients who experience intolerable side-effects or who fail to respond to the maximum recommended dose of oral baclofen.

Palatability and oral cavity tolerability of THC:CBD oromucosal spray and possible improvement measures in multiple sclerosis patients with resistant spasticity: a pilot study.

PubMed

Lus, Giacomo; Cantello, Roberto; Danni, Maura Chiara; Rini, Agusto; Sarchielli, Paola; Tassinari, Tiziana; Signoriello, Elisabetta

2018-04-01

Complaints about Δ 9 -tetrahydrocannabinol (THC):cannabidiol (CBD) oromucosal spray (Sativex ® ; GW Pharma Ltd, Salisbury, UK) in the management of multiple sclerosis spasticity include unpleasant taste and oral mucosal anomalies. This pilot study assessed the use of sugar-free chewing gum and/or a refrigerated bottle of THC:CBD oromucosal spray to mitigate these effects. Patients with multiple sclerosis spasticity (n = 52) at six sites in Italy who were receiving THC:CBD oromucosal spray and had associated oral mucosal effects were randomized into Group A (chewing gum; n = 15); Group B (cold bottle; n = 20); and Group C (cold bottle + chewing gum; n = 17). Taste perception in patients receiving chewing gum ± cold bottle intervention (Groups A and C combined) was significantly (p = 0.0001) improved from baseline to week 4 while maintaining spasticity control. Patient comfort, satisfaction and treatment adherence may benefit from these interventions.

What place for ▾ cannabis extract in MS?

PubMed

2012-12-01

Multiple sclerosis (MS) is a neurological condition that is estimated to affect around 60,000 people in England and Wales, with a lifetime risk in the UK of 1 in 1,000.(1,2) Spasticity (an increase in muscle tone) is a common symptom of MS, resulting in muscle spasms, immobility, disturbed sleep and pain.(3,4) Complex drug combinations are sometimes necessary to manage symptoms of MS, but these are often only partially effective and associated with unacceptable side effects.(5) Cannabis extract containing delta9-tetrahydrocannabinol (dronabinol) and cannabidiol are the principal extracts from the cannabis plant present in a licensed preparation (▾Sativex - GW Pharma Ltd), the first cannabinoid preparation to be approved for medical use. Sativex has been licensed "for symptom improvement in adult patients with moderate to severe spasticity due to MS who have not responded adequately to other anti-spasticity medication and who demonstrate clinically significant improvement in spasticity related symptoms during an initial trial of therapy".(6) Here we review the evidence for cannabis extract and its place in the treatment of the condition.

Establishing a rat model of spastic cerebral palsy by targeted ethanol injection

PubMed Central

Yu, Yadong; Li, Liang; Shao, Xinzhong; Tian, Fangtao; Sun, Qinglu

2013-01-01

Spastic cerebral palsy is generally considered to result from cerebral cortical or pyramidal tract damage. Here, we precisely targeted the left pyramidal tract of 2-month-old Sprague-Dawley rats placed on a stereotaxic instrument under intraperitoneal anesthesia. Based on the rat brain stereotaxic map, a 1-mm hole was made 10 mm posterior to bregma and 0.8 mm left of sagittal suture. A microsyringe was inserted perpendicularly to the surface of the brain to a depth of 9.7 mm, and 15 μL of ethanol was slowly injected to establish a rat model of spastic cerebral palsy. After modeling, the rats appeared to have necrotic voids in the pyramidal tract and exhibited typical signs and symptoms of flexion spasms that lasted for a long period of time. These findings indicate that this is an effective and easy method of establishing a rat model of spastic cerebral palsy with good re-producibility. Ethanol as a chemical ablation agent specifically and thoroughly damages the pyramidal tract, and therefore, the animals display flexion spasms, which are a typical symptom of the disease. PMID:25206647

Mucopolysaccharidosis type IV

MedlinePlus

... include: Abnormal development of bones, including the spine Bell-shaped chest with ribs flared out at the ... may slip and damage the spinal cord, causing paralysis. Surgery to correct such problems should be done ...

Ramsay Hunt Syndrome

MedlinePlus

... spinning or moving (vertigo) A change in taste perception or loss of taste Dry mouth and eyes ... of one-sided facial paralysis and hearing loss. Risk factors Anyone who has had chickenpox can develop ...

Development of hand rehabilitation system for paralysis patient - universal design using wire-driven mechanism.

PubMed

Yamaura, Hiroshi; Matsushita, Kojiro; Kato, Ryu; Yokoi, Hiroshi

2009-01-01

We have developed a hand rehabilitation system for patients suffering from paralysis or contracture. It consists of two components: a hand rehabilitation machine, which moves human finger joints with motors, and a data glove, which provides control of the movement of finger joints attached to the rehabilitation machine. The machine is based on the arm structure type of hand rehabilitation machine; a motor indirectly moves a finger joint via a closed four-link mechanism. We employ a wire-driven mechanism and develop a compact design that can control all three joints (i.e., PIP, DIP and MP ) of a finger and that offers a wider range of joint motion than conventional systems. Furthermore, we demonstrate the hand rehabilitation process, finger joints of the left hand attached to the machine are controlled by the finger joints of the right hand wearing the data glove.

Left lower lobe atelectasis and consolidation following cardiac surgery: the effect of topical cooling on the phrenic nerve

DOE Office of Scientific and Technical Information (OSTI.GOV)

Benjamin, J.J.; Cascade, P.N.; Rubenfire, M.

1982-01-01

Retrospective and prospective analyses of chest radiographs of patients following coronary artery bypass surgery were undertaken. Left lower lobe pulmonary infiltrate and/or atelectasis developed in 13 of 40 (32.5%) patients who were operated upon without topical cooling of the heart with ice, and in 77 of 122 (63.1%) patients in one group and 34 of 40 (85.0%) patients in another group who were operated upon with topical cooling of the heart with ice. This difference was highly significant (p<0.001). Of the patients in one group in whom left lower lobe abnormality developed, 69.2% had paralysis or paresis of the leftmore » hemidiaphragm. It is evident that application of ice to the phrenic nerve can lead to temporary paralysis of the left leaf of the diaphragm, with subsequent development of left lower lobe pulmonary infiltrate and/or atelectasis.« less

Literature study on clinical treatment of facial paralysis in the last 20 years using Web of Science

PubMed Central

Zhang, Xiaoge; Feng, Ling; Du, Liang; Zhang, Anxiang; Tang, Tian

2012-01-01

BACKGROUND: Facial paralysis is defined as severe or complete loss of facial muscle motor function. OBJECTIVE: The study was undertaken to explore a bibliometric approach to quantitatively assess the research on clinical treatment of facial paralysis using rehabilitation, physiotherapy and acupuncture using Web of Science from 1992 to 2011. DESIGN: Bibliometric approach. DATA RETRIEVAL: A bibliometric analysis based on the publications on Web of Science was performed using key words such as “facial paralysis”, “rehabilitation”, “physiotherapy” and “acupuncture”. INCLUSIVE CRITERIA: (1) Research articles on the clinical treatment of facial paralysis using acupuncture or physiotherapy (e.g. exercise, electro-stimulation) and other rehabilitation methods; (2) researches on human and animal fundamentals, clinical trials and case reports; (3) Article types: article, review, proceedings paper, note, letter, editorial material, discussion, book chapter. (4) Publication year: 1992–2011 inclusive. Exclusion criteria: (1) Articles on the causes and diagnosis on facial paralysis; (2) Type of articles: correction; (3) Articles from following databases: all databases related to social science and chemical databases in Web of Science. MAIN OUTCOME MEASURES: (1) Overall number of publications; (2) number of publications annually; (3) number of citations received annually; (4) top cited paper; (5) subject categories of publication; (6) the number of countries in which the article is published; (7) distribution of output in journals. RESULTS: Overall population stands at 3 543 research articles addressing the clinical treatment of facial paralysis in Web of Science during the study period. There is also a markedly increase in the number of publications on the subject “facial paralysis treatments using rehabilitation” during the first decade of the 21st century, except in 2004 and 2006 when there are perceptible drops in the number of articles published. The only other year during the study period saw such a drop is 1993. Specifically, there are 192 published articles on facial paralysis treated by rehabilitation in the past two decades, far more than the output of physiotherapy treatment. Physiotherapy treatment scored only 25 articles including acupuncture treatment, with over 80% of these written by Chinese researchers and clinicians. Ranked by regions, USA is by far the most productive country in terms of the number of publications on facial paralysis rehabilitation and physiotherapy research. Seeing from another angle, the journals that focus on otolaryngology published the most number of articles in rehabilitation and physiotherapy studies, whereas most acupuncture studies on facial paralysis were published in the alternative and complementary medicine journals. CONCLUSION: Study of facial paralysis remains an area of active investigation and innovation. Further clinical studies in humans addressing the use of growth factors or stem cells continue to successful facial nerve regeneration. PMID:25767492

Drug-resistant MS spasticity treatment with Sativex(®) add-on and driving ability.

PubMed

Freidel, M; Tiel-Wilck, K; Schreiber, H; Prechtl, A; Essner, U; Lang, M

2015-01-01

The aim of the present observational study was to determine the effects of a delta-9-tetrahydrocannabinol (THC) and cannabidiol (CBD) oromucosal spray (Sativex(®) spray), brand name Sativex(®), indicated for drug-resistant MS spasticity, on the driving ability of treated MS patients. The study was conducted over a period of 4-6 weeks. Thirty-three MS patients with moderate to severe treatment-resistant spasticity and planned to begin add-on treatment with Sativex(®) were enrolled at three specialized MS centres in Germany. A set of five driving test procedures from a validated computerized test battery was used to evaluate the driving ability of eligible patients. Tests were performed by patients at baseline and repeated after 4-6 weeks of treatment with Sativex(®) oromucosal spray. According to German normative data, the test thresholds achieved by the general population served as a reference to allow for a fitness/unfitness to drive classification. Patients showed comparable driving test results at baseline and at final visits. Only two patients changed classification shifting from 'unfit' to drive to 'fit' and vice versa. The mean severity of spasticity, as self-reported by the patients, improved with statistical significance. Sativex(®) was generally well tolerated. Treatment of MS patients with Sativex(®) does not negatively impact on driving ability and may improve moderate to severe treatment-resistant MS spasticity. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Relationship of spasticity to knee angular velocity and motion during gait in cerebral palsy.

PubMed

Damiano, Diane L; Laws, Edward; Carmines, Dave V; Abel, Mark F

2006-01-01

This study investigated the effects of spasticity in the hamstrings and quadriceps muscles on gait parameters including temporal spatial measures, knee position, excursion and angular velocity in 25 children with spastic diplegic cerebral palsy (CP) as compared to 17 age-matched peers. While subjects were instructed to relax, an isokinetic device alternately flexed and extended the left knee at one of the three constant velocities 30 degrees/s, 60 degrees/s and 120 degrees/s, while surface electromyography (EMG) electrodes over the biceps femoris and the rectus femoris recorded muscle activity. Patients then participated in 3D gait analysis at a self-selected speed. Results showed that, those with CP who exhibited heightened stretch responses (spasticity) in both muscles, had significantly slower knee angular velocities during the swing phase of gait as compared to those with and without CP who did not exhibit stretch responses at the joint and the tested speeds. The measured amount (torque) of the resistance to passive flexion or extension was not related to gait parameters in subjects with CP; however, the rate of change in resistance torque per unit angle change (stiffness) at the fastest test speed of 120 degrees/s showed weak to moderate relationships with knee angular velocity and motion during gait. For the subset of seven patients with CP who subsequently underwent a selective dorsal rhizotomy, knee angular extension and flexion velocity increased post-operatively, suggesting some degree of causality between spasticity and movement speed.

Endoscopic laser arytenoidectomy with the use of CO2 laser

NASA Astrophysics Data System (ADS)

Malandrakis, S.; Prokopakis, E. P.; Sidoris, M.; Karatzanis, A.; Velegrakis, G.; Helidonis, Emmanuel S.

2004-06-01

Upper airway obstruction due to bilateral vocal cord paralysis presents a serious challenge to the Otolaryngologist. Various surgical techniques have been advocated for the management of patients with vocal cord patients with vocal cord paralysis. Among these techniques, the individual use of laser CO2 arytenoidectomy and posterior cordotomy has gained wide acceptance. In this report, we describe our experience in the management of bilateral vocal cord paralysis by combining posterior partial cordotomy as described by Dennis and Kashima with total arytenoidectomy as described by Ossoff. We report the long term results in the management of 24 patients with bilateral vocal cord paralysis treated in our Department during the last 9 years.

Detection of chronic bee paralysis virus and acute bee paralysis virus in Uruguayan honeybees.

PubMed

Antúnez, Karina; D' Alessandro, Bruno; Corbella, Eduardo; Zunino, Pablo

2005-09-01

Chronic bee paralysis virus (CBPV) causes a disease characterized by trembling, flightless, and crawling bees, while Acute bee paralysis virus (ABPV) is commonly detected in apparently healthy colonies, usually associated to Varroa destructor. Both viruses had been detected in most regions of the world, except in South America. In this work, we detected CBPV and ABPV in samples of Uruguayan honeybees by RT-PCR. The detection of both viruses in different provinces and the fact that most of the analyzed samples were infected, suggest that, they are widely spread in the region. This is the first record of the presence of CBPV and ABPV in Uruguay and South America.

Use of botulinum toxin in stroke patients with severe upper limb spasticity.

PubMed

Bhakta, B B; Cozens, J A; Bamford, J M; Chamberlain, M A

1996-07-01

Spasticity can contribute to poor recovery of upper limb function after stroke. This is a preliminary evaluation of the impact of botulinum toxin treatment on disability caused by upper limb spasticity after stroke. Seventeen patients with severe spasticity and a non-functioning arm were treated with intramuscular botulinum A neurotoxin (median age at treatment 54.5 years; median time between onset of stroke and treatment 1.5 years). Baseline and assessments two weeks after treatment were compared to assess efficacy. The duration of improvement in disability was documented. Outcome measures used were; passive range of movement at the shoulder, elbow, wrist, and fingers; modified Ashworth scale to assess spasticity of biceps and forearm finger flexors; an eight point scale to assess the degree of difficulty experienced by the patient or carer for each functional problem defined before treatment; the presence of upper limb pain. The biceps, forearm finger flexors, and flexor carpiulnaris were treated with intramuscular botulinum toxin. Up to a total dose of 400-1000 mouse units (MU) of Dysport (Speywood) or 100-200 MU of BOTOX (Allergan) was used in each patient. Functional problems reported by the patients before treatment were difficulty with cleaning the palm, cutting fingernails, putting the arm through a sleeve, standing and walking balance, putting on gloves, and rolling over in bed. Hand hygiene improved in 14 of 17 patients; difficulty with sleeves improved in four of 16; standing and walking balance improved in one of four; shoulder pain improved in six of nine; wrist pain improved in five of six. Passive range of movement at shoulder, elbow, and wrist improved after treatment. Benefit was noted within two weeks and lasted one to 11 months. No adverse effects occurred. This preliminary study suggests that intramuscular botulinum toxin is a safe and effective treatment for reducing disability in patients with severe upper limb spasticity.

Contralesional Cathodal versus Dual Transcranial Direct Current Stimulation for Decreasing Upper Limb Spasticity in Chronic Stroke Individuals: A Clinical and Neurophysiological Study.

PubMed

Del Felice, Alessandra; Daloli, Verena; Masiero, Stefano; Manganotti, Paolo

2016-12-01

Different transcranial direct current stimulation (tDCS) paradigms have been implemented to treat poststroke spasticity, but discordant results have been reported. This study aimed to determine the efficacy and persistence of dual tDCS (anode over affected motor cortex [M1] and cathode over contralateral M1) compared with cathodal tDCS (cathode over contralateral M1) on upper limb (UL) functional, behavioral, and neurophysiological measures in chronic poststroke individuals. Ten subjects with UL spasticity (7 men; mean 62 years; 8 ischemic stroke; years from event: 2.3 years) were enrolled in a cross-over, double-blinded study. Cathodal and dual tDCS, both preceded by 1 week of sham stimulation 1 month before real stimulation, were applied with 3 months interval. Stimulating paradigm was 20 minutes for five consecutive days in each block. Evaluations were performed before (T1), after real or sham treatment (T2), and after 1 (T3), 4 (T4), and 8 weeks (T5). Functional, behavioral, and neurophysiological tests were performed at each time. Both tDCS paradigms decreased spasticity, increased strength, and ameliorated behavioral scales. Cathodal tDCS was superior to dual tDCS in reducing UL distal spasticity immediately after treatment (T2: cathodal > dual: P = .023) and provided a higher and longer lasting reduction at proximal districts (T3: cathodal > dual: P = .042; T4: cathodal > dual: P = .028; T5: cathodal > dual: P = .05). These findings are supported by an H-reflex modulation (overall time effect P > .002). Cathodal tDCS is slightly more effective than dual tDCS in reducing distal UL spasticity in chronic poststroke subjects. A modulation of spinal inhibitory mechanisms, demonstrated by H-reflex modifications, supports this finding. Copyright © 2016 National Stroke Association. Published by Elsevier Inc. All rights reserved.

Gait asymmetry, ankle spasticity, and depression as independent predictors of falls in ambulatory stroke patients

PubMed Central

Wei, Ta-Sen; Liu, Peng-Ta; Chang, Liang-Wey; Liu, Sen-Yung

2017-01-01

Background Falls are the leading cause of injury in stroke patients. However, the cause of a fall is complicated, and several types of risk factors are involved. Therefore, a comprehensive model to predict falls with high sensitivity and specificity is needed. Methods This study was a prospective study of 112 inpatients in a rehabilitation ward with follow-up interviews in patients’ homes. Evaluations were performed 1 month after stroke and included the following factors: (1) status of cognition, depression, fear of fall and limb spasticity; (2) functional assessments [walking velocity and the Functional Independence Measure (FIM)]; and (3) objective, computerized gait and balance analyses. The outcome variable was the number of accidental falls during the 6-month follow-up period after baseline measurements. Results The non-faller group exhibited significantly better walking velocity and FIM scale compared to the faller group (P < .001). The faller group exhibited higher levels of spasticity in the affected limbs, asymmetry of gait parameters in single support (P < .001), double support (P = .027), and step time (P = .003), and lower stability of center of gravity in the medial-lateral direction (P = .008). Psychological assessments revealed that the faller group exhibited more severe depression and lower confidence without falling. A multivariate logistic regression model identified three independent predictors of falls with high sensitivity (82.6%) and specificity (86.5%): the asymmetry ratio of single support [adjusted odds ratio, aOR = 2.2, 95% CI (1.2–3.8)], the level of spasticity in the gastrocnemius [aOR = 3.2 (1.4–7.3)], and the degree of depression [aOR = 1.4 (1.2–1.8)]. Conclusions This study revealed depression, in additional to gait asymmetry and spasticity, as another independent factor for predicting falls. These results suggest that appropriate gait training, reduction of ankle spasticity, and aggressive management of depression may be critical to prevent falls in stroke patients. PMID:28542281

Randomized Controlled Trial on Effectiveness of Intermittent Serial Casting on Spastic Equinus Foot in Children with Cerebral Palsy After Botulinum Toxin-A Treatment.

PubMed

Dursun, Nigar; Gokbel, Tugba; Akarsu, Melike; Dursun, Erbil

2017-04-01

Physical therapy (PT) and botulinum toxin-A (BTX-A) injections are widely used in the treatment of spastic equinus foot due to cerebral palsy. The aim of this study was to show effects of intermittent serial casting (SC) in addition to standard treatment on spasticity, passive range of motion (PROM), and gait. Fifty-one ambulatory patients, treated by BTX-A to plantar flexor muscles, were randomly assigned to casting or control groups in a 2:1 ratio. Both groups received PT for 3 weeks. Casting group additionally received intermittent SC during 3 consecutive weekends. Assessments included Modified Ashworth Scale (MAS), Tardieu Scale, Observational Gait Scale (OGS), and Physician Global Assessment at baseline and posttreatment weeks 4 and 12. Significant improvements in PROM, MAS, Tardieu Scale, and OGS were recorded in both groups (P < 0.001 for all). Average changes in MAS, PROM, angle of catch, spasticity angle, and OGS of the casting group were significantly higher than those of the controls at week 4 (P = 0.006, P = 0.002, P < 0.001, P = 0.005, P = 0.011), and 12 (P = 0.013, P < 0.001, P < 0.001, P = 0.011, P < 0.001). Follow-up Physician Global Assessment also favored casting group (P < 0.001 for both). Combining intermittent SC with BTX-A injections and PT might provide additional benefits for spastic equinus foot. Complete the self-assessment activity and evaluation online at http://www.physiatry.org/JournalCME CME OBJECTIVES: Upon completion of this article, the reader should be able to: (1) identify treatment options for spastic equinus goot in children with cerebral palsy; (2) explain different approaches of serial casting with an additional model of intermittent casting; and (3) describe the potential benefits of combined treatment modalities, including intermittent serial casting, for spastic equinus foot in children with cerebral palsy. Advanced ACCREDITATION: The Association of Academic Physiatrists is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.The Association of Academic Physiatrists designates this activity for a maximum of 1.5 AMA PRA Category 1 Credit(s)™. Physicians should only claim credit commensurate with the extent of their participation in the activity.

Temporary suspension of acute facial paralysis using the S-S Cable Suture (Medical U&A, Tokyo, Japan).

PubMed

Ozaki, Mine; Takushima, Akihiko; Momosawa, Akira; Kurita, Masakazu; Harii, Kiyonori

2008-07-01

For a treatment of facial paralysis, suture suspension of soft tissue is considered effective due to its less invasiveness and relatively simple technique, with minimal bruising and rapid recovery. However, suture suspension effect may not last for a long period of time. We obtained good outcome with temporary static suture suspension in 5 cases of severe facial paralysis in the intervening period between the onset of paralysis and expected spontaneous recovery. We used the S-S Cable Suture (Medical U&A, Tokyo, Japan), which was based on the modification of previously established method using the Gore-Tex cable suture originally reported by Sasaki et al in 2002. Because of the ease of technique and relatively strong lifting capability of the malar pad, we recommend it as a useful procedure for a patient suffering acute facial paralysis with possible spontaneous recovery for an improved quality of life by the quick elimination of facial distortion.

Vocal fold paralysis secondary to phonotrauma.

PubMed

Klein, Travis A L; Gaziano, Joy E; Ridley, Marion B

2014-01-01

A unique case of acute onset vocal fold paralysis secondary to phonotrauma is presented. The cause was forceful vocalization by a drill instructor on a firearm range. Imaging studies revealed extensive intralaryngeal and retropharyngeal hemorrhage. Laryngoscopy showed a complete left vocal fold paralysis. Relative voice rest was recommended, and the patient regained normal vocal fold mobility and function after approximately 12 weeks. Copyright © 2014 The Voice Foundation. All rights reserved.

Hypokalemic periodic paralysis as first sign of thyrotoxicosis.

PubMed

Trifanescu, R A; Danciulescu Miulescu, R; Carsote, M; Poiana, C

2013-03-15

periodic paralysis related to hypokalemia is seldom reported in thyrotoxicosis, and it usually occurs in Asian males. Two Romanian (Caucasian) young patients presented with hypokalemic paralysis. TSH, FT4, TT3 was measured by immunochemiluminescence. Case report 1. Patient O.R, aged 19, presented marked asthenia and lower limbs paralysis, following high carbohydrate meal. He declared 10 kg weight loss on hypocaloric diet and mild sweating. Biochemical data revealed moderate hypokalemia (K+=2.6 mmol/L) and thyrotoxicosis (TSH<0.03 mIU/L, FT4=30 pmol/L, TT3=315 ng/dL). Case report 2. Patient T.A., aged 18, presented 2 episodes of weakness and flaccid paralysis, with hypokalemia, precipitated by effort, without any sign of thyrotoxicosis. Biochemical data revealed severe hypokalemia (K+=1.8 mmol/L) and thyrotoxicosis (TSH<0.03 mIU/L, FT4=24 pmol/L, TT3=190 ng/dL). Treatment with intravenous potassium, thereafter methimazole and propranolol were administered in both cases, with the maintenance of normal kalemia and thyrotoxicosis' control. these 2 cases of hypokalemic periodic paralysis occurring in young Caucasian teenagers with mild thyrotoxicosis underlined the importance of thyroid screening in patients with symptomatic hypokalemia, even in the absence of symptoms and signs of thyrotoxicosis.

Initiation of bladder voiding with epidural stimulation in paralyzed, step trained rats.

PubMed

Gad, Parag N; Roy, Roland R; Zhong, Hui; Lu, Daniel C; Gerasimenko, Yury P; Edgerton, V Reggie

2014-01-01

The inability to control timely bladder emptying is one of the most serious challenges among the several functional deficits that occur after a complete spinal cord injury. Having demonstrated that electrodes placed epidurally on the dorsum of the spinal cord can be used in animals and humans to recover postural and locomotor function after complete paralysis, we hypothesized that a similar approach could be used to recover bladder function after paralysis. Also knowing that posture and locomotion can be initiated immediately with a specific frequency-dependent stimulation pattern and that with repeated stimulation-training sessions these functions can improve even further, we reasoned that the same two strategies could be used to regain bladder function. Recent evidence suggests that rats with severe paralysis can be rehabilitated with a multisystem neuroprosthetic training regime that counteracts the development of neurogenic bladder dysfunction. No data regarding the acute effects of locomotion on bladder function, however, were reported. In this study we show that enabling of locomotor-related spinal neuronal circuits by epidural stimulation also influences neural networks controlling bladder function and can play a vital role in recovering bladder function after complete paralysis. We have identified specific spinal cord stimulation parameters that initiate bladder emptying within seconds of the initiation of epidural stimulation. The clinical implications of these results are substantial in that this strategy could have a major impact in improving the quality of life and longevity of patients while simultaneously dramatically reducing ongoing health maintenance after a spinal cord injury.

Initiation of Bladder Voiding with Epidural Stimulation in Paralyzed, Step Trained Rats

PubMed Central

Gad, Parag N.; Roy, Roland R.; Zhong, Hui; Lu, Daniel C.; Gerasimenko, Yury P.; Edgerton, V. Reggie

2014-01-01

The inability to control timely bladder emptying is one of the most serious challenges among the several functional deficits that occur after a complete spinal cord injury. Having demonstrated that electrodes placed epidurally on the dorsum of the spinal cord can be used in animals and humans to recover postural and locomotor function after complete paralysis, we hypothesized that a similar approach could be used to recover bladder function after paralysis. Also knowing that posture and locomotion can be initiated immediately with a specific frequency-dependent stimulation pattern and that with repeated stimulation-training sessions these functions can improve even further, we reasoned that the same two strategies could be used to regain bladder function. Recent evidence suggests that rats with severe paralysis can be rehabilitated with a multisystem neuroprosthetic training regime that counteracts the development of neurogenic bladder dysfunction. No data regarding the acute effects of locomotion on bladder function, however, were reported. In this study we show that enabling of locomotor-related spinal neuronal circuits by epidural stimulation also influences neural networks controlling bladder function and can play a vital role in recovering bladder function after complete paralysis. We have identified specific spinal cord stimulation parameters that initiate bladder emptying within seconds of the initiation of epidural stimulation. The clinical implications of these results are substantial in that this strategy could have a major impact in improving the quality of life and longevity of patients while simultaneously dramatically reducing ongoing health maintenance after a spinal cord injury. PMID:25264607

Gaze Behaviour during Interception in Children with Spastic Unilateral Cerebral Palsy

ERIC Educational Resources Information Center

van Kampen, P. M.; Ledebt, A.; Smorenburg, A. R. P.; Vermeulen, R. J.; Kelder, M. E.; van der Kamp, J.; Savelsbergh, G. J. P.

2012-01-01

Anticipatory gaze behaviour during interceptive movements was investigated in children with Spastic Unilateral Cerebral Palsy (SUCP), and related to the side of the intracerebral lesion. Five children with lesions of the right hemisphere (RHL) and five children with lesions of the left hemisphere (LHL) had to walk towards and intercept a ball that…

The Impact of Contrastive Stress on Vowel Acoustics and Intelligibility in Dysarthria

ERIC Educational Resources Information Center

Connaghan, Kathryn P.; Patel, Rupal

2017-01-01

Purpose: To compare vowel acoustics and intelligibility in words produced with and without contrastive stress by speakers with spastic (mixed-spastic) dysarthria secondary to cerebral palsy (DYS[subscript CP]) and healthy controls (HCs). Method: Fifteen participants (9 men, 6 women; age M = 42 years) with DYS[subscript CP] and 15 HCs (9 men, 6…

Mirror Visual Feedback Induces Lower Neuromuscular Activity in Children with Spastic Hemiparetic Cerebral Palsy

ERIC Educational Resources Information Center

Feltham, Max G.; Ledebt, Annick; Deconinck, Frederik J. A.; Savelsbergh, Geert J. P.

2010-01-01

The study examined the effects of mirror feedback information on neuromuscular activation during bimanual coordination in eight children with spastic hemiparetic cerebral palsy (SHCP) and a matched control group. The "mirror box" creates a visual illusion, which gives rise to a visual perception of a zero lag, symmetric movement between the two…

Assessing the immediate impact of botulinum toxin injection on impedance of spastic muscle.

PubMed

Li, Xiaoyan; Shin, Henry; Li, Le; Magat, Elaine; Li, Sheng; Zhou, Ping

2017-05-01

This study aimed to investigate the immediate impacts of Botulinum Toxin A (BoNT-A) injections on the inherent electrical properties of spastic muscles using a newly developed electrical impedance myography (EIM) technique. Impedance measures were performed before and after a BoNT-A injection in biceps brachii muscles of 14 subjects with spasticity. Three major impedance variables, resistance (R), reactance (X) and phase angle (θ) were obtained from three different configurations, and were evaluated using the conventional EIM frequency at 50kHz as well as multiple frequency analysis. Statistical analysis demonstrated a significant decrease of resistance in the injected muscles (Multiple-frequency: R pre =25.17±1.94Ohm, R post =23.65±1.63Ohm, p<0.05; 50kHz: R pre =29.06±2.16Ohm, R post =27.7±1.89Ohm, p<0.05). Despite this decrease, there were no substantial changes in the reactance, phase angle, or anisotropy features after a BoNT-A injection. The significant changes of muscle resistance were most likely associated with the liquid injection of the BoNT-A-saline solution rather than the immediate toxin effects on the muscle. This study demonstrated high sensitivity of the EIM technique in the detection of alterations to muscle composition. Copyright © 2017 IPEM. Published by Elsevier Ltd. All rights reserved.

Constructing an adaptive care model for the management of disease-related symptoms throughout the course of multiple sclerosis--performance improvement CME.

PubMed

Miller, Aaron E; Cohen, Bruce A; Krieger, Stephen C; Markowitz, Clyde E; Mattson, David H; Tselentis, Helen N

2014-01-01

Symptom management remains a challenging clinical aspect of MS. To design a performance improvement continuing medical education (PI CME) activity for better clinical management of multiple sclerosis (MS)-related depression, fatigue, mobility impairment/falls, and spasticity. Ten volunteer MS centers participated in a three-stage PI CME model: A) baseline assessment; B) practice improvement CME intervention; C) reassessment. Expert faculty developed performance measures and activity intervention tools. Designated MS center champions reviewed patient charts and entered data into an online database. Stage C data were collected eight weeks after implementation of the intervention and compared with Stage A baseline data to measure change in performance. Aggregate data from the 10 participating MS centers (405 patient charts) revealed performance improvements in the assessment of all four MS-related symptoms. Statistically significant improvements were found in the documented assessment of mobility impairment/falls (p=0.003) and spasticity (p<0.001). For documentation of care plans, statistically significant improvements were reported for fatigue (p=0.007) and mobility impairment/falls (p=0.040); non-significant changes were noted for depression and spasticity. Our PI CME interventions demonstrated performance improvement in the management of MS-related symptoms. This PI CME model (available at www.achlpicme.org/ms/toolkit) offers a new perspective on enhancing symptom management in patients with MS.

Advances in Management of Esophageal Motility Disorders.

PubMed

Kahrilas, Peter J; Bredenoord, Albert J; Carlson, Dustin A; Pandolfino, John E

2018-04-24

The widespread adoption of high-resolution manometry (HRM) has led to a restructuring in the classification of esophageal motility disorder classification summarized in the Chicago Classification, currently in version 3.0. It has become apparent that the cardinal feature of achalasia, impaired lower esophageal sphincter relaxation, can occur in several disease phenotypes: without peristalsis, with premature (spastic) distal esophageal contractions, with panesophageal pressurization, or even with preserved peristalsis. Furthermore, despite these advances in diagnostics, no single manometric pattern is perfectly sensitive or specific for idiopathic achalasia and complimentary assessments with provocative maneuvers during HRM or interrogating the esophagogastric junction with the functional luminal imaging probe during endoscopy can be useful in clarifying equivocal or inexplicable HRM findings. Using these tools, we have come to conceptualize esophageal motility disorders as characterized by obstructive physiology at the esophagogastric junction, smooth muscle esophagus, or both. Recognizing obstructive physiology as a primary target of therapy has become particularly relevant with the development of a minimally invasive technique for performing a calibrated myotomy of the esophageal circular muscle, the POEM procedure. Now and going forward, optimal management is to render treatment in a phenotype-specific manner: e.g. POEM calibrated to patient-specific physiology for spastic achalasia and spastic disorders of the smooth muscle esophagus, more conservative strategies (pneumatic dilation) for the disorders limited to the sphincter. Copyright © 2018 AGA Institute. Published by Elsevier Inc. All rights reserved.

Shoulder tenotomies to improve passive motion and relieve pain in patients with spastic hemiplegia after upper motor neuron injury.

PubMed

Namdari, Surena; Alosh, Hassan; Baldwin, Keith; Mehta, Samir; Keenan, Mary Ann

2011-07-01

Shoulder adduction and internal rotation contractures commonly develop in patients with spastic hemiplegia after upper motor neuron (UMN) injury. Contractures are often painful, macerate skin, and impair axillary hygiene. We hypothesize that shoulder tenotomies are an effective means of pain relief and passive motion restoration in patients without active upper extremity motor function. A consecutive series of 36 adults (10 men, 26 women) with spastic hemiplegia from UMN injury, shoulder adduction, and internal rotation contractures, and no active movement, who underwent shoulder tenotomies of the pectoralis major, latissimus dorsi, teres major, and subscapularis were evaluated. Patients were an average age of 52.2 years. Pain, passive motion, and satisfaction were considered preoperatively and postoperatively. Average follow-up was 14.3 months. Preoperatively, all patients had limited passive motion that interfered with passive functions. Nineteen patients had pain. After surgery, passive extension, flexion, abduction, and external rotation improved from 50%, 27%, 27%, and 1% to 85%, 70%, 66%, and 56%, respectively, compared with the normal contralateral side (P < .001). All patients with preoperative pain had improved pain relief at follow-up, with 18 (95%) being pain-free. Thirty-five (97%) were satisfied with the outcome of surgery, and all patients reported improved axillary hygiene and skin care. Age, gender, etiology, and chronicity of UMN injury were not associated with improvement in motion. We observed improvements in passive ROM and high patient satisfaction with surgery at early follow-up. Patients who had pain with passive motion preoperatively had significant improvements in pain after shoulder tenotomy. Shoulder tenotomy to relieve spastic contractures resulting from UMN injury can be an effective means of pain relief and improved passive range of motion in patients without active motor function. Copyright © 2011 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Mosby, Inc. All rights reserved.

Prolonged stretching of the ankle plantarflexors elicits muscle-tendon adaptations relevant to ankle gait kinetics in children with spastic cerebral palsy.

PubMed

Martín Lorenzo, Teresa; Rocon, Eduardo; Martínez Caballero, Ignacio; Ramírez Barragán, Ana; Lerma Lara, Sergio

2017-11-01

Tissue related ankle hyper-resistance has been reported to contribute to equinus gait in children with spastic cerebral palsy. Hence, ankle plantarflexor stretching programs have been developed in order to restore passive ankle dorsiflexion. Despite high quality evidence on the limited effects of stretching on passive joint mobility, further muscle-tendon adaptations have been reported which may impact gait performance. As such, children with spastic cerebral palsy subject to long-term manual static stretching achieved dorsiflexion gains through the reduction of muscle and fascicle strain whilst preserving tendon strain, and prolonged use of ankle-foot orthoses achieved similar dorsiflexion gains through increased tendon strain whilst preserving muscle and fascicle strain. The latter concurred with normalization of early stance plantarflexor moment yet reductions in push-off plantarflexor moment given the increase in tendon compliance. Therefore, similar limited gains in passive ankle joint mobility in response to stretching may be achieved either by preserving/restoring optimal muscle-tendon function, or at the expense of muscle-tendon function and thus contributing gait impairments. The largest increase in ankle passive joint mobility in children with SCP has been obtained through prolonged plantarflexor stretching through ankle casting combined with botulinum neurotoxin type A. However, to our knowledge, there are no published studies on muscle-tendinous adaptations to ankle casting combined with botulinum toxin type A and its effect on ankle joint gait kinetics. Therefore, we hypothesized that ankle casting elicits muscle-tendon adaptations which concur with altered ankle joint kinetics during the stance phase of gait in children with SCP. More information is needed about the relationships between muscle structure and function, and the effect of specific interventions designed to alter muscle properties and associated functional outcomes in children with spastic cerebral palsy. Copyright © 2017 Elsevier Ltd. All rights reserved.

Practice guideline update summary: Botulinum neurotoxin for the treatment of blepharospasm, cervical dystonia, adult spasticity, and headache

PubMed Central

Simpson, David M.; Hallett, Mark; Ashman, Eric J.; Comella, Cynthia L.; Green, Mark W.; Gronseth, Gary S.; Armstrong, Melissa J.; Gloss, David; Potrebic, Sonja; Jankovic, Joseph; Karp, Barbara P.; Naumann, Markus; So, Yuen T.; Yablon, Stuart A.

2016-01-01

Objective: To update the 2008 American Academy of Neurology (AAN) guidelines regarding botulinum neurotoxin for blepharospasm, cervical dystonia (CD), headache, and adult spasticity. Methods: We searched the literature for relevant articles and classified them using 2004 AAN criteria. Results and recommendations: Blepharospasm: OnabotulinumtoxinA (onaBoNT-A) and incobotulinumtoxinA (incoBoNT-A) are probably effective and should be considered (Level B). AbobotulinumtoxinA (aboBoNT-A) is possibly effective and may be considered (Level C). CD: AboBoNT-A and rimabotulinumtoxinB (rimaBoNT-B) are established as effective and should be offered (Level A), and onaBoNT-A and incoBoNT-A are probably effective and should be considered (Level B). Adult spasticity: AboBoNT-A, incoBoNT-A, and onaBoNT-A are established as effective and should be offered (Level A), and rimaBoNT-B is probably effective and should be considered (Level B), for upper limb spasticity. AboBoNT-A and onaBoNT-A are established as effective and should be offered (Level A) for lower-limb spasticity. Headache: OnaBoNT-A is established as effective and should be offered to increase headache-free days (Level A) and is probably effective and should be considered to improve health-related quality of life (Level B) in chronic migraine. OnaBoNT-A is established as ineffective and should not be offered for episodic migraine (Level A) and is probably ineffective for chronic tension-type headaches (Level B). PMID:27164716

Effects of a single session of whole body vibration on ankle plantarflexion spasticity and gait performance in patients with chronic stroke: a randomized controlled trial.

PubMed

Chan, Kwan-Shan; Liu, Chin-Wei; Chen, Tien-Wen; Weng, Ming-Cheng; Huang, Mao-Hsiung; Chen, Chia-Hsin

2012-12-01

To investigate the effects of a single session of whole body vibration training on ankle plantarflexion spasticity and gait performance in chronic stroke patients. Randomized controlled trial. Rehabilitation unit in university hospital. Thirty subjects with chronic stroke were randomized into either a control group (n = 15) or a group receiving a single session of whole body vibration (n = 15). The intervention group was actually treated with whole body vibration while the control group was treated with placebo treatment. The spastic changes were measured clinically and neurophysiologically. Subjective evaluation of ankle spasticity was performed via a visual analogue scale. Gait performances were evaluated by the timed up and go test, 10-meter walk test and cadence. A forceplate was used for measuring foot pressure. The changes between whole body vibration and control groups were significantly different in Modified Ashworth Scale (1.33, 95% confidence interval (CI) = 1.06~1.60). The H (max)/M (max) ratio (0.14, 95% CI = 0.01~0.26) and visual analogue scale (1.87, 95% CI = 1.15~2.58) were significantly decreased. Whole body vibration could significantly improve gait velocity, timed up and go test (6.03, 95% CI = 3.17~8.89) and 10-meter walk test (1.99, 95% CI = 0.11~3.87). The uneven body weight posture on bilateral feet was also improved after vibration. These results suggest that a single session of whole body vibration training can reduce ankle plantarflexion spasticity in chronic stroke patients, thereby potentially increasing ambulatory capacity.

Health Authorities Data Collection of THC:CBD Oromucosal Spray (L'Agenzia Italiana del Farmaco Web Registry): Figures after 1.5 Years.

PubMed

Patti, Francesco

2016-01-01

In Italy, all prescriptions for THC:CBD oromucosal spray for treatment of multiple sclerosis (MS) spasticity are linked to the official Agenzia Italiana del Farmaco (AIFA) web-based registry, which tracks the effectiveness and tolerability of medications in a prospective and observational manner. AIFA e-registry data for THC:CBD oromucosal spray collected between January 2014 and February 2015 for 1,534 patients from 30 large Italian specialized MS centres were compiled. Patients had a long disease history (17.6 ± 8.6 years) and significant impairment (mean Expanded Disability Status Scale score 6.4 ± 1.2). MS spasticity was evaluated using the 0-10 numerical rating scale (NRS). After the first month titration and trial period, 61.9% of patients achieved sufficient improvement in spasticity (≥20% NRS) to qualify for continued treatment. After 6 months, clinically meaningful ≥30% NRS improvement was recorded in 40.2% of patients continuing with treatment. Spasticity-associated symptoms such as cramps and nocturnal spasms improved in most responding patients. Mean reported doses of THC:CBD oromucosal spray (6.2-6.7 sprays/day) were lower than those reported in clinical trials. Adverse events (mainly mild to moderate) were reported by 15% of patients; no new safety concerns beyond the approved label were identified. The results of the AIFA e-registry analysis align with those of other THC:CBD observational projects and reaffirm the characteristics of this therapeutic option in the management of treatment-resistant MS spasticity, a frequently overlooked symptom. © 2016 S. Karger AG, Basel.

Effectiveness of Oral Baclofen in the Treatment of Spasticity in Children and Adolescents With Cerebral Palsy.

PubMed

Navarrete-Opazo, Angela A; Gonzalez, Waleska; Nahuelhual, Paula

2016-04-01

To systematically review the effectiveness of oral baclofen versus placebo or other antispastic oral medications in terms of body function, level of activity, and quality of life in children and adolescents with spastic cerebral palsy who are younger than 18 years. Cochrane Library, Health Science Databases, DARE, LILACS, Embase, MEDLINE, OTseeker, PEDro, PsycINFO, SpeechBITE, ScienceDirect, Scopus, Trip, ClinicalTrials.gov, Google Scholar, OpenGrey, and manual search. Randomized or not randomized controlled trials and cohort studies comparing the effect of any dosage of oral baclofen with that of no treatment, placebo, or another antispastic medication in children and adolescents with spastic cerebral palsy were selected. Following the Cochrane Handbook for Systematic Reviews of Interventions guidelines, 2 reviewers independently searched articles in databases from their inceptions until October 2014. Six randomized controlled trials involving a total of 130 patients were selected. Studies show a great variability in motor classification, dosage of baclofen, and outcome measures. There is conflicting evidence on the effectiveness of oral baclofen in reducing muscle tone or improving motor function or the level of activity. The overall methodological quality of the studies was low. The main qualitative limitations of the studies correspond to serious risk of bias, inconsistency of results, unpowered sample size, and publication bias. There are insufficient data to support or refute the use of oral baclofen for reducing spasticity or improving motor function in children and adolescents with spastic cerebral palsy. Copyright © 2016 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

The benefits of a comprehensive rehabilitation program in patients diagnosed with spastic quadriplegia

PubMed Central

Rogoveanu, OC; Tuțescu, NC; Kamal, D; Alexandru, DO; Kamal, C; Streba, CT; Trăistaru, MR

2016-01-01

Spastic quadriplegia has as an etiopathogenic substrate, a non-progressive brain lesion; however, the clinical manifestations of the disease evolve over time. Children diagnosed with spastic quadriplegia show a variety of symptoms in different areas: sensorimotor, emotional, cognitive, and social. The purpose of this study was to assess the functional status in patients diagnosed with spastic quadriplegia, who followed a complex medical rehabilitation program, during a year, and highlight the importance of using physical and kinetic techniques in improving their status. A total of 10 children diagnosed with spastic quadriplegia were included in the study and the Gross Motor Function Classification System (GMFCS) and manual ability classification system (MACS) were used to evaluate the functionality status of each patient. Every patient was evaluated initially (T1), after six months of program (T2), and after they completed the study. All the children were originally monitored daily, for 5 days per week for a period of one month, then two times a week for a year. A statistically significant difference regarding the modification of the GMFCS and MACS stage was found, which occurred between the first and the third evaluation. The inverse correlation of the statistical significance between the ages of patients and the decrease in GMFCS or MACS stage was highlighted; the younger the patient, the more the scale decreased. A direct link between the gross motor function and the manual ability was noticed. Applying a complex rehabilitation program has proven efficient by improving both the gross motor functionality and the manual ability. PMID:27974931

The benefits of a comprehensive rehabilitation program in patients diagnosed with spastic quadriplegia.

PubMed

Rogoveanu, O C; Tuțescu, N C; Kamal, D; Alexandru, D O; Kamal, C; Streba, C T; Trăistaru, M R

2016-01-01

Spastic quadriplegia has as an etiopathogenic substrate, a non-progressive brain lesion; however, the clinical manifestations of the disease evolve over time. Children diagnosed with spastic quadriplegia show a variety of symptoms in different areas: sensorimotor, emotional, cognitive, and social. The purpose of this study was to assess the functional status in patients diagnosed with spastic quadriplegia, who followed a complex medical rehabilitation program, during a year, and highlight the importance of using physical and kinetic techniques in improving their status. A total of 10 children diagnosed with spastic quadriplegia were included in the study and the Gross Motor Function Classification System (GMFCS) and manual ability classification system (MACS) were used to evaluate the functionality status of each patient. Every patient was evaluated initially (T1), after six months of program (T2), and after they completed the study. All the children were originally monitored daily, for 5 days per week for a period of one month, then two times a week for a year. A statistically significant difference regarding the modification of the GMFCS and MACS stage was found, which occurred between the first and the third evaluation. The inverse correlation of the statistical significance between the ages of patients and the decrease in GMFCS or MACS stage was highlighted; the younger the patient, the more the scale decreased. A direct link between the gross motor function and the manual ability was noticed. Applying a complex rehabilitation program has proven efficient by improving both the gross motor functionality and the manual ability.

Effect of Baseline Spastic Hemiparesis on Recovery of Upper-Limb Function Following Botulinum Toxin Type A Injections and Postinjection Therapy

PubMed Central

Chang, Chia-Lin; Munin, Michael C.; Skidmore, Elizabeth R.; Niyonkuru, Christian; Huber, Lynne M.; Weber, Douglas J.

2015-01-01

Objective To determine whether baseline hand spastic hemiparesis assessed by the Chedoke-McMaster Assessment influences functional improvement after botulinum toxin type A (BTX-A) injections and postinjection therapy. Design Prospective cohort study. Setting Outpatient spasticity clinic. Participants Participants (N = 14) with spastic hemiparesis divided into 2 groups: Chedoke-McMaster Assessment Hand-Higher Function (stage≥4, n = 5) and Chedoke-McMaster Assessment Hand-Lower Function (stage = 2 or 3, n = 9). Interventions Upper-limb BTX-A injections followed by 6 weeks of postinjection therapy. Main Outcome Measures Primary outcomes were Motor Activity Log-28 and Motor Activity Log items. Secondary outcomes were Action Research Arm Test (ARAT), Motor Activity Log-Self-Report, and Modified Ashworth Scale (MAS). Measures were assessed at baseline (preinjection), 6 weeks, 9 weeks, and 12 weeks postinjection. Results Primary and secondary outcomes improved significantly over time in both groups. Although no significant differences in ARAT or MAS change scores were noted between groups, Chedoke-McMaster Assessment Hand-Higher Function group demonstrated greater change on Motor Activity Log-28 (P = .013) from baseline to 6 weeks and Motor Activity Log items (P = .006) from baseline to 12 weeks compared to Chedoke-McMaster Assessment Hand-Lower Function group. Conclusions BTX-A injections and postinjection therapy improved hand function and reduced spasticity for both Chedoke-McMaster Assessment Hand-Higher Function and Chedoke-McMaster Assessment Hand-Lower Function groups. Clinicians should expect to see larger gains for persons with less baseline impairment. PMID:19735772

Delayed finger tapping and cognitive responses in preterm-born male teenagers with mild spastic diplegia.

PubMed

Gao, Fei; Mei, Xi; Chen, Andrew C N

2015-02-01

Information on fine motor and basic cognitive functions in spastic diplegia is sparse in the literature. The aim of this study was to investigate index finger's tapping speed and cognitive functions in categorization and old/new recognition of pictures in patients with mild spastic diplegia. Fifteen preterm-born male teenagers with mild spastic diplegia and 15 healthy male teenagers participated in this study. Finger-tapping tests and cognitive tests were performed on all participants. Outcomes were compared between the two groups. In the finger-tapping tests, the tapping speed was significantly slower in patients than in controls. In the tests of tapping one key persistently and tapping two keys alternately, the reaction time gaps between the left and right digits were larger in patients than in controls. In the categorization tests, the accuracies and reaction times for animal/plant and girl face pictures, but not for boy face pictures, were significantly worse in patients than in controls. In the recognition tests, the accuracies for old/new, animal/plant, and boy/girl face pictures were significantly lower in patients than in controls. The reaction times for old/new, animal/plant, and new face pictures, but not for old face pictures, were significantly longer in patients compared with controls. Our results demonstrate delayed finger tapping and cognitive responses in preterm-born male teenagers with mild spastic diplegia. Our experimental paradigm is sensitive for the study of fine motor and cognitive functions between patients and healthy controls. Copyright © 2015 Elsevier Inc. All rights reserved.

Long-Term Data of Efficacy, Safety, and Tolerability in a Real-Life Setting of THC/CBD Oromucosal Spray-Treated Multiple Sclerosis Patients.

PubMed

Paolicelli, Damiano; Direnzo, Vita; Manni, Alessia; D'Onghia, Mariangela; Tortorella, Carla; Zoccolella, Stefano; Di Lecce, Valentina; Iaffaldano, Antonio; Trojano, Maria

2016-07-01

Delta-9-tetrahydrocannabinol (THC)/cannabidiol (CBD) oromucosal spray was approved as add-on therapy for spasticity in patients with multiple sclerosis (MS). We show our 40-week postmarketing experience regarding efficacy and safety of THC/CBD spray in an Italian cohort of 102 MS patients. Patients were evaluated using the Expanded Disability Status Scale (EDSS) score, the Numerical Rating Scale (NRS) for spasticity, the Ambulation Index (AI), and Timed 25-Foot Walk (T25-FW) at the beginning of treatment and then every 3 months. After 4 weeks, if a clinically significant improvement in spasticity (at least 20% of baseline NRS score) was not seen, administration of the drug was stopped. In our cohort, patients received an average of 6.5 ± 1.6 sprays each day. The mean reduction to the NRS spasticity score was 2.5 ± 1.2 points (P < .0001). Thirty-seven patients (36.2%) discontinued the treatment. The incidence of adverse events (AEs) was 40.2%. Fifty-eight patients (56.9%) were also assessed using the NRS for pain, and 46 patients (45.1%) with bladder dysfunction were assessed for the IPSS (International Prostatic Symptoms Score) score, showing a significant improvement in these scales (P = .011 and P = .001, respectively). In conclusion, treatment with THC/CBD spray appears to be a valid answer to some of the unmet needs in MS patients, such as spasticity and other refractory-to-treatment symptoms. © 2015, The American College of Clinical Pharmacology.

[Effectiveness of acupuncture in spasticity of the post-stroke patient. Systematic review].

PubMed

Rodríguez-Mansilla, Juan; Espejo-Antúnez, Luis; Bustamante-López, Ana Isabel

2016-04-01

To determine the effectiveness of acupuncture for reducing spasticity in post-stroke patients. Literature review. The literature search was performed using scientific databases from January 2000 to January 2013. Out of the 110 studies that were found, nine random and controlled trials were included. Inclusion criteria were based on clinical trials in which participants were over 18 years old, who were suffering with post-stroke spasticity, and one of the experimental groups was treated with acupuncture. The variables were the passive resistance to stretching of the affected limb, and the degree of personal dependence. The variables were assessed by the Modified Ashworth Scale and Barthel Index. The search was performed in the PUBMED, COCHRANE Library, PEDro, Dialnet, CSIC, CINAHL, databases. Search terms included the combination of keywords "acupuncture"; "muscle spasticity"; "stroke". Passive resistance to stretching, the degree of personal dependence, and motor function of the affected limb showed statistically significant improvements in at least one study included; however, these improvements are not clinically relevant changes. Passive resistance improved in the elbow, ankle, knee, and wrist. An increased joint range was observed, except for the elbow, forearm, and thumb. Improved of the patient dependency was also observed. Although improvements relative to the reduction of spasticity are shown, the results have failed to demonstrate the effectiveness of the technique for this ailment. It would take a greater number of studies to calculate the size of the reported effects with homogeneous procedures in the design as well as in the duration, frequency, and measurement tools. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

In Vivo Evidence for Lysosome Depletion and Impaired Autophagic Clearance in Hereditary Spastic Paraplegia Type SPG11

PubMed Central

Varga, Rita-Eva; Khundadze, Mukhran; Damme, Markus; Nietzsche, Sandor; Hoffmann, Birgit; Stauber, Tobias; Koch, Nicole; Hennings, J. Christopher; Franzka, Patricia; Huebner, Antje K.; Kessels, Michael M.; Biskup, Christoph; Jentsch, Thomas J.; Qualmann, Britta; Braulke, Thomas; Kurth, Ingo; Beetz, Christian; Hübner, Christian A.

2015-01-01

Hereditary spastic paraplegia (HSP) is characterized by a dying back degeneration of corticospinal axons which leads to progressive weakness and spasticity of the legs. SPG11 is the most common autosomal-recessive form of HSPs and is caused by mutations in SPG11. A recent in vitro study suggested that Spatacsin, the respective gene product, is needed for the recycling of lysosomes from autolysosomes, a process known as autophagic lysosome reformation. The relevance of this observation for hereditary spastic paraplegia, however, has remained unclear. Here, we report that disruption of Spatacsin in mice indeed causes hereditary spastic paraplegia-like phenotypes with loss of cortical neurons and Purkinje cells. Degenerating neurons accumulate autofluorescent material, which stains for the lysosomal protein Lamp1 and for p62, a marker of substrate destined to be degraded by autophagy, and hence appears to be related to autolysosomes. Supporting a more generalized defect of autophagy, levels of lipidated LC3 are increased in Spatacsin knockout mouse embryonic fibrobasts (MEFs). Though distinct parameters of lysosomal function like processing of cathepsin D and lysosomal pH are preserved, lysosome numbers are reduced in knockout MEFs and the recovery of lysosomes during sustained starvation impaired consistent with a defect of autophagic lysosome reformation. Because lysosomes are reduced in cortical neurons and Purkinje cells in vivo, we propose that the decreased number of lysosomes available for fusion with autophagosomes impairs autolysosomal clearance, results in the accumulation of undegraded material and finally causes death of particularly sensitive neurons like cortical motoneurons and Purkinje cells in knockout mice. PMID:26284655

In Vivo Evidence for Lysosome Depletion and Impaired Autophagic Clearance in Hereditary Spastic Paraplegia Type SPG11.

PubMed

Varga, Rita-Eva; Khundadze, Mukhran; Damme, Markus; Nietzsche, Sandor; Hoffmann, Birgit; Stauber, Tobias; Koch, Nicole; Hennings, J Christopher; Franzka, Patricia; Huebner, Antje K; Kessels, Michael M; Biskup, Christoph; Jentsch, Thomas J; Qualmann, Britta; Braulke, Thomas; Kurth, Ingo; Beetz, Christian; Hübner, Christian A

2015-08-01

Hereditary spastic paraplegia (HSP) is characterized by a dying back degeneration of corticospinal axons which leads to progressive weakness and spasticity of the legs. SPG11 is the most common autosomal-recessive form of HSPs and is caused by mutations in SPG11. A recent in vitro study suggested that Spatacsin, the respective gene product, is needed for the recycling of lysosomes from autolysosomes, a process known as autophagic lysosome reformation. The relevance of this observation for hereditary spastic paraplegia, however, has remained unclear. Here, we report that disruption of Spatacsin in mice indeed causes hereditary spastic paraplegia-like phenotypes with loss of cortical neurons and Purkinje cells. Degenerating neurons accumulate autofluorescent material, which stains for the lysosomal protein Lamp1 and for p62, a marker of substrate destined to be degraded by autophagy, and hence appears to be related to autolysosomes. Supporting a more generalized defect of autophagy, levels of lipidated LC3 are increased in Spatacsin knockout mouse embryonic fibrobasts (MEFs). Though distinct parameters of lysosomal function like processing of cathepsin D and lysosomal pH are preserved, lysosome numbers are reduced in knockout MEFs and the recovery of lysosomes during sustained starvation impaired consistent with a defect of autophagic lysosome reformation. Because lysosomes are reduced in cortical neurons and Purkinje cells in vivo, we propose that the decreased number of lysosomes available for fusion with autophagosomes impairs autolysosomal clearance, results in the accumulation of undegraded material and finally causes death of particularly sensitive neurons like cortical motoneurons and Purkinje cells in knockout mice.

Randomised double blind placebo controlled trial of the effect of botulinum toxin on walking in cerebral palsy

PubMed Central

Ubhi, T; Bhakta, B; Ives, H; Allgar, V; Roussounis, S

2000-01-01

BACKGROUND—Cerebral palsy is the commonest cause of severe physical disability in childhood. For many years treatment has centred on the use of physiotherapy and orthotics to overcome the problems of leg spasticity, which interferes with walking and can lead to limb deformity. Intramuscular botulinum toxin (BT-A) offers a targeted form of therapy to reduce spasticity in specific muscle groups.
AIMS—To determine whether intramuscular BT-A can improve walking in children with cerebral palsy.
DESIGN—Randomised, double blind, placebo controlled trial.
METHODS—Forty patients with spastic diplegia or hemiplegia were enrolled. Twenty two received botulinum toxin and 18 received placebo. The primary outcome measure was video gait analysis and secondary outcome measures were gross motor function measure (GMFM), physiological cost index (PCI), and passive ankle dorsiflexion.
RESULTS—Video gait analysis showed clinically and statistically significant improvement in initial foot contact following BT-A at six weeks and 12 weeks compared to placebo. Forty eight per cent of BT-A treated children showed clinical improvement in VGA compared to 17% of placebo treated children. The GMFM (walking dimension) showed a statistically significant improvement in favour of the botulinum toxin treated group. Changes in PCI and passive ankle dorsiflexion were not statistically significant.
CONCLUSION—The study gives further support to the use of intramuscular botulinum toxin type A as an adjunct to conventional physiotherapy and orthoses to reduce spasticity and improve functional mobility in children with spastic diplegic or hemiplegic cerebral palsy.

 PMID:11087280

Ascending paralysis associated with HIV infection

PubMed Central

Afzal, Aasim; Benjamin, Mina; Gummelt, Kyle L.; Afzal, Sadaf; Tribble, Marc

2015-01-01

We present two patients with a high viral load of HIV-1 who developed symptoms of ascending paralysis leading to respiratory failure and autonomic instability. One patient had symptom improvement with highly active antiretroviral therapy (HAART) and a subsequent decrease in viral load. The other patient improved with intravenous immunoglobulin therapy and did not show much improvement on HAART alone. There are several proposed mechanisms for peripheral neuropathies seen in HIV-infected patients, including a direct action of HIV on the nerve by neurotropic strains or formation of autoantibodies against nerve elements. The comparison of the response to different therapies in these two cases highlights the importance of understanding different pathophysiologies, as the treatment modality may differ. PMID:25552790

A list of the 70 species of Australian ticks; diagnostic guides to and species accounts of Ixodes holocyclus (paralysis tick), Ixodes cornuatus (southern paralysis tick) and Rhipicephalus australis (Australian cattle tick); and consideration of the place of Australia in the evolution of ticks with comments on four controversial ideas.

PubMed

Barker, Stephen C; Walker, Alan R; Campelo, Dayana

2014-10-15

Seventy species of ticks are known from Australia: 14 soft ticks (family Argasidae) and 56 hard ticks (family Ixodidae). Sixteen of the 70 ticks in Australia may feed on humans and domestic animals (Barker and Walker 2014). The other 54 species of ticks in Australia feed only on wild mammals, reptiles and birds. At least 12 of the species of ticks in Australian also occur in Papua New Guinea. We use an image-matching system much like the image-matching systems of field guides to birds and flowers to identify Ixodes holocyclus (paralysis tick), Ixodes cornuatus (southern paralysis tick) and Rhipicephalus (Boophilus) australis (Australian cattle tick). Our species accounts have reviews of the literature on I. holocyclus (paralysis tick) from the first paper on the biology of an Australian tick by Bancroft (1884), on paralysis of dogs by I. holocyclus, to papers published recently, and of I. cornuatus (southern paralysis tick) and Rhipicephalus (Boophilus) australis (Australian cattle tick). We comment on four controversial questions in the evolutionary biology of ticks: (i) were labyrinthodont amphibians in Australia in the Devonian the first hosts of soft, hard and nuttalliellid ticks?; (ii) are the nuttalliellid ticks the sister-group to the hard ticks or the soft ticks?; (iii) is Nuttalliella namaqua the missing link between the soft and hard ticks?; and (iv) the evidence for a lineage of large bodied parasitiform mites (ticks plus the holothyrid mites plus the opiliocarid mites). Copyright © 2014. Published by Elsevier Ltd.

Benefits of aerobic exercise after stroke.

PubMed

Potempa, K; Braun, L T; Tinknell, T; Popovich, J

1996-05-01

The debilitating loss of function after a stroke has both primary and secondary effects on sensorimotor function. Primary effects include paresis, paralysis, spasticity, and sensory-perceptual dysfunction due to upper motor neuron damage. Secondary effects, contractures and disuse muscle atrophy, are also debilitating. This paper presents theoretical and empirical benefits of aerobic exercise after stroke, issues relevant to measuring peak capacity, exercise training protocols, and the clinical use of aerobic exercise in this patient population. A stroke, and resulting hemiparesis, produces physiological changes in muscle fibres and muscle metabolism during exercise. These changes, along with comorbid cardiovascular disease, must be considered when exercising stroke patients. While few studies have measured peak exercise capacity in hemiparetic populations, it has been consistently observed in these studies that stroke patients have a lower functional capacity than healthy populations. Hemiparetic patients have low peak exercise responses probably due to a reduced number of motor units available for recruitment during dynamic exercise, the reduced oxidative capacity of paretic muscle, and decreased overall endurance. Consequently, traditional methods to predict aerobic capacity are not appropriate for use with stroke patients. Endurance exercise training is increasingly recognised as an important component in rehabilitation. An average improvement in maximal oxygen consumption (VO2max) of 13.3% in stroke patients who participated in a 10-week aerobic exercise training programme has been reported compared with controls. This study underscored the potential benefits of aerobic exercise training in stroke patients. In this paper, advantages and disadvantages of exercise modalities are discussed in relation to stroke patients. Recommendations are presented to maximise physical performance and minimise potential cardiac risks during exercise.

Two Boys with Multiple Disabilities Increasing Adaptive Responding and Curbing Dystonic/Spastic Behavior via a Microswitch-Based Program

ERIC Educational Resources Information Center

Lancioni, Giulio E.; Singh, Nirbhay N.; O'Reilly, Mark F.; Sigafoos, Jeff; Didden, Robert; Oliva, Doretta

2009-01-01

A recent study has shown that microswitch clusters (i.e., combinations of microswitches) and contingent stimulation could be used to increase adaptive responding and reduce dystonic/spastic behavior in two children with multiple disabilities [Lancioni, G. E., Singh, N. N., Oliva, D., Scalini, L., & Groeneweg, J. (2003). Microswitch clusters to…

Clinical Characteristics of Impaired Trunk Control in Children with Spastic Cerebral Palsy

ERIC Educational Resources Information Center

Heyrman, Lieve; Desloovere, Kaat; Molenaers, Guy; Verheyden, Geert; Klingels, Katrijn; Monbaliu, Elegast; Feys, Hilde

2013-01-01

This study aimed to identify clinical characteristics of impaired trunk control in hundred children with spastic CP (mean age 11.4 [plus or minus] 2.1 years, range 8-15 years). Assessment of trunk control was performed with the Trunk Control Measurement Scale (TCMS). Trunk control was clearly impaired, indicated by a median total TCMS score of…

REEP1 Mutation Spectrum and Genotype/Phenotype Correlation in Hereditary Spastic Paraplegia Type 31

ERIC Educational Resources Information Center

Beetz, Christian; Schule, Rebecca; Deconinck, Tine; Tran-Viet, Khanh-Nhat; Zhu, Hui; Kremer, Berry P. H.; Frints, Suzanna G. M.; van Zelst-Stams, Wendy A. G.; Byrne, Paula; Otto, Susanne; Nygren, Anders O. H.; Baets, Jonathan; Smets, Katrien; Ceulemans, Berten; Dan, Bernard; Nagan, Narasimhan; Kassubek, Jan; Klimpe, Sven; Klopstock, Thomas; Stolze, Henning; Smeets, Hubert J. M.; Schrander-Stumpel, Constance T. R. M.; Hutchinson, Michael; van de Warrenburg, Bart P.; Braastad, Corey; Deufel, Thomas; Pericak-Vance, Margaret; Schols, Ludger; de Jonghe, Peter; Zuchner, Stephan

2008-01-01

Mutations in the receptor expression enhancing protein 1 (REEP1) have recently been reported to cause autosomal dominant hereditary spastic paraplegia (HSP) type SPG31. In a large collaborative effort, we screened a sample of 535 unrelated HSP patients for "REEP1" mutations and copy number variations. We identified 13 novel and 2 known "REEP1"…

Controlled Study of the Effects of Continuous Intrathecal Baclofen Infusion in Non-Ambulant Children with Cerebral Palsy

ERIC Educational Resources Information Center

Morton, Richard E.; Gray, Natalie; Vloeberghs, Michael

2011-01-01

Aim: To measure changes in children with severe spastic cerebral palsy (CP) after continuous intrathecal baclofen (ITB) infusion over 18 months and to compare the results with those of a comparison group awaiting treatment. Method: Thirty-eight children with severe spastic CP considered suitable for ITB were assessed when first seen, just before…

Changes in Muscle Activation Following Ankle Strength Training in Children with Spastic Cerebral Palsy: An Electromyography Feasibility Case Report

ERIC Educational Resources Information Center

Olsen, Jamie E.; Ross, Sandy A.; Foreman, Matthew H.; Engsberg, Jack R.

2013-01-01

Children with cerebral palsy (CP) are likely to experience decreased participation in activities and less competence in activities of daily living. Studies of children with spastic CP have shown that strengthening programs produce positive results in strength, gait, and functional outcomes (measured by the Gross Motor Function Measure). No…

Intrathecal Baclofen in Children with Spastic Cerebral Palsy: A Double-Blind, Randomized, Placebo-Controlled, Dose-Finding Study

ERIC Educational Resources Information Center

Hoving, Marjanke A.; van Raak, Elisabeth P. M.; Spincemaille, Geert H. J. J.; Palmans, Liesbeth J.; Sleypen, Frans A. M.; Vles, Johan S. H.

2007-01-01

Intrathecal baclofen (ITB) therapy can be very effective in the treatment of intractable spasticity, but its effectiveness and safety have not yet been thoroughly studied in children with cerebral palsy (CP). The aims of this double-blind, randomized, placebo-controlled, dose-finding study were to select children eligible for continuous ITB…

Neonatal peripheral facial paralysis' evaluation with photogrammetry: A case report.

PubMed

da Fonseca Filho, Gentil Gomes; de Medeiros Cirne, Gabriele Natane; Cacho, Roberta Oliveira; de Souza, Jane Carla; Nagem, Danilo; Cacho, Enio Walker Azevedo; Moran, Cristiane Aparecida; Abreu, Bruna; Pereira, Silvana Alves

2015-12-01

Facial paralysis in newborns can leave functional sequelae. Determining the evolution and amount of functional losses requires consistent evaluation methods that measure, quantitatively, the evolution of clinical functionality. This paper reports an innovative method of facial assessment for the case of a child 28 days of age with unilateral facial paralysis. The child had difficulty breast feeding, and quickly responded to the physical therapy treatment. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

A preliminary study of simple voice assessment in a routine clinical setting to predict vocal cord paralysis after thyroid or parathyroid surgery.

PubMed

Hanna, B C; Brooker, D S

2008-02-01

To determine if a simple assessment of vocal cord function performed in a routine clinical setting can predict vocal cord paralysis on laryngoscopy in patients following thyroid or parathyroid surgery. Prospective observational cohort study of patients undergoing thyroid or parathyroid surgery. Vocal cord function was assessed in terms of an abnormality of voice or cough reported by the patient or detected by the examiner. Laryngoscopy was performed by indirect mirror examination or fibreoptic nasopharyngolaryngoscopy. Examiners included otolaryngology consultants, registrars, senior house officers and a general practitioner with a special interest in otolaryngology. The patient was often examined by a different clinician before and after surgery. Eighty-six patients participated in the study between August 2003 and July 2004. Eleven had a change in their voice postoperatively and one patient had difficulty coughing. Six of these patients had a vocal cord paralysis. A further five apparently asymptomatic patients had a vocal cord paralysis. The positive predictive value of voice assessment for vocal cord paralysis was 55%. In a routine clinical setting where the assessment of vocal cord function is often reported by different clinicians of varying experience at each patient attendance, the assessment of a patient's voice following thyroid or parathyroid surgery is not adequately predictive of vocal cord paralysis on indirect laryngoscopy or fibreoptic nasopharyngolaryngoscopy. Further study is required to determine reasons for this.

Two Distinct Types of Hypercontractile Esophagus: Classic and Spastic Jackhammer

PubMed Central

Hong, Yun Soo; Min, Yang Won; Rhee, Poong-Lyul

2016-01-01

Hypercontractile esophagus (nicknamed jackhammer esophagus) is a recently defined disease within the esophageal motility disorders classification. Responses to treatments for jackhammer esophagus have been inconsistent in previous trials, possibly due to its heterogeneous manifestation. Thus, we reviewed 10 patients diagnosed with jackhammer esophagus and compared their clinical and manometric features at baseline. Additionally, manometric and symptomatic responses after treatment with known smooth muscle relaxants, including anticholinergic drugs (cimetropium bromide and scopolamine butylbromide) and a phosphodiesterase-5 inhibitor (sildenafil) were compared. We observed two distinct subgroups in the findings: one with hypercontractility and normal distal latencies (“classic jackhammer esophagus,” n=7) and the other with hypercontractility and short distal latencies (“spastic jackhammer esophagus,” n=3). The two types also differed in their responses to medications in that symptoms improved upon treatment with an anticholinergic agent in classic jackhammer esophagus patients, while spastic jackhammer esophagus was unresponsive to both the anticholinergic drugs and the phosphodiesterase-5 inhibitor. In conclusion, hypercontractile esophagus may be a heterogeneous disease with different underlying pathophysiologies. We introduced two novel terms, “classic jackhammer esophagus” and “spastic jackhammer esophagus,” to distinguish the two types. PMID:27458179

Quantifying Spasticity With Limited Swinging Cycles Using Pendulum Test Based on Phase Amplitude Coupling.

PubMed

Yeh, Chien Hung; Young, Hsu Wen Vincent; Wang, Cheng Yen; Wang, Yung Hung; Lee, Po Lei; Kang, Jiunn Horng; Lo, Men Tzung

2016-10-01

Parameters derived from the goniometer measures in the Pendulum test are insufficient in describing the function of abnormal muscle activity in the spasticity. To explore a quantitative evaluation of muscle activation-movement interaction, we propose a novel index based on phase amplitude coupling (PAC) analysis with the consideration of the relations between movement and surface electromyography (SEMG) activity among 22 hemiplegic stroke patients. To take off trend and noise, we use the empirical mode decomposition (EMD) to obtain intrinsic mode functions (IMFs) of the angular velocity due to its superior decomposing ability in nonlinear oscillations. Shannon entropy based on angular velocity (phase)-envelope of EMG (amplitude) distribution was calculated to demonstrate characteristics of the coupling between SEMG activity and joint movement. We also compare our results with those from traditional methods such as the normalized relaxation index derived from the Pendulum test and the mean root mean square (RMS) of the SEMG signals in the study. Our results show effective discrimination ability between spastic and nonaffected limbs using our method . This study indicates the feasibility of using the novel indices based on the PAC in evaluation the spasticity among the hemiplegic stroke patients with less than three swinging cycles.

Autosomal recessive spastic paraplegia (SPG30) with mild ataxia and sensory neuropathy maps to chromosome 2q37.3.

PubMed

Klebe, Stephan; Azzedine, Hamid; Durr, Alexandra; Bastien, Patrick; Bouslam, Naima; Elleuch, Nizar; Forlani, Sylvie; Charon, Celine; Koenig, Michel; Melki, Judith; Brice, Alexis; Stevanin, Giovanni

2006-06-01

The hereditary spastic paraplegias (HSPs) are a clinically and genetically heterogeneous group of neurodegenerative diseases characterized by progressive spasticity in the lower limbs. Twenty-nine different loci (SPG) have been mapped so far, and 11 responsible genes have been identified. Clinically, one distinguishes between pure and complex HSP forms which are variably associated with numerous combinations of neurological and extra-neurological signs. Less is known about autosomal recessive forms (ARHSP) since the mapped loci have been identified often in single families and account for only a small percentage of patients. We report a new ARHSP locus (SPG30) on chromosome 2q37.3 in a consanguineous family with seven unaffected and four affected members of Algerian origin living in Eastern France with a significant multipoint lod score of 3.8. Ten other families from France (n = 4), Tunisia (n = 2), Algeria (n = 3) and the Czech Republic (n = 1) were not linked to the newly identified locus thus demonstrating further genetic heterogeneity. The phenotype of the linked family consists of spastic paraparesis and peripheral neuropathy associated with slight cerebellar signs confirmed by cerebellar atrophy on one CT scan.

A new approach to assess the spasticity in hamstrings muscles using mechanomyography antagonist muscular group.

PubMed

Krueger, Eddy; Scheeren, Eduardo M; Nogueira-Neto, Guilherme N; Button, Vera Lúcia da S N; Nohama, Percy

2012-01-01

Several pathologies can cause muscle spasticity. Modified Ashworth scale (MAS) can rank spasticity, however its results depend on the physician subjective evaluation. This study aims to show a new approach to spasticity assessment by means of MMG analysis of hamstrings antagonist muscle group (quadriceps muscle). Four subjects participated in the study, divided into two groups regarding MAS (MAS0 and MAS1). MMG sensors were positioned over the muscle belly of rectus femoris (RF), vastus lateralis (VL) and vastus medialis (VM) muscles. The range of movement was acquired with an electrogoniometer placed laterally to the knee. The system was based on a LabVIEW acquisition program and the MMG sensors were built with triaxial accelerometers. The subjects were submitted to stretching reflexes and the integral of the MMG (MMG(INT)) signal was calculated to analysis. The results showed that the MMG(INT) was greater to MAS1 than to MAS0 [muscle RF (p = 0.004), VL (p = 0.001) and VM (p = 0.007)]. The results showed that MMG was viable to detect a muscular tonus increase in antagonist muscular group (quadriceps femoris) of spinal cord injured volunteers.

Etiologic profile of spastic quadriplegia in children.

PubMed

Venkateswaran, Sunita; Shevell, Michael I

2007-09-01

The etiologic profile and possible predictors of etiology in children with spastic quadriplegia were assessed in a consecutive cohort of children with this motor impairment. Medical records from a single pediatric neurology practice over a 14-year interval were retrospectively and systematically reviewed. Variables comprised possible demographic, prenatal, perinatal, and postnatal risk factors. Of the 99 patients included in the study, 39 were premature (<37 weeks gestation). The overall etiologic yield was 83%. The top three diagnoses were hypoxic-ischemic perinatal asphyxia (33%), periventricular leukomalacia (15%), and central nervous system infections (11%). In premature children, the most common diagnoses were periventricular leukomalacia (33%), perinatal asphyxia (26%), and central nervous system infections (15%). In term-born children, the most frequent diagnoses were perinatal asphyxia (37%), metabolic disease (12%), and structural malformation or infection (9% each). Factors predicting the identification of an etiology included male sex (P = 0.05), low birth weight (P = 0.003), prematurity (P = 0.01), perinatal complications (P = 0.002), and neonatal encephalopathy (P = 0.006). The etiologic yield in patients with spastic quadriplegia was 83%, with differing underlying etiologies depending on gestational age. These results should help guide physicians in investigating possible underlying etiologies in patients with spastic quadriplegia.