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Sample records for proximal brachial amyotrophic

  1. Amyotrophic Lateral Sclerosis Regional Variants (Brachial Amyotrophic Diplegia, Leg Amyotrophic Diplegia, and Isolated Bulbar Amyotrophic Lateral Sclerosis).

    PubMed

    Jawdat, Omar; Statland, Jeffrey M; Barohn, Richard J; Katz, Jonathan S; Dimachkie, Mazen M

    2015-11-01

    Amyotrophic lateral sclerosis (ALS), a rapidly progressive, invariably fatal disease, involves mixed upper and lower motor neurons in different spinal cord regions. Patients with bulbar onset progress more rapidly than patients with limb onset or with a lower motor neuron presentation. Recent descriptions of regional variants suggest some patients have ALS isolated to a single spinal region for many years, including brachial amyotrophic diplegia, leg amyotrophic diplegia, and isolated bulbar palsy. Clearer definitions of regional variants will have implications for prognosis, understanding the pathophysiology of ALS, identifying genetic factors related to slower disease progression, and future planning of clinical trials.

  2. Proximal versus Distal Nerve Transfer for Biceps Reinnervation—A Comparative Study in a Rat’s Brachial Plexus Injury Model

    PubMed Central

    McGrath, Aleksandra M.; Lu, Johnny Chuieng-Yi; Chang, Tommy Naj-Jen; Fang, Frank

    2016-01-01

    Background: The exact role of proximal and distal nerve transfers in reconstruction strategies of brachial plexus injury remains controversial. We compared proximal with distal nerve reconstruction strategies in a rat model of brachial plexus injury. Methods: In rats, the C6 spinal nerve with a nerve graft (proximal nerve transfer model, n = 30, group A) and 50% of ulnar nerve (distal nerve transfer model, n = 30, group B) were used as the donor nerves. The targets were the musculocutaneous nerve and the biceps muscle. Outcomes were recorded at 4, 8, 12, and 16 weeks postoperatively. Outcome parameters included grooming test, biceps muscle weight, compound muscle action potentials, tetanic contraction force, and axonal morphology of the donor and target nerves. Results: The axonal morphology of the 2 donor nerves revealed no significant difference. Time interval analysis in the proximal nerve transfer group showed peak axon counts at 12 weeks and a trend of improvement in all functional and physiologic parameters across all time points with statistically significant differences for grooming test, biceps compound action potentials, tetanic muscle contraction force, and muscle weight at 16 weeks. In contrast, in the distal nerve transfer group, the only statistically significant difference was observed between the 4 and 8 week time points, followed by a plateau from 8 to 16 weeks. Conclusions: Outcomes of proximal nerve transfers are ultimately superior to distal nerve transfers in our experimental model. Possible explanations for the superior results include a reduced need for cortical adaptation and higher proportions of motor units in the proximal nerve transfers. PMID:28293499

  3. CONSIDERATION OF DOSE LIMITS FOR ORGANS AT RISK OF THORACIC RADIOTHERAPY: ATLAS FOR LUNG, PROXIMAL BRONCHIAL TREE, ESOPHAGUS, SPINAL CORD, RIBS, AND BRACHIAL PLEXUS

    PubMed Central

    Kong, Feng-Ming (Spring); Ritter, Timothy; Quint, Douglas J.; Senan, Suresh; Gaspar, Laurie E.; Komaki, Ritsuko U.; Hurkmans, Coen W.; Timmerman, Robert; Bezjak, Andrea; Bradley, Jeffrey D.; Movsas, Benjamin; Marsh, Lon; Okunieff, Paul; Choy, Hak; Curran, Walter J.

    2012-01-01

    Purpose To review the dose limits and standardize the three-dimenional (3D) radiographic definition for the organs at risk (OARs) for thoracic radiotherapy (RT), including the lung, proximal bronchial tree, esophagus, spinal cord, ribs, and brachial plexus. Methods and Materials The present study was performed by representatives from the Radiation Therapy Oncology Group, European Organization for Research and Treatment of Cancer, and Soutwestern Oncology Group lung cancer committees. The dosimetric constraints of major multicenter trials of 3D-conformal RT and stereotactic body RT were reviewed and the challenges of 3D delineation of these OARs described. Using knowledge of the human anatomy and 3D radiographic correlation, draft atlases were generated by a radiation oncologist, medical physicist, dosimetrist, and radiologist from the United States and reviewed by a radiation oncologist and medical physicist from Europe. The atlases were then critically reviewed, discussed, and edited by another 10 radiation oncologists. Results Three-dimensional descriptions of the lung, proximal bronchial tree, esophagus, spinal cord, ribs, and brachial plexus are presented. Two computed tomography atlases were developed: one for the middle and lower thoracic OARs (except for the heart) and one focusing on the brachial plexus for a patient positioned supine with their arms up for thoracic RT. The dosimetric limits of the key OARs are discussed. Conclusions We believe these atlases will allow us to define OARs with less variation and generate dosimetric data in a more consistent manner. This could help us study the effect of radiation on these OARs and guide high-quality clinical trials and individualized practice in 3D-conformal RT and stereotactic body RT. PMID:20934273

  4. Brachial plexopathy

    MedlinePlus

    ... the muscles Weakness of hand flexing A detailed history may help determine the cause of the brachial plexopathy. Age and sex are important, because some brachial plexus problems are more common in certain groups. For example, young men more often have inflammatory or post-viral ...

  5. Brachial plexopathy

    PubMed Central

    Khadilkar, Satish V.; Khade, Snehaldatta S.

    2013-01-01

    Brachial plexus injury can occur as a result of trauma, inflammation or malignancies, and associated complications. The current topic is concerned with various forms of brachial plexopathy, its clinical features, pathophysiology, imaging findings, and management. Idiopathic brachial neuritis (IBN), often preceded with antecedent events such as infection, commonly present with abruptonset painful asymmetric upper limb weakness with associated wasting around the shoulder girdle and arm muscles. Idiopathic hypertrophic brachial neuritis, a rare condition, is usually painless to begin with, unlike IBN. Hereditary neuralgic amyotrophy is an autosomal-dominant disorder characterized by repeated episodes of paralysis and sensory disturbances in an affected limb, which is preceded by severe pain. While the frequency of the episodes tends to decrease with age, affected individuals suffer from residual deficits. Neurogenic thoracic outlet syndrome affects the lower trunk of the brachial plexus. It is diagnosed on the basis of electrophysiology and is amenable to surgical intervention. Cancer-related brachial plexopathy may occur secondary to metastatic infiltration or radiation therapy. Traumatic brachial plexus injury is commonly encountered in neurology, orthopedic, and plastic surgery set-ups. Trauma may be a direct blow or traction or stretch injury. The prognosis depends on the extent and site of injury as well as the surgical expertise. PMID:23661957

  6. Brachial neuritis.

    PubMed

    Dillin, L; Hoaglund, F T; Scheck, M

    1985-07-01

    Brachial neuritis is an unusual syndrome of unknown etiology that can be confused with other causes of pain or weakness, or both, of the shoulder and arm. It is important to distinguish this disorder because of its dramatic symptoms and relatively good prognosis. Sharp pain, usually in the elbow or shoulder, marks the onset of brachial neuritis, but is relatively short-lived. Weakness generally occurs as the pain is subsiding and most frequently involves the deltoid, spinati, serratus anterior, biceps, and triceps. Paresthesias, atrophy, and sensory loss are inconstant features. Electromyographic findings of fibrillation potentials and positive waves characteristically are found in a pattern indicating combined nerve-root and peripheral nerve involvement. Electromyography more frequently than clinical examination shows that the lesion is bilateral, and also is of both diagnostic and prognostic value. Other laboratory studies serve only to exclude other causes of shoulder pain. The clinical course is variable, but in 90 per cent of patients complete recovery occurs within three years. Recurrences are uncommon.

  7. Brachial Plexus Injuries

    MedlinePlus

    ... to the shoulder, arm, and hand. Brachial plexus injuries are caused by damage to those nerves. Symptoms ... sensation in the arm or hand Brachial plexus injuries can occur as a result of shoulder trauma, ...

  8. Posterior subscapular dissection: An improved approach to the brachial plexus for human anatomy students.

    PubMed

    Hager, Shaun; Backus, Timothy Charles; Futterman, Bennett; Solounias, Nikos; Mihlbachler, Matthew C

    2014-05-01

    Students of human anatomy are required to understand the brachial plexus, from the proximal roots extending from spinal nerves C5 through T1, to the distal-most branches that innervate the shoulder and upper limb. However, in human cadaver dissection labs, students are often instructed to dissect the brachial plexus using an antero-axillary approach that incompletely exposes the brachial plexus. This approach readily exposes the distal segments of the brachial plexus but exposure of proximal and posterior segments require extensive dissection of neck and shoulder structures. Therefore, the proximal and posterior segments of the brachial plexus, including the roots, trunks, divisions, posterior cord and proximally branching peripheral nerves often remain unobserved during study of the cadaveric shoulder and brachial plexus. Here we introduce a subscapular approach that exposes the entire brachial plexus, with minimal amount of dissection or destruction of surrounding structures. Lateral retraction of the scapula reveals the entire length of the brachial plexus in the subscapular space, exposing the brachial plexus roots and other proximal segments. Combining the subscapular approach with the traditional antero-axillary approach allows students to observe the cadaveric brachial plexus in its entirety. Exposure of the brachial dissection in the subscapular space requires little time and is easily incorporated into a preexisting anatomy lab curriculum without scheduling additional time for dissection.

  9. Brachial plexopathy as a rare presenting manifestation of scorpion envenomation.

    PubMed

    Rubin, Devon I; Vavra, Michael

    2011-07-01

    We report a patient who experienced a rare manifestation of an acute, severe brachial plexopathy as the initial complication of scorpion (presumed Hemiscorpius lepturus species) envenomation. Features suggesting conduction block, due to either proximal demyelination or ion channel dysfunction, along with axonal loss were seen on serial electrophysiological studies. Possible mechanisms of the brachial plexopathy include direct compression from tissue edema or a toxic effect on the membrane channels along the nerve.

  10. Recurrent brachial plexus neuropathy.

    PubMed

    Bradley, W G; Madrid, R; Thrush, D C; Campbell, M J

    1975-09-01

    The clinical, electrophysiological and pathological changes in 3 patients with recurrent attacks of non-traumatic brachial plexus neuropathy have been described. Two had recurrent attacks and a dominant family history of similar attacks, together with evidence of lesser degrees of nerve involvement outside the brachial plexus. In one patient the attacks were moderately painful, while in the other there was little or no pain. Only one showed undue slowing of motor nerve conduction during ischaemia, but in both cases the sural nerves had the changes of tomaculous neuropathy, with many sausage-shaped swellings of the myelin sheaths, and extensive segmental demyelination and remyelination. The third patient had two attacks of acute brachial plexus neuropathy which were both extremely painful. The clinical features were compatible with a diagnosis of neuralgic amuotrophy. In the second attack, there was vagus nerve involvement and the sural nerve showed evidence of healed extensive segmental demyelination. The various syndromes presenting with acute non-traumatic brachial plexus neuropathy are reviewed, and a tentative nonsological classification advanced. Most patients fall into the category of acute, painful paralysis with amyotrophy, with no family history and no evidence of lesions outside the brachial plexus. It is suggested that the term "neuralgic amyotrophy" be restricted to this group. Patients with features outside this clinical picture probably suffer from other disease entities presenting with brachial plexus neuropathy. The familial cases constitute one or more aetioliogical subgroups, differing from neuralgic amyotrophy in the frequency of recurrences, the relative freedom from pain in the attacks, the frequency of nerve lesions outside the brachial plexus, and of hypotelorism. Individual attacks of acute brachial plexus neuropathy, however, may be identical in patients with the different diseases, and further pathological and biochemical studies are

  11. Amyotrophic Lateral Sclerosis

    MedlinePlus

    Amyotrophic lateral sclerosis Overview By Mayo Clinic Staff Amyotrophic lateral sclerosis (a-my-o-TROE-fik LAT-ur-ul skluh-ROE-sis), or ALS, is a progressive nervous system (neurological) disease that ...

  12. Obstetrical brachial plexus palsy.

    PubMed

    Romaña, M C; Rogier, A

    2013-01-01

    Obstetrical brachial plexus palsy is considered to be the result of a trauma during the delivery, even if there remains some controversy surrounding the causes. Although most babies recover spontaneously in the first 3 months of life, a small number remains with poor recovery which requires surgical brachial plexus exploration. Surgical indications depend on the type of lesion (producing total or partial palsy) and particularly the nonrecovery of biceps function by the age of 3 months. In a global palsy, microsurgery will be mandatory and the strategy for restoration will focus first on hand reinnervation and secondarily on providing elbow flexion and shoulder stability. Further procedures may be necessary during growth in order to avoid fixed contractured deformities or to give or increase strength of important muscle functions like elbow flexion or wrist extension. The author reviews the history of obstetrical brachial plexus injury, epidemiology, and the specifics of descriptive and functional anatomy in babies and children. Clinical manifestations at birth are directly correlated with the anatomical lesion. Finally, operative procedures are considered, including strategies of reconstruction with nerve grafting in infants and secondary surgery to increase functional capacity at later ages. However, normal function is usually not recovered, particularly in total brachial plexus palsy.

  13. Traumatic Brachial Artery Injuries

    PubMed Central

    Ergunes, Kazim; Yilik, Levent; Ozsoyler, Ibrahim; Kestelli, Mert; Ozbek, Cengiz; Gurbuz, Ali

    2006-01-01

    We performed this retrospective study to analyze our strategies for managing and surgically treating brachial artery injuries. Fifty-seven patients with a total of 58 traumatic brachial artery injuries underwent surgery at our institution, from August 1996 through November 2004. Fifty-four patients were male and 3 were female (age range, 7 to 75 years; mean, 29.4 years). Forty-four of the patients had penetrating injuries (18 had stab wounds; 16, window glass injuries; and 10, industrial accidents), 10 had blunt trauma injuries (traffic accidents), and 3 had gunshot injuries. Fourteen patients (24.6%) had peripheral nerve injury. All patients underwent Doppler ultrasonographic examination. The repair of the 58 arterial injuries involved end-to-end anastomosis for 32 injuries (55.2%), reverse saphenous vein graft interpositional grafts for 18 (31%), and primary repair for 8 (13.8%). Venous continuity was achieved in 11 (84.6%) of 13 patients who had major venous injuries. Nine of the 57 patients (15.8%) required primary fasciotomy. Follow-up showed that 5 of the 14 patients with peripheral nerve injury had apparent disabilities due to nerve injury. One patient underwent amputation. There were no deaths. We believe that good results can be achieved in patients with brachial artery injuries by use of careful physical examination, Doppler ultrasonography, and restoration of viability with vascular repair and dbridement of nonviable tissues. Traumatic neurologic injury frequently leads to disability of the extremities. PMID:16572866

  14. [Amyotrophic neuralgia associated with bilateral phrenic paralysis treated with non-invasive mechanical ventilation].

    PubMed

    García García, María Del Carmen; Hernández Borge, Jacinto; Antona Rodríguez, María José; Pires Gonçalves, Pedro; García García, Gema

    2015-09-07

    Amyotrophic neuralgia is an uncommon neuropathy characterized by severe unilateral shoulder pain. Isolated or concomitant involvement of other peripheral motor nerves depending on the brachial plexus such as phrenic or laryngeal nerves is unusual(1). Its etiology is unknown, yet several explanatory factors have been proposed. Phrenic nerve involvement, either unilateral or bilateral, is exceedingly rare. Diagnosis relies on anamnesis, functional and imaging investigations and electromyogram. We report the case of a 48-year-old woman with a past history of renal transplantation due to proliferative glomerulonephritis with subsequent transplant rejection, who was eventually diagnosed with amyotrophic neuralgia with bilateral phrenic involvement, and who required sustained non-invasive mechanical ventilation.

  15. Amyotrophic Lateral Sclerosis

    MedlinePlus

    Amyotrophic lateral sclerosis (ALS) is a nervous system disease that attacks nerve cells called neurons in your brain and spinal cord. These neurons ... breathing machine can help, but most people with ALS die from respiratory failure. The disease usually strikes ...

  16. [Amyotrophic lateral sclerosis].

    PubMed

    Veldink, J H; Weikamp, J; Schelhaas, H J; van den Berg, L H

    2010-01-01

    Amyotrophic lateral sclerosis is one of the most severe and disabling diseases of the nervous system. Amyotrophic lateral sclerosis leads to the progressive weakening of the muscles in the arms, legs, face, mouth and trunk. The onset of the disease is insidious, starting with weakness in the hands or feet or with slurred speech. The weakness worsens and patients pass away as a result of weakness of the respiratory muscles on average within 3 years of the onset of the disease. In the Netherlands, approximately 400 patients are diagnosed with amyotrophic lateral sclerosis every year. There is no diagnostic test for this neuro-muscular disease; the diagnosis is established by excluding other disorders that resemble amyotrophic lateral sclerosis. Only one drug is able to inhibit the progression of the disease to any extent: riluzole. Treatment, therefore, is mainly focused on supportive measures and those which enhance the quality of life optimally.

  17. [Ankle brachial index measurement].

    PubMed

    Rucigaj, Tanja Planinsek

    2014-10-01

    Ultrasound examinations are noninvasive diagnostic methods which, along with appropriate history and clinical examination, provide basic information on the etiology and spread of the disease, as well as on treatment options required in patients with chronic venous insufficiency and arterial flow impairment. Doppler flow meter offers useful data on venous blood return, primarily in great veins, while both deep and superficial veins as well as arteries can be visualized and data on venous and arterial hemodynamics obtained by duplex ultrasonography. In addition, Doppler flow meter provides data on the peripheral arterial system action through ankle brachial index measurement, which will guide the choice of compression therapy when deciding on the treatment of peripheral arterial disease and mixed arteriovenous leg ulcers. However, diagnosis of arterial insufficiency requires additional examinations.

  18. Brachial plexus injury in newborns

    MedlinePlus

    Gentle massage of the arm and range-of-motion exercises are recommended for mild cases. The infant ... the American College of Obstetricians and Gynecologists' Task Force on Neonatal Brachial Plexus Palsy. Obstet Gynecol . 2014 ...

  19. 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

  20. MRI of the Brachial Plexus: Modified Imaging Technique Leading to a Better Characterization of Its Anatomy and Pathology

    PubMed Central

    Torres, Carlos; Mailley, Kathleen; del Carpio O’Donovan, Raquel

    2013-01-01

    Summary Magnetic resonance imaging (MRI) is the imaging modality of choice for the evaluation of the brachial plexus due to its superior soft tissue resolution and multiplanar capabilities. The evaluation of the brachial plexus however represents a diagnostic challenge for the clinician and the radiologist. The imaging assessment of the brachial plexus, in particular, has been traditionally challenging due to the complexity of its anatomy, its distribution in space and due to technical factors. Herein, we describe a modified technique used in our institution for the evaluation of the brachial plexus which led to a substantial decrease in scanning time and to better visualization of all the segments of the brachial plexus from the roots to the branches, in only one or two images, facilitating therefore the understanding of the anatomy and the interpretation of the study. To our knowledge, we are the first group to describe this technique of imaging the brachial plexus. We illustrate the benefit of this modified technique with an example of a patient with a lesion in the proximal branches of the left brachial plexus that was clinically suspected but missed on conventional brachial plexus imaging for six consecutive years. In addition, we review the common and infrequent benign and malignant pathology that can affect the brachial plexus. PMID:24355190

  1. Use of StarClose for brachial artery closure after percutaneous endovascular interventions.

    PubMed

    Puggioni, Alessandra; Boesmans, Evelyne; Deloose, Koen; Peeters, Patrick; Bosiers, Marc

    2008-01-01

    The objective of this study was to evaluate a percutaneous extravascular closure device (StarClose, Abbott Vascular, Redwood City, CA) after brachial endovascular approach. From 2004 to 2006, 29 patients received StarClose for brachial closure. Primary endpoints were successful deployment and absence of procedure-related morbidity, secondary endpoints were brachial artery patency on duplex and absence of late (> 30 days) complications. The device was successfully deployed in all patients. In two patients (6.8%) local complications occurred: one patient developed a large hematoma successfully treated with prolonged compression and a second patient presented with brachial artery occlusion requiring operative intervention. After a mean follow-up of 7.5+/-7.2 months, all patients had a palpable brachial/radial pulse; none had signs of infection, distal embolization or neurological deficits. On ultrasound b-mode imaging, the clip was visible as a 4 mm echolucent area at the outer anterior wall of the artery. Based on the peak systolic velocity ratios between the site of StarClose and proximal brachial artery (mean 1.08+/-0.2), none of the studied patients had a significant stenosis at the site of closure. StarClose is safe and effective in providing hemostasis following interventional procedures through the brachial artery; further advantages include patients comfort and early discharge.

  2. Amyotrophic Lateral Sclerosis Research Program

    DTIC Science & Technology

    2010-08-01

    U.S. Army Medical Research and Materiel Command Amyotrophic Lateral Sclerosis Research Program Report Documentation Page Form ApprovedOMB No. 0704...to 00-00-2010 4. TITLE AND SUBTITLE Amyotrophic Lateral Sclerosis Research Program 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM ELEMENT...research programs such as the Amyotrophic Lateral Sclerosis Research Program (ALSRP) is allo- cated via specific guidance from Congress. Proposal

  3. Amyotrophic lateral sclerosis.

    PubMed

    Malik, Rabia; Lui, Andrew; Lomen-Hoerth, Catherine

    2014-11-01

    Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting 20,000 to 30,000 people in the United States. The mainstay of care of patients affected by this disease is supportive and given the multifaceted nature of their needs is provided most efficiently through multidisciplinary clinics that have shown to prolong survival and improve quality of life. The authors discuss in detail evidence-based management of individuals affected by this condition.

  4. Conjugal amyotrophic lateral sclerosis

    PubMed Central

    Dewitt, John D.; Kwon, Julia; Burton, Rebecca

    2012-01-01

    Amyotrophic lateral sclerosis (ALS) is a disease characterized by progressive degeneration of motor neurons in the motor cortex, brainstem, and spinal cord. The incidence of sporadic ALS is 1.5 to 2.7 in 100,000, and the prevalence is 5.2 to 6.0 in 100,000. Conjugal ALS is even rarer than sporadic ALS. We report a case of conjugal ALS encountered in our outpatient neurology clinic. PMID:22275781

  5. Changes in Spinal Cord Architecture after Brachial Plexus Injury in the Newborn

    ERIC Educational Resources Information Center

    Korak, Klaus J.; Tam, Siu Lin; Gordon, Tessa; Frey, Manfred; Aszmann, Oskar C.

    2004-01-01

    Obstetric brachial plexus palsy is a devastating birth injury. While many children recover spontaneously, 20-25% are left with a permanent impairment of the affected limb. So far, concepts of pathology and recovery have focused on the injury of the peripheral nerve. Proximal nerve injury at birth, however, leads to massive injury-induced…

  6. Axillary artery injury as a complication of proximal humerus fractures.

    PubMed

    McLaughlin, J A; Light, R; Lustrin, I

    1998-01-01

    Proximal humerus fractures are common injuries and represent approximately 5% of all fractures. These fractures are infrequently associated with neurovascular injuries. Brachial plexus injuries are uncommon, whereas axillary artery injuries are rare. A review of 19 previously reported cases of axillary artery injury after proximal humerus fracture revealed that 84% occurred in patients older than 50 years, 53% were associated with brachial plexus injury, and 21% resulted in upper extremity amputation. This study describes a case of axillary artery injury after proximal humerus fracture and, on the basis of a literature review, offers suggestions for the early diagnosis and effective treatment of this uncommon injury.

  7. What causes amyotrophic lateral sclerosis?

    PubMed Central

    Martin, Sarah; Al Khleifat, Ahmad; Al-Chalabi, Ammar

    2017-01-01

    Amyotrophic lateral sclerosis is a neurodegenerative disease predominantly affecting upper and lower motor neurons, resulting in progressive paralysis and death from respiratory failure within 2 to 3 years. The peak age of onset is 55 to 70 years, with a male predominance. The causes of amyotrophic lateral sclerosis are only partly known, but they include some environmental risk factors as well as several genes that have been identified as harbouring disease-associated variation. Here we review the nature, epidemiology, genetic associations, and environmental exposures associated with amyotrophic lateral sclerosis.

  8. Severe Brachial Plexus Injuries in American Football.

    PubMed

    Daly, Charles A; Payne, S Houston; Seiler, John G

    2016-11-01

    This article reports a series of severe permanent brachial plexus injuries in American football players. The authors describe the mechanisms of injury and outcomes from a more contemporary treatment approach in the form of nerve transfer tailored to the specific injuries sustained. Three cases of nerve transfer for brachial plexus injury in American football players are discussed in detail. Two of these patients regained functional use of the extremity, but 1 patient with a particularly severe injury did not regain significant function. Brachial plexus injuries are found along a spectrum of brachial plexus stretch or contusion that includes the injuries known as "stingers." Early identification of these severe brachial plexus injuries allows for optimal outcomes with timely treatment. Diagnosis of the place of a given injury along this spectrum is difficult and requires a combination of imaging studies, nerve conduction studies, and close monitoring of physical examination findings over time. Although certain patients may be at higher risk for stingers, there is no evidence to suggest that this correlates with a higher risk of severe brachial plexus injury. Unfortunately, no equipment or strengthening program has been shown to provide a protective effect against these severe injuries. Patients with more severe injuries likely have less likelihood of functional recovery. In these patients, nerve transfer for brachial plexus injury offers the best possibility of meaningful recovery without significant morbidity. [ Orthopedics. 2016; 39(6):e1188-e1192.].

  9. Optineurin and amyotrophic lateral sclerosis.

    PubMed

    Maruyama, Hirofumi; Kawakami, Hideshi

    2013-07-01

    Amyotrophic lateral sclerosis is a devastating disease, and thus it is important to identify the causative gene and resolve the mechanism of the disease. We identified optineurin as a causative gene for amyotrophic lateral sclerosis. We found three types of mutations: a homozygous deletion of exon 5, a homozygous Q398X nonsense mutation and a heterozygous E478G missense mutation within its ubiquitin-binding domain. Optineurin negatively regulates the tumor necrosis factor-α-induced activation of nuclear factor kappa B. Nonsense and missense mutations abolished this function. Mutations related to amyotrophic lateral sclerosis also negated the inhibition of interferon regulatory factor-3. The missense mutation showed a cyotoplasmic distribution different from that of the wild type. There are no specific clinical symptoms related to optineurin. However, severe brain atrophy was detected in patients with homozygous deletion. Neuropathologically, an E478G patient showed transactive response DNA-binding protein of 43 kDa-positive neuronal intracytoplasmic inclusions in the spinal and medullary motor neurons. Furthermore, Golgi fragmentation was identified in 73% of this patient's anterior horn cells. In addition, optineurin is colocalized with fused in sarcoma in the basophilic inclusions of amyotrophic lateral sclerosis with fused in sarcoma mutations, and in basophilic inclusion body disease. These findings strongly suggest that optineurin is involved in the pathogenesis of amyotrophic lateral sclerosis.

  10. Neurinomas of the brachial plexus: case report.

    PubMed

    Forte, A; Gallinaro, L S; Bertagni, A; Montesano, G; Prece, V; Illuminati, G

    1999-01-01

    Neurinomas, also referred to as neurilemmomas and schwannomas, are rare benign tumours of the peripheral nerves, a low proportion of which arise from the brachial plexus. Authors report a case of an ancient schwannoma arising from the brachial plexus. The tumour, usually asymptomatic, may cause sensory radicular symptoms, or rarely motor deficits in the involved arm. Enucleation of the tumour from the nerve without damage to any of the fascicles is the correct treatment.

  11. Acromioclavicular joint dislocation with associated brachial plexus injury

    PubMed Central

    Gallagher, Charles Alexander; Blakeney, William; Zellweger, René

    2014-01-01

    We present the case of a 32-year-old female who sustained a left acromioclavicular (AC) joint type V injury and brachial plexus injury. The patient's AC joint injury was identified 6 days after she was involved in a motorbike accident where she sustained multiple other injuries. She required operative fixation of the AC joint using a locking compression medial proximal tibial plate. At 3 months post operatively, the patient was found to have a subluxed left shoulder as a result of an axonal injury to the upper trunk of the brachial plexus. In addition, the tibial plate had cut out. The plate was subsequently removed. At 8 months the glenohumeral articulation had been restored and the patient had clinically regained significant shoulder function. After 15 months the patient was pain free and could complete all her activities of daily living without impediment. She returned to playing competitive pool after 24 months. PMID:24855076

  12. Amyotrophic lateral sclerosis mimic syndromes

    PubMed Central

    Ghasemi, Majid

    2016-01-01

    Amyotrophic lateral sclerosis (ALS) misdiagnosis has many broad implications for the patient and the neurologist. Potentially curative treatments exist for certain ALS mimic syndromes, but delay in starting these therapies may have an unfavorable effect on outcome. Hence, it is important to exclude similar conditions. In this review, we discuss some of the important mimics of ALS. PMID:27326363

  13. Systematic evaluation of brachial plexus injuries.

    PubMed

    Haynes, S

    1993-01-01

    Brachial plexus injuries offer a unique challenge to the athletic trainer because of their relatively high frequency rate in contact sports and because of the complexity of the neuroanatomy in the cervical area. During a game, athletic trainers must make a fast, accurate decision regarding a player's return to competition. It is imperative that the athletic trainer be able to quickly differentiate between minor injuries and more serious injuries warranting removal from the game and/or physician referral. A systematic approach to the evaluation of a brachial plexus injury is essential to ensure proper treatment. This paper will present a structured approach to an on-the-field assessment of brachial plexus injuries.

  14. Acoustic reflex patterns in amyotrophic lateral sclerosis.

    PubMed

    Canale, Andrea; Albera, Roberto; Lacilla, Michelangelo; Canosa, Antonio; Albera, Andrea; Sacco, Francesca; Chiò, Adriano; Calvo, Andrea

    2017-02-01

    The aim of the study is to investigate acoustic reflex testing in amyotrophic lateral sclerosis patients. Amplitude, latency, and rise time of stapedial reflex were recorded for 500 and 1000 Hz contralateral stimulus. Statistical analysis was performed by the Wilcoxon test and the level of significance was set at 5 %. Fifty-one amyotrophic lateral sclerosis patients and ten sex- and age-matched control subjects were studied. Patients were further divided in two groups: amyotrophic lateral sclerosis-bulbar (38 cases, with bulbar signs at evaluation) and amyotrophic lateral sclerosis-spinal (13 cases, without bulbar signs at evaluation). Stapedial reflex was present in all patients. There was a statistically significant difference in the mean amplitude, latency, and rise time between the amyotrophic lateral sclerosis patients as compared with the controls. Amplitude was lower in both the amyotrophic lateral sclerosis-bulbar and the amyotrophic lateral sclerosis-spinal patients than in the controls (p < 0.05) and rise time was longer in both patient groups compared with the controls (p < 0.05). These results confirm the presence of abnormal acoustic reflex patterns in amyotrophic lateral sclerosis cases with bulbar signs and, moreover, suggesting a possible subclinical involvement of the stapedial motor neuron even in amyotrophic lateral sclerosis-spinal patients. Amplitude and rise time seem to be good sensitive parameters for investigating subclinical bulbar involvement.

  15. Magnetic resonance neurography of the brachial plexus

    PubMed Central

    Upadhyaya, Vaishali; Upadhyaya, Divya Narain; Kumar, Adarsh; Pandey, Ashok Kumar; Gujral, Ratni; Singh, Arun Kumar

    2015-01-01

    Magnetic Resonance Imaging (MRI) is being increasingly recognised all over the world as the imaging modality of choice for brachial plexus and peripheral nerve lesions. Recent refinements in MRI protocols have helped in imaging nerve tissue with greater clarity thereby helping in the identification, localisation and classification of nerve lesions with greater confidence than was possible till now. This article on Magnetic Resonance Neurography (MRN) is based on the authors’ experience of imaging the brachial plexus and peripheral nerves using these protocols over the last several years. PMID:26424974

  16. Immune system alterations in amyotrophic lateral sclerosis.

    PubMed

    Hovden, H; Frederiksen, J L; Pedersen, S W

    2013-11-01

    Amyotrophic lateral sclerosis is a disease of which the underlying cause and pathogenesis are unknown. Cumulatative data clearly indicates an active participation by the immune system in the disease. An increasingly recognized theory suggests a non-cell autonomous mechanism, meaning that multiple cells working together are necessary for the pathogenesis of the disease. Observed immune system alterations could indicate an active participation in this mechanism. Damaged motor neurons are able to activate microglia, astrocytes and the complement system, which further can influence each other and contribute to neurodegeneration. Infiltrating peripheral immune cells appears to correlate with disease progression, but their significance and composition is unclear. The deleterious effects of this collaborating system of cells appear to outweigh the protective aspects, and revealing this interplay might give more insight into the disease. Markers from the classical complement pathway are elevated where its initiator C1q appears to derive primarily from motor neurons. Activated microglia and astrocytes are found in close proximity to dying motor neurons. Their activation status and proliferation seemingly increases with disease progression. Infiltrating monocytes, macrophages and T cells are associated with these areas, although with mixed reports regarding T cell composition. This literature review will provide evidence supporting the immune system as an important part of ALS disease mechanism and present a hypothesis to direct the way for further studies.

  17. Proximal Nephron

    PubMed Central

    Zhuo, Jia L.; Li, Xiao C.

    2013-01-01

    The kidney plays a fundamental role in maintaining body salt and fluid balance and blood pressure homeostasis through the actions of its proximal and distal tubular segments of nephrons. However, proximal tubules are well recognized to exert a more prominent role than distal counterparts. Proximal tubules are responsible for reabsorbing approximately 65% of filtered load and most, if not all, of filtered amino acids, glucose, solutes, and low molecular weight proteins. Proximal tubules also play a key role in regulating acid-base balance by reabsorbing approximately 80% of filtered bicarbonate. The purpose of this review article is to provide a comprehensive overview of new insights and perspectives into current understanding of proximal tubules of nephrons, with an emphasis on the ultrastructure, molecular biology, cellular and integrative physiology, and the underlying signaling transduction mechanisms. The review is divided into three closely related sections. The first section focuses on the classification of nephrons and recent perspectives on the potential role of nephron numbers in human health and diseases. The second section reviews recent research on the structural and biochemical basis of proximal tubular function. The final section provides a comprehensive overview of new insights and perspectives in the physiological regulation of proximal tubular transport by vasoactive hormones. In the latter section, attention is particularly paid to new insights and perspectives learnt from recent cloning of transporters, development of transgenic animals with knockout or knockin of a particular gene of interest, and mapping of signaling pathways using microarrays and/or physiological proteomic approaches. PMID:23897681

  18. What has changed in brachial plexus surgery?

    PubMed Central

    de Rezende, Marcelo Rosa; Silva, Gustavo Bersani; de Paula, Emygdio José Leomil; Junior, Rames Mattar; de Camargo, Olavo Pires

    2013-01-01

    Brachial plexus injuries, in all their severity and complexity, have been extensively studied. Although brachial plexus injuries are associated with serious and often definitive sequelae, many concepts have changed since the 1950s, when this pathological condition began to be treated more aggressively. Looking back over the last 20 years, it can be seen that the entire approach, from diagnosis to treatment, has changed significantly. Some concepts have become better established, while others have been introduced; thus, it can be said that currently, something can always be offered in terms of functional recovery, regardless of the degree of injury. Advances in microsurgical techniques have enabled improved results after neurolysis and have made it possible to perform neurotization, which has undoubtedly become the greatest differential in treating brachial plexus injuries. Improvements in imaging devices and electrical studies have allowed quick decisions that are reflected in better surgical outcomes. In this review, we intend to show the many developments in brachial plexus surgery that have significantly changed the results and have provided hope to the victims of this serious injury. PMID:23644864

  19. [Idiopathic brachial neuralgia after cesarean section].

    PubMed

    Rihane, B; Le Borgne, J M; Bélair, C

    2002-11-01

    We report a case of idiopathic brachial nevralgia of the right shoulder in a 30-year-old female, after caesarean section, under spinal anaesthesia. Two days after surgery, intense cervical pain appeared on the second day, associated with rapid collapse of muscular shoulder belt. Full recovery occurred in four months.

  20. Diagnosis of amyotrophic lateral sclerosis.

    PubMed

    Rowland, L P

    1998-10-01

    This review of the differential diagnosis of amyotrophic lateral sclerosis focuses on two themes. The first is practical, how to establish the diagnosis based primarily on clinical findings buttressed by electrodiagnosis. The main considerations are multifocal motor neuropathy and cervical spondylotic myelopathy. The second theme is the relationship of motor neuron disease to other conditions, including benign fasciculation (Denny-Brown, Foley syndrome), paraneoplastic syndromes, lymphoproliferative disease, radiation damage, monomelic amyotrophy (Hirayama syndrome), as well as an association with parkinsonism, dementia and multisystem disorders of the central nervous system.

  1. Diffusion tensor imaging analysis of sequential spreading of disease in amyotrophic lateral sclerosis confirms patterns of TDP-43 pathology.

    PubMed

    Kassubek, Jan; Müller, Hans-Peter; Del Tredici, Kelly; Brettschneider, Johannes; Pinkhardt, Elmar H; Lulé, Dorothée; Böhm, Sarah; Braak, Heiko; Ludolph, Albert C

    2014-06-01

    Diffusion tensor imaging can identify amyotrophic lateral sclerosis-associated patterns of brain alterations at the group level. Recently, a neuropathological staging system for amyotrophic lateral sclerosis has shown that amyotrophic lateral sclerosis may disseminate in a sequential regional pattern during four disease stages. The objective of the present study was to apply a new methodological diffusion tensor imaging-based approach to automatically analyse in vivo the fibre tracts that are prone to be involved at each neuropathological stage of amyotrophic lateral sclerosis. Two data samples, consisting of 130 diffusion tensor imaging data sets acquired at 1.5 T from 78 patients with amyotrophic lateral sclerosis and 52 control subjects; and 55 diffusion-tensor imaging data sets at 3.0 T from 33 patients with amyotrophic lateral sclerosis and 22 control subjects, were analysed by a tract of interest-based fibre tracking approach to analyse five tracts that become involved during the course of amyotrophic lateral sclerosis: the corticospinal tract (stage 1); the corticorubral and the corticopontine tracts (stage 2); the corticostriatal pathway (stage 3); the proximal portion of the perforant path (stage 4); and two reference pathways. The statistical analyses of tracts of interest showed differences between patients with amyotrophic lateral sclerosis and control subjects for all tracts. The significance level of the comparisons at the group level was lower, the higher the disease stage with corresponding involved fibre tracts. Both the clinical phenotype as assessed by the amyotrophic lateral sclerosis functional rating scale-revised and disease duration correlated significantly with the resulting staging scheme. In summary, the tract of interest-based technique allowed for individual analysis of predefined tract structures, thus making it possible to image in vivo the disease stages in amyotrophic lateral sclerosis. This approach can be used not only for

  2. Treatment Options for Brachial Plexus Injuries

    PubMed Central

    Sakellariou, Vasileios I.; Badilas, Nikolaos K.; Stavropoulos, Nikolaos A.; Mazis, George; Kotoulas, Helias K.; Kyriakopoulos, Stamatios; Tagkalegkas, Ioannis; Sofianos, Ioannis P.

    2014-01-01

    The incidence of brachial plexus injuries is rapidly growing due to the increasing number of high-speed motor-vehicle accidents. These are devastating injuries leading to significant functional impairment of the patients. The purpose of this review paper is to present the available options for conservative and operative treatment and discuss the correct timing of intervention. Reported outcomes of current management and future prospects are also analysed. PMID:24967125

  3. Genetics Home Reference: amyotrophic lateral sclerosis

    MedlinePlus

    ... DW. Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Neuron. 2013 Aug 7;79( ... Miller CC, Shaw CE. Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type ...

  4. Lightning strike-induced brachial plexopathy.

    PubMed

    Bhargava, Amita N; Kasundra, Gaurav M; Khichar, Subhakaran; Bhushan, Bharat S K

    2014-10-01

    We describe a patient who presented with a history of lightning strike injury. Following the injury, he sustained acute right upper limb weakness with pain. Clinically, the lesion was located to the upper and middle trunk of the right brachial plexus, and the same confirmed with electrophysiological studies. Nerve damage due to lightning injuries is considered very rare, and a plexus damage has been described infrequently, if ever. Thus, the proposed hypothesis that lightning rarely causes neuropathy, as against high-voltage electric current, due to its shorter duration of exposure not causing severe burns which lead to nerve damage, needs to be reconsidered.

  5. Brachial plexopathy: recurrent cancer or radiation

    SciTech Connect

    Lederman, R.J.; Wilbourn, A.J.

    1984-10-01

    We reviewed clinical and electrodiagnostic features of 16 patients with neoplastic brachial plexopathy (NBP) and 17 patients with radiation-induced plexopathy (RBP). The groups were similar in symptom-free interval after cancer diagnosis and location of the plexus lesions. NBP patients had pain and Horner's syndrome; RBP patients had paresthesias, but rarely Horner's. NBP patients presented earlier after symptom onset and had a shorter course. RBP patients more frequently had abnormal sensory and normal motor nerve conduction studies and characteristically had fasciculations or myokymia on EMG.

  6. Lightning strike-induced brachial plexopathy

    PubMed Central

    Bhargava, Amita N.; Kasundra, Gaurav M.; Khichar, Subhakaran; Bhushan, Bharat S. K.

    2014-01-01

    We describe a patient who presented with a history of lightning strike injury. Following the injury, he sustained acute right upper limb weakness with pain. Clinically, the lesion was located to the upper and middle trunk of the right brachial plexus, and the same confirmed with electrophysiological studies. Nerve damage due to lightning injuries is considered very rare, and a plexus damage has been described infrequently, if ever. Thus, the proposed hypothesis that lightning rarely causes neuropathy, as against high-voltage electric current, due to its shorter duration of exposure not causing severe burns which lead to nerve damage, needs to be reconsidered. PMID:25288846

  7. Shoulder pain and isolated brachial plexopathy

    PubMed Central

    Kishan, Amar U; Syed, Sana; Fiorito-Torres, Franchesca; Thakore-James, Manisha

    2012-01-01

    Pancoast syndrome, classically considered as a constellation of (1) pain along the C8–T2 dermatomes, (2) weakness and atrophy of the hand and (3) Horner's syndrome, often presents a diagnostic challenge. In fact, it may manifest as a singular orthopaedic complaint, prompting a futile barrage of tests and referrals. The authors present the case of an elderly man who initially presented with severe shoulder pain. Due to progressive pain and weakness, he was referred to rheumatology and was treated with corticosteroid injections for a presumed musculoskeletal lesion. Ultimately, he manifested gross muscular atrophy and worsening pain, prompting a referral to neurology. An electromyogram (EMG) suggested a lower brachial plexopathy, and a follow-up brachial plexus MRI identified a large Pancoast tumour. Unfortunately, his disease was rapidly progressive, and he passed away within 2 months. While the MRI remains the gold standard for diagnosing Pancoast syndrome, an EMG can facilitate diagnosis in difficult cases such as this one. PMID:22744250

  8. Lateral approach for supraclavicular brachial plexus block

    PubMed Central

    Sahu, DK; Sahu, Anjana

    2010-01-01

    A lateral approach described by Volker Hempel and Dr. Dilip Kotharihas been further studied, evaluated and described in detail in the present study. The aim of this study was to evaluate lateral approach of supraclavicular brachial plexus block, mainly in terms of successes rate and complication rate. The study was conducted in secondary level hospital and tertiary level hospital from 2004 to 2008. It was a prospective nonrandomized open-level study. Eighty-two patients of both sexes, aged between 18 and 65 years with ASA Grade I and II scheduled to undergo elective major surgery of the upper limb below the midarm, were selected for this new lateral approach of brachial plexus block. The onset and duration of sensory and motor block, any complications and need for supplement anaesthesia were observed. Success and complication rate were calculated in percentage. Average onset and duration of sensory and motor block was calculated as mean ± SD and percentage. Out of 82 patients, 75 (92%) have got successful block with no significant complication in any case. PMID:20885867

  9. Exercise and amyotrophic lateral sclerosis.

    PubMed

    de Almeida, J P Lopes; Silvestre, R; Pinto, A C; de Carvalho, M

    2012-02-01

    Amyotrophic Lateral Sclerosis (ALS) is a progressive and fatal neurodegenerative disease in which much burden is geared towards end-of-life care. Particularly in the earlier stages of ALS, many people have found both physiological and psychological boosts from various types of physical exercise for disused muscles. Proper exercise is important for preventing atrophy of muscles from disuse-a key for remaining mobile for as long as possible-and as long as it is possible to exercise comfortably and safely, for preserving cardiovascular fitness. However, the typical neuromuscular patient features a great physical inactivity and disuse weakness, and for that reason many controversial authors have contested exercise in these patients during years, especially in ALS which is rapidly progressive. There is an urgent need for dissecting in detail the real risks or benefits of exercise in controlled clinical trials to demystify this ancient paradigm. Yet, recent research studies document significant benefits in terms of survival and quality of life in ALS, poor cooperation, small sample size, uncontrolled and short-duration trials, remain the main handicaps. Sedentary barriers such as early fatigue and inherent muscle misuse should be overcome, for instance with body-weight supporting systems or non-invasive ventilation, and exercise should be faced as a potential non-monotonous way for contributing to better health-related quality of life.

  10. Amyotrophic Lateral Sclerosis: A Historical Perspective.

    PubMed

    Katz, Jonathan S; Dimachkie, Mazen M; Barohn, Richard J

    2015-11-01

    This article looks back in time to see where the foundational basis for the understanding of amyotrophic lateral sclerosis originated. This foundation was created primarily in France by Jean-Martin Charcot and his fellow countrymen and disciples, along with key contributions from early clinicians in England and Germany. The early work on amyotrophic lateral sclerosis provides a useful foundation for today's clinicians with respect to tying together genetic and biologic aspects of the disorder that have been discovered over the past few decades.

  11. Proximity fuze

    DOEpatents

    Harrison, Thomas R.

    1989-08-22

    A proximity fuze system includes an optical ranging apparatus, a detonation circuit controlled by the optical ranging apparatus, and an explosive charge detonated by the detonation cirtcuit. The optical ranging apparatus includes a pulsed laser light source for generating target ranging light pulses and optical reference light pulses. A single lens directs ranging pulses to a target and collects reflected light from the target. An optical fiber bundle is used for delaying the optical reference pulses to correspond to a predetermined distance from the target. The optical ranging apparatus includes circuitry for providing a first signal depending upon the light pulses reflected from the target, a second signal depending upon the light pulses from the optical delay fiber bundle, and an output signal when the first and second signals coincide with each other. The output signal occurs when the distance from the target is equal to the predetermined distance form the target. Additional circuitry distinguishes pulses reflected from the target from background solar radiation.

  12. Proximity fuze

    DOEpatents

    Harrison, T.R.

    1987-07-10

    A proximity fuze system includes an optical ranging apparatus, a detonation circuit controlled by the optical ranging apparatus, and an explosive charge detonated by the detonation circuit. The optical ranging apparatus includes a pulsed laser light source for generating target ranging light pulses and optical reference light pulses. A single lens directs ranging pulses to a target and collects reflected light from the target. An optical fiber bundle is used for delaying the optical reference pulses to correspond to a predetermined distance from the target. The optical ranging apparatus includes circuitry for providing a first signal depending upon the light pulses reflected from the target, a second signal depending upon the light pulses from the optical delay fiber bundle, and an output signal when the first and second signals coincide with each other. The output signal occurs when the distance from the target is equal to the predetermined distance from the target. Additional circuitry distinguishes pulses reflected from the target from background solar radiation. 3 figs.

  13. Proximity fuze

    SciTech Connect

    Harrison, T.R.

    1989-08-22

    A proximity fuze system is described. It includes an optical ranging apparatus, a detonation circuit controlled by the optical ranging apparatus, and an explosive charge detonated by the detonation circuit. The optical ranging apparatus includes a pulsed laser light source for generating target ranging light pulses and optical reference light pulses. A single lens directs ranging pulses to a target and collects reflected light from the target. An optical fiber bundle is used for delaying the optical reference pulses to correspond to a predetermined distance from the target. The optical ranging apparatus includes circuitry for providing a first signal depending upon the light pulses reflected from the target, a second signal depending upon the light pulses from the optical delay fiber bundle, and an output signal when the first and second signals coincide with each other. The output signal occurs when the distance from the target is equal to the predetermined distance from the target. Additional circuitry distinguishes pulses reflected from the target from background solar radiation.

  14. Brachial Artery Reconstruction in Trauma Using Reversed Arm Vein from the Injured Upper Limb

    PubMed Central

    Harnarayan, Patrick

    2016-01-01

    Background: Brachial artery repair may be technically challenging with a paucity of guidelines. The use arm vein (AV) from the traumatized limb is herein described. Methods: Data were prospectively collected from 2002 to 2016 on brachial artery injury including age, sex, mechanism/site of injury, and repair technique. Categories included AV and non-arm vein (NAV) groups. One-year outcomes were noted. Results: All 31 cases studied were of men with an age range of 16 to 73 years (mean = 28). Injuries included 13 gunshots, 7 stabbings, 6 glass injuries, 2 dislocated elbows, 1 crush, 1 impalement, and 1 avulsion. Site of injuries included the antecubital region in 25, midbrachial in 5, and proximal brachial in 1, with 4 associated fractures. Repair was done using reversed AV from the traumatized limb in 15 cases and NAV in 16. In the AV group, the adjacent basilic vein was used in 9 cases, the adjacent cephalic vein in 3, and the distal (or wrist area) cephalic vein in 3. The limb salvage rates in the AV versus NAV groups were 100% and 94%, respectively (Fisher’s exact t test, P = 1.00), with no major technique-related complications. Conclusions: The outcomes of using reversed AV from the traumatized limb are equivalent to those of other standard techniques such as primary repair, polytetrafluoroethylene, or reversed great saphenous vein, with a 1-year limb salvage rate of 100%. Additionally, advantages include decreased wound complications, better vein graft--artery caliber match, and shorter operating times while maintaining acceptable patency rates. PMID:27826464

  15. Amyotrophic lateral sclerosis: the role of exercise.

    PubMed

    Lisle, Stuart; Tennison, Matthew

    2015-01-01

    Amyotrophic lateral sclerosis (ALS) is a chronic progressive neurodegenerative disease affecting both the upper and lower motor neurons. Given the deterioration of skeletal muscle function, historically there has been concern regarding exercise and its affect on ALS. This article reviews and explains current research, helping patients, caregivers, and providers be equipped better to make decisions regarding the treatment of ALS with exercise.

  16. Clinical Psychology and Amyotrophic Lateral Sclerosis

    PubMed Central

    Pagnini, Francesco; Rossi, Gabriella; Lunetta, Christian; Banfi, Paolo; Corbo, Massimo

    2010-01-01

    Amyotrophic lateral sclerosis is a fatal and progressive disease, characterized by progressive muscles weakness, with consequent loss of physical capacities. Psychologists can play an important role in ALS care, by providing clinical activities in every step of the disease, including support and counseling activities directed to patients, their caregivers and to physicians. PMID:21833203

  17. Superficial brachial artery: A possible cause for idiopathic median nerve entrapment neuropathy.

    PubMed

    Nkomozepi, Pilani; Xhakaza, Nkosi; Swanepoel, Elaine

    2017-02-15

    Nerve entrapment syndromes occur because of anatomic constraints at specific locations in both upper and lower limbs. Anatomical locations prone to nerve entrapment syndromes include sites where a nerve courses through fibro-osseous or fibromuscular tunnels or penetrates a muscle. The median nerve (MN) can be entrapped by the ligament of Struthers; thickened biceps aponeurosis; between the superficial and deep heads of the pronator teres muscle and by a thickened proximal edge of flexor digitorum superficialis muscle. A few cases of MN neuropathies encountered are reported to be idiopathic. The superficial branchial artery (SBA) is defined as the artery running superficial to MN or its roots. This divergence from normal anatomy may be the possible explanation for idiopathic median nerve entrapment neuropathy. This study presents three cases with unilateral presence of the SBA encountered during routine undergraduate dissection at the University of Johannesburg. Case 1: SBA divided into radial and ulnar arteries. Brachial artery (BA) terminated as deep brachial artery. Case 2: SBA continued as radial artery (RA). BA terminated as ulnar artery (UA), anterior and posterior interosseous arteries. Case 3: SBA continued as UA. BA divided into radial and common interosseous arteries. Arteries that take an unusual course are more vulnerable to iatrogenic injury during surgical procedures and may disturb the evaluation of angiographic images during diagnosis. In particular, the presence of SBA may be a course of idiopathic neuropathies.

  18. Polycyclic aromatic hydrocarbons, brachial artery distensibility and blood pressure among children residing near an oil refinery

    PubMed Central

    Trasande, Leonardo; Urbina, Elaine M.; Khoder, Mamdouh; Alghamdi, Mansour; Shabaj, Ibrahim; Alam, Mohammed S.; Harrison, Roy M.; Shamy, Magdy

    2017-01-01

    Background Polycyclic aromatic hydrocarbons (PAH) are produced by the burning and processing of fuel oils, and have been associated with oxidant stress, insulin resistance and hypertension in adults. Few studies have examined whether adolescents are susceptible to cardiovascular effects of PAHs. Objective To study associations of PAH exposure with blood pressure (BP) and brachial artery distensibility (BAD), an early marker of arterial wall stiffness, in young boys attending three schools in Jeddah, Saudi Arabia in varying proximity to an oil refinery. Methods Air samples collected from the three schools were analyzed for PAHs. PAH metabolites (total hydroxyphenanthrenes and 1-hydroxypyrene) were measured in urine samples from 184 adolescent males, in whom anthropometrics, heart rate, pulse pressure, brachial artery distensibility and blood pressure were measured. Descriptive, bivariate and multivariable analyses were performed to assess relationships of school location and urinary PAH metabolites with cardiovascular measures. Results Total suspended matter was significantly higher (444 ± 143 µg/m3) at the school near the refinery compared to a school located near a ring road (395 ± 65 µg/m3) and a school located away from vehicle traffic (232 ± 137 µg/m3), as were PAHs. Systolic (0.47 SD units, p = 0.006) and diastolic (0.53 SD units, p < 0.001) BP Z-scores were highest at the school near the refinery, with a 4.36-fold increase in prehypertension (p = 0.001), controlling for confounders. No differences in pulse pressure, BAD and heart rate were noted in relationship to school location. Urinary total hydroxyphenanthrenes and 1-hydroxypyrene were not associated with cardiovascular outcomes. Conclusions Proximity to an oil refinery in Saudi Arabia is associated with prehypertension and increases in PAH and particulate matter exposures. Further study including insulin resistance measurements, better control for confounding, and longitudinal measurement is

  19. Epidemiology of amyotrophic lateral sclerosis.

    PubMed

    Kurtzke, J F

    1982-01-01

    Motor neuron disease (MND) is used in this paper as the generic label, encompassing the clinical variants of amyotrophic lateral sclerosis (ALS), progressive myelopathic muscular atrophy (PMMA), and progressive bulbar palsy (PBP). ALS is limited to instances of anterior horn cell plus pyramidal tract involvement. When only anterior horn cell lesions are inferred, either PMMA or PBP is used, depending on the levels of involvement; when both cord and brain stem are affected. PBP is the designation. Mortality data on MND have been available for a number of countries since 1949. The coding used under international rules has varied considerably over this interval. Before 1969, hereditary muscular atrophies were included. Since 1979, no subdivision by type of MND is possible. International death rates for MND have all been rather close to 1 per 100,000 population per year, though perhaps nearer to 1.4 on the average in recent years. There has been an increasing proportion of MND deaths coded to ALS between 1949 and 1977. There is no notable geographic variation among countries, nor within countries such as the U.S. and Denmark. A slight upward trend in death rates over time in the U.S. is matched by a slight decrease in Denmark. Death rates from all sources indicate a male preponderance for ALS or MND as a whole, at about 1.5 to 1, male to female. There is also a consistent predilection by age, with few deaths under age 50 or so and a clear maximum in age-specific death rates at about age 70. This holds for both sexes. In the U.S., there is also a white-nonwhite difference, with a ratio of about 1.6:1 but with age and sex differences similar to whites. Average annual incidence rates from among white occidental populations range mostly between 0.6 and 1.8 per 100,000 population for MND and about 0.8 and 1.5 per 100,000 for ALS. Again a male predilection is seen. There is a clear maximum in age-specific incidence rates at about age 65 in all surveys except that of

  20. Automated analysis of brachial ultrasound time series

    NASA Astrophysics Data System (ADS)

    Liang, Weidong; Browning, Roger L.; Lauer, Ronald M.; Sonka, Milan

    1998-07-01

    Atherosclerosis begins in childhood with the accumulation of lipid in the intima of arteries to form fatty streaks, advances through adult life when occlusive vascular disease may result in coronary heart disease, stroke and peripheral vascular disease. Non-invasive B-mode ultrasound has been found useful in studying risk factors in the symptom-free population. Large amount of data is acquired from continuous imaging of the vessels in a large study population. A high quality brachial vessel diameter measurement method is necessary such that accurate diameters can be measured consistently in all frames in a sequence, across different observers. Though human expert has the advantage over automated computer methods in recognizing noise during diameter measurement, manual measurement suffers from inter- and intra-observer variability. It is also time-consuming. An automated measurement method is presented in this paper which utilizes quality assurance approaches to adapt to specific image features, to recognize and minimize the noise effect. Experimental results showed the method's potential for clinical usage in the epidemiological studies.

  1. Morphology of brachial plexus and axillary artery in bonobo (Pan paniscus).

    PubMed

    Kikuchi, Y; Oishi, M; Shimizu, D

    2011-02-01

    A left brachial plexus and axillary artery of bonobo (Pan paniscus) were examined, and the interrelation between the brachial plexus and the axillary artery was discussed. This is the first report of the brachial plexus and the axillary artery of bonobo. The bonobo brachial plexus formed very similar pattern to that of other ape species and human. On the other hand, the branches of the bonobo axillary artery had uncommon architecture in comparison with human case. The axillary artery did not penetrate the brachial plexus and passes through all way along anterior to the brachial plexus. Only 4.9% of human forelimbs have this pattern. Moreover, the brachial artery runs through superficially anterior to branches of the brachial plexus.

  2. Proximal Tibial Bone Graft

    MedlinePlus

    ... All Site Content AOFAS / FootCareMD / Treatments Proximal Tibial Bone Graft Page Content What is a bone graft? Bone grafts may be needed for various ... the proximal tibia. What is a proximal tibial bone graft? Proximal tibial bone graft (PTBG) is a ...

  3. Evaluation and management of amyotrophic lateral sclerosis.

    PubMed

    Valadi, Nojan

    2015-06-01

    Motor neuron diseases can cause progressive impairment of voluntary muscles of movement, respiration, speech, and swallowing. This review discusses the most common motor neuron disease, amyotrophic lateral sclerosis (ALS). It reviews the evaluation, diagnosis, and management of ALS, and its epidemiology, pathophysiology, and management. A coordinated approach by the primary care physician and neurologist is necessary with a focus on treatment options, durable medical equipment needs, and end-of-life discussions.

  4. Massive hemothorax: A rare complication after supraclavicular brachial plexus block.

    PubMed

    Singh, Shiv Kumar; Katyal, Surabhi; Kumar, Amit; Kumar, Pawan

    2014-01-01

    Plexus block is the preferred anesthesia plan for upper limb surgeries. Among the known complications, hematoma formation following the vascular trauma is often occur but this complication is frequently underreported. We present a case where a massive hemothorax developed post operatively in a patient who underwent resection of giant cell tumor of the right hand radius bone followed by arthroplasty under brachial plexus block using supraclavicular approach. This case report attempts to highlight the essence of remaining vigilant postoperatively for first initial days after brachial plexus block, especially after failed or multiple attempts. Ultrasound guided technique in combination with nerve stimulator has proven to be more reliable and safer than traditional techniques.

  5. Acute presentation of brachial plexus schwannoma secondary to infarction.

    PubMed

    Sidani, Charif; Saraf-Lavi, Efrat; Lyapichev, Kirill A; Nadji, Mehrdad; Levi, Allan D

    2015-06-01

    Schwannomas of the brachial plexus are rare and typically present as slowly growing masses. We describe a case of a 37-year-old female who presented with acute onset of severe left upper extremity pain. Magnetic resonance imaging (MRI) showed a 2.3 × 2.1 cm peripherally enhancing centrally cystic lesion in the left axilla, along the cords of the left brachial plexus, with significant surrounding edema and enhancement. The mass was surgically removed. Pathology was consistent with a schwannoma with infarction. The pain completely resolved immediately after surgery.

  6. High-fat and ketogenic diets in amyotrophic lateral sclerosis.

    PubMed

    Paganoni, Sabrina; Wills, Anne-Marie

    2013-08-01

    Amyotrophic lateral sclerosis is a fatal neurodegenerative disease. Epidemiologic data suggest that malnutrition is a common feature in amyotrophic lateral sclerosis and being overweight or obese confers a survival advantage in this patient population. In amyotrophic lateral sclerosis mouse models, a high-fat diet has been shown to lead to weight gain and prolonged survival. However, little research has been conducted to test whether nutritional interventions might ameliorate the disease course in humans. Here we review the currently available evidence supporting the potential role of dietary interventions as a therapeutic tool for amyotrophic lateral sclerosis. Ultimately, determining whether a high-fat or ketogenic diet could be beneficial in amyotrophic lateral sclerosis will require large randomized, placebo-controlled clinical trials.

  7. The natural history and management of brachial plexus birth palsy.

    PubMed

    Buterbaugh, Kristin L; Shah, Apurva S

    2016-12-01

    Brachial plexus birth palsy (BPBP) is an upper extremity paralysis that occurs due to traction injury of the brachial plexus during childbirth. Approximately 20 % of children with brachial plexus birth palsy will have residual neurologic deficits. These permanent and significant impacts on upper limb function continue to spur interest in optimizing the management of a problem with a highly variable natural history. BPBP is generally diagnosed on clinical examination and does not typically require cross-sectional imaging. Physical examination is also the best modality to determine candidates for microsurgical reconstruction of the brachial plexus. The key finding on physical examination that determines need for microsurgery is recovery of antigravity elbow flexion by 3-6 months of age. When indicated, both microsurgery and secondary shoulder and elbow procedures are effective and can substantially improve functional outcomes. These procedures include nerve transfers and nerve grafting in infants and secondary procedures in children, such as botulinum toxin injection, shoulder tendon transfers, and humeral derotational osteotomy.

  8. Psychiatric disorders prior to amyotrophic lateral sclerosis

    PubMed Central

    Goldacre, Raph; Talbot, Kevin; Goldacre, Michael J.

    2016-01-01

    It is recognized that neuropsychiatric conditions are overrepresented in amyotrophic lateral sclerosis (ALS) patient kindreds and psychiatric symptoms may precede the onset of motor symptoms. Using a hospital record linkage database, hospitalization with a diagnosis of schizophrenia, bipolar disorder, depression, or anxiety was significantly associated with a first diagnosis of ALS within the following year. This is likely to specifically reflect the clinicopathological overlap of ALS with frontotemporal dementia. A diagnosis of depression was significantly associated with a first record of ALS ≥5 years later, in keeping with growing evidence for major depressive disorder as an early marker of cerebral neurodegeneration. Ann Neurol 2016;80:935–938 PMID:27761925

  9. Pemphigus vulgaris and amyotrophic lateral sclerosis

    PubMed Central

    Mokhtari, Fatemeh; Matin, Marzieh; Rajati, Fatemeh

    2016-01-01

    Pemphigus vulgaris (PV) is an autoimmune bullous and erosive mucocutaneous disease. Rarely, it occurs in patients with other autoimmune disease. The relation between PV and neurological disorders is unclear and needs to be more studied. Here, we report a case of amyotrophic lateral sclerosis (ALS), followed by dermatologic involvement. Histopathological evidence and direct immunofluorescence are consistent with PV. Systemic corticosteroid and azathioprine were effective in the treatment of mucocutaneous lesions. PV seems to be accidentally associated with ALS. Expression of major histocompatibility complex Class II in autoimmune disease and production of autoantibodies have been proposed to describe the association of PV with ALS. PMID:28163728

  10. The changing scene of amyotrophic lateral sclerosis.

    PubMed

    Robberecht, Wim; Philips, Thomas

    2013-04-01

    Several recent breakthroughs have provided notable insights into the pathogenesis of amyotrophic lateral sclerosis (ALS), with some even shifting our thinking about this neurodegenerative disease and raising the question as to whether this disorder is a proteinopathy, a ribonucleopathy or both. In addition, these breakthroughs have revealed mechanistic links between ALS and frontotemporal dementia, as well as between ALS and other neurodegenerative diseases, such as the cerebellar atrophies, myotonic dystrophy and inclusion body myositis. Here, we summarize the new findings in ALS research, discuss what they have taught us about this disease and examine issues that are still outstanding.

  11. Quantifying disease progression in amyotrophic lateral sclerosis.

    PubMed

    Simon, Neil G; Turner, Martin R; Vucic, Steve; Al-Chalabi, Ammar; Shefner, Jeremy; Lomen-Hoerth, Catherine; Kiernan, Matthew C

    2014-11-01

    Amyotrophic lateral sclerosis (ALS) exhibits characteristic variability of onset and rate of disease progression, with inherent clinical heterogeneity making disease quantitation difficult. Recent advances in understanding pathogenic mechanisms linked to the development of ALS impose an increasing need to develop strategies to predict and more objectively measure disease progression. This review explores phenotypic and genetic determinants of disease progression in ALS, and examines established and evolving biomarkers that may contribute to robust measurement in longitudinal clinical studies. With targeted neuroprotective strategies on the horizon, developing efficiencies in clinical trial design may facilitate timely entry of novel treatments into the clinic.

  12. Amyotrophic lateral sclerosis: one or multiple causes?

    PubMed Central

    Bastos, Aline Furtado; Orsini, Marco; Machado, Dionis; Mello, Mariana Pimentel; Nader, Sergio; Silva, Júlio Guilherme; da Silva Catharino, Antonio M.; de Freitas, Marcos R.G.; Pereira, Alessandra; Pessoa, Luciane Lacerda; Sztajnbok, Flavio R.; Leite, Marco Araújo; Nascimento, Osvaldo J.M.; Bastos, Victor Hugo

    2011-01-01

    The Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease in the adulthood, and it is characterized by rapid and progressive compromise of the upper and lower motor neurons. The majority of the cases of ALS are classified as sporadic and, until now, a specific cause for these cases still is unknown. To present the different hypotheses on the etiology of ALS. It was carried out a search in the databases: Bireme, Scielo and Pubmed, in the period of 1987 to 2011, using the following keywords: Amyotrophic lateral sclerosis, motor neuron disease, etiology, causes and epidemiology and its similar in Portuguese and Spanish. It did not have consensus as regards the etiology of ALS. Researches demonstrates evidences as regards intoxication by heavy metals, environmental and occupational causes, genetic mutations (superoxide dismutase 1), certain viral infections and the accomplishment of vigorous physical activity for the development of the disease. There is still no consensus regarding the involved factors in the etiology of ALS. In this way, new research about these etiologies are necessary, for a better approach of the patients, promoting preventive programs for the disease and improving the quality of life of the patients. PMID:21785676

  13. [An autopsy case of amyotrophic lateral sclerosis associated with cervical syringomyelia].

    PubMed

    Hamada, K; Sudoh, K; Fukaura, H; Yanagihara, T; Hamada, T; Tashiro, K; Isu, T

    1990-06-01

    An autopsied case of amyotrophic lateral sclerosis complicated by cervical syringomyelia was reported. The case was a 59-year-old man, who first noticed weakness of both lower extremities at 54-year-old. The weakness spread to both upper extremities within 2 years. Cervical myelography revealed multi-level cervical spondylosis and anterior fusion of C5-C7 was done. But the weakness and atrophy of proximal muscle, diminished deep tendon reflex on upper extremities, hyperreflexia and pathological reflexes on both legs, tongue fasciculation and respiratory muscle weakness developed successively, and the patient died of respiratory distress at 59-year-old. Autopsy revealed multiple independent four syrinxes located at the level between C2-C7. One of these syrinxes had ependymal cell lining and thought to be idiopathic syringomyelia. The other three syrinxes were considered to be the cavitation in association with cervical spondylotic myelopathy. Degeneration and decreasing of spinal anterior horn cells, atrophy of medullary pyramis and Bunina bodies were observed as features of typical amyotrophic lateral sclerosis. Cervical spondylosis as causative lesion of multiple syrinxes was discussed, and relationship between ALS and the syrinxes was not indicated clearly.

  14. Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients.

    PubMed

    Watanabe, Hazuki; Atsuta, Naoki; Nakamura, Ryoichi; Hirakawa, Akihiro; Watanabe, Hirohisa; Ito, Mizuki; Senda, Jo; Katsuno, Masahisa; Izumi, Yuishin; Morita, Mitsuya; Tomiyama, Hiroyuki; Taniguchi, Akira; Aiba, Ikuko; Abe, Koji; Mizoguchi, Kouichi; Oda, Masaya; Kano, Osamu; Okamoto, Koichi; Kuwabara, Satoshi; Hasegawa, Kazuko; Imai, Takashi; Aoki, Masashi; Tsuji, Shoji; Nakano, Imaharu; Kaji, Ryuji; Sobue, Gen

    2015-06-01

    Our objective was to elucidate the clinical factors affecting functional decline and survival in Japanese amyotrophic lateral sclerosis (ALS) patients. We constructed a multicenter prospective ALS cohort that included 451 sporadic ALS patients in the analysis. We longitudinally utilized the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) as the functional scale, and determined the timing of introduction of a tracheostomy for positive-pressure ventilation and death. A joint modelling approach was employed to identify prognostic factors for functional decline and survival. Age at onset was a common prognostic factor for both functional decline and survival (p < 0.001, p < 0.001, respectively). Female gender (p = 0.019) and initial symptoms, including upper limb weakness (p = 0.010), lower limb weakness (p = 0.008) or bulbar symptoms (p = 0.005), were related to early functional decline, whereas neck weakness as an initial symptom (p = 0.018), non-use of riluzole (p = 0.030) and proximal dominant muscle weakness in the upper extremities (p = 0.01) were related to a shorter survival time. A decline in the ALSFRS-R score was correlated with a shortened survival time (p < 0.001). In conclusion, the factors affecting functional decline and survival in ALS were common in part but different to some extent. This difference has not been previously well recognized but is informative in clinical practice and for conducting trials.

  15. Brachial plexus lesions in patients with cancer: 100 cases

    SciTech Connect

    Kori, S.H.; Foley, K.M.; Posner, J.B.

    1981-01-01

    In patients with cancer, brachial plexus signs are usually caused by tumor infiltration or injury from radiation therapy (RT). We analyzed 100 cases of brachial plexopathy to determine which clinical criteria helped differentiate tumor from radiation injury. Seventy-eight patients had tumor and 22 had radiation injury. Severe pain occurred in 80% of tumor patients but in only 19% of patients with radiation injury. The lower trunk was involved in 72% of the tumors. Seventy-eight percent of the radiation injuries affected the upper plexus (C5-6). Horner syndrome was more common in tumor, and lymphedema in radiation injury. The time from RT to onset of plexus symptoms, and the dose of RT, also differed.

  16. Recent advances in the management of brachial plexus injuries

    PubMed Central

    Bhandari, Prem Singh; Maurya, Sanjay

    2014-01-01

    Management of brachial plexus injury is a demanding field of hand and upper extremity surgery. With currently available microsurgical techniques, functional gains are rewarding in upper plexus injuries. However, treatment options in the management of flail and anaesthetic limb are still evolving. Last three decades have witnessed significant developments in the management of these injuries, which include a better understanding of the anatomy, advances in the diagnostic modalities, incorporation of intra-operative nerve stimulation techniques, more liberal use of nerve grafts in bridging nerve gaps, and the addition of new nerve transfers, which selectively neurotise the target muscles close to the motor end plates. Newer research works on the use of nerve allografts and immune modulators (FK 506) are under evaluation in further improving the results in nerve reconstruction. Direct reimplantation of avulsed spinal nerve roots into the spinal cord is another area of research in brachial plexus reconstruction. PMID:25190913

  17. Massive hemothorax: A rare complication after supraclavicular brachial plexus block

    PubMed Central

    Singh, Shiv Kumar; Katyal, Surabhi; Kumar, Amit; Kumar, Pawan

    2014-01-01

    Plexus block is the preferred anesthesia plan for upper limb surgeries. Among the known complications, hematoma formation following the vascular trauma is often occur but this complication is frequently underreported. We present a case where a massive hemothorax developed post operatively in a patient who underwent resection of giant cell tumor of the right hand radius bone followed by arthroplasty under brachial plexus block using supraclavicular approach. This case report attempts to highlight the essence of remaining vigilant postoperatively for first initial days after brachial plexus block, especially after failed or multiple attempts. Ultrasound guided technique in combination with nerve stimulator has proven to be more reliable and safer than traditional techniques. PMID:25886347

  18. Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis.

    PubMed

    Vercruysse, Pauline; Sinniger, Jérôme; El Oussini, Hajer; Scekic-Zahirovic, Jelena; Dieterlé, Stéphane; Dengler, Reinhard; Meyer, Thomas; Zierz, Stephan; Kassubek, Jan; Fischer, Wilhelm; Dreyhaupt, Jens; Grehl, Torsten; Hermann, Andreas; Grosskreutz, Julian; Witting, Anke; Van Den Bosch, Ludo; Spreux-Varoquaux, Odile; Ludolph, Albert C; Dupuis, Luc

    2016-04-01

    Amyotrophic lateral sclerosis, the most common adult-onset motor neuron disease, leads to death within 3 to 5 years after onset. Beyond progressive motor impairment, patients with amyotrophic lateral sclerosis suffer from major defects in energy metabolism, such as weight loss, which are well correlated with survival. Indeed, nutritional intervention targeting weight loss might improve survival of patients. However, the neural mechanisms underlying metabolic impairment in patients with amyotrophic lateral sclerosis remain elusive, in particular due to the lack of longitudinal studies. Here we took advantage of samples collected during the clinical trial of pioglitazone (GERP-ALS), and characterized longitudinally energy metabolism of patients with amyotrophic lateral sclerosis in response to pioglitazone, a drug with well-characterized metabolic effects. As expected, pioglitazone decreased glycaemia, decreased liver enzymes and increased circulating adiponectin in patients with amyotrophic lateral sclerosis, showing its efficacy in the periphery. However, pioglitazone did not increase body weight of patients with amyotrophic lateral sclerosis independently of bulbar involvement. As pioglitazone increases body weight through a direct inhibition of the hypothalamic melanocortin system, we studied hypothalamic neurons producing proopiomelanocortin (POMC) and the endogenous melanocortin inhibitor agouti-related peptide (AGRP), in mice expressing amyotrophic lateral sclerosis-linked mutant SOD1(G86R). We observed lower Pomc but higher Agrp mRNA levels in the hypothalamus of presymptomatic SOD1(G86R) mice. Consistently, numbers of POMC-positive neurons were decreased, whereas AGRP fibre density was elevated in the hypothalamic arcuate nucleus of SOD1(G86R) mice. Consistent with a defect in the hypothalamic melanocortin system, food intake after short term fasting was increased in SOD1(G86R) mice. Importantly, these findings were replicated in two other amyotrophic

  19. Ultrasonographic evaluation of brachial plexus tumors in five dogs.

    PubMed

    Rose, Scott; Long, Craig; Knipe, Marguerite; Hornof, Bill

    2005-01-01

    Five dogs with unilateral thoracic limb lameness, neurologic deficits, muscle atrophy, and pain, or a combination of these signs, were examined using ultrasonograghy. Large, hypoechoic tubular masses that displaced vessels and destroyed the normal architecture were found in each dog. The affected axilla of each patient was then imaged with computed tomography or magnetic resonance to fully assess the extent of the masses. We describe the use of ultrasound in screening patients for brachial plexus tumors.

  20. A case-control study of amyotrophic lateral sclerosis.

    PubMed

    Savettieri, G; Salemi, G; Arcara, A; Cassata, M; Castiglione, M G; Fierro, B

    1991-01-01

    A retrospective case-control study was conducted using 46 patients affected by amyotrophic lateral sclerosis and 92 closely matched healthy controls. Cases were ascertained through typical clinical and instrumental findings. Putative risk factors (bone fractures or major trauma, exposure to domestic animals, surgical operations, disease among first degree relatives and others) were investigated anamnestically using a standard questionnaire. Using Mantel-Haenzsel estimates of the odds ratio, no association was found between amyotrophic lateral sclerosis and the investigated variables.

  1. [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.

  2. Amyotrophic lateral sclerosis associated with pregnancy.

    PubMed

    Tyagi, A; Sweeney, B J; Connolly, S

    2001-12-01

    Amyotrophic lateral sclerosis (ALS) is the most common, progressive motor neurone disease but is rare in the obstetric population. Only 4 cases have been described in the English literature since 1975. We describe a 29 year old woman who presented with ataxia, lower limb weakness and dysarthria 4 weeks after the birth of her first child. The symptoms had onset during the pregnancy but had not been considered remarkable. There were clinical features of upper and lower motor neurone involvement without any sensory loss. MRI of brain and spine was normal. CSF analysis was negative. EMG studies confirmed the presence of widespread anterior horn cell dysfunction compatible with ALS. The patient was commenced on Riluzole and has progressed clinically, at 12 months post diagnosis.

  3. Amyotrophic lateral sclerosis and environmental factors

    PubMed Central

    Bozzoni, Virginia; Pansarasa, Orietta; Diamanti, Luca; Nosari, Guido; Cereda, Cristina; Ceroni, Mauro

    2016-01-01

    Summary Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder that affects central and peripheral motor neuron cells. Its etiology is unknown, although a relationship between genetic background and environmental factors may play a major role in triggering the neurodegeneration. In this review, we analyze the role of environmental factors in ALS: heavy metals, electromagnetic fields and electric shocks, pesticides, β-N-methylamino-L-alanine, physical activity and the controversial role of sports. The literature on the single issues is analyzed in an attempt to clarify, as clearly as possible, whether each risk factor significantly contributes to the disease pathogenesis. After summarizing conflicting observations and data, the authors provide a final synthetic statement. PMID:27027889

  4. Dysarthria in amyotrophic lateral sclerosis: A review.

    PubMed

    Tomik, Barbara; Guiloff, Roberto J

    2010-01-01

    Dysarthria is a motor disorder of speech characterized by abnormalities of the articulation and intelligibility of speech. Phonation and the rate of facial movements may also be affected. Understanding the nature and course of dysarthria in amyotrophic lateral sclerosis (ALS) is important because loss of communication prevents patients from participating in many activities, may lead to social isolation, and reduces the quality of life. The goal of management of dysarthria in ALS patients is to optimize communication effectiveness for as long as possible. The information about dysarthria in ALS is dispersed in physiological, pathological, speech therapy, otorhinolaringological and neurological publications. This review summarizes the current state of knowledge on the clinical features, differential diagnosis, pathophysiology, investigations and management of dysarthria in ALS patients. There is a need to compare the different methods used to assess dysarthria and for controlled clinical trials to assess therapeutic strategies.

  5. A comprehensive review of amyotrophic lateral sclerosis

    PubMed Central

    Zarei, Sara; Carr, Karen; Reiley, Luz; Diaz, Kelvin; Guerra, Orleiquis; Altamirano, Pablo Fernandez; Pagani, Wilfredo; Lodin, Daud; Orozco, Gloria; Chinea, Angel

    2015-01-01

    Amyotrophic lateral sclerosis (ALS) is a late-onset fatal neurodegenerative disease affecting motor neurons with an incidence of about 1/100,000. Most ALS cases are sporadic, but 5–10% of the cases are familial ALS. Both sporadic and familial ALS (FALS) are associated with degeneration of cortical and spinal motor neurons. The etiology of ALS remains unknown. However, mutations of superoxide dismutase 1 have been known as the most common cause of FALS. In this study, we provide a comprehensive review of ALS. We cover all aspects of the disease including epidemiology, comorbidities, environmental risk factor, molecular mechanism, genetic factors, symptoms, diagnostic, treatment, and even the available supplement and management of ALS. This will provide the reader with an advantage of receiving a broad range of information about the disease. PMID:26629397

  6. Brachial artery aneurysms associated with arteriovenous access for hemodialysis.

    PubMed

    Chemla, Eric; Nortley, Mei; Morsy, Mohamed

    2010-01-01

    Brachial artery aneurysm (BAA) is a rare condition. We describe a series of cases of BAA with arteriovenous access. Thirteen patients were retrospectively identified between January 2006 and July 2009 using a patient database. All were associated with brachio-cephalic fistulas. Mean age was 51.2 +/- 13.8 years. Twelve males (93.3%) were identified. Characteristics were: diabetes 1, hypertension 8, hypercholesterolemia 2, ischemic heart disease 2, family history of aneurysmal disease 2. Five BAA developed after access ligation, eight while it was working, one after trauma. One was associated with a venous aneurysm. While the average life of the access was 161 +/- 115 months, the average time for BAA formation was 40 +/- 35.8 months. BAA was asymptomatic in three patients, whereas 10 presented with ischemic and neurologic symptoms. None presented with a rupture. All patients underwent surgical repair, seven an aneurysm excision and end-to-end reconstruction of the brachial artery. Venous conduits were utilized: four long saphenous veins, one cephalic, and one basilic vein. All patients had patent brachial arteries with a complete relief of symptoms at 14 months. BAA is a rare but significant complication of vascular access. The surgical approaches presented offer a reasonable outcome.

  7. Evaluation of an education day for families of children with obstetrical brachial plexus palsy.

    PubMed

    Ho, Emily S; Ulster, Alissa A

    2011-09-01

    Children with obstetrical brachial plexus palsy may have chronic physical impairment in their affected upper extremity. Affected children and their families may benefit from psychosocial interventions including therapeutic relationships with health professionals, meeting other families living with obstetrical brachial plexus palsy, support groups, and social work. One method of addressing psychosocial needs is through a support and education day. The purpose of this quality improvement project is to evaluate parental perceptions of a support and education day called the "Brachial Plexus Family Day." Families of children with obstetrical brachial plexus palsy who attended the Brachial Plexus Family Day completed a questionnaire to evaluate the different programs offered during the day. The families also ranked the importance of different psychosocial supports offered in the clinic. Sixty-three out of 69 families completed the questionnaire. Each program of the Brachial Plexus Family Day was rated as good or excellent by the respondents. Ninety-seven percent of respondents rated meeting other families and children with obstetrical brachial plexus palsy as helpful supports. Attending a Brachial Plexus Family day event (86%), followed by connecting with a doctor (60%), and physical or occupational therapist (59%) were the highest ranked supports reported by the families. The parents and caregivers that attended the Brachial Plexus Family Day rated the program highly. This group also valued the opportunity to connect with other families and children affected with the same condition.

  8. Delayed rupture of a pseudoaneurysm in the brachial artery of a burn reconstruction patient

    PubMed Central

    2013-01-01

    A brachial artery pseudoaneurysm is a rare but serious condition that can be limb threatening. A number of reports have found that it may be the result of damage to the blood vessels around the brachial artery, either directly or indirectly, due to trauma or systemic diseases. We present our experience of delayed pseudoaneurysm rupture of the brachial artery in a rehabilitation patient with burns of the upper extremity who underwent fasciotomy and musculocutaneous flap coverage. We also provide a review of the brachial artery pseudoaneurysm. PMID:23758847

  9. [Modified grant method protocol for dissecting and identifying the brachial plexus].

    PubMed

    Arakawa, Takamitsu; Setsu, Tomiyoshi; Terashima, Toshio

    2004-03-01

    Dissection of the brachial plexus is an important part in the anatomical course, but it is difficult for medical students to identify individual nerves of the brachial plexus due to its complexity and numerous variations. We have recently adopted the Grant method (1991) to guide students in the successful identification of this plexus. However, according to the Grant method the part of the upper limb including the brachial plexus is dissected before the neck part, which makes it impossible to identify the roots, trunks, and cords of the brachial plexus, and to identify the nerve branches extending from the brachial plexus. Here, we propose of anatomical dissection protocol of the brachial plexus a modified Grant method for medical students and instructors. The points of the modified protocols are: (1) to dissect the brachial plexus after the dissection of the neck part, (2) to identify the nerve trunks at the scalenus gap after dissecting the lateral, medial and posterior cords. The modified Grant method can be adapted to any other dissecting protocol of the brachial plexus, and will allow students to cope with many variations of the brachial plexus when they occur.

  10. Magnetic resonance imaging of traumatic and non-traumatic brachial plexopathies

    PubMed Central

    Fan, Yiru Lorna; Othman, Mohamad Isham Bin; Dubey, Niraj; Peh, Wilfred CG

    2016-01-01

    Adult-onset brachial plexopathy can be classified into traumatic and non-traumatic aetiologies. Traumatic brachial plexopathies can affect the pre- or postganglionic segments of the plexus. Non-traumatic brachial plexopathies may be due to neoplasia, radiotherapy, thoracic outlet syndrome and idiopathic neuralgic amyotrophy. Conventional magnetic resonance imaging (MRI) is useful to localise the area of injury or disease, and identify the likely cause. This review discusses some of the common causes of adult-onset brachial plexopathy and their imaging features on MRI. We also present a series of cases to illustrate some of these causes and their MRI findings. PMID:27779278

  11. Associations and clinical relevance of aortic-brachial artery stiffness mismatch, aortic reservoir function, and central pressure augmentation.

    PubMed

    Schultz, Martin G; Hughes, Alun D; Davies, Justin E; Sharman, James E

    2015-10-01

    Central augmentation pressure (AP) and index (AIx) predict cardiovascular events and mortality, but underlying physiological mechanisms remain disputed. While traditionally believed to relate to wave reflections arising from proximal arterial impedance (and stiffness) mismatching, recent evidence suggests aortic reservoir function may be a more dominant contributor to AP and AIx. Our aim was therefore to determine relationships among aortic-brachial stiffness mismatching, AP, AIx, aortic reservoir function, and end-organ disease. Aortic (aPWV) and brachial (bPWV) pulse wave velocity were measured in 359 individuals (aged 61 ± 9, 49% male). Central AP, AIx, and aortic reservoir indexes were derived from radial tonometry. Participants were stratified by positive (bPWV > aPWV), negligible (bPWV ≈ aPWV), or negative stiffness mismatch (bPWV < aPWV). Left-ventricular mass index (LVMI) was measured by two-dimensional-echocardiography. Central AP and AIx were higher with negative stiffness mismatch vs. negligible or positive stiffness mismatch (11 ± 6 vs. 10 ± 6 vs. 8 ± 6 mmHg, P < 0.001 and 24 ± 10 vs. 24 ± 11 vs. 21 ± 13%, P = 0.042). Stiffness mismatch (bPWV-aPWV) was negatively associated with AP (r = -0.18, P = 0.001) but not AIx (r = -0.06, P = 0.27). Aortic reservoir pressure strongly correlated to AP (r = 0.81, P < 0.001) and AIx (r = 0.62, P < 0.001) independent of age, sex, heart rate, mean arterial pressure, and height (standardized β = 0.61 and 0.12, P ≤ 0.001). Aortic reservoir pressure independently predicted abnormal LVMI (β = 0.13, P = 0.024). Positive aortic-brachial stiffness mismatch does not result in higher AP or AIx. Aortic reservoir function, rather than discrete wave reflection from proximal arterial stiffness mismatching, provides a better model description of AP and AIx and also has clinical relevance as evidenced by an independent association of aortic reservoir pressure with LVMI.

  12. Development of a novel experimental rat model for neonatal pre-ganglionic upper brachial plexus injury.

    PubMed

    Ochiai, Hidenobu; Ikeda, Tomoaki; Mishima, Kenichi; Yoshikawa, Tetsuya; Aoo, Naoya; Iwasaki, Katsunori; Fujiwara, Michihiro; Ikenoue, Tsuyomu; Nakano, Shinichi; Wakisaka, Shinichiro

    2002-09-15

    A neonatal upper brachial plexus injury, referred to as Erb's palsy, is a serious obstetric problem. Some surgical methods are used to treat this injury, but they are inadequate. To seek new treatments for Erb's palsy, we used a model for cervical preganglionic root transection in neonate rats and evaluated the behavioral and histological compatibility of this model with Erb's palsy. Two groups were used in this study. In the group, receiving the Erb operation, the left anterior and posterior roots of spinal vertebra C5-C7 were transected at the preganglionic level, and the results were compared with those of a group that received a sham operation. In the group, receiving the Erb operation, walking difficulties and behavioral abnormalities were observed. These observations were noted on the side where the transection took place, and the problems were attributed to proximal muscle weakness in the forelimb. Additionally, the forepaw grip was not impaired. Furthermore, in this group, the number of anterior horn cells in the cervical cord on the transected side was significantly lower than that on the contralateral side (P < 0.001). The results of this study indicate that the model fulfills the criteria for the clinical symptoms of Erb's palsy and that it may also serve as a new method for enabling treatment of the condition.

  13. Proximal humerus fractures.

    PubMed

    Price, Matthew C; Horn, Pamela L; Latshaw, James C

    2013-01-01

    Proximal humerus fractures are among the most common fractures associated with osteoporosis. With an aging population, incidence of these fractures will only increase. The proximal humerus not only forms the lateral portion of the shoulder articulation but also has significant associations with musculoskeletal and neurovascular structures. As a result, fractures of the proximal humerus can significantly impact not only the function of the shoulder joint, but the health and function of the entire upper extremity as well. Understanding of these fractures, the management options, and associated nursing care, can help reduce morbidity rate and improve functional outcomes.

  14. Correlation between ultrasound imaging, cross-sectional anatomy, and histology of the brachial plexus: a review.

    PubMed

    van Geffen, Geert J; Moayeri, Nizar; Bruhn, Jörgen; Scheffer, Gert J; Chan, Vincent W; Groen, Gerbrand J

    2009-01-01

    The anatomy of the brachial plexus is complex. To facilitate the understanding of the ultrasound appearance of the brachial plexus, we present a review of important anatomic considerations. A detailed correlation of reconstructed, cross-sectional gross anatomy and histology with ultrasound sonoanatomy is provided.

  15. 38 CFR 3.318 - Presumptive service connection for amyotrophic lateral sclerosis.

    Code of Federal Regulations, 2011 CFR

    2011-07-01

    ... connection for amyotrophic lateral sclerosis. 3.318 Section 3.318 Pensions, Bonuses, and Veterans' Relief... sclerosis. (a) Except as provided in paragraph (b) of this section, the development of amyotrophic lateral... under this section: (1) If there is affirmative evidence that amyotrophic lateral sclerosis was...

  16. 38 CFR 3.318 - Presumptive service connection for amyotrophic lateral sclerosis.

    Code of Federal Regulations, 2012 CFR

    2012-07-01

    ... connection for amyotrophic lateral sclerosis. 3.318 Section 3.318 Pensions, Bonuses, and Veterans' Relief... sclerosis. (a) Except as provided in paragraph (b) of this section, the development of amyotrophic lateral... under this section: (1) If there is affirmative evidence that amyotrophic lateral sclerosis was...

  17. Apo-Ferritin as a Therapeutic Treatment for Amyotrophic Lateral Sclerosis

    DTIC Science & Technology

    2012-09-01

    for Amyotrophic Lateral Sclerosis PRINCIPAL INVESTIGATOR: James R. Connor, Ph.D...August 2012 4. TITLE AND SUBTITLE 5a. CONTRACT NUMBER Apo-Ferritin as a Therapeutic Treatment for Amyotrophic Lateral Sclerosis 5b. GRANT NUMBER...accumulation and deposition have been reported in patients with amyotrophic lateral sclerosis (ALS). Previous work in mutant SOD1 mice mouse models of ALS

  18. 38 CFR 3.318 - Presumptive service connection for amyotrophic lateral sclerosis.

    Code of Federal Regulations, 2014 CFR

    2014-07-01

    ... connection for amyotrophic lateral sclerosis. 3.318 Section 3.318 Pensions, Bonuses, and Veterans' Relief... sclerosis. (a) Except as provided in paragraph (b) of this section, the development of amyotrophic lateral... under this section: (1) If there is affirmative evidence that amyotrophic lateral sclerosis was...

  19. 38 CFR 3.318 - Presumptive service connection for amyotrophic lateral sclerosis.

    Code of Federal Regulations, 2013 CFR

    2013-07-01

    ... connection for amyotrophic lateral sclerosis. 3.318 Section 3.318 Pensions, Bonuses, and Veterans' Relief... sclerosis. (a) Except as provided in paragraph (b) of this section, the development of amyotrophic lateral... under this section: (1) If there is affirmative evidence that amyotrophic lateral sclerosis was...

  20. Finger movement at birth in brachial plexus birth palsy

    PubMed Central

    Nath, Rahul K; Benyahia, Mohamed; Somasundaram, Chandra

    2013-01-01

    AIM: To investigate whether the finger movement at birth is a better predictor of the brachial plexus birth injury. METHODS: We conducted a retrospective study reviewing pre-surgical records of 87 patients with residual obstetric brachial plexus palsy in study 1. Posterior subluxation of the humeral head (PHHA), and glenoid retroversion were measured from computed tomography or Magnetic resonance imaging, and correlated with the finger movement at birth. The study 2 consisted of 141 obstetric brachial plexus injury patients, who underwent primary surgeries and/or secondary surgery at the Texas Nerve and Paralysis Institute. Information regarding finger movement was obtained from the patient’s parent or guardian during the initial evaluation. RESULTS: Among 87 patients, 9 (10.3%) patients who lacked finger movement at birth had a PHHA > 40%, and glenoid retroversion < -12°, whereas only 1 patient (1.1%) with finger movement had a PHHA > 40%, and retroversion < -8° in study 1. The improvement in glenohumeral deformity (PHHA, 31.8% ± 14.3%; and glenoid retroversion 22.0° ± 15.0°) was significantly higher in patients, who have not had any primary surgeries and had finger movement at birth (group 1), when compared to those patients, who had primary surgeries (nerve and muscle surgeries), and lacked finger movement at birth (group 2), (PHHA 10.7% ± 15.8%; Version -8.0° ± 8.4°, P = 0.005 and P = 0.030, respectively) in study 2. No finger movement at birth was observed in 55% of the patients in this study group. CONCLUSION: Posterior subluxation and glenoid retroversion measurements indicated significantly severe shoulder deformities in children with finger movement at birth, in comparison with those lacked finger movement. However, the improvement after triangle tilt surgery was higher in patients who had finger movement at birth. PMID:23362472

  1. Postoperative analgesia comparing levobupivacaine and ropivacaine for brachial plexus block

    PubMed Central

    Watanabe, Kunitaro; Tokumine, Joho; Lefor, Alan Kawarai; Moriyama, Kumi; Sakamoto, Hideaki; Inoue, Tetsuo; Yorozu, Tomoko

    2017-01-01

    Abstract Background: On a pharmacologic basis, levobupivacaine is expected to last longer than ropivacaine. However, most reports of these anesthetics for brachial plexus block do not suggest a difference in analgesic effect. The aim of this study is to compare the postoperative analgesic effects of levobupivacaine and ropivacaine when used for treating ultrasound-guided brachial plexus block. Methods: A total of 62 patients undergoing orthopedic surgery procedures were prospectively enrolled and randomized to receive levobupivacaine (group L, N = 31) or ropivacaine (group R, N = 31). The duration of analgesia, offset time of motor block, need for rescue analgesics, and sleep disturbance on the night of surgery were recorded. Pain score was recorded on the day of surgery, and on postoperative days 1 and 2. Results: There was no difference in the time interval until the first request for pain medication comparing the two groups (group L: 15.6 [11.4, 16.8] hours; group R: 12.5 [9.4, 16.0] hours, P = 0.32). There was no difference in the duration of motor block (group L: 12.2 [7.6, 14.4] hours; group R: 9.4 [7.9, 13.2] hours, P = 0.44), pain score (P = 0.92), need for rescue analgesics (group L: 55%; group R: 65%, P = 0.6), or rate of sleep disturbance (group L: 61%, group R: 58%, P = 1.0) on comparing the two groups. Conclusions: There was no difference in postoperative analgesia comparing levobupivacaine and ropivacaine when used for brachial plexus block. PMID:28328862

  2. Dexamethasone added to lidocaine prolongs axillary brachial plexus blockade.

    PubMed

    Movafegh, Ali; Razazian, Mehran; Hajimaohamadi, Fatemeh; Meysamie, Alipasha

    2006-01-01

    Different additives have been used to prolong regional blockade. We designed a prospective, randomized, double-blind study to evaluate the effect of dexamethasone added to lidocaine on the onset and duration of axillary brachial plexus block. Sixty patients scheduled for elective hand and forearm surgery under axillary brachial plexus block were randomly allocated to receive either 34 mL lidocaine 1.5% with 2 mL of isotonic saline chloride (control group, n = 30) or 34 mL lidocaine 1.5% with 2 mL of dexamethasone (8 mg) (dexamethasone group, n = 30). Neither epinephrine nor bicarbonate was added to the treatment mixture. We used a nerve stimulator and multiple stimulations technique in all of the patients. After performance of the block, sensory and motor blockade of radial, median, musculocutaneous, and ulnar nerves were recorded at 5, 15, and 30 min. The onset time of the sensory and motor blockade was defined as the time between last injection and the total abolition of the pinprick response and complete paralysis. The duration of sensory and motor blocks were considered as the time interval between the administration of the local anesthetic and the first postoperative pain and complete recovery of motor functions. Sixteen patients were excluded because of unsuccessful blockade. The duration of surgery and the onset times of sensory and motor block were similar in the two groups. The duration of sensory (242 +/- 76 versus 98 +/- 33 min) and motor (310 +/- 81 versus 130 +/- 31 min) blockade were significantly longer in the dexamethasone than in the control group (P < 0.01). We conclude that the addition of dexamethasone to lidocaine 1.5% solution in axillary brachial plexus block prolongs the duration of sensory and motor blockade.

  3. High resolution neurography of the brachial plexus by 3 Tesla magnetic resonance imaging.

    PubMed

    Cejas, C; Rollán, C; Michelin, G; Nogués, M

    2016-01-01

    The study of the structures that make up the brachial plexus has benefited particularly from the high resolution images provided by 3T magnetic resonance scanners. The brachial plexus can have mononeuropathies or polyneuropathies. The mononeuropathies include traumatic injuries and trapping, such as occurs in thoracic outlet syndrome due to cervical ribs, prominent transverse apophyses, or tumors. The polyneuropathies include inflammatory processes, in particular chronic inflammatory demyelinating polyneuropathy, Parsonage-Turner syndrome, granulomatous diseases, and radiation neuropathy. Vascular processes affecting the brachial plexus include diabetic polyneuropathy and the vasculitides. This article reviews the anatomy of the brachial plexus and describes the technique for magnetic resonance neurography and the most common pathologic conditions that can affect the brachial plexus.

  4. Amyotrophic Lateral Sclerosis: New Perpectives and Update.

    PubMed

    Orsini, Marco; Oliveira, Acary Bulle; Nascimento, Osvaldo J M; Reis, Carlos Henrique Melo; Leite, Marco Antonio Araujo; de Souza, Jano Alves; Pupe, Camila; de Souza, Olivia Gameiro; Bastos, Victor Hugo; de Freitas, Marcos R G; Teixeira, Silmar; Bruno, Carlos; Davidovich, Eduardo; Smidt, Benny

    2015-09-24

    Amyotrophic lateral sclerosis (ALS), Charcot's disease or Lou Gehrig's disease, is a term used to cover the spetrum of syndromes caracterized by progressive degeneration of motor neurons, a paralytic disorder caused by motor neuron degeneration. Currently, there are approximately 25,000 patients with ALS in the USA, with an average age of onset of 55 years. The incidence and prevalence of ALS are 1-2 and 4-6 per 100,000 each year, respectively, with a lifetime ALS risk of 1/600 to 1/1000. It causes progressive and cumulative physical disabilities, and leads to eventual death due to respiratory muscle failure. ALS is diverse in its presentation, course, and progression. We do not yet fully understand the causes of the disease, nor the mechanisms for its progression; thus, we lack effective means for treating this disease. In this chapter, we will discuss the diagnosis, treatment, and how to cope with impaired function and end of life based on of our experience, guidelines, and clinical trials. Nowadays ALS seems to be a more complex disease than it did two decades - or even one decade - ago, but new insights have been plentiful. Clinical trials should be seen more as experiments on pathogenic mechanisms. A medication or combination of medications that targets more than one pathogenic pathway may slow disease progression in an additive or synergistic fashion.

  5. Controversies and priorities in amyotrophic lateral sclerosis

    PubMed Central

    Turner, Martin R; Hardiman, Orla; Benatar, Michael; Brooks, Benjamin R; Chio, Adriano; de Carvalho, Mamede; Ince, Paul G; Lin, Cindy; Miller, Robert G; Mitsumoto, Hiroshi; Nicholson, Garth; Ravits, John; Shaw, Pamela J; Swash, Michael; Talbot, Kevin; Traynor, Bryan J; den Berg, Leonard H Van; Veldink, Jan H; Vucic, Steve; Kiernan, Matthew C

    2015-01-01

    Summary Two decades after the discovery that 20% of familial amyotrophic lateral sclerosis (ALS) cases were linked to mutations in the superoxide dismutase-1 (SOD1) gene, a substantial proportion of the remainder of cases of familial ALS have now been traced to an expansion of the intronic hexanucleotide repeat sequence in C9orf72. This breakthrough provides an opportunity to re-evaluate longstanding concepts regarding the cause and natural history of ALS, coming soon after the pathological unification of ALS with frontotemporal dementia through a shared pathological signature of cytoplasmic inclusions of the ubiquitinated protein TDP-43. However, with profound clinical, prognostic, neuropathological, and now genetic heterogeneity, the concept of ALS as one disease appears increasingly untenable. This background calls for the development of a more sophisticated taxonomy, and an appreciation of ALS as the breakdown of a wider network rather than a discrete vulnerable population of specialised motor neurons. Identification of C9orf72 repeat expansions in patients without a family history of ALS challenges the traditional division between familial and sporadic disease. By contrast, the 90% of apparently sporadic cases and incomplete penetrance of several genes linked to familial cases suggest that at least some forms of ALS arise from the interplay of multiple genes, poorly understood developmental, environmental, and age-related factors, as well as stochastic events. PMID:23415570

  6. Narrative discourse deficits in amyotrophic lateral sclerosis

    PubMed Central

    Menaged, Anna; Olm, Christopher; McMillan, Corey T.; Boller, Ashley; Irwin, David J.; McCluskey, Leo; Elman, Lauren; Grossman, Murray

    2014-01-01

    Objective: We examined narrative discourse in amyotrophic lateral sclerosis (ALS) to assess the role of executive functioning in support of language and the neuroanatomical basis for such support. Methods: We analyzed a semistructured speech sample in 26 patients with ALS and 19 healthy seniors for narrative discourse features of coherence. Regression analyses related a measure of discourse coherence (“local connectedness”) to gray matter atrophy and reduced white matter fractional anisotropy. Results: Patients with ALS were impaired relative to controls on measures of discourse adequacy, including local connectedness and maintenance of the theme. These discourse measures were related to measures of executive functioning but not to motor functioning. Regressions related local connectedness to gray matter atrophy in ventral and dorsal prefrontal regions and to reduced fractional anisotropy in white matter tracts mediating projections between prefrontal regions. Conclusion: Patients with ALS exhibit deficits in their ability to organize narrative discourse. These deficits appear to be related in part to executive limitations. Consistent with the hypothesis that ALS is a multisystem disorder, this deficit is related to disease in prefrontal regions. PMID:24991038

  7. Risk factors for amyotrophic lateral sclerosis

    PubMed Central

    Ingre, Caroline; Roos, Per M; Piehl, Fredrik; Kamel, Freya; Fang, Fang

    2015-01-01

    Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease. It is typically fatal within 2–5 years of symptom onset. The incidence of ALS is largely uniform across most parts of the world, but an increasing ALS incidence during the last decades has been suggested. Although recent genetic studies have substantially improved our understanding of the causes of ALS, especially familial ALS, an important role of non-genetic factors in ALS is recognized and needs further study. In this review, we briefly discuss several major genetic contributors to ALS identified to date, followed by a more focused discussion on the most commonly examined non-genetic risk factors for ALS. We first review factors related to lifestyle choices, including smoking, intake of antioxidants, physical fitness, body mass index, and physical exercise, followed by factors related to occupational and environmental exposures, including electromagnetic fields, metals, pesticides, β-methylamino-L-alanine, and viral infection. Potential links between ALS and other medical conditions, including head trauma, metabolic diseases, cancer, and inflammatory diseases, are also discussed. Finally, we outline several future directions aiming to more efficiently examine the role of non-genetic risk factors in ALS. PMID:25709501

  8. Epidemiologic correlates of sporadic amyotrophic lateral sclerosis

    SciTech Connect

    Armon, C.; Kurland, L.T.; Daube, J.R.; O'Brien, P.C. )

    1991-07-01

    The authors evaluated 74 selected patients with amyotrophic lateral sclerosis (ALS) and 201 matched controls for risk factors for ALS by a case-control design and a sequential questionnaire/interview technique to quantitate biographic data. They analyzed occupational and recreational data only for 47 male patients and 47 corresponding patient controls; data for women were insufficient. They used nonparametric analyses to evaluate five primary comparisons of ALS patients with controls: (1) more hard physical labor, p not significant (NS); (2) greater frequency of neurodegenerative disease in family members, p NS; (3) greater exposure to lead, p less than 0.05; (4) more years lived in a rural community, p NS; and (5) more trauma or major surgery, p NS. Men with ALS had worked more frequently at blue-collar jobs (although not a statistically significant difference, p = 0.10) and at welding or soldering (p less than 0.01). These results suggest that there may be an association between ALS in men and exposure to lead vapor. The limited nature of the association favors a multifactorial etiologic mechanism of ALS.

  9. Rodent Models of Amyotrophic Lateral Sclerosis.

    PubMed

    Philips, Thomas; Rothstein, Jeffrey D

    2015-06-01

    Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease affecting upper and lower motor neurons in the central nervous system. Patients with ALS develop extensive muscle wasting and atrophy leading to paralysis and death 3 to 5 years after disease onset. The condition may be familial (fALS 10%) or sporadic ALS (sALS, 90%). The large majority of fALS cases are due to genetic mutations in the Superoxide dismutase 1 gene (SOD1, 15% of fALS) and repeat nucleotide expansions in the gene encoding C9ORF72 (∼ 40% to 50% of fALS and ∼ 10% of sALS). Studies suggest that ALS is mediated through aberrant protein homeostasis (i.e., ER stress and autophagy) and/or changes in RNA processing (as in all non-SOD1-mediated ALS). In all of these cases, animal models suggest that the disorder is mediated non-cell autonomously, i.e., not only motor neurons are involved, but glial cells including microglia, astrocytes, and oligodendrocytes, and other neuronal subpopulations are also implicated in the pathogenesis. Provided in this unit is a review of ALS rodent models, including discussion of their relative advantages and disadvantages. Emphasis is placed on correlating the model phenotype with the human condition and the utility of the model for defining the disease process. Information is also presented on RNA processing studies in ALS research, with particular emphasis on the newest ALS rodent models.

  10. Decisional Capacity in Amyotrophic Lateral Sclerosis.

    PubMed

    Khin Khin, Eindra; Minor, Darlinda; Holloway, Amanda; Pelleg, Ayla

    2015-06-01

    The cognitive and behavioral changes that can be observed in the neurodegenerative terminal disease amyotrophic lateral sclerosis (ALS), once characterized as purely a motor neuron disease, have become increasingly recognized over the past century. Detecting cognitive deficits earlier and identifying continued changes at regular intervals can lead to improved care, proactive treatments, and earlier discussions about end-of-life wishes. Although medical decisional capacity is required for every treatment decision made, its importance becomes paramount when making decisions on complex medical treatments that will invariably and significantly affect quality of life or life itself. In this review, we conducted a critical analysis of the evidence-based literature on the cognitive and behavioral impairments in ALS that can compromise medical decisional capacity. We review specific ALS-related clinical scenarios in which decisional capacity is of utmost importance and discuss a practical framework for cognitive and behavioral assessment that can be routinely and efficiently used, while being mindful of the confounding factors associated with ALS. Finally, we review models for preserving patient choices that can be used in patients with ALS to help safeguard autonomy and retain dignity toward the end of life.

  11. Amyotrophic Lateral Sclerosis: New Perpectives and Update

    PubMed Central

    Orsini, Marco; Oliveira, Acary Bulle; Nascimento, Osvaldo J.M.; Reis, Carlos Henrique Melo; Leite, Marco Antonio Araujo; de Souza, Jano Alves; Pupe, Camila; de Souza, Olivia Gameiro; Bastos, Victor Hugo; de Freitas, Marcos R.G.; Teixeira, Silmar; Bruno, Carlos; Davidovich, Eduardo; Smidt, Benny

    2015-01-01

    Amyotrophic lateral sclerosis (ALS), Charcot’s disease or Lou Gehrig’s disease, is a term used to cover the spetrum of syndromes caracterized by progressive degeneration of motor neurons, a paralytic disorder caused by motor neuron degeneration. Currently, there are approximately 25,000 patients with ALS in the USA, with an average age of onset of 55 years. The incidence and prevalence of ALS are 1-2 and 4-6 per 100,000 each year, respectively, with a lifetime ALS risk of 1/600 to 1/1000. It causes progressive and cumulative physical disabilities, and leads to eventual death due to respiratory muscle failure. ALS is diverse in its presentation, course, and progression. We do not yet fully understand the causes of the disease, nor the mechanisms for its progression; thus, we lack effective means for treating this disease. In this chapter, we will discuss the diagnosis, treatment, and how to cope with impaired function and end of life based on of our experience, guidelines, and clinical trials. Nowadays ALS seems to be a more complex disease than it did two decades – or even one decade – ago, but new insights have been plentiful. Clinical trials should be seen more as experiments on pathogenic mechanisms. A medication or combination of medications that targets more than one pathogenic pathway may slow disease progression in an additive or synergistic fashion. PMID:26487927

  12. Selective attention impairment in amyotrophic lateral sclerosis.

    PubMed

    Volpato, Chiara; Prats Sedano, Maria Angeles; Silvoni, Stefano; Segato, Nicoletta; Cavinato, Marianna; Merico, Antonio; Piccione, Francesco; Palmieri, Arianna; Birbaumer, Niels

    2016-01-01

    Objective of this study was to evaluate attentional control mechanisms in amyotrophic lateral sclerosis (ALS) using an auditory event-related potentials (ERPs) paradigm. Fifteen mild to moderate ALS patients and 15 healthy controls were administered a brief neuropsychological test battery and an ERPs paradigm assessing selective attention. Four types of auditory stimuli were presented in random order: short standard (200 Hz, 200 ms), long standard (200 Hz, 500 ms), short deviant (1000 Hz, 200 ms) and long deviant (1000 Hz, 500 ms). Participants had to respond to the long deviant stimuli only. During the task the electroencephalogram (EEG) was recorded. The N200, P300 and re-orienting negativity (RON) ERP components were analysed. Compared to controls ALS patients showed reduced amplitudes and delayed latencies of N200, P300 and RON. These results could be attributable to both an alteration in change detection resulting in a reduction of the allocation and re-orientation of attentional resources or a general slowing or reduction of neural processing efficiency in the same system. The ERPs results support the hypothesis that ALS involves extramotor cognitive functions including auditory attentional processing at all processing stages, early (200 ms) and late (300-600 ms). These data prove the usefulness and sensitivity of the auditory ERPs in detection of cognitive functions in ALS patients.

  13. Respiratory exercise in amyotrophic lateral sclerosis.

    PubMed

    Pinto, Susana; Swash, Michael; de Carvalho, Mamede

    2012-01-01

    We have evaluated the potential role of respiratory exercise by implementing specific inspiratory muscle training in a selected population of early-affected amyotrophic lateral sclerosis (ALS) patients. We studied 26 patients with ALS with normal respiratory function using two groups of patients in a parallel, control-group, randomized, delayed-start design. Patients in the first group (G1) started the active inspiratory exercise programme at entry and were followed for eight months, while the second group (G2) of patients followed a placebo exercise programme for the first four months and then active exercise for the second four-month period. The primary outcome measure was the ALSFRS. Respiratory tests, neurophysiological measurements, fatigue and quality of life scales were secondary outcomes. Analysis of covariance was used to compare changes between and within groups. Results showed that there was no significant difference between the two patient groups. Within-group analysis suggested that inspiratory exercise promotes a transient improvement in the respiratory subscore and in the maximal voluntary ventilation, peak expiratory flow, and sniff inspiratory pressure. In conclusion, there was no clear positive or negative outcome of the respiratory exercise protocol we have proposed, but we cannot rule out a minor positive effect. Exercise regimes merit more detailed clinical evaluation in ALS.

  14. Characteristics of pain in amyotrophic lateral sclerosis

    PubMed Central

    Hanisch, Frank; Skudlarek, Anika; Berndt, Janine; Kornhuber, Malte E

    2015-01-01

    Background Pain is an often underestimated and neglected symptom in amyotrophic lateral sclerosis (ALS). Methods In a cross-sectional survey, 46 patients with ALS, 46 age- and gender matched population-based controls, and 23 diseased controls with myotonic dystrophy type 2 (DM2) were screened for occurrence, type, distribution, and treatment of pain and cramps. Data were collected with the use of the short form brief pain inventory (BPI). Results Pain was reported in 78% of ALS patients,79% of DM2 patients, and 54% of controls (P < 0.05). More ALS patients than controls reported moderate to severe pain (42% vs. 20%). Pain in ALS patients interfered significantly more with daily activities than in controls (median pain interference score: 3.0 vs. 1.2, P < 0.05), especially enjoyment of life (5.0 vs. 1.0) and mood (3.0 vs. 1.0). There was no correlation between the duration of the disease and the severity of pain. Movement-induced cramps were reported in 63% of ALS patients, mostly in the distal extremities. There was no difference in the duration of ALS disease between patients reporting cramps and those who did not. Discussion Our study showed that pain was a relatively frequent symptom which had an important impact on the quality of life. Pain that requires treatment can occur at every stage of ALS. PMID:25642388

  15. Reverse split hand syndrome: Dissociated intrinsic hand muscle atrophy pattern in Hirayama disease/brachial monomelic amyotrophy.

    PubMed

    Singh, Ravinder-Jeet; Preethish-Kumar, Veeramani; Polavarapu, Kiran; Vengalil, Seena; Prasad, Chandrajit; Nalini, Atchayaram

    2017-02-01

    Preferential involvement of C7, C8, T1 level anterior horn cells is a typical feature in Hirayama disease/brachial monomelic amyotrophy (BMMA). There are no clinico-electrophysiological studies to substantiate the peculiar pattern of muscle involvement. Thirty subjects, 10 in each group of BMMA, amyotrophic lateral sclerosis (ALS) and age-matched normal healthy subjects underwent detailed clinical and electrophysiological testing. Results showed that the mean age at evaluation for BMMA and ALS patients was 25.8 ± 3.8 and 51.8 ± 9.5 years, respectively; illness duration was 8.1 ± 5.7 years and 11.14 ± 2.85 months, respectively. Clinically, all BMMA patients had reverse of split hand (RSH) syndrome [abductor digiti minimi (ADM) affected more than abductor pollicis brevis (APB)], while 7/10 ALS patients had classical split hand syndrome (APB affected more than ADM). In BMMA, the compound muscle action potential (CMAP) of APB was preserved but reduced/absent in ADM compared to the ALS group which demonstrated reverse findings. APB/ADM ratio was >0.8 in the BMMA group (>1.4 in 80%), around 1.0 in normal controls (none had >1.4) and <0.8 in ALS (70% having values <0.6). In conclusion, RSH syndrome may provide valuable diagnostic clues to differentiate this relatively self-restricted disease from progressive degenerative disease like ALS.

  16. Mitochondrial dysfunction in blood cells from amyotrophic lateral sclerosis patients.

    PubMed

    Ehinger, Johannes K; Morota, Saori; Hansson, Magnus J; Paul, Gesine; Elmér, Eskil

    2015-06-01

    Mitochondrial dysfunction is implicated in amyotrophic lateral sclerosis, where the progressive degeneration of motor neurons results in muscle atrophy, paralysis and death. Abnormalities in both central nervous system and muscle mitochondria have previously been demonstrated in patient samples, indicating systemic disease. In this case-control study, venous blood samples were acquired from 24 amyotrophic lateral sclerosis patients and 21 age-matched controls. Platelets and peripheral blood mononuclear cells were isolated and mitochondrial oxygen consumption measured in intact and permeabilized cells with additions of mitochondrial substrates, inhibitors and titration of an uncoupler. Respiratory values were normalized to cell count and for two markers of cellular mitochondrial content, citrate synthase activity and mitochondrial DNA, respectively. Mitochondrial function was correlated with clinical staging of disease severity. Complex IV (cytochrome c-oxidase)-activity normalized to mitochondrial content was decreased in platelets from amyotrophic lateral sclerosis patients both when normalized to citrate synthase activity and mitochondrial DNA copy number. In mononuclear cells, complex IV-activity was decreased when normalized to citrate synthase activity. Mitochondrial content was increased in amyotrophic lateral sclerosis patient platelets. In mononuclear cells, complex I activity declined and mitochondrial content increased progressively with advancing disease stage. The findings are, however, based on small subsets of patients and need to be confirmed. We conclude that when normalized to mitochondria-specific content, complex IV-activity is reduced in blood cells from amyotrophic lateral sclerosis patients and that there is an apparent compensatory increase in cellular mitochondrial content. This supports systemic involvement in amyotrophic lateral sclerosis and suggests further study of mitochondrial function in blood cells as a future biomarker for the

  17. Amyotrophic lateral sclerosis treatment with ultramicronized palmitoylethanolamide: a case report.

    PubMed

    Clemente, Simonetta

    2012-11-01

    Amyotrophic lateral sclerosis is a fatal neurodegenerative disease characterized by progressive degeneration of motor neurons which leads to muscular atrophy, paralysis and death in 3-5 years from starting symptoms. This disorder is accompanied by noteworthy spinal inflammation mediated in particular by microglia and mast cells. No effective therapy is available. This report describes the effects of administering the anti-inflammatory agent palmitoylethanolamide in a case of sporadic amyotrophic lateral sclerosis. Palmitoylethanolamide treatment led to an improved clinical picture, as evidenced by electromyographic analysis and pulmonary function. Conceivably, the action of palmitoylethanolamide could result, in part, from its ability to dampen mast cell and microglia activation.

  18. Capacitive proximity sensor

    DOEpatents

    Kronberg, J.W.

    1994-05-31

    A proximity sensor based on a closed field circuit is disclosed. The circuit comprises a ring oscillator using a symmetrical array of plates that creates an oscillating displacement current. The displacement current varies as a function of the proximity of objects to the plate array. Preferably the plates are in the form of a group of three pair of symmetric plates having a common center, arranged in a hexagonal pattern with opposing plates linked as a pair. The sensor produces logic level pulses suitable for interfacing with a computer or process controller. The proximity sensor can be incorporated into a load cell, a differential pressure gauge, or a device for measuring the consistency of a characteristic of a material where a variation in the consistency causes the dielectric constant of the material to change. 14 figs.

  19. Capacitive proximity sensor

    DOEpatents

    Kronberg, James W.

    1994-01-01

    A proximity sensor based on a closed field circuit. The circuit comprises a ring oscillator using a symmetrical array of plates that creates an oscillating displacement current. The displacement current varies as a function of the proximity of objects to the plate array. Preferably the plates are in the form of a group of three pair of symmetric plates having a common center, arranged in a hexagonal pattern with opposing plates linked as a pair. The sensor produces logic level pulses suitable for interfacing with a computer or process controller. The proximity sensor can be incorporated into a load cell, a differential pressure gauge, or a device for measuring the consistency of a characteristic of a material where a variation in the consistency causes the dielectric constant of the material to change.

  20. Proximal Hamstring Repair Strength

    PubMed Central

    Harvey, Margaret Ann; Singh, Hardeep; Obopilwe, Elifho; Charette, Ryan; Miller, Suzanne

    2015-01-01

    Background: Proximal hamstring repair for complete ruptures has become a common treatment. There is no consensus in the literature about postoperative rehabilitation protocols following proximal hamstring repair. Some protocols describe bracing to prevent hip flexion or knee extension while others describe no immobilization. There are currently no biomechanical studies evaluating proximal hamstring repairs; nor are there any studies evaluating the effect of different hip flexion angles on these repairs. Hypothesis: As hip flexion increases from 0° to 90°, there will be a greater gap with cyclical loading. Study Design: Controlled laboratory study. Methods: Proximal hamstring insertions were detached from the ischial tuberosity in 24 cadavers and were repaired with 3 single-loaded suture anchors in the hamstring footprint with a Krakow suture technique. Cyclic loading from 10 to 125 N at 1 Hz was then performed for 0°, 45°, and 90° of hip flexion for 1500 cycles. Gap formation, stiffness, yield load, ultimate load, and energy to ultimate load were compared between groups using paired t tests. Results: Cyclic loading demonstrated the least amount of gap formation (P < .05) at 0° of hip flexion (2.39 mm) and most at 90° of hip flexion (4.19 mm). There was no significant difference in ultimate load between hip flexion angles (326, 309, and 338 N at 0°, 45°, and 90°, respectively). The most common mode of failure occurred with knot/suture failure (n = 17). Conclusion: Increasing hip flexion from 0° to 90° increases the displacement across proximal hamstring repairs. Postoperative bracing that limits hip flexion should be considered. Clinical Relevance: Repetitive motion involving hip flexion after a proximal hamstring repair may cause compromise of the repair. PMID:26665049

  1. Effect of Collateral Sprouting on Donor Nerve Function After Nerve Coaptation: A Study of the Brachial Plexus

    PubMed Central

    Reichert, Paweł; Kiełbowicz, Zdzisław; Dzięgiel, Piotr; Puła, Bartosz; Wrzosek, Marcin; Bocheńska, Aneta; Gosk, Jerzy

    2016-01-01

    Background The aim of the present study was to evaluate the donor nerve from the C7 spinal nerve of the rabbit brachial plexus after a coaptation procedure. Assessment was performed of avulsion of the C5 and C6 spinal nerves treated by coaptation of these nerves to the C7 spinal nerve. Material/Methods After nerve injury, fourteen rabbits were treated by end-to-side coaptation (ETS), and fourteen animals were treated by side-to-side coaptation (STS) on the right brachial plexus. Electrophysiological and histomorphometric analyses and the skin pinch test were used to evaluate the outcomes. Results There was no statistically significant difference in the G-ratio proximal and distal to the coaptation in the ETS group, but the differences in the axon, myelin sheath and fiber diameters were statistically significant. The comparison of the ETS and STS groups distal to the coaptation with the controls demonstrated statistically significant differences in the fiber, axon, and myelin sheath diameters. With respect to the G-ratio, the ETS group exhibited no significant differences relative to the control, whereas the G-ratio in the STS group and the controls differed significantly. In the electrophysiological study, the ETS and STS groups exhibited major changes in the biceps and subscapularis muscles. Conclusions The coaptation procedure affects the histological structure of the nerve donor, but it does not translate into changes in nerve conduction or the sensory function of the limb. The donor nerve lesion in the ETS group is transient and has minimal clinical relevance. PMID:26848925

  2. Traumatic proximal tibiofibular dislocation.

    PubMed

    Burgos, J; Alvarez-Montero, R; Gonzalez-Herranz, P; Rapariz, J M

    1997-01-01

    Proximal tibiofibular dislocation is an exceptional lesion. Rarer still is its presentation in childhood. We describe the clinical case of a 6-year-old boy, the victim of a road accident. He had a tibiofibular dislocation associated with a metaphyseal fracture of the tibia.

  3. Steerable Capacitive Proximity Sensor

    NASA Technical Reports Server (NTRS)

    Jenstrom, Del T.; Mcconnell, Robert L.

    1994-01-01

    Steerable capacitive proximity sensor of "capaciflector" type based partly on sensing units described in GSC-13377 and GSC-13475. Position of maximum sensitivity adjusted without moving sensor. Voltage of each driven shield adjusted separately to concentrate sensing electric field more toward one side or other.

  4. Proximate Analysis of Coal

    ERIC Educational Resources Information Center

    Donahue, Craig J.; Rais, Elizabeth A.

    2009-01-01

    This lab experiment illustrates the use of thermogravimetric analysis (TGA) to perform proximate analysis on a series of coal samples of different rank. Peat and coke are also examined. A total of four exercises are described. These are dry exercises as students interpret previously recorded scans. The weight percent moisture, volatile matter,…

  5. Dose Constraints to Prevent Radiation-Induced Brachial Plexopathy in Patients Treated for Lung Cancer

    SciTech Connect

    Amini, Arya; Yang Jinzhong; Williamson, Ryan; McBurney, Michelle L.; Erasmus, Jeremy; Allen, Pamela K.; Karhade, Mandar; Komaki, Ritsuko; Liao, Zhongxing; Gomez, Daniel; Cox, James; Dong, Lei; Welsh, James

    2012-03-01

    Purpose: As the recommended radiation dose for non-small-cell lung cancer (NSCLC) increases, meeting dose constraints for critical structures like the brachial plexus becomes increasingly challenging, particularly for tumors in the superior sulcus. In this retrospective analysis, we compared dose-volume histogram information with the incidence of plexopathy to establish the maximum dose tolerated by the brachial plexus. Methods and Materials: We identified 90 patients with NSCLC treated with definitive chemoradiation from March 2007 through September 2010, who had received >55 Gy to the brachial plexus. We used a multiatlas segmentation method combined with deformable image registration to delineate the brachial plexus on the original planning CT scans and scored plexopathy according to Common Terminology Criteria for Adverse Events version 4.03. Results: Median radiation dose to the brachial plexus was 70 Gy (range, 56-87.5 Gy; 1.5-2.5 Gy/fraction). At a median follow-up time of 14.0 months, 14 patients (16%) had brachial plexopathy (8 patients [9%] had Grade 1, and 6 patients [7%] had Grade {>=}2); median time to symptom onset was 6.5 months (range, 1.4-37.4 months). On multivariate analysis, receipt of a median brachial plexus dose of >69 Gy (odds ratio [OR] 10.091; 95% confidence interval [CI], 1.512-67.331; p = 0.005), a maximum dose of >75 Gy to 2 cm{sup 3} of the brachial plexus (OR, 4.909; 95% CI, 0.966-24.952; p = 0.038), and the presence of plexopathy before irradiation (OR, 4.722; 95% CI, 1.267-17.606; p = 0.021) were independent predictors of brachial plexopathy. Conclusions: For lung cancers near the apical region, brachial plexopathy is a major concern for high-dose radiation therapy. We developed a computer-assisted image segmentation method that allows us to rapidly and consistently contour the brachial plexus and establish the dose limits to minimize the risk of brachial plexopathy. Our results could be used as a guideline in future prospective

  6. Distinction between neoplastic and radiation-induced brachial plexopathy, with emphasis on the role of EMG

    SciTech Connect

    Harper, C.M. Jr.; Thomas, J.E.; Cascino, T.L.; Litchy, W.J.

    1989-04-01

    The results of clinical, radiologic, and electrophysiologic studies are retrospectively reviewed for 55 patients with neoplastic and 35 patients with radiation-induced brachial plexopathy. The presence or absence of pain as the presenting symptom, temporal profile of the illness, presence of a discrete mass on CT of the plexus, and presence of myokymic discharges on EMG contributed significantly to the prediction of the underlying cause of the brachial plexopathy. The distribution of weakness and the results of nerve conduction studies were of no help in distinguishing neoplastic from radiation-induced brachial plexopathy.

  7. Avulsion of the brachial plexus in a great horned owl (Bubo virginaus)

    USGS Publications Warehouse

    Moore, M.P.; Stauber, E.; Thomas, N.J.

    1989-01-01

    Avulsion of the brachial plexus was documented in a Great Horned Owl (Bubo virginianus). A fractured scapula was also present. Cause of these injuries was not known but was thought to be due to trauma. Differentiation of musculoskeletal injury from peripheral nerve damage can be difficult in raptors. Use of electromyography and motor nerve conduction velocity was helpful in demonstrating peripheral nerve involvement. A brachial plexus avulsion was suspected on the basis of clinical signs, presence of electromyographic abnormalities in all muscles supplied by the nerves of the brachial plexus and absence of median-ulnar motor nerve conduction velocities.

  8. Spatiotemporal Coupling of the Tongue in Amyotrophic Lateral Sclerosis

    ERIC Educational Resources Information Center

    Kuruvilla, Mili S.; Green, Jordan R.; Yunusova, Yana; Hanford, Kathy

    2012-01-01

    Purpose: The primary aim of the investigation was to identify deficits in spatiotemporal coupling between tongue regions in amyotrophic lateral sclerosis (ALS). The relations between disease-related changes in tongue movement patterns and speech intelligibility were also determined. Methods: The authors recorded word productions from 11…

  9. A perspective on stem cell modeling of amyotrophic lateral sclerosis

    PubMed Central

    de Boer, A Sophie; Eggan, Kevin

    2015-01-01

    Amyotrophic lateral sclerosis is a complex neurodegenerative disease. Limitations in animal models have impeded progress in studying disease pathology and potential drug discovery. Here, we will review recent advances in the development of stem cell models for the study of ALS. Additionally, we will discuss the progress toward therapeutic development derived from these stem cell based assays. PMID:26505672

  10. Amyotrophic Lateral Sclerosis: An Introduction to Psychosocial and Behavioral Adaptations.

    ERIC Educational Resources Information Center

    Hoffman, R. Leigh; Decker, Thomas W.

    1993-01-01

    Defines amyotrophic lateral sclerosis (ALS) as motor-neuron disease that is terminal. Discusses symptoms associated with ALS and identifies treatment options. Reviews psychological and behavioral adaptations in regard to ALS clients, their families, and professionals who work with them. Discusses support groups as method of reducing stress for ALS…

  11. Amyotrophic Lateral Sclerosis Patients' Perspectives on Use of Mechanical Ventilation.

    ERIC Educational Resources Information Center

    Young, Jenny M.; And Others

    1994-01-01

    Interviewed 13 amyotrophic lateral sclerosis patients. All believed that they alone should make decision regarding use of mechanical ventilation. Factors they considered important were quality of life, severity of disability, availability of ventilation by means of nasal mask, possible admission to long-term care facility, ability to discontinue…

  12. Incidence of amyotrophic lateral sclerosis in Rhineland-Palatinate, Germany.

    PubMed

    Wolf, Joachim; Wöhrle, Johannes C; Palm, Frederick; Nix, Wilfred A; Maschke, Matthias; Safer, Anton; Becher, Heiko; Grau, Armin J

    2014-06-01

    There is a lack of prospective and population based epidemiological data on amyotrophic lateral sclerosis in Germany to date. The ALS registry Rhineland-Palatinate was established to investigate the incidence, course and phenotypic variety of ALS in this south-west German state of about 4 million inhabitants. During the period 2010-2011, consecutive incident patients with amyotrophic lateral sclerosis according to the revised El Escorial criteria were included and followed up using multiple overlapping sources of case ascertainment. One hundred and forty-six patients were enrolled. The annual crude incidence for amyotrophic lateral sclerosis in Rhineland-Palatinate was 1.8/100,000 person-years (95% CI 1.6-2.2). Male to female ratio was 1.1:1. Incidence increased with age reaching a peak in the 70-74 years age group and declined thereafter. Late-onset ALS (≥ 75 years) was found in 14.4% of patients. About 32% of patients presented with bulbar onset. In conclusion, incidence rate of amyotrophic lateral sclerosis in Rhineland-Palatinate is within the range of other prospective population based registers in Europe and North America. Gender ratio is nearly balanced.

  13. Subacute diabetic proximal neuropathy

    NASA Technical Reports Server (NTRS)

    Pascoe, M. K.; Low, P. A.; Windebank, A. J.; Litchy, W. J.

    1997-01-01

    OBJECTIVE: To evaluate the clinical, electrophysiologic, autonomic, and neuropathologic characteristics and the natural history of subacute diabetic proximal neuropathy and its response to immunotherapy. MATERIAL AND METHODS: For the 12-year period from 1983 to 1995, we conducted a retrospective review of medical records of Mayo Clinic patients with diabetes who had subacute onset and progression of proximal weakness. The responses of treated versus untreated patients were compared statistically. RESULTS: During the designated study period, 44 patients with subacute diabetic proximal neuropathy were encountered. Most patients were middle-aged or elderly, and no sex preponderance was noted. The proximal muscle weakness often was associated with reduced or absent lower extremity reflexes. Associated weight loss was a common finding. Frequently, patients had some evidence of demyelination on nerve conduction studies, but it invariably was accompanied by concomitant axonal degeneration. The cerebrospinal fluid protein concentration was usually increased. Diffuse and substantial autonomic failure was generally present. In most cases, a sural nerve biopsy specimen suggested demyelination, although evidence of an inflammatory infiltrate was less common. Of 12 patients who received treatment (with prednisone, intravenous immune globulin, or plasma exchange), 9 had improvement of their conditions, but 17 of 29 untreated patients (59%) with follow-up also eventually had improvement, albeit at a much slower rate. Improvement was usually incomplete. CONCLUSION: We suggest that the entity of subacute diabetic proximal neuropathy is an extensive and severe variant of bilateral lumbosacral radiculoplexopathy, with some features suggestive of an immune-mediated cause. It differs from chronic inflammatory demyelinating polyradiculoneuropathy in that most cases have a more restricted distribution and seem to be monophasic and self-limiting. The efficacy of immunotherapy is unproved

  14. Lack of evidence of the effectiveness of primary brachial plexus surgery for infants (under the age of two years) diagnosed with obstetric brachial plexus palsy.

    PubMed

    Bialocerkowski, Andrea; Gelding, Bronwyn

    2006-12-01

    Background  Obstetric brachial plexus palsy, which occurs in 1-3 per 1000 live births, results from traction and/or compression of the brachial plexus in utero, during descent through the birth canal or during delivery. This results in a spectrum of injuries that range in extent of damage and severity and can lead to a lifelong impairment and functional difficulties associated with the use of the affected upper limb. Most infants diagnosed with obstetric brachial plexus palsy receive treatment, such as surgery to the brachial plexus, physiotherapy or occupational therapy, within the first months of life. However, there is controversy regarding the most effective form of management. This review follows on from our previous systematic review which investigated the effectiveness of primary conservative management in infants with obstetric brachial plexus palsy. This systematic review focuses on the effects of primary surgery. Objectives  The objective of this review was to systematically assess and collate all available evidence on effectiveness of primary brachial plexus surgery for infants with obstetric brachial plexus palsy. Search strategy  A systematic literature search was performed using 13 databases: TRIP, MEDLINE, CINAHL, Web of Science, Proquest 5000, Evidence Based Medicine Reviews, Expanded Academic ASAP, Meditext, Science Direct, the Physiotherapy Evidence Database, Proquest Digital Dissertations, Open Archives Initiative Search Engine, the Australian Digital Thesis program. Those studies that were reported in English and published between July 1992 to June 2004 were included in this review. Selection criteria  Quantitative studies that investigated the effectiveness of primary brachial plexus surgery for infants with obstetric brachial plexus palsy were eligible for inclusion into this review. This excluded studies where infants were solely managed conservatively or with pharmacological agents, or underwent surgery for the management of

  15. Percutaneous gastrojejunostomy in amyotrophic lateral sclerosis.

    PubMed

    Strong, M J; Rowe, A; Rankin, R N

    1999-10-31

    We have performed a retrospective review of the use of a percutaneous gastrojejunostomy in patients with amyotrophic lateral sclerosis (ALS). Forty-one patients with initial bulbar manifestations of ALS and 32 patients with initial limb manifestations underwent a percutaneous gastrojejunostomy under fluoroscopic control using the Rankin gastrojejunostomy tube. Survival characteristics were compared with 86 bulbar onsetting and 207 limb onsetting ALS patients who did not require nutritional support. The 30-day mortality rate was 9.6% (respiratory death in three bulbar onsetting patients and four limb onsetting patients) and the 30 day morbidity rate was 4.1% (one operative site infection and intraperitoneal leakage in two patients). The most frequent long-term complication was the requirement for tube changing (blockage in six; dislodgment in two). Gastric reflux was not described amongst the treated patients. Overall survivorship (symptom onset to death) was less in the bulbar onsetting patients receiving a gastrojejunostomy tube than in the control population (median survival 22.0 vs. 33.7 months, respectively, P=0.005). As a group, the median survivorship for limb onsetting patients was not different for those receiving a gastrojejunostomy than for those who did not. However, a significant reduction in survival was observed in limb onsetting patients receiving a gastrojejunostomy early in the course of their disease (P=0.001) compared to those with a longer duration prior to the procedure. This was not observed in the bulbar onsetting patients. In both patient populations, no relationship was observed between survival post-gastrojejunostomy and the severity of pulmonary involvement at the time of the intervention, serum chloride, or age at onset. These studies demonstrate that a percutaneous gastrojejunostomy is a well-tolerated and safe alternative technique for enteral nutritional support in ALS patients. It also offers the advantage of not requiring either a

  16. Upper Limb Multifactorial Movement Analysis in Brachial Plexus Birth Injury

    PubMed Central

    Bahm, Jorg

    2016-01-01

    Multifactorial motion analysis was first established for gait and then developed in the upper extremity. Recordings of infrared light reflecting sensitive passive markers in space, combined with surface eletromyographic recordings and/or transmitted forces, allow eclectic study of muscular coordination in the upper limb. Brachial plexus birth injury is responsible for various patterns of muscle weakness, imbalance, and/or simultaneous activation, soft tissue contractures, and bone-joint deformities, leading to individual motion patterns and adaptations, which we studied by means of motion analysis tools. We describe the technical development and examination setup to evaluate motion impairment and present first clinical results. Motion analysis is a reliable objective assessment tool allowing precise pre- and postoperative multimodal evaluation of upper limb function. Level of evidence: II. PMID:28077954

  17. Brachial plexus injury: the London experience with supraclavicular traction lesions.

    PubMed

    Birch, Rolfe

    2009-01-01

    In this article, the author details the experiences of his hospital and other London hospitals in treating brachial plexus injury. As noted, important advances have been made in methods of diagnosis and repair. Myelography was replaced by CT scan and later by MRI. Among the topics the author explores are diagnosis (including pain, the presence or absence of the Tinel sign, and the irradiation of pins and needles) and the principles of repair. The author emphasizes that it is imperative that ruptured nerves be repaired as soon as possible, with the closed traction lesion coming, in urgency, close behind reattachment of the amputated hand or repair of a great artery and a trunk nerve in the combined lesion. Finally, the article concludes that the surgeon must be actively engaged in the whole process of rehabilitation and treatment of pain. This is part of a Point-Counterpoint discussion with Dr. David G. Kline's presentation of "A Personal Experience."

  18. Surgical treatment of brachial plexus injuries in adults.

    PubMed

    Ricardo, Monreal

    2005-12-01

    We carried out a retrospective review of 32 consecutive patients (30 adults and two children) with total or partial lesions of the brachial plexus who had surgical repair using nerve grafting, neurotisation, and neurolysis between January 1991 and December 2003. The outcome measures of muscular strength were correlated with the type of lesion, age, preoperative time, length and number of grafts, and time to reinnervation of the biceps. The function of the upper limb was also evaluated. There was a significant correlation between muscular strength after surgical repair and both the preoperative time and the length of the nerve graft. There was also a significant correlation between muscular strength and the number of grafts. Muscular strength was better when the neurolysis was done before six months. When neurosurgical repair and reconstructive procedures were performed, the function of the upper limb was improved.

  19. Obstetrical brachial plexus injury (OBPI): Canada's national clinical practice guideline

    PubMed Central

    Coroneos, Christopher J; Voineskos, Sophocles H; Christakis, Marie K; Thoma, Achilleas; Bain, James R; Brouwers, Melissa C

    2017-01-01

    Objective The objective of this study was to establish an evidence-based clinical practice guideline for the primary management of obstetrical brachial plexus injury (OBPI). This clinical practice guideline addresses 4 existing gaps: (1) historic poor use of evidence, (2) timing of referral to multidisciplinary care, (3) Indications and timing of operative nerve repair and (4) distribution of expertise. Setting The guideline is intended for all healthcare providers treating infants and children, and all specialists treating upper extremity injuries. Participants The evidence interpretation and recommendation consensus team (Canadian OBPI Working Group) was composed of clinicians representing each of Canada's 10 multidisciplinary centres. Outcome measures An electronic modified Delphi approach was used for consensus, with agreement criteria defined a priori. Quality indicators for referral to a multidisciplinary centre were established by consensus. An original meta-analysis of primary nerve repair and review of Canadian epidemiology and burden were previously completed. Results 7 recommendations address clinical gaps and guide identification, referral, treatment and outcome assessment: (1) physically examine for OBPI in newborns with arm asymmetry or risk factors; (2) refer newborns with OBPI to a multidisciplinary centre by 1 month; (3) provide pregnancy/birth history and physical examination findings at birth; (4) multidisciplinary centres should include a therapist and peripheral nerve surgeon experienced with OBPI; (5) physical therapy should be advised by a multidisciplinary team; (6) microsurgical nerve repair is indicated in root avulsion and other OBPI meeting centre operative criteria; (7) the common data set includes the Narakas classification, limb length, Active Movement Scale (AMS) and Brachial Plexus Outcome Measure (BPOM) 2 years after birth/surgery. Conclusions The process established a new network of opinion leaders and researchers for further

  20. OCT/PS-OCT imaging of brachial plexus neurovascular structures

    NASA Astrophysics Data System (ADS)

    Raphael, David T.; Zhang, Jun; Zhang, Yaoping; Chen, Zhongping; Miller, Carol; Zhou, Li

    2004-07-01

    Introduction: Optical coherence tomography (OCT) allows high-resolution imaging (less than 10 microns) of tissue structures. A pilot study with OCT and polarization-sensitive OCT (PS-OCT) was undertaken to image ex-vivo neurovascular structures (vessels, nerves) of the canine brachial plexus. Methods: OCT is an interferometry-based optical analog of B-mode ultrasound, which can image through non-transparent biological tissues. With approval of the USC Animal Care and Use Committee, segments of the supra- and infraclavicular brachial plexus were excised from euthanized adult dogs, and the ex-vivo specimens were placed in cold pH-buffered physiologic solution. An OCT beam, in micrometer translational steps, scanned the fixed-position bisected specimens in transverse and longitudinal views. Two-dimensional images were obtained from identified arteries and nerves, with specific sections of interest stained with hematoxylin-eosin for later imaging through a surgical microscope. Results: with the beam scan direction transverse to arteries, the resulting OCT images showed an identifiable arterial lumen and arterial wall tissue layers. By comparison, transverse beam OCT images of nerves revealed a multitude of smaller nerve bundles contained within larger circular-shaped fascicles. PS-OCT imaging was helpful in showing the characteristic birefringence exhibited by arrayed neural structures. Discussion: High-resolution OCT imaging may be useful in the optical identification of neurovascular structures during attempted regional nerve blockade. If incorporated into a needle-shaped catheter endoscope, such a technology could prevent intraneural and intravascular injections immediately prior to local anesthetic injection. The major limitation of OCT is that it can form a coherent image of tissue structures only to a depth of 1.5 - 2 mm.

  1. Morphometric Atlas Selection for Automatic Brachial Plexus Segmentation

    SciTech Connect

    Van de Velde, Joris; Wouters, Johan; Vercauteren, Tom; De Gersem, Werner; Duprez, Fréderic; De Neve, Wilfried; Van Hoof, Tom

    2015-07-01

    Purpose: The purpose of this study was to determine the effects of atlas selection based on different morphometric parameters, on the accuracy of automatic brachial plexus (BP) segmentation for radiation therapy planning. The segmentation accuracy was measured by comparing all of the generated automatic segmentations with anatomically validated gold standard atlases developed using cadavers. Methods and Materials: Twelve cadaver computed tomography (CT) atlases (3 males, 9 females; mean age: 73 years) were included in the study. One atlas was selected to serve as a patient, and the other 11 atlases were registered separately onto this “patient” using deformable image registration. This procedure was repeated for every atlas as a patient. Next, the Dice and Jaccard similarity indices and inclusion index were calculated for every registered BP with the original gold standard BP. In parallel, differences in several morphometric parameters that may influence the BP segmentation accuracy were measured for the different atlases. Specific brachial plexus-related CT-visible bony points were used to define the morphometric parameters. Subsequently, correlations between the similarity indices and morphometric parameters were calculated. Results: A clear negative correlation between difference in protraction-retraction distance and the similarity indices was observed (mean Pearson correlation coefficient = −0.546). All of the other investigated Pearson correlation coefficients were weak. Conclusions: Differences in the shoulder protraction-retraction position between the atlas and the patient during planning CT influence the BP autosegmentation accuracy. A greater difference in the protraction-retraction distance between the atlas and the patient reduces the accuracy of the BP automatic segmentation result.

  2. Restoration and protection of brachial plexus injury: hot topics in the last decade.

    PubMed

    Zhang, Kaizhi; Lv, Zheng; Liu, Jun; Zhu, He; Li, Rui

    2014-09-15

    Brachial plexus injury is frequently induced by injuries, accidents or birth trauma. Upper limb function may be partially or totally lost after injury, or left permanently disabled. With the development of various medical technologies, different types of interventions are used, but their effectiveness is wide ranging. Many repair methods have phasic characteristics, i.e., repairs are done in different phases. This study explored research progress and hot topic methods for protection after brachial plexus injury, by analyzing 1,797 articles concerning the repair of brachial plexus injuries, published between 2004 and 2013 and indexed by the Science Citation Index database. Results revealed that there are many methods used to repair brachial plexus injury, and their effects are varied. Intervention methods include nerve transfer surgery, electrical stimulation, cell transplantation, neurotrophic factor therapy and drug treatment. Therapeutic methods in this field change according to the hot topic of research.

  3. Screening for Peripheral Artery Disease and Cardiovascular Disease Risk Assessment with Ankle Brachial Index in Adults

    MedlinePlus

    Understanding Task Force Recommendations Screening for Peripheral Artery Disease and Cardiovascular Disease Risk Assessment with Ankle Brachial Index in Adults The U.S. Preventive Services Task Force (Task Force) has issued a ...

  4. Restoration and protection of brachial plexus injury: hot topics in the last decade

    PubMed Central

    Zhang, Kaizhi; Lv, Zheng; Liu, Jun; Zhu, He; Li, Rui

    2014-01-01

    Brachial plexus injury is frequently induced by injuries, accidents or birth trauma. Upper limb function may be partially or totally lost after injury, or left permanently disabled. With the development of various medical technologies, different types of interventions are used, but their effectiveness is wide ranging. Many repair methods have phasic characteristics, i.e., repairs are done in different phases. This study explored research progress and hot topic methods for protection after brachial plexus injury, by analyzing 1,797 articles concerning the repair of brachial plexus injuries, published between 2004 and 2013 and indexed by the Science Citation Index database. Results revealed that there are many methods used to repair brachial plexus injury, and their effects are varied. Intervention methods include nerve transfer surgery, electrical stimulation, cell transplantation, neurotrophic factor therapy and drug treatment. Therapeutic methods in this field change according to the hot topic of research. PMID:25374596

  5. Reliability of 3D upper limb motion analysis in children with obstetric brachial plexus palsy.

    PubMed

    Mahon, Judy; Malone, Ailish; Kiernan, Damien; Meldrum, Dara

    2017-03-01

    Kinematics, measured by 3D upper limb motion analysis (3D-ULMA), can potentially increase understanding of movement patterns by quantifying individual joint contributions. Reliability in children with obstetric brachial plexus palsy (OBPP) has not been established.

  6. Contralateral Spinal Accessory Nerve Transfer: A New Technique in Panavulsive Brachial Plexus Palsy.

    PubMed

    Zermeño-Rivera, Jaime; Gutiérrez-Amavizca, Bianca Ethel

    2015-06-01

    Brachial plexus avulsion results from excessive stretching and can occur secondary to motor vehicle accidents, mainly in motorcyclists. In a 28-year-old man with panavulsive brachial plexus palsy, we describe an alternative technique to repair brachial plexus avulsion and to stabilize and preserve shoulder function by transferring the contralateral spinal accessory nerve to the suprascapular nerve. We observed positive clinical and electromyographic results in sternocleidomastoid, trapezius, supraspinatus, infraspinatus, pectoralis, triceps, and biceps, with good outcome and prognosis for shoulder function at 12 months after surgery. This technique provides a unique opportunity for patients suffering from severe brachial plexus injuries and lacking enough donor nerves to obtain shoulder stability and mobility while avoiding bone fusion and preserving functionality of the contralateral shoulder with favorable postoperative outcomes.

  7. Neuroanatomy of the brachial plexus: normal and variant anatomy of its formation.

    PubMed

    Johnson, Elizabeth O; Vekris, Marios; Demesticha, Theano; Soucacos, Panayotis N

    2010-03-01

    The brachial plexus is the complex network of nerves, extending from the neck to the axilla, which supplies motor, sensory, and sympathetic fibers to the upper extremity. Typically, it is formed by the union of the ventral primary rami of the spinal nerves, C5-C8 & T1, the so-called "roots" of the brachial plexus. By examining the neural architecture of the brachial plexus, the most constant arrangement of nerve fibers can be delineated, and the most predominate variations in the neural architecture defined. A thorough understanding of the neuroanatomy of the brachial plexus, with an appreciation of the possible anatomic variations that may occur is necessary for effective clinical practice.

  8. Brachial Plexus Injury from CT-Guided RF Ablation Under General Anesthesia

    SciTech Connect

    Shankar, Sridhar Sonnenberg, Eric van; Silverman, Stuart G.; Tuncali, Kemal; Flanagan, Hugh L.; Whang, Edward E.

    2005-06-15

    Brachial plexus injury in a patient under general anesthesia (GA) is not uncommon, despite careful positioning and, particularly, awareness of the possibility. The mechanism of injury is stretching and compression of the brachial plexus over a prolonged period. Positioning the patient within the computed tomography (CT) gantry for abdominal or chest procedures can simulate a surgical procedure, particularly when GA is used. The potential for brachial plexus injury is increased if the case is prolonged and the patient's arms are raised above the head to avoid CT image degradation from streak artifacts. We report a case of profound brachial plexus palsy following a CT-guided radiofrequency ablation procedure under GA. Fortunately, the patient recovered completely. We emphasize the mechanism of injury and detail measures to combat this problem, such that radiologists are aware of this potentially serious complication.

  9. Investigation of brachial plexus traction lesions by peripheral and spinal somatosensory evoked potentials.

    PubMed Central

    Jones, S J

    1979-01-01

    Peripheral, spinal and cortical somatosensory evoked potentials were recorded in 26 patients with unilateral traction injuries of the brachial plexus ganglia. Of 10 cases explored surgically the recordings correctly anticipated the major site of the lesion in eight. PMID:422958

  10. Proximity Networks and Epidemics

    NASA Astrophysics Data System (ADS)

    Guclu, Hasan; Toroczkai, Zoltán

    2007-03-01

    We presented the basis of a framework to account for the dynamics of contacts in epidemic processes, through the notion of dynamic proximity graphs. By varying the integration time-parameter T, which is the period of infectivity one can give a simple account for some of the differences in the observed contact networks for different diseases, such as smallpox, or AIDS. Our simplistic model also seems to shed some light on the shape of the degree distribution of the measured people-people contact network from the EPISIM data. We certainly do not claim that the simplistic graph integration model above is a good model for dynamic contact graphs. It only contains the essential ingredients for such processes to produce a qualitative agreement with some observations. We expect that further refinements and extensions to this picture, in particular deriving the link-probabilities in the dynamic proximity graph from more realistic contact dynamics should improve the agreement between models and data.

  11. Tetraplegia or paraplegia with brachial diparesis? What is the most appropriate designation for the motor deficit in patients with lower cervical spinal cord injury?

    PubMed

    Figueiredo, Nicandro; Figueiredo, Iara Eberhard; Resnick, Daniel

    2013-02-01

    The authors seek to clarify the nomenclature used to describe cervical spinal cord injuries, particularly the use of the terms "tetraplegia", "quadriplegia", "quadriparesis", "tetraparesis", "incomplete quadriplegia" or "incomplete tetraplegia" when applied to patients with lower cervical cord injuries. A review of the origin of the terms and nomenclature used currently to describe the neurological status of patients with SCI in the literature was performed. The terms "tetraplegia", "quadriplegia", "quadriparesis", "tetraparesis", "incomplete quadriplegia" or "incomplete tetraplegia" have been used very often to describe patients with complete lower cervical SCI despite the fact that the clinical scenario is all the same for most of these patients. Most of these patients have total loss of the motor voluntary movements of their lower trunk and inferior limbs, and partial impairment of movement of their superior limbs, preserving many motor functions of the proximal muscles of their arms (superior limbs). A potentially better descriptive term may be "paraplegia with brachial diparesis". In using the most appropriate terminology, the patients with lower cervical SCI currently referred as presenting with "tetraplegia", "quadriplegia", "quadriparesis", "tetraparesis", "incomplete quadriplegia" or "incomplete tetraplegia", might be better described as having "paraplegia with brachial diparesis".

  12. Diffuse aneurysmal degeneration of the brachial artery after long-standing high-flow arteriovenous fistula closure for hemodialysis at elbow level.

    PubMed

    De Santis, Francesco; Martini, Guido; Mani, Gabriele; Bernhard, Othmar

    2014-07-01

    While the possibility of development of a panarterial dilatation proximal to a long-standing high-flow posttraumatic arteriovenous fistula is well known, to the best of our knowledge, this event has never been described after vascular access for hemodialysis closure. We describe a man in whom a diffuse aneurysmal degeneration of the brachial artery has been highlighted 6 months after long-standing high-flow arteriovenous fistula closure. A 47-year-old man developed a painful pulsatile mass in the anterior distal third of his arm 6 months after long-standing high-flow arteriovenous fistula closure at the level of his elbow. A computed tomography scan revealed multiple "true" aneurysms of the brachial artery (BA) that appeared enlarged in toto. One of these aneurysms (near the BA bifurcation) presented with significant thrombus stratification. Surgery was recommended because of the major risk of peripheral embolization. Considering the anatomic characteristics of both the BA and aneurysm, no arterial substitution was performed and, after removal of the thrombus, the aneurysm diameter was reduced via direct arterial wall suture. The patient was discharged under oral anticoagulation. Aneurysmal degeneration of the donor artery after vascular access is relatively rare but represents a challenging problem. Operative or conservative management of these aneurysms should evaluate both the possible aneurysm-related complications and the feasibility of vascular reconstruction. In this context, the risk of additional donor artery and/or vascular reconstruction enlargement over time should also be considered.

  13. Vascular patterns of upper limb: an anatomical study with accent on superficial brachial artery

    PubMed Central

    Kachlik, David; Konarik, Marek; Baca, Vaclav

    2011-01-01

    The aim of the study was to evaluate the terminal segmentation of the axillary artery and to present four cases of anomalous branching of the axillary artery, the superficial brachial artery (arteria brachialis superficialis), which is defined as the brachial artery that runs superficially to the median nerve. Totally, 130 cadaveric upper arms embalmed by classical formaldehyde technique from collections of the Department of Anatomy, Third Faculty of Medicine, Charles University in Prague, were macroscopically dissected with special focus on the branching arrangement of the axillary artery. The most distal part of the axillary artery (infrapectoral part) terminated in four cases as a bifurcation into two terminal branches: the superficial brachial artery and profunda brachii artery, denominated according to their relation to the median nerve. The profunda brachii artery primarily gave rise to the main branches of the infrapectoral part of the axillary artery. The superficial brachial artery descended to the cubital fossa where it assumed the usual course of the brachial artery in two cases and in the other two cases its branches (the radial and ulnar arteries) passed superficially to the flexors. The incidence of the superficial brachial artery in our study was 5% of cases. The reported incidence is a bit contradictory, from 0.12% to 25% of cases. The anatomical knowledge of the axillary region is of crucial importance for neurosurgeons and specialists using the radiodiagnostic techniques, particularly in cases involving traumatic injuries. The improved knowledge would allow more accurate diagnostic interpretations and surgical treatment. PMID:21342134

  14. Targeted Riluzole Delivery by Antioxidant Nanovectors for Treating Amyotrophic Lateral Sclerosis

    DTIC Science & Technology

    2013-10-01

    treating amyotrophic lateral sclerosis . PRINCIPAL INVESTIGATOR: Raymond J. Grill CONTRACTING ORGANIZATION: University of Texas Health...treating Amyotrophic lateral sclerosis 5a. CONTRACT NUMBER W 5b. GRANT NUMBER W81XWH-12-1-0612 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S...survival in a mouse model of amyotrophic lateral sclerosis . This project involves work performed at both UT-Health and Rice University; combining the

  15. A case of amyotrophic lateral sclerosis presented as oropharyngeal Dysphagia.

    PubMed

    Noh, Eun Ji; Park, Moo In; Park, Seun Ja; Moon, Won; Jung, Hyun Joo

    2010-07-01

    Amyotrophic lateral sclerosis is a rare disease. It is a fatal neurodegenerative disease characterized by progressive muscular paralysis reflecting degeneration of motor neurons which leads to muscle weakness and muscle wasting. Respiratory failure limits survival to 2-5 years after disease onset. Several clinical manifestations including dysphagia can result in reductions in both the quality of life and life expectancy. Dysphagia occurs at onset in about one third of case, although generally it occurs in later stage of the disease. Evaluation of dysphagia includes video-fluoroscopic swallow study, radiological esophagogram, flexible endoscopic examination, ultrasound examination, conventional manometry and electromyography. We report a case of amyotrophic lateral sclerosis in a 54-year-old man presenting oropharyngeal dysphagia which was diagnosed by high resolution esophageal manometry presenting abnormality of the upper esophageal sphincter.

  16. Motor recovery and the breathing arm after brachial plexus surgical repairs, including re-implantation of avulsed spinal roots into the spinal cord.

    PubMed

    Htut, M; Misra, V P; Anand, P; Birch, R; Carlstedt, T

    2007-04-01

    Forty-four patients with severe traction brachial plexus avulsion injuries were studied following surgical repairs. In eight patients, re-implanting avulsed spinal roots directly to the spinal cord was performed with other repairs and motor recovery in the proximal limb was similar to that achieved by conventional nerve grafts and transfers when assessed using the MRC clinical grades, Narakas scores, EMG and Transcranial Magnetic Stimulation (TMS). Thirty-four of the 37 patients had co-contractions of agonist and antagonist muscle groups. Spontaneous contractions of limb muscles in synchrony with respiration, the "breathing arm", were noted in 26 of 37 patients: in three patients, the source of the breathing arm was from spinal cord re-connection, providing evidence of regeneration from the CNS to the periphery. Our study shows that re-connection of avulsed spinal roots can produce good motor recovery and provides a clinical model for developing new treatments which may enhance nerve regeneration.

  17. Echosonography with proximity sensors

    NASA Astrophysics Data System (ADS)

    Thaisiam, W.; Laithong, T.; Meekhun, S.; Chaiwathyothin, N.; Thanlarp, P.; Danworaphong, S.

    2013-03-01

    We propose the use of a commercial ultrasonic proximity sensor kit for profiling an altitude-varying surface by employing echosonography. The proximity sensor kit, two identical transducers together with its dedicated operating circuit, is used as a profiler for the construction of an image. Ultrasonic pulses are emitted from one of the transducers and received by the other. The time duration between the pulses allows us to determine the traveling distance of each pulse. In the experiment, the circuit is used with the addition of two copper wires for directing the outgoing and incoming signals to an oscilloscope. The time of flight of ultrasonic pulses can thus be determined. Square grids of 5 × 5 cm2 are made from fishing lines, forming pixels in the image. The grids are designed to hold the detection unit in place, about 30 cm above a flat surface. The surface to be imaged is constructed to be height varying and placed on the flat surface underneath the grids. Our result shows that an image of the profiled surface can be created by varying the location of the detection unit along the grid. We also investigate the deviation in relation to the time of flight of the ultrasonic pulse. Such an experiment should be valuable for conveying the concept of ultrasonic imaging to physical and medical science undergraduate students. Due to its simplicity, the setup could be made in any undergraduate laboratory relatively inexpensively and it requires no complex parts. The results illustrate the concept of echosonography.

  18. Amyotrophic lateral sclerosis-like presentation in a HIV-positive patient.

    PubMed

    Anand, Kuljeet Singh; Wadhwa, Ankur; Garg, Jyoti; Mahajan, Rakesh Kumar

    2014-01-01

    There has been several reports of an MND like syndrome in HIV-1 infection, however the data is still sparse. Furthermore, HIV-associated amyotrophic lateral sclerosis (ALS) syndrome differs from the classical ALS in some key aspects.. A 44-year-old male presented with a history of insidious onset and gradually progressive asymmetric weakness of lower limbs. He also complained of thinning in both legs, the left leg more than the right since 1 year along with spontaneous twitching of muscles in both the thighs. On neurological examination, the assessment of higher mental functions was normal. There were no cranial nerve deficits. Motor power was grade 5/5 (Medical Research Council scale) in both the upper limbs and 4+ at hips and knees bilaterally, 5 at right ankle, and 4+ at left ankle. All the deep tendon reflexes were brisk with extensor planter responses. There were no cerebellar signs or sensory deficits. HIV-1 was reactive in enzyme-linked immunosorbent assay. Electrophysiological studies were conducted per the MND protocol.None of the nerves studied showed an abnormal drop in compound muscle action potential amplitude with proximal stimulation. There was evidence of diffuse spontaneous activity, which manifests as fibrillation and fasciculation potentials in most muscles tested . Overall there seems to be sufficient evidence to implicate HIV as a potential cause of an ALS-like disorder, but one must also consider the possibility of coincidental HIV infection in patients who have sporadic ALS.

  19. The interaction between breathing and swallowing in amyotrophic lateral sclerosis.

    PubMed

    Erdem, Nazan Simsek; Karaali, Kamil; Ünal, Ali; Kızılay, Ferah; Öğüş, Candan; Uysal, Hilmi

    2016-12-01

    The aim of the study is to determine the association between respiratory swallow patterns in amyotrophic lateral sclerosis (ALS) patients. Furthermore, it aims to clarify the role of the dysphagia limit in defining the relationship between swallowing disorders and respiratory disorders. Functional rating scales were used to describe swallowing and respiratory function. Swallowing was observed using the dysphagia limit. Dysphagia limit is the volume at which a second or more swallows are required to swallow the whole bolus. Laryngeal and chest movement sensors, pulmonary function tests, submental, and diaphragm electromyography activity were used to evaluate the relationship between swallowing and respiratory phase. Of the 27 patients included in the study, 14 were dysphagic and 13 were non-dysphagic. Tests showed normal respiratory function in 11 of the non-dysphagic patients and 3 of the dysphagic patients. There was a high correlation between the dysphagia limit and Amyotrophic Lateral Sclerosis Functional Rating Scale swallowing parameters. Non-dysphagic patients were able to swallow during inspiration but only six patients in the dysphagic group were able to swallow during inspiration. The occurrence of dysphagia in ALS is related to piecemeal deglutition and respiration consistency during swallowing. Detecting the timing of disturbances in the relationship between swallowing and respiration may be a way of identifying dysphagia. Dysphagia limit may be a useful, complementary test for assessing swallowing disturbances in amyotrophic lateral sclerosis.

  20. Compromising abnormalities of the brachial plexus as displayed by magnetic resonance imaging.

    PubMed

    Collins, J D; Shaver, M L; Disher, A C; Miller, T Q

    1995-01-01

    Magnetic resonance images (MRI) of brachial plexus anatomy bilaterally, not possible by plain radiographs or CT, were presented to the Vascular Surgery, Neurology, and the Neurosurgery departments. Patients were requested for MRI of their brachial plexus. They were referred for imaging and the imaging results were presented to the faculty and housestaff. Our technique was accepted and adopted to begin referrals for MRI evaluation of brachial plexopathy. Over 175 patients have been studied. Eighty-five patients were imaged with the 1.5 Tesla magnet (Signa; General Electric Medical Systems, Milwaukee, WI) 3-D reconstruction MRI. Coronal, transverse (axial), oblique transverse, and sagittal plane T1-weighted and selected T2-weighted pulse sequences were obtained at 4-5 mm slice thickness, 40-45 full field of view, and a 512 x 256 size matrix. Saline water bags were used to enhance the signal between the neck and the thorax. Sites of brachial plexus compromise were demonstrated. Our technique with 3-D reconstruction increased the definition of brachial plexus pathology. The increased anatomical definition enabled the vascular surgeons and neurosurgeons to improve patient care. Brachial plexus in vivo anatomy as displayed by MRI, magnetic resonance angiography (MRA), and 3-D reconstruction offered an opportunity to augment the teaching of clinical anatomy to medical students and health professionals. Selected case presentations (bodybuilder, anomalous muscle, fractured clavicle, thyroid goiter, silicone breast implant rupture, and cervical rib) demonstrated compromise of the brachial plexus displayed by MRI. The MRI and 3-D reconstruction techniques, demonstrating the bilateral landmark anatomy, increased the definition of the clinical anatomy and resulted in greater knowledge of patient care management.

  1. Brachial artery injury following opened elbow dislocation associated with accessory brachial artery: two rare entities in a 17-year -old girl: case report.

    PubMed

    Hajji, Rita; Zrihni, Youssef; Naouli, Hamza; Bouarhroum, Abdellatif

    2015-01-01

    Elbow dislocations are the most frequently encountered after shoulder dislocations. In their vast majority, these injuries carry a good prognosis. Although, concomitant arterial injury is rare and make them more serious. In this paper, we report a case of a 17 year old woman with opened elbow dislocation with arterial injury associated to an artery variation: "accessory brachial artery".

  2. Brachial artery injury following opened elbow dislocation associated with accessory brachial artery: two rare entities in a 17-year –old girl: case report

    PubMed Central

    Hajji, Rita; Zrihni, Youssef; Naouli, Hamza; Bouarhroum, Abdellatif

    2015-01-01

    Elbow dislocations are the most frequently encountered after shoulder dislocations. In their vast majority, these injuries carry a good prognosis. Although, concomitant arterial injury is rare and make them more serious. In this paper, we report a case of a 17 year old woman with opened elbow dislocation with arterial injury associated to an artery variation: "accessory brachial artery" PMID:26161188

  3. Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome.

    PubMed

    Wakerley, Benjamin R; Yuki, Nobuhiro

    2014-03-01

    The pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome is defined by rapidly progressive oropharyngeal and cervicobrachial weakness associated with areflexia in the upper limbs. Serial nerve conduction studies suggest that PCB represents a localised subtype of Guillain-Barré syndrome characterised by axonal rather than demyelinating neuropathy. Many neurologists are unfamiliar with PCB, which is often misdiagnosed as brainstem stroke, myasthenia gravis or botulism. The presence of additional ophthalmoplegia and ataxia indicates overlap with Fisher syndrome. Half of patients with PCB carry IgG anti-GT1a antibodies which often cross-react with GQ1b, whereas most patients with Fisher syndrome carry IgG anti-GQ1b antibodies which always cross-react with GT1a. Significant overlap between the clinical and serological profiles of these patients supports the view that PCB and Fisher syndrome form a continuous spectrum. In this review, we highlight the clinical features of PCB and outline new diagnostic criteria.

  4. Microscopic anatomy of brachial plexus branches in Wistar rats.

    PubMed

    Santos, Ana Paula; Suaid, Carla Adelino; Fazan, Valéria Paula Sassoli; Barreira, Amilton Antunes

    2007-05-01

    In the present study, we analyze the morphology and morphometry of the lateral proper digital nerve of the third finger, and of the proximal and distal segments of the ulnar, median, and radial nerves, in Wistar rats 4 or 7 weeks old. The fascicular area and diameter were generally significantly greater in the proximal compared to distal segments and tended to be larger in 7-week-old compared to 4-week-old rats (e.g., median nerve area of 0.13 mm(2) for the proximal and 0.07 mm(2) for distal segments in 4-week-old rats, and 0.17 and 0.10 mm(2), respectively, for the proximal and distal segments of 7-week-old rats). The number of fascicles was significantly greater while the number of myelinated fibers was significantly less in the distal segments (e.g., 1,359 and 509 myelinated fibers, respectively, in the proximal and distal segments of the radial nerve 4-week-old rats). There was no significant difference in these parameters between the two age groups. The diameter of the myelinated fibers and their respective axons increased from 4 to 7 weeks of age (e.g., myelinated fiber diameter of 4.10 microm in 4-week-old animals and 4.7 microm in the ulnar nerve proximal segment of 7-week-old rats). The g-ratio regression line (axon diameter vs. fiber diameter quotient) was outlined for all the nerves studied here. Differences in myelinated fiber density were detected between the segments of the radial nerve, accompanying the number of myelinated fibers. Detailed knowledge of the microscopic anatomy of rat forelimb nerves provides control data for comparison with studies of experimentally induced neuropathies, which can shed more light on human neuropathies.

  5. Bilateral Brachial Plexus Home Going Catheters After Digital Amputation for Patient With Upper Extremity Digital Gangrene

    PubMed Central

    Abd-Elsayed, Alaa A; Seif, John; Guirguis, Maged; Zaky, Sherif; Mounir-Soliman, Loran

    2011-01-01

    Peripheral nerve catheter placement is used to control surgical pain. Performing bilateral brachial plexus block with catheters is not frequently performed; and in our case sending patient home with bilateral brachial plexus catheters has not been reported up to our knowledge. Our patient is a 57 years old male patient presented with bilateral upper extremity digital gangrene on digits 2 through 4 on both sides with no thumb involvement. The plan was to do the surgery under sequential axillary blocks. On the day of surgery a right axillary brachial plexus block was performed under ultrasound guidance using 20 ml of 0.75% ropivacaine. Patient was taken to the OR and the right fingers amputation was carried out under mild sedation without problems. Left axillary brachial plexus block was then done as the surgeon was closing the right side, two hours after the first block was performed. The left axillary block was done also under ultrasound using 20 ml of 2% mepivacaine. The brachial plexus blocks were performed in a sequential manner. Surgery was unremarkable, and patient was transferred to post anesthetic care unit in stable condition. Over that first postoperative night, the patient complained of severe pain at the surgical sites with minimal pain relief with parentral opioids. We placed bilateral brachial plexus catheters (right axillary and left infra-clavicular brachial plexus catheters). Ropivacaine 0.2% infusion was started at 7 ml per hour basal rate only with no boluses on each side. The patient was discharged home with the catheters in place after receiving the appropriate education. On discharge both catheters were connected to a single ON-Q (I-flow Corporation, Lake Forest, CA) ball pump with a 750 ml reservoir using a Y connection and were set to deliver a fixed rate of 7 ml for each catheter. The brachial plexus catheters were removed by the patient on day 5 after surgery without any difficulty. Patient's postoperative course was otherwise unremarkable

  6. Application of exercise transcutaneous oxygen pressure measurements for detection of proximal lower extremity arterial disease: a case report.

    PubMed

    Mahe, Guillaume; Kalra, Manju; Abraham, Pierre; Liedl, David A; Wennberg, Paul W

    2015-06-01

    Proximal claudication is secondary to ischemia caused by peripheral artery disease (PAD), whereas proximal pseudo-claudication is secondary to other disease processes such as hip arthritis, spinal stenosis, neuropathy, and so forth. The differentiation between the two can be challenging. Exercise transcutaneous oxygen pressure measurement (exercise-TcPO2) allows noninvasive detection of flow-reducing lesions in the proximal arteries and tributaries of the lower extremity arterial tree. We present the first case report in the United States using an exercise-TcPO2 algorithm. A 71-year-old diabetic patient with proximal left-sided and right-calf claudication with indeterminate ankle-brachial indices underwent an exercise-TcPO2 study before and after endovascular intervention. Four TcPO2 probes were placed: one at chest level (reference probe), one on each buttock, and one on the symptomatic calf. The Delta from Resting Oxygen Pressure (DROP) index was calculated at each probe site using a previously validated protocol. Proximal left- and right-calf ischemia were confirmed by the initial exercise-TcPO2, and, after endovascular treatment of the left iliac artery lesion, improvements in proximal exercise-TcPO2 values were found. These data suggest that exercise-TcPO2 can be useful in PAD evaluation in patients with non-compressible arteries and/or proximal claudication.

  7. Use of a Collagen-Based Device for Closure of Low Brachial Artery Punctures

    SciTech Connect

    Belenky, A. Aranovich, D.; Greif, F.; Bachar, G.; Bartal, G.; Atar, E.

    2007-04-15

    Purpose. To report our experience with the Angioseal vascular closure device for hemostasis of distal brachial artery puncture. Methods. Between September 2003 and August 2005, 64 Angioseal vascular closure devices were inserted in 64 patients (40 men, 24 women; mean age 65 years) immediately after diagnostic or therapeutic arterial angiographies performed through a 5 Fr to 7 Fr sheath via the distal brachial artery. Ultrasound examination of the brachial artery preceded the angiography in all cases and only arteries wider than 4 mm were closed by the Angioseal. In cases of a sonographically evident thin subcutaneous space of the cubital fossa, tissue tumescence, using 1% Lidocaine, was performed prior to the arterial closure. Results. The deployment success rate was 100%. No major complications were encountered; only 2 patients developed puncture site hematoma, and these were followed conservatively. Conclusions. Closure of low brachial artery punctures with the Angioseal is simple and safe. No additional manual compression is required. We recommend its use after brachial artery access interventions, through appropriately wide arteries, to improve early patient ambulation and potentially reduce possible puncture site complications.

  8. Genetic contribution to brachial artery flow-mediated dilation: The Northern Manhattan Family Study

    PubMed Central

    Suzuki, Keiko; Juo, Suh-Hang Hank; Rundek, Tanja; Boden-Albala, Bernadette; Disla, Norbelina; Liu, Rui; Park, Naeun; Di Tullio, Marco R.; Sacco, Ralph L.; Homma, Shunichi

    2007-01-01

    Background Brachial artery flow-mediated dilation (FMD) is a non-invasive measure of endothelial function. Endothelial dysfunction has been associated with traditional vascular risk factors and increased risk of cardiovascular disease. The importance of genetic contribution to FMD and baseline brachial artery diameter has not been shown in Hispanic populations. The purpose of this study was to estimate the heritability of FMD. Methods Flow mediated dilation and brachial artery diameter were measured in a subset of Caribbean Hispanic families from the ongoing Northern Manhattan Family Study (NOMAFS), which studies the contribution of genetics to stroke and cardiovascular risk factors. The age- and sex-adjusted heritability of FMD was estimated using variance component methods. Results The current data include 620 subjects (97 probands and 523 relatives) from 97 families. The age and sex-adjusted heritability of brachial artery diameter was 0.57 (p < 0.01). The age- and sex-adjusted heritability of FMD was 0.20 (p = 0.01). After additional adjustment for systolic and diastolic blood pressure, body mass index, smoking, lipid, diabetes mellitus, medication, and baseline brachial artery diameter, the heritability of FMD was 0.17 (p = 0.01). Conclusions We found modest heritability of FMD. FMD might be a reasonable phenotype for further investigation of genetic contribution to atherosclerosis. PMID:17462653

  9. Above Elbow Amputation Under Brachial Plexus Block at Supraclavicular and Interscalene Levels

    PubMed Central

    Ahmad, Hassan; Yadagiri, Manjula; Macrosson, Duncan; Majeed, Amer

    2015-01-01

    Introduction: The brachial plexus block is a commonly performed procedure in the anesthetic practice today. It is performed for analgesia as well as anesthesia for upper limb procedures. It has been used for amputation and replantation surgeries of the upper limb. Case presentation: We present the case of a 68-year-old gentleman who had brachial plexus block at supraclavicular and interscalene levels as the sole anesthetic for undergoing above elbow amputation. He was deemed to be very high risk for a general anesthetic as he suffered from severe chronic obstructive pulmonary disease (COPD) and a very poor exercise tolerance (NYHA Class III). The supraclavicular brachial plexus block was supplemented with an interscalene brachial plexus block due to inadequate surgical anesthesia encountered with the former. The procedure was successfully completed under regional anesthesia. Conclusions: The brachial plexus block can be performed at different levels in the same patient to achieve desired results, while employing sound anatomical knowledge and adhering to the maximum safe dose limit of the local anesthetic. PMID:26705518

  10. Trifurcation of superficial brachial artery: a rare case with its clinico-embryological implications.

    PubMed

    Gupta, N; Anshu, A; Dada, R

    2014-01-01

    Literatures on vasculature of upper limbs are crammed with reports of distinctly deviant version of normally prevalent vessels having modified origins, altered branching and odd courses. A unique anatomical variation in vascular pattern was observed during routine dissection of right upper limb in gross anatomy laboratory, AIIMS, New Delhi, India. The brachial artery was placed superficial to median nerve in the arm and therefore it was called superficial brachial artery. In the cubital fossa, 2.8 cm distal to intercondylar line of elbow joint, this superficial brachial artery terminated by trifurcation into radial, common interosseous and ulnar branches. Strikingly the ulnar branch, after its origin ran superficially over the median nerve and epitrochlear superficial flexor group of muscles of forearm in succession for the initial third of its course in the forearm, consequently it was addressed as superficial ulnar artery. The existence of superficial brachial artery in place of normal brachial artery, its termination by trifurcation into radial, common interosseous and superficial ulnar arteries with remarkably different courses, leads to confusing disposition of structures in the arm, cubital fossa and in the forearm and collectively makes this myriad of anatomical variations even rarer. The clinico-embryological revelations for combination of these unconventional observations, apprises and guides the specialized medical personnel attempting blind and invasive procedures in brachium and ante-brachium. This case report depicts the anatomical perspective and clinical implications on confronting a rare variant vasculature architecture pattern of upper limb.

  11. A case of relapsing-remitting facial palsy and ipsilateral brachial plexopathy caused by HSV-1.

    PubMed

    Alstadhaug, Karl B; Kvarenes, Hanne W; Prytz, Jan; Vedeler, Christian

    2016-05-01

    The etiologies of Bell's palsy and brachial neuritis remain uncertain, and the conditions rarely co-occur or reoccur. Here we present a woman in her twenties who had several relapsing-remitting episodes with left-sided facial palsy and brachial neuropathy. The episodes always started with painful left-sided oral blisters. Repeat PCRs HSV-1 DNA from oral vesicular lesions were positive. Extensive screening did not reveal any other underlying cause. Findings on MRI T2-weighted brachial plexus STIR images, using a 3.0-Tesla scanner during an episode, were compatible with brachial plexus neuritis. Except a mannose-binding lectin deficiency, a congenital complement deficiency that is frequently found in the general Caucasian population, no other immunodeficiency was demonstrated in our patient. In vitro resistance to acyclovir was tested negative, but despite prophylactic treatment with the drug in high doses, relapses recurred. To our knowledge, this is the first ever reported documentation of relapsing-remitting facial and brachial plexus neuritis caused by HSV-1.

  12. Some Properties of Fuzzy Soft Proximity Spaces

    PubMed Central

    Demir, İzzettin; Özbakır, Oya Bedre

    2015-01-01

    We study the fuzzy soft proximity spaces in Katsaras's sense. First, we show how a fuzzy soft topology is derived from a fuzzy soft proximity. Also, we define the notion of fuzzy soft δ-neighborhood in the fuzzy soft proximity space which offers an alternative approach to the study of fuzzy soft proximity spaces. Later, we obtain the initial fuzzy soft proximity determined by a family of fuzzy soft proximities. Finally, we investigate relationship between fuzzy soft proximities and proximities. PMID:25793224

  13. Some properties of fuzzy soft proximity spaces.

    PubMed

    Demir, İzzettin; Özbakır, Oya Bedre

    2015-01-01

    We study the fuzzy soft proximity spaces in Katsaras's sense. First, we show how a fuzzy soft topology is derived from a fuzzy soft proximity. Also, we define the notion of fuzzy soft δ-neighborhood in the fuzzy soft proximity space which offers an alternative approach to the study of fuzzy soft proximity spaces. Later, we obtain the initial fuzzy soft proximity determined by a family of fuzzy soft proximities. Finally, we investigate relationship between fuzzy soft proximities and proximities.

  14. 76 FR 78823 - Schedule for Rating Disabilities; Evaluation of Amyotrophic Lateral Sclerosis

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-12-20

    ... Sclerosis AGENCY: Department of Veterans Affairs. ACTION: Final rule. SUMMARY: The Department of Veterans... criterion provided for amyotrophic lateral sclerosis (ALS) to provide an evaluation of 100 percent for any... revise the evaluation criterion for amyotrophic lateral sclerosis (ALS) in the VA Schedule for...

  15. Brachioradial arteries with anastomotic arteries connecting to brachial arteries bilaterally.

    PubMed

    Hong, Tong; Qiuhong, Dan; Haipeng, Cai

    2010-01-01

    We present a patient with a failed radial coronary angioplasty as a result of bilateral brachioradial arteries, the radial arteries anomalously originating from the axillary arteries. We review the literature concerning abnormal origins of the radial artery and propose the left ulnar artery as optimal access of choice in cases with a right brachioradial artery of relatively small size in its proximal part.

  16. Constraint-Induced Movement Therapy for Children with Obstetric Brachial Plexus Palsy: Two Single-Case Series

    ERIC Educational Resources Information Center

    Buesch, Francisca Eugster

    2010-01-01

    The objective of this pilot study was to investigate the feasibility of constraint-induced movement therapy (CIMT) in children with obstetric brachial plexus palsy and receive preliminary information about functional improvements. Two patients (age 12 years) with obstetric brachial plexus palsy were included for a 126-h home-based CIMT…

  17. Hand Function in Children with an Upper Brachial Plexus Birth Injury: Results of the Nine-Hole Peg Test

    ERIC Educational Resources Information Center

    Immerman, Igor; Alfonso, Daniel T.; Ramos, Lorna E.; Grossman, Leslie A.; Alfonso, Israel; Ditaranto, Patricia; Grossman, John A. I.

    2012-01-01

    Aim: The aim of this study was to evaluate hand function in children with Erb upper brachial plexus palsy. Method: Hand function was evaluated in 25 children (eight males; 17 females) with a diagnosed upper (C5/C6) brachial plexus birth injury. Of these children, 22 had undergone primary nerve reconstruction and 13 of the 25 had undergone…

  18. Characteristic features of hereditary neuropathy with liability to pressure palsy (HNPP) presenting with brachial plexopathy in soldiers.

    PubMed

    Kim, Kyoung-Eun

    2014-11-15

    A brachial plexus lesion is not common in hereditary neuropathy with liability to pressure palsy (HNPP). We report the clinical and electrodiagnostic features of young soldiers with HNPP presenting with brachial plexopathy. By reviewing 2year medical records from Korean military hospitals, we identified soldiers with brachial plexus lesions. Among them, patients diagnosed with HNPP were determined and clinical and electrophysiological findings were compared between HNPP and non-HNPP patients with a brachial plexus lesion. Thirteen patients (6.8%) were diagnosed with HNPP among 189 patients with a brachial plexus lesion. Push-ups, as either a punishment or an exercise, was the most frequent preceding event in HNPP patients (76.9%), whereas it was rare in non-HNPP patients. The distal motor latency of the median nerve showed the highest sensitivity (90.9%) and specificity (100%) for HNPP in patients with a brachial plexus lesion. In conclusion, HNPP should be suspected in patients with brachial plexopathy if brachial plexopathy develops after push-ups or if the distal motor latency of median nerves is prolonged.

  19. Association between alcohol consumption and amyotrophic lateral sclerosis: a meta-analysis of five observational studies.

    PubMed

    E, Meng; Yu, Sufang; Dou, Jianrui; Jin, Wu; Cai, Xiang; Mao, Yiyang; Zhu, Daojian; Yang, Rumei

    2016-08-01

    The purpose of this study is to examine the association between alcohol consumption and amyotrophic lateral sclerosis. Published literature on the association between alcohol consumption and amyotrophic lateral sclerosis was retrieved from the PubMed and Embase databases. Two authors independently extracted the data. The quality of the identified studies was evaluated according to the Newcastle-Ottawa scale. Subgroup and sensitivity analyses were performed and publication bias was assessed. Five articles, including one cohort study and seven case-control studies, and a total of 431,943 participants, were identified. The odds ratio for the association between alcohol consumption and amyotrophic lateral sclerosis was 0.57 (95 % confidence interval 0.51-0.64). Subgroup and sensitivity analyses confirmed the result. Evidence for publication bias was detected. Alcohol consumption reduced the risk of developing amyotrophic lateral sclerosis compared with non-drinking. Alcohol, therefore, has a potentially neuroprotective effect on the development of amyotrophic lateral sclerosis.

  20. Effect of Addition of Fentanyl to Xylocaine Hydrochloride in Brachial Plexus Block by Supraclavicular Approach

    PubMed Central

    Paluvadi, Venkata Raghavendra; Manne, Venkata Sesha Sai Krishna

    2017-01-01

    Aim: This study was designed to quantitatively compare the effects of 1.5% xylocaine with 1.5% xylocaine and fentanyl (1 μg/kg) mixture for supraclavicular brachial plexus block. Materials and Methods: Sixty patients between the age group of 20–60 and scheduled for upper limb surgery were divided into two groups (xylocaine group and xylocaine plus fentanyl group). After performing supraclavicular brachial plexus block, an assessment was made for onset of analgesia, duration and degree of analgesia, block intensity, and for any other side effects. Results: Mean duration of analgesia is Group I is 2.1 h and in Group II is 8.1 h; a four-fold increase in duration of analgesia. Conclusion: Addition of fentanyl to xylocaine for supraclavicular brachial plexus block has no significant effect on onset or quality of analgesia, but duration of analgesia is significantly prolonged. PMID:28298769

  1. Different Learning Curves for Axillary Brachial Plexus Block: Ultrasound Guidance versus Nerve Stimulation

    PubMed Central

    Luyet, C.; Schüpfer, G.; Wipfli, M.; Greif, R.; Luginbühl, M.; Eichenberger, U.

    2010-01-01

    Little is known about the learning of the skills needed to perform ultrasound- or nerve stimulator-guided peripheral nerve blocks. The aim of this study was to compare the learning curves of residents trained in ultrasound guidance versus residents trained in nerve stimulation for axillary brachial plexus block. Ten residents with no previous experience with using ultrasound received ultrasound training and another ten residents with no previous experience with using nerve stimulation received nerve stimulation training. The novices' learning curves were generated by retrospective data analysis out of our electronic anaesthesia database. Individual success rates were pooled, and the institutional learning curve was calculated using a bootstrapping technique in combination with a Monte Carlo simulation procedure. The skills required to perform successful ultrasound-guided axillary brachial plexus block can be learnt faster and lead to a higher final success rate compared to nerve stimulator-guided axillary brachial plexus block. PMID:21318138

  2. Does C5 or C6 Radiculopathy Affect the Signal Intensity of the Brachial Plexus on Magnetic Resonance Neurography?

    PubMed Central

    Seo, Tae Gyu; Kim, In-Soo; Son, Eun Seok

    2016-01-01

    Patients with C5 or C6 radiculopathy complain of shoulder area pain or shoulder girdle weakness. Typical idiopathic neuralgic amyotrophy (INA) is also characterized by severe shoulder pain, followed by paresis of shoulder girdle muscles. Recent studies have demonstrated that magnetic resonance neurography (MRN) of the brachial plexus and magnetic resonance imaging (MRI) of the shoulder in patients with INA show high signal intensity (HSI) or thickening of the brachial plexus and changes in intramuscular denervation of the shoulder girdle. We evaluated the value of brachial plexus MRN and shoulder MRI in four patients with typical C5 or C6 radiculopathy. HSI of the brachial plexus was noted in all patients and intramuscular changes were observed in two patients who had symptoms over 4 weeks. Our results suggest that HSI or thickening of the brachial plexus and changes in intramuscular denervation of the shoulder girdle on MRN and MRI may not be specific for INA. PMID:27152289

  3. Epithelioid hemangioma of brachial artery: report of a case and review of the literature

    PubMed Central

    Moira, Ragazzi; Giuseppe, Falco; Riccardo, Valli; Nicola, Rocco; Daniele, Bordoni; Pierfrancesco, Cadenelli; Antonio, Della Corte Gianni; Antonello, Accurso; Bruno, Amato; Giovanni, Casali; Guglielmo, Ferrari

    2015-01-01

    Epithelioid hemangioma (EH) is an uncommon benign vascular lesion, also known as angioblastic lymphoid (or angiolymphoid) hyperplasia with eosinophilia, characterized by an unclear etiopathogenesis. It usually affects young to middle-aged adults and develops in the head and neck region, as painless cutaneous or subcutaneous reddish papules or nodules. Large vessels involvement is extremely rare, and to date only two cases affecting the brachial artery have been cited in literature. In this report we present a further case of EH of the brachial artery and review the pertinent literature. PMID:28352744

  4. Hereditary Neuropathy with Liability to Pressure Palsy Presenting as an Acute Brachial Plexopathy: A Lover's Palsy

    PubMed Central

    Wedderburn, Sarah; Pateria, Puraskar; Panegyres, Peter K.

    2014-01-01

    It is generally regarded that patients with hereditary neuropathy to pressure palsies, due to a deletion in the PMP22 gene, show recurrent pressure palsy and generalised peripheral neuropathy (pes cavus and hammer toes sometimes develop). Brachial plexopathy is rarely identified as a first presentation of hereditary neuropathy to pressure palsies. We describe a young man who developed a painless flail upper limb with a clinical diagnosis of a brachial plexopathy after his partner slept on his arm – a PMP22 deletion was found. His father, who had a symmetrical polyneuropathy without recurrent mononeuropathies, shared the PMP22 deletion. PMID:25685136

  5. [Anesthetic management of a patient with amyotrophic lateral sclerosis].

    PubMed

    Mashio, H; Ito, Y; Yanagita, Y; Fujisawa, E; Hada, K; Goda, Y; Kawahigashi, H

    2000-02-01

    A 49-year-old male with amyotrophic lateral sclerosis (ALS) was scheduled for gastrectomy. Anesthetic management was performed under general anesthesia with sevoflurane and epidural anesthesia with lidocaine. He showed increased response to vecuronium under monitoring of neuromuscular block. But he responded favorably to anticholineesterase. He had little pain and showed no progress in neurological symptoms in the postoperative period. Neuromuscular monitoring is essential in administrating non-depolarizing neuromuscular blocking agents to patients with ALS, and epidural anesthesia may be useful for perioperative management of patients with ALS.

  6. [Amyotrophic lateral sclerosis in totally locked-in state].

    PubMed

    Oyanagi, Kiyomitsu; Mochizuki, Yoko; Nakayama, Yuki; Hayashi, Kentaro; Shimizu, Toshio; Nagao, Masahiro; Hashimoto, Tomoyo; Yamazaki, Mineo; Matsubara, Shiro; Komori, Takashi

    2013-01-01

    Seven autopsy patients with amyotrophic lateral sclerosis (ALS) in totally locked-in state (TLS) were examined neuropathologically. The patients were composed of 4 males and 3 females, and 3 with familial, 1 sporadic but with mutation in SOD1 gene, and 3 sporadic patients with unremarkable gene mutation. The brains weighed 715, 783, 1,019, 1,050, 1,170, 1,190 or 1,233 g. The tegmentum of the brain stem was markedly degenerated in every patient, and the tracts relating to the somatic sensory and auditory were involved in the lesions.

  7. An Autopsy Case of Amyotrophic Lateral Sclerosis with Diaphragm Pacing

    PubMed Central

    Ito, Hisashi; Kamei, Tetsumasa; Odake, Sanae; Nakano, Masayuki; Okeda, Riki; Kohriki, Shunsaku; Kawachi, Jun; Onders, Raymond P.; Yoshii, Fumihito

    2016-01-01

    Respiratory insufficiency is a critical problem in amyotrophic lateral sclerosis (ALS) patients. We herein present the case of an autopsied patient with sporadic ALS who underwent diaphragm pacing (DP). The pathology showed several localized adhesions with a markedly atrophied diaphragm. A marked loss of motor neurons with Bunina bodies and phosphorylated TDP-43 positive inclusions was found in the spinal cord and primary motor cortex. Mild hyalinization and a few multinucleated giant cells were present around the electrode tracks in the diaphragm. However, no infiltration of inflammatory cells was detected. Our findings suggest that full-time DP might not cause severe damage to adjacent diaphragm tissue. PMID:27904119

  8. Neuropathology of Amyotrophic Lateral Sclerosis and Its Variants.

    PubMed

    Saberi, Shahram; Stauffer, Jennifer E; Schulte, Derek J; Ravits, John

    2015-11-01

    The neuropathologic molecular signature common to almost all sporadic amyotrophic lateral sclerosis (ALS) and most familial ALS is TDP-43 immunoreactive neuronal cytoplasmic inclusions. The neuropathologic and molecular neuropathologic features of ALS variants, primarily lateral sclerosis and progressive muscular atrophy, are less certain but also seem to share the primary features of ALS. Genetic causes, including mutations in SOD1, TDP-43, FUS, and C9orf72, all have distinctive molecular neuropathologic signatures. Neuropathology will continue to play an increasingly key role in solving the puzzle of ALS pathogenesis.

  9. Ensuring continued progress in biomarkers for amyotrophic lateral sclerosis

    PubMed Central

    Turner, Martin R; Benatar, Michael

    2015-01-01

    Multiple candidate biomarkers for amyotrophic lateral sclerosis (ALS) have emerged across a range of platforms. Replication of results, however, has been absent in all but a few cases, and the range of control samples has been limited. If progress toward clinical translation is to continue, the specific biomarker needs of ALS, which differ from those of other neurodegenerative disorders, as well as the challenges inherent to longitudinal ALS biomarker cohorts, must be understood. Appropriate application of multimodal approaches, international collaboration, presymptomatic studies, and biomarker integration into future therapeutic trials are among the essential priorities going forward. PMID:25288265

  10. An Autopsy Case of Amyotrophic Lateral Sclerosis with Diaphragm Pacing.

    PubMed

    Ito, Hisashi; Kamei, Tetsumasa; Odake, Sanae; Nakano, Masayuki; Okeda, Riki; Kohriki, Shunsaku; Kawachi, Jun; Onders, Raymond P; Yoshii, Fumihito

    Respiratory insufficiency is a critical problem in amyotrophic lateral sclerosis (ALS) patients. We herein present the case of an autopsied patient with sporadic ALS who underwent diaphragm pacing (DP). The pathology showed several localized adhesions with a markedly atrophied diaphragm. A marked loss of motor neurons with Bunina bodies and phosphorylated TDP-43 positive inclusions was found in the spinal cord and primary motor cortex. Mild hyalinization and a few multinucleated giant cells were present around the electrode tracks in the diaphragm. However, no infiltration of inflammatory cells was detected. Our findings suggest that full-time DP might not cause severe damage to adjacent diaphragm tissue.

  11. A patient with amyotrophic lateral sclerosis and atypical clinical and electrodiagnostic features: a case report

    PubMed Central

    2011-01-01

    Introduction Amyotrophic lateral sclerosis is a rapidly progressive, fatal neurodegenerative disorder for which there is no effective treatment. The diagnosis is dependent on the clinical presentation and consistent electrodiagnostic studies. Typically, there is a combination of upper and lower motor neuron signs as well as electrodiagnostic studies indicative of diffuse motor axonal injury. The presentation of amyotrophic lateral sclerosis, however, may be variable. At the same time, the diagnosis is essential for patient prognosis and management. It is therefore important to appreciate the range of possible presentations of amyotrophic lateral sclerosis. Case presentation We present the case of a 57-year-old Caucasian man with pathological findings on postmortem examination consistent with amyotrophic lateral sclerosis but atypical clinical and electrodiagnostic features. He died after a rapid course of progressive weakness. The patient did not respond to immunosuppressive therapy. Conclusion Amyotrophic lateral sclerosis should be considered in patients with a rapidly progressive, unexplained neuropathic process. This should be true even if there are atypical clinical and electrodiagnostic findings. Absence of response to therapy and the development of upper motor neuron signs should reinforce the possibility that amyotrophic lateral sclerosis may be present. Since amyotrophic lateral sclerosis is a fatal illness, however, the possibility of this disease in patients with atypical clinical features should not diminish the need for a thorough diagnostic evaluation and treatment trials. PMID:22047468

  12. Chitotriosidase - a putative biomarker for sporadic amyotrophic lateral sclerosis

    PubMed Central

    2013-01-01

    Background Potential biomarkers to aid diagnosis and therapy need to be identified for Amyotrophic Lateral Sclerosis, a progressive motor neuronal degenerative disorder. The present study was designed to identify the factor(s) which are differentially expressed in the cerebrospinal fluid (CSF) of patients with sporadic amyotrophic lateral sclerosis (SALS; ALS-CSF), and could be associated with the pathogenesis of this disease. Results Quantitative mass spectrometry of ALS-CSF and control-CSF (from orthopaedic surgical patients undergoing spinal anaesthesia) samples showed upregulation of 31 proteins in the ALS-CSF, amongst which a ten-fold increase in the levels of chitotriosidase-1 (CHIT-1) was seen compared to the controls. A seventeen-fold increase in the CHIT-1 levels was detected by ELISA, while a ten-fold elevated enzyme activity was also observed. Both these results confirmed the finding of LC-MS/MS. CHIT-1 was found to be expressed by the Iba-1 immunopositive microglia. Conclusion Elevated CHIT-1 levels in the ALS-CSF suggest a definitive role for the enzyme in the disease pathogenesis. Its synthesis and release from microglia into the CSF may be an aligned event of neurodegeneration. Thus, high levels of CHIT-1 signify enhanced microglial activity which may exacerbate the process of neurodegeneration. In view of the multifold increase observed in ALS-CSF, it can serve as a potential CSF biomarker for the diagnosis of SALS. PMID:24295388

  13. Anthropometry of Arm: Nutritional Risk Indicator in Amyotrophic Lateral Sclerosis.

    PubMed

    Salvioni, Cristina Cleide Dos Santos; Stanich, Patricia; Oliveira, Acary Souza Bulle; Orsini, Marco

    2015-12-29

    The aim of the paper is to examine the correlation between clinical data, nutritional, respiratory and functional parameters in amyotrophic lateral sclerosis (ALS). This is a descriptive study of 111 ALS patients [91 spinal onset (GS) and 20 bulbar onset (GB)] carried on using nutritional and respiratory parameters and amyotrophic lateral sclerosis functional rating scale (ALSFRS). ALSFRS was analyzed in the main domains (D1, D2 and D3). Forced vital capacity and anthropometric measurements, there was significant association for GS and GB, and in GS there was positive correlation with midarm circumference (MAC) (r=0.30; P=0.020), midarm muscle circumference (r=0.29; P=0.026), arm muscle area (r=0.28; P=0.033) and protein-caloric malnutrition score (r=0.27; P=0.039), while for GB only with body weight (r=0.64; P=0.024). On correlation of nutritional parameters and ALSFRS for GS patients we observed that MAC and %MAC presented positive association with both issues of D1 and D2. For GB, the total score in addition to correlate positively with anthropometric parameters related to lean body mass also presented negative association with a parameter associated with body fat. In summary, it is suggested that the application of anthropometry of arm could be useful in routine monitoring of ALS patients.

  14. Case-control study of amyotrophic lateral sclerosis

    SciTech Connect

    Deapen, D.M.; Henderson, B.E.

    1986-05-01

    The authors conducted a study of 518 amyotrophic lateral sclerosis patients identified between 1977 and 1979 and 518 controls to investigate putative risk factors for this disease. Occupations at risk of electrical exposure were reported more often by patients (odds ratio (OR) = 3.8, 95% confidence interval (CI) = 1.4-13.0) as were electrical shocks producing unconsciousness (OR = 2.8, 95% CI = 1.0-9.9). Although an overall excess of physical trauma associated with unconsciousness was observed in the amyotrophic lateral sclerosis patients (OR = 1.6, 95% CI = 1.0-2.4), the effect was inversely associated with duration of the unconscious episodes, suggesting an effect of recall bias. Only slight differences were found for surgical traumata to the nervous system. Parkinsonism was reported more often among first degree relatives of cases (OR = 2.7, 95% CI = 1.1-7.6). The frequencies of prior poliomyelitis or other central nervous system diseases were similar for patients and controls. Occupational exposure to selected toxic substances was similar for patients and controls except for the manufacture of plastics (OR = 3.7, 95% CI = 1.0-20.5), although few details of these exposures were provided. No differences in occupations with exposure to animal skins or hides were observed.

  15. Anthropometry of Arm: Nutritional Risk Indicator in Amyotrophic Lateral Sclerosis

    PubMed Central

    Stanich, Patricia; Oliveira, Acary Souza Bulle; Orsini, Marco

    2015-01-01

    The aim of the paper is to examine the correlation between clinical data, nutritional, respiratory and functional parameters in amyotrophic lateral sclerosis (ALS). This is a descriptive study of 111 ALS patients [91 spinal onset (GS) and 20 bulbar onset (GB)] carried on using nutritional and respiratory parameters and amyotrophic lateral sclerosis functional rating scale (ALSFRS). ALSFRS was analyzed in the main domains (D1, D2 and D3). Forced vital capacity and anthropometric measurements, there was significant association for GS and GB, and in GS there was positive correlation with midarm circumference (MAC) (r=0.30; P=0.020), midarm muscle circumference (r=0.29; P=0.026), arm muscle area (r=0.28; P=0.033) and protein-caloric malnutrition score (r=0.27; P=0.039), while for GB only with body weight (r=0.64; P=0.024). On correlation of nutritional parameters and ALSFRS for GS patients we observed that MAC and %MAC presented positive association with both issues of D1 and D2. For GB, the total score in addition to correlate positively with anthropometric parameters related to lean body mass also presented negative association with a parameter associated with body fat. In summary, it is suggested that the application of anthropometry of arm could be useful in routine monitoring of ALS patients. PMID:26788263

  16. Is SOD1 loss of function involved in amyotrophic lateral sclerosis?

    PubMed

    Saccon, Rachele A; Bunton-Stasyshyn, Rosie K A; Fisher, Elizabeth M C; Fratta, Pietro

    2013-08-01

    Mutations in the gene superoxide dismutase 1 (SOD1) are causative for familial forms of the neurodegenerative disease amyotrophic lateral sclerosis. When the first SOD1 mutations were identified they were postulated to give rise to amyotrophic lateral sclerosis through a loss of function mechanism, but experimental data soon showed that the disease arises from a--still unknown--toxic gain of function, and the possibility that loss of function plays a role in amyotrophic lateral sclerosis pathogenesis was abandoned. Although loss of function is not causative for amyotrophic lateral sclerosis, here we re-examine two decades of evidence regarding whether loss of function may play a modifying role in SOD1-amyotrophic lateral sclerosis. From analysing published data from patients with SOD1-amyotrophic lateral sclerosis, we find a marked loss of SOD1 enzyme activity arising from almost all mutations. We continue to examine functional data from all Sod1 knockout mice and we find obvious detrimental effects within the nervous system with, interestingly, some specificity for the motor system. Here, we bring together historical and recent experimental findings to conclude that there is a possibility that SOD1 loss of function may play a modifying role in amyotrophic lateral sclerosis. This likelihood has implications for some current therapies aimed at knocking down the level of mutant protein in patients with SOD1-amyotrophic lateral sclerosis. Finally, the wide-ranging phenotypes that result from loss of function indicate that SOD1 gene sequences should be screened in diseases other than amyotrophic lateral sclerosis.

  17. Radiation-induced brachial plexopathy: Neurological follow-up in 161 recurrence-free breast cancer patients

    SciTech Connect

    Olsen, N.K.; Pfeiffer, P.; Johannsen, L.; Schroder, H.; Rose, C. )

    1993-04-30

    The purpose was to assess the incidence and clinical manifestations of radiation-induced brachial plexopathy in breast cancer patients, treated according to the Danish Breast Cancer Cooperative Group protocols. One hundred and sixty-one recurrence-free breast cancer patients were examined for radiation-induced brachial plexopathy after a median follow-up period of 50 months (13-99 months). After total mastectomy and axillary node sampling, high-risk patients were randomized to adjuvant therapy. One hundred twenty-eight patients were treated with postoperative radiotherapy with 50 Gy in 25 daily fractions over 5 weeks. In addition, 82 of these patients received cytotoxic therapy (cyclophosphamide, methotrexate, and 5-fluorouracil) and 46 received tamoxifen. Five percent and 9% of the patients receiving radiotherapy had disabling and mild radiation-induced brachial plexopathy, respectively. Radiation-induced brachial plexopathy was more frequent in patients receiving cytotoxic therapy (p = 0.04) and in younger patients (p = 0.04). The clinical manifestations were paraesthesia (100%), hypaesthesia (74%), weakness (58%), decreased muscle stretch reflexes (47%), and pain (47%). The brachial plexus is more vulnerable to large fraction size. Fractions of 2 Gy or less are advisable. Cytotoxic therapy adds to the damaging effect of radiotherapy. Peripheral nerves in younger patients seems more vulnerable. Radiation-induced brachial plexopathy occurs mainly as diffuse damage to the brachial plexus. 24 refs., 9 tabs.

  18. Luxation de l’épaule compliquée de paralysie du plexus brachial

    PubMed Central

    Lukulunga, Loubet Unyendje; Moussa, Abdou Kadri; Mahfoud, Mustapha; EL Bardouni, Ahmed; Berrada, Mohamed Saleh; El Yaacoubi, Moradh

    2014-01-01

    Les auteurs rapportent l'observation d'une paralysie totale du plexus brachial survenue trois mois après un épisode de luxation antéro-interne sous coracoïdienne associée à une fracture du trochiter chez une patiente âgée de 88 ans. PMID:25426187

  19. Cost analysis of brachial plexus injuries: variability of compensation by insurance companies before and after surgery.

    PubMed

    Felici, N; Zaami, S; Ciancolini, G; Marinelli, E; Tagliente, D; Cannatà, C

    2014-04-01

    Traumatic paralysis of the brachial plexus is an extremely disabling pathology. The type of trauma most frequently suffered by this group of patients is due to motorcycle injuries. It therefore affects a population of young patients. In the majority of cases, these patients receive compensation for permanent damage from insurance companies. Surgery of the brachial plexus enables various forms of functional recovery, depending on the number of roots of the brachial plexus involved in the injury. The aim of this study is to compare the functional deficit and the extent of the related compensation before and after surgical intervention, and to evaluate the saving in economic terms (understood as the cost of compensation paid by insurance companies) obtainable through surgical intervention. The authors analysed the functional recovery obtained through surgery in 134 patients divided into 4 groups on the basis of the number of injured roots. The levels of compensation payable to the patient before surgical intervention, and 3 years after, were then compared. The results showed that the saving obtainable through surgical treatment of brachial plexus injuries may exceed 65% of the economic value of the compensation that would have been attributable to the same patients if they had not undergone surgical treatment.

  20. Mononeuritis multiplex with brachial plexus neuropathy coincident with Mycoplasma pneumoniae infection.

    PubMed

    Kidron, D; Barron, S A; Mazliah, J

    1989-01-01

    Mycoplasma pneumoniae infection has been associated with a variety of neurologic complications involving the central nervous system, the peripheral nervous system and muscle. We present a patient who developed a previously unreported complication: mononeuritis multiplex. This consisted of a severe brachial plexus neuropathy with contralateral cervical monoradiculopathy.

  1. A young man with intimomedial mucoid degeneration of the brachial artery.

    PubMed

    Raber, Menno H; Meerwaldt, Robbert; van Det, Rob J

    2011-03-01

    Intimomedial mucoid degeneration is a rare disorder and has been described as a distinctly different entity from Erdheim's cystic medial necrosis. Most studies show a strong predominance in African American females with hypertension. In our case report, we describe the presence of a large brachial aneurysm in a young white male with intimomedial mucoid degeneration.

  2. Robot-Assisted Surgery of the Shoulder Girdle and Brachial Plexus

    PubMed Central

    Facca, Sybille; Hendriks, Sarah; Mantovani, Gustavo; Selber, Jesse C.; Liverneaux, Philippe

    2014-01-01

    New developments in the surgery of the brachial plexus include the use of less invasive surgical approaches and more precise techniques. The theoretical advantages of the use of robotics versus endoscopy are the disappearance of physiological tremor, three-dimensional vision, high definition, magnification, and superior ergonomics. On a fresh cadaver, a dissection space was created and maintained by insufflation of CO2. The supraclavicular brachial plexus was dissected using the da Vinci robot (Intuitive Surgical, Sunnyvale, CA). A segment of the C5 nerve root was grafted robotically. A series of eight clinical cases of nerve damage around the shoulder girdle were operated on using the da Vinci robot. The ability to perform successful microneural repair was confirmed in both the authors' clinical and experimental studies, but the entire potential of robotically assisted microneural surgery was not realized during these initial cases because an open incision was still required. Robotic-assisted surgery of the shoulder girdle and brachial plexus is still in its early stages. It would be ideal to have even finer and more suitable instruments to apply fibrin glue or electrostimulation in nerve surgery. Nevertheless, the prospects of minimally invasive techniques would allow acute and subacute surgical approach of traumatic brachial plexus palsy safely, without significant and cicatricial morbidity. PMID:24872778

  3. Bilateral transit time assessment of upper and lower limbs as a surrogate ankle brachial index marker.

    PubMed

    Foo, Jong Yong Abdiel

    2008-01-01

    Ankle brachial index is useful in monitoring the pathogenesis of peripheral arterial occlusive diseases. Sphygmomanometer is the standard instrument widely used but frequent prolonged monitoring can be less comfortable for patients. Pulse transit time is known to be inversely correlated with blood pressure and a ratio-based pulse transit time measurement has been proposed as a surrogate ankle brachial index marker. In this study, 17 normotensive adults (9 men; aged 25.4 +/- 3.9 years) were recruited. Two postural change test activities were performed to induce changes in the stiffness of the arterial wall of the moved periphery. Results showed that only readings from the limbs that adopted a new posture registered significant blood pressure and pulse transit time changes (P < .05). Furthermore, there was significant correlation between the ankle brachial index and pulse transit time ratio measure for both test activities (R(2) > or = 0.704). The findings herein suggest that pulse transit time ratio is a surrogate and accommodating ankle brachial index marker.

  4. Brachial plexus compression due to subclavian artery pseudoaneurysm from internal jugular vein catheterization

    PubMed Central

    Mol, T. N.; Gupta, A.; Narain, U.

    2017-01-01

    Internal jugular vein (IJV) catheterization has become the preferred approach for temporary vascular access for hemodialysis. However, complications such as internal carotid artery puncture, vessel erosion, thrombosis, and infection may occur. We report a case of brachial plexus palsy due to compression by right subclavian artery pseudoaneurysm as a result of IJV catheterization in a patient who was under maintenance hemodialysis. PMID:28356671

  5. Digital infarction in a hemodialysis patient due to embolism from a thrombosed brachial arteriovenous fistula.

    PubMed

    Yj, Anupama

    2015-10-01

    Acute onset of digital ischemia and infarction is an unusual complication in patients undergoing hemodialysis. This is a report of a patient on regular hemodialysis who presented with acute distal extremity ischemia, progressing to digital infarction and on evaluation was found to have thrombosis of brachial arteriovenous fistula with embolization to the distal arteries causing digital artery occlusion.

  6. Analysis of the neurofilament heavy subunit (NFH) gene in familial amyotrophic lateral sclerosis

    SciTech Connect

    Rooke, K.; Rouleau, G.A.; Figlewicz, D.A.

    1994-09-01

    Amyotrophic lateral sclerosis (ALS) is a fatal, adult-onset, degenerative disorder of the motor neurons in the cortex, brainstem and spinal cord. Approximately 10% of ALS cases are familial (FALS) and are inherited as an age-dependent autosomal dominant trait. Mutations in the Cu/Zn superoxide dismutase (SOD-1) gene on chromosome 21 have been found in a subset of cases. However, for the remaining FALS cases, the etiology is unknown. The abnormal accumulation of neurofilaments in the cell body and proximal axon of motor neurons is a characteristic pathological finding in ALS. Furthermore, aberrant neuronal swellings that closely resemble those found in ALS have been reported in transgenic mice overexpressing NFH. The C-terminal region of NFH contains a unique functional domain with multiple repeats of the amino acids (Lys-Ser-Pro) (KSP) and forms the side-arms which appear, at the level of electron microscopy, to cross-link neurofilaments. Recently, deletions in the DSP repeat domain have been identified in five ALS patients diagnosed as sporadic cases of the disease. Based on these findings, we propose to analyze all 4 exons of the NFH gene for variation in FALS. DNA from 110 FALS cases has been amplified by the polymerase chain reaction (PCR) and analyzed by single strand conformation polymorphism (SSCP) analysis. Exon 2, exon 3 and the KSP repeat domain (part of exon 4) appear normal in all our FALS individuals under several different SSCP conditions. The analysis of exon 1 and the remainder of exon 4 has yet to be completed.

  7. Hirayama Disease with Proximal Involvement

    PubMed Central

    2016-01-01

    Hirayama disease is a slowly progressing benign motor neuron disease that affects the distal upper limb. A 29-year-old man visited the hospital with a 1-year history of weakened left proximal upper limb. He was diagnosed with Hirayama disease 9 years ago, while there was no further progression of the muscle weakness afterward. Atrophy and weakness was detected in proximal upper limb muscles. Magnetic resonance imaging and somatosensory evoked potentials were normal. Needle electromyography showed abnormal findings in proximal upper limb muscles. Our patient had Hirayama disease involving the proximal portion through secondary progression. Clinical manifestation and accurate electromyography may be useful for diagnosis. Rare cases with progression patterns as described here are helpful and have clinical meaning for clinicians. PMID:27550499

  8. Tools for proximal soil sensing

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Proximal soil sensing (i.e. near-surface geophysical methods) are used to study soil phenomena across spatial scales. Geophysical methods exploit contrasts in physical properties (dielectric permittivity, apparent electrical conductivity or resistivity, magnetic susceptibility) to indirectly measur...

  9. The impact of handgrip exercise duty cycle on brachial artery flow-mediated dilation.

    PubMed

    King, Trevor J; Slattery, David J; Pyke, Kyra E

    2013-07-01

    Endothelial function is essential for vasoprotection and regulation of vascular tone. Using handgrip exercise (HGEX) to increase blood flow-associated shear stress is an increasingly popular method for assessing brachial artery endothelial function via flow-mediated dilation (FMD). However, different exercise duty cycles [ratio of handgrip relaxation: contraction (seconds)] produce different patterns of brachial artery shear stress with distinct antegrade/retrograde magnitudes. To determine the impact of HGEX duty cycle on brachial artery %FMD, three distinct duty cycles were employed while maintaining a uniform mean shear stress. Brachial artery diameter and mean blood velocity were assessed via echo and Doppler ultrasound in 16 healthy male subjects. Shear stress was estimated as shear rate (SR = blood velocity/brachial artery diameter) and the target mean SR during HGEX was 75 s(-1). Subjects performed three 6-min HGEX trials on each of 2 days (like trials averaged). In each trial, subjects performed one of the three randomly ordered HGEX duty cycles (1:1, 3:1, 5:1). %FMD was calculated from baseline to the end of HGEX and (subset N = 10) during each minute of HGEX. Data are mean ± SD. As intended, mean SR was uniform across duty cycles (6 min HGEX average: 72.9 ± 4.9s(-1), 72.6 ± 3.6s(-1), 72.8 ± 3.5 s(-1), p = 0.835), despite differences in antegrade/retrograde SR (p < 0.001). End-exercise %FMD (4.0 ± 1.3 %, 4.1 ± 2.2 %, 4.2 ± 1.4 %, p = 0.860) and %FMD during exercise (p = 0.939) were not different between duty cycles. These data indicate that the endothelium responds to the mean shear stress and is not specifically sensitive to the contraction/relaxation or retrograde shear stress created by a range of HGEX protocols.

  10. Application of botulinum toxin to treat sialorrhea in amyotrophic lateral sclerosis patients: a literature review

    PubMed Central

    de Oliveira, Ademar Francisco; Silva, Gêssyca Adryene de Menezes; Almeida, Débora Milenna Xavier

    2016-01-01

    ABSTRACT Amyotrophic lateral sclerosis is a progressive and fatal neurodegenerative disease characterized by the degeneration of motor neurons, which are the central nervous system cells that control voluntary muscle movements. The excessive salivation (sialorrhea) is present in approximately 50% of amyotrophic lateral sclerosis cases. Thus, some alternative therapeutic methods are sought, such as anticholinergic drugs and surgery. Recently the use of botulinum toxin applied at a midpoint of the salivary glands, often guided by ultrasound, have demonstrated positive results. The objective was to review the literature to demonstrate an alternative method to treatments of sialorrhea in patients with amyotrophic lateral sclerosis. In recent studies, the efficacy of botulinum toxin is confirmed, although new applications are required. Since the side effects are negligible, this is an alternative to treat amyotrophic lateral sclerosis, and other patients with diseases that present sialorrhea. PMID:27759834

  11. Radiation dose to the brachial plexus in nasopharyngeal carcinoma treated with intensity-modulated radiation therapy: An increased risk of an excessive dose to the brachial plexus adjacent to gross nodal disease

    PubMed Central

    FENG, GUOSHENG; LU, HEMING; LIANG, YUAN; CHEN, HUASHENG; SHU, LIUYANG; LU, SHUI; ZHU, JIANFANG; GAO, WEIWEI

    2012-01-01

    This retrospective study aimed to evaluate the dose to the brachial plexus in patients with nasopharyngeal carcinoma (NPC) treated with intensity-modulated radiation therapy (IMRT). Twenty-eight patients were selected and the brachial plexus was delineated retrospectively. Brachial plexus adjacent/not adjacent to nodes were defined and abbreviated as BPAN and BPNAN, respectively. Dose distribution was recalculated and a dose-volume histogram was generated based on the original treatment plan. The maximum dose to the left brachial plexus was 59.12–78.47 Gy, and the percentage of patients receiving the maximum dose exceeding 60, 66 and 70 Gy was 96.4, 57.1 and 25.0%, respectively; the maximum dose to the right brachial plexus was 59.74–80.31 Gy, and the percentage of patients exposed to a maximum dose exceeding 60, 66 and 70 Gy was 96.4, 64.3 and 39.3%, respectively. For the left brachial plexus, the maximum doses to the BPANs and the BPNANs were 72.84±3.91 and 64.81±3.47 Gy, respectively (p<0.001). For the right brachial plexus, the maximum doses to the BPANs and the BPNANs were 72.91±4.74 and 64.91±3.52 Gy, respectively (p<0.001). The difference between the left BPANs and the left BPNANs was statistically significant not only for V60 (3.60 vs. 1.01 cm3, p=0.028) but also for V66 (1.26 vs. 0.11 cm3, p=0.046). There were significant differences in V60 (3.68 vs. 1.16 cm3, p<0.001) and V66 (1.83 vs. 1.23 cm3, p=0.012) between the right BPANs and the right BPNANs. In conclusion, a large proportion of patients were exposed to the maximum dose to the brachial plexus exceeding the Radiation Therapy Oncology Group-recommended restraints when the brachial plexus was not outlined. The BPANs are at a significantly higher risk of receiving an excessive radiation dose when compared to the BPNANs. A further study is underway to test whether brachial plexus contouring assists in the dose reduction to the brachial plexus for IMRT optimization. PMID:22970028

  12. Diagnostic Value and Surgical Implications of the 3D DW-SSFP MRI On the Management of Patients with Brachial Plexus Injuries

    PubMed Central

    Qin, Ben-Gang; Yang, Jian-Tao; Yang, Yi; Wang, Hong-Gang; Fu, Guo; Gu, Li-Qiang; Li, Ping; Zhu, Qing-Tang; Liu, Xiao-Lin; Zhu, Jia-Kai

    2016-01-01

    Three-dimensional diffusion-weighted steady-state free precession (3D DW-SSFP) of high-resolution magnetic resonance has emerged as a promising method to visualize the peripheral nerves. In this study, the application value of 3D DW-SSFP brachial plexus imaging in the diagnosis of brachial plexus injury (BPI) was investigated. 33 patients with BPI were prospectively examined using 3D DW-SSFP MR neurography (MRN) of brachial plexus. Results of 3D DW-SSFP MRN were compared with intraoperative findings and measurements of electromyogram (EMG) or somatosensory evoked potentials (SEP) for each injured nerve root. 3D DW-SSFP MRN of brachial plexus has enabled good visualization of the small components of the brachial plexus. The postganglionic section of the brachial plexus was clearly visible in 26 patients, while the preganglionic section of the brachial plexus was clearly visible in 22 patients. Pseudomeningoceles were commonly observed in 23 patients. Others finding of MRN of brachial plexus included spinal cord offset (in 16 patients) and spinal cord deformation (in 6 patients). As for the 3D DW-SSFP MRN diagnosis of preganglionic BPI, the sensitivity, the specificity and the accuracy were respectively 96.8%, 90.29%, and 94.18%. 3D DW-SSFP MRN of brachial plexus improve visualization of brachial plexus and benefit to determine the extent of injury. PMID:27782162

  13. c-jun-N-Terminal Kinase (JNK) for the Treatment of Amyotrophic Lateral Sclerosis

    DTIC Science & Technology

    2013-10-01

    Treatment of Amyotrophic Lateral Sclerosis ” PRINCIPAL INVESTIGATOR: Dr. Philip LoGrasso CONTRACTING ORGANIZATION: The Scripps Research... Lateral Sclerosis ” 5a. CONTRACT NUMBER W81XWH-12-1-0431 5b. GRANT NUMBER W81XWH-12-1-0431 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) Philip...Annual 3. DATES COVERED 30September2012-29September2013 4. TITLE AND SUBTITLE “c-jun-N-Terminal Kinase (JNK) for the Treatment of Amyotrophic

  14. c-jun-N-Terminal Kinase (JNK) for the Treatment of Amyotrophic Lateral Sclerosis

    DTIC Science & Technology

    2015-03-01

    1 AWARD NUMBER: W81XWH-12-1-0431 TITLE: “c-jun-N- Terminal Kinase (JNK) for the Treatment of Amyotrophic Lateral Sclerosis” PRINCIPAL...TITLE AND SUBTITLE “c-jun-N- Terminal Kinase (JNK) for the Treatment of Amyotrophic Lateral Scelerosis” 5a. CONTRACT NUMBER 5b. GRANT NUMBER...ABSTRACT Abstract: 250 aminopyrazoles, a new class of c-jun-N- terminal kinase (JNK) inhibitors, have been synthesized and the biochemical IC50 has

  15. Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis.

    PubMed

    Kühnlein, Peter; Gdynia, Hans-Jürgen; Sperfeld, Anne-Dorte; Lindner-Pfleghar, Beate; Ludolph, Albert Christian; Prosiegel, Mario; Riecker, Axel

    2008-07-01

    Amyotrophic lateral sclerosis (ALS) is the most common neurodegenerative disease of the motor system. Bulbar symptoms such as dysphagia and dysarthria are frequent features of ALS and can result in reductions in life expectancy and quality of life. These dysfunctions are assessed by clinical examination and by use of instrumented methods such as fiberendoscopic evaluation of swallowing and videofluoroscopy. Laryngospasm, another well-known complication of ALS, commonly comes to light during intubation and extubation procedures in patients undergoing surgery. Laryngeal and pharyngeal complications are treated by use of an array of measures, including body positioning, compensatory techniques, voice and breathing exercises, communication devices, dietary modifications, various safety strategies, and neuropsychological assistance. Meticulous monitoring of clinical symptoms and close cooperation within a multidisciplinary team (physicians, speech and language therapists, occupational therapists, dietitians, caregivers, the patients and their relatives) are vital.

  16. Rehabilitation in amyotrophic lateral sclerosis: why it matters.

    PubMed

    Majmudar, Salony; Wu, Jason; Paganoni, Sabrina

    2014-07-01

    Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that results in a constellation of problematic symptoms and a high patient and caregiver burden. Multidisciplinary care includes rehabilitation interventions that have the goal of assisting people to teach their fullest potential despite the presence of a disabling disease. Given the progressive nature of ALS, the clinician must be aware of the expected disease trajectory and apply appropriate interventions at each stage. This review will present rehabilitation strategies that can be utilized to maximize patient independence, function, safety, and quality of life, and to minimize disease-related symptoms. The role of bracing, exercise, assistive devices, and adaptive equipment will be discussed. At each disease stage, an experienced rehabilitation team is well positioned to make a significant impact on the life of ALS patients.

  17. Comprehensive care of amyotrophic lateral sclerosis patients: a care model.

    PubMed

    Güell, Maria Rosa; Antón, Antonio; Rojas-García, Ricardo; Puy, Carmen; Pradas, Jesus

    2013-12-01

    Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease that presents with muscle weakness, causing progressive difficulty in movement, communication, eating and ultimately, breathing, creating a growing dependence on family members and other carers. The ideal way to address the problems associated with the disease, and the decisions that must be taken, is through multidisciplinary teams. The key objectives of these teams are to optimise medical care, facilitate communication between team members, and thus to improve the quality of care. In our centre, we have extensive experience in the care of patients with ALS through an interdisciplinary team whose aim is to ensure proper patient care from the hospital to the home setting. In this article, we describe the components of the team, their roles and our way of working.

  18. Elastic head support for persons with amyotrophic lateral sclerosis.

    PubMed

    Hansen, Andrew; Bedore, Beau; Nickel, Eric; Hanowski, Kristin; Tangen, Sonya; Goldish, Gary

    2014-01-01

    This article describes an inexpensive elastic head support for persons with amyotrophic lateral sclerosis (ALS) and neck muscle weakness and also presents a case series to examine its effectiveness. The device offers support to the head while the user is seated, standing, and walking, providing support for persons in various stages of ALS. The head support system was tested in seven male patients with ALS. Before and after the 2 wk trial, the subjects answered questions related to their communication efficacy, difficulty swallowing, level of neck discomfort, number of hours being upright before neck discomfort, comfort in social settings, and rating of perceived dyspnea. Subjects also answered specific questions related to the elastic head support after the 2 wk trial. The results suggested that the elastic head support is useful for some, but not all, patients.

  19. Current Therapy of Drugs in Amyotrophic Lateral Sclerosis

    PubMed Central

    Lu, Haiyan; Le, Wei Dong; Xie, Ya-Ying; Wang, Xiao-Ping

    2016-01-01

    Amyotrophic lateral sclerosis (ALS), commonly termed as motor neuron disease (MND) in UK, is a chronically lethal disorder among the neurodegenerative diseases, meanwhile. ALS is basically irreversible and progressive deterioration of upper and lower motor neurons in the motor cortex, brain stem and medulla spinalis. Riluzole, used for the treatment of ALS, was demonstrated to slightly delay the initiation of respiratory dysfunction and extend the median survival of patients by a few months. In this study, the key biochemical defects were discussed, such as: mutant Cu/Zn superoxide dismutase, mitochondrial protectants, and anti-excitotoxic/ anti-oxidative / anti-inflammatory/ anti-apoptotic agents, so the related drug candidates that have been studied in ALS models would possibly be further used in ALS patients. PMID:26786249

  20. Projected increase in amyotrophic lateral sclerosis from 2015 to 2040

    PubMed Central

    Arthur, Karissa C.; Calvo, Andrea; Price, T. Ryan; Geiger, Joshua T.; Chiò, Adriano; Traynor, Bryan J.

    2016-01-01

    Although amyotrophic lateral sclerosis (ALS) is relatively rare, the socioeconomic significance of the disease is extensive. It is therefore vital to project the epidemiologic trend of ALS. To date, there have been few published studies attempting to estimate the number and distribution of ALS cases in the upcoming years. Here we show that the number of ALS cases across the globe will increase from 222,801 in 2015 to 376,674 in 2040, representing an increase of 69%. This increase is predominantly due to ageing of the population, particularly among developing nations. This projection is likely an underestimate due to improving healthcare and economic conditions. The results should be used to inform healthcare policy to more efficiently allocate healthcare resources. PMID:27510634

  1. Research advances in amyotrophic lateral sclerosis, 2009 to 2010.

    PubMed

    Traub, Rebecca; Mitsumoto, Hiroshi; Rowland, Lewis P

    2011-02-01

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of upper and lower motor neurons that causes progressive weakness and death. The breadth of research in ALS continues to grow with exciting new discoveries in disease pathogenesis and potential future therapeutics. There is a growing list of identified mutations in familial ALS, including those in genes encoding TDP-43 and FUS/TLS, which are expanding our understanding of the role of RNA modulation in ALS pathogenesis. There is a greater appreciation for the role of glial cells in motor neuron disease. Mitochondrial dysfunction is also being shown to be critical for motor neuron degeneration. In addition to pharmacotherapy, there are promising early developments with therapeutic implications in the areas of RNA interference, stem cell therapies, viral vector-mediated gene therapy, and immunotherapy. With greater understanding of ALS pathogenesis and exciting new therapeutic technologies, there is hope for future progress in treating this disease.

  2. Autoimmunity in Amyotrophic Lateral Sclerosis: Past and Present

    PubMed Central

    Pagani, Mario Rafael; Gonzalez, Laura Elisabeth; Uchitel, Osvaldo Daniel

    2011-01-01

    Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting particularly motor neurons for which no cure or effective treatment is available. Although the cause of ALS remains unknown, accumulative evidence suggests an autoimmune mechanism of pathogenesis. In this paper, we will summarize the current research related to autoimmunity in the sporadic form of ALS and discuss the potential underlying pathogenic mechanisms and perspectives. Presented data supports the view that humoral immune responses against motor nerve terminals can initiate a series of physiological changes leading to alteration of calcium homeostasis. In turn, loss of calcium homeostasis may induce neuronal death through apoptotic signaling pathways. Additional approaches identifying specific molecular features of this hypothesis are required, which will hopefully allow us to develop techniques of early diagnosis and effective therapies. PMID:21826267

  3. Therapeutic applications of mesenchymal stem cells for amyotrophic lateral sclerosis

    PubMed Central

    2014-01-01

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting the neuromuscular system and does not have a known singular cause. Genetic mutations, extracellular factors, non-neuronal support cells, and the immune system have all been shown to play varied roles in clinical and pathological disease progression. The therapeutic plasticity of mesenchymal stem cells (MSCs) may be well matched to this complex disease pathology, making MSCs strong candidates for cellular therapy in ALS. In this review, we summarize a variety of explored mechanisms by which MSCs play a role in ALS progression, including neuronal and non-neuronal cell replacement, trophic factor delivery, and modulation of the immune system. Currently relevant techniques for applying MSC therapy in ALS are discussed, focusing in particular on delivery route and cell source. We include examples from in vitro, preclinical, and clinical investigations to elucidate the remaining progress that must be made to understand and apply MSCs as a treatment for ALS. PMID:25157751

  4. Amyotrophic Lateral Sclerosis and Metabolomics: Clinical Implication and Therapeutic Approach

    PubMed Central

    Kumar, Alok; Ghosh, Devlina; Singh, R. L.

    2013-01-01

    Amyotrophic lateral sclerosis (ALS) is one of the most common motor neurodegenerative disorders, primarily affecting upper and lower motor neurons in the brain, brainstem, and spinal cord, resulting in paralysis due to muscle weakness and atrophy. The majority of patients die within 3–5 years of symptom onset as a consequence of respiratory failure. Due to relatively fast progression of the disease, early diagnosis is essential. Metabolomics offer a unique opportunity to understand the spatiotemporal metabolic crosstalks through the assessment of body fluids and tissue. So far, one of the most challenging issues related to ALS is to understand the variation of metabolites in body fluids and CNS with the progression of disease. In this paper we will review the changes in metabolic profile in response to disease progression condition and also see the therapeutic implication of various drugs in ALS patients. PMID:26317018

  5. Cerebrovascular injury as a risk factor for amyotrophic lateral sclerosis

    PubMed Central

    Turner, Martin R; Goldacre, Raph; Talbot, Kevin; Goldacre, Michael J

    2016-01-01

    Objective To use an unbiased method to test a previously reported association between cerebral arteriovenous malformation (AVM) embolisation and the subsequent development of amyotrophic lateral sclerosis (ALS). Methods A hospital record linkage database was used to create cohorts of individuals coded as having cerebral and peripheral vessel AVMs, stroke (separately for haemorrhagic and ischaemic), transient ischaemic attack (TIA) and subarachnoid haemorrhage (SAH). The rate ratio for subsequent ALS was compared to a reference cohort. Results An increased rate ratio for ALS was found in relation to prior AVM (2.69; p=0.005), all strokes (1.38; p<0.001), and TIA (1.47; p<0.001). Conclusions Cerebrovascular injury from a variety of causes, rather than the presence of AVM or the associated embolisation procedure per se, may be a risk factor for ALS within the context of a more complex multiple-hit model of pathogenesis. PMID:26260352

  6. Age, gender, kinship and caregiver burden in amyotrophic lateral sclerosis.

    PubMed

    Tramonti, Francesco; Bongioanni, Paolo; Leotta, Rebecca; Puppi, Irene; Rossi, Bruno

    2015-01-01

    Amyotrophic lateral sclerosis is a neurodegenerative disease that affects the motor neurons and causes progressive physical impairment. Also, other functions, such as breathing, swallowing and speech are compromised, and the loss of independence makes caregiver burden extremely high. The present study aimed at evaluating the differences in the caregiver burden due to age, gender and kinship. Women reported a higher physical and social burden than men, and partners scored higher in several dimensions of the caregiver burden when compared to sons and daughters. With respect to adult child caregivers, daughters reported higher levels of developmental burden than sons. Age has a significant impact on the caregiver burden, especially for the time dedicated to assistance and physical burden; disease severity is significantly related to the physical burden as well, and also with the developmental burden.

  7. Myostatin as a therapeutic target in Amyotrophic lateral sclerosis.

    PubMed

    Walsh, Frank S; Rutkowski, Julia Lynn

    2012-11-01

    Amyotrophic Lateral Sclerosis is a devastating neurological disease that is inevitably fatal after 3-5years duration. Treatment options are minimal and as such new therapeutic modalities are required. In this review, we discuss the role of the myostatin pathway as a modulator of skeletal muscle mass and therapeutic approaches using biological based therapies. Both monoclonal antibodies to myostatin and a soluble receptor decoy to its high affinity receptor have been used in clinical trials of neuromuscular diseases and while there have been efficacy signals with the latter approach there have also been safety issues. Our approach is to target the high affinity receptor-binding site on myostatin and to develop a next generation set of therapeutic reagents built on a novel protein scaffold. This is the natural single domain VNAR found in sharks which is extremely versatile and has the ability to develop products with superior properties compared to existing therapeutics.

  8. Amyotrophic lateral sclerosis: increased solubility of skin collagen

    NASA Technical Reports Server (NTRS)

    Ono, S.; Yamauchi, M.

    1992-01-01

    We studied the solubility of skin collagen from six patients with amyotrophic lateral sclerosis (ALS) and six controls. The amount of collagen extracted with neutral salt solution was significantly greater in patients with ALS than in controls. In addition, there was a statistically significant increase in the proportion of collagen extracted from ALS patients with increased duration of illness. The collagen solubilized by pepsin and cyanogen bromide treatments was significantly higher in ALS patients than in controls, and its proportion was positively and significantly associated with duration of illness in ALS patients. These results indicate that the metabolism of skin collagen may be affected in the disease process of ALS, causing an increase in immature soluble collagen in the tissue, which is the opposite to that which occurs in the normal aging process.

  9. Therapeutic progress in amyotrophic lateral sclerosis-beginning to learning.

    PubMed

    Kumar, Vijay; Islam, Asimul; Hassan, Md Imtaiyaz; Ahmad, Faizan

    2016-10-04

    Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease associated with motor neuron degeneration, muscle weakness, paralysis and finally death. The proposed mechanisms of ALS include glutamate excitotoxicity, oxidative stress, inflammation, mitochondrial dysfunction, apoptosis and proteasomal dysfunction. Although numerous pathological mechanisms have been explained, ALS remains incurable disease because of failure of clinical trials and lack of any effective therapy. The rapid advancement in genetic discoveries in ALS emphasizes the point that ALS is a multi-subtype syndrome rather than a single disease. This can be argued as one of the single reason why many previous therapeutic drug trials have failed. Efforts to develop novel ALS treatments which target specific pathomechanisms are currently being pursued. Herein, we review the recent discovery and preclinical characterization of neuroprotective compounds and compare their effects on disease onset, duration and survival. Furthermore, the structure-activity relationships of these agents are analyzed with the overall goal of developing a screening strategy for future clinical applications.

  10. Comprehensive Rehabilitative Care Across the Spectrum of Amyotrophic Lateral Sclerosis

    PubMed Central

    Paganoni, Sabrina; Karam, Chafic; Joyce, Nanette; Bedlack, Richard; Carter, Gregory T

    2016-01-01

    Background Amyotrophic lateral sclerosis (ALS or Lou Gehrig’s disease) is a neurodegenerative disease that results in progressive muscle weakness and wasting. There is no known cure and the disease is uniformly fatal. Purpose This review discusses current concepts in ALS care, from breaking the diagnosis to end-of-life care. People with ALS have several multidisciplinary needs due to a complex and dynamic disease process. They benefit from rehabilitation interventions that are individualized and have the goal of optimizing independence, function, and safety. These strategies also help minimize symptomatic burden and maximize quality of life. Conclusion Patient-centered, multidisciplinary care has a significant impact on the life of people with ALS and is the current standard of care for this patient population. PMID:26409693

  11. Alternative Fuels in Epilepsy and Amyotrophic Lateral Sclerosis.

    PubMed

    Tefera, Tesfaye W; Tan, Kah Ni; McDonald, Tanya S; Borges, Karin

    2016-11-21

    This review summarises the recent findings on metabolic treatments for epilepsy and Amyotrophic Lateral Sclerosis (ALS) in honour of Professor Ursula Sonnewald. The metabolic impairments in rodent models of these disorders as well as affected patients are being discussed. In both epilepsy and ALS, there are defects in glucose uptake and reduced tricarboxylic acid (TCA) cycling, at least in part due to reduced amounts of C4 TCA cycle intermediates. In addition there are impairments in glycolysis in ALS. A reduction in glucose uptake can be addressed by providing the brain with alternative fuels, such as ketones or medium-chain triglycerides. As anaplerotic fuels, such as the triglyceride of heptanoate, triheptanoin, refill the TCA cycle C4/C5 intermediate pool that is deficient, they are ideal to boost TCA cycling and thus the oxidative metabolism of all fuels.

  12. Impact of Expiratory Strength Training in Amyotrophic Lateral Sclerosis

    PubMed Central

    Plowman, Emily K.; Watts, Stephanie A.; Tabor, Lauren; Robison, Raele; Gaziano, Joy; Domer, Amanda S.; Richter, Joel; Vu, Tuan; Gooch, Clifton

    2016-01-01

    Introduction We evaluated the feasibility and impact of Expiratory Muscle Strength Training (EMST) on respiratory and bulbar function in persons with amyotrophic lateral sclerosis (ALS). Methods 25 ALS patients participated in this delayed intervention open-label clinical trial. Following a lead-in period, patients completed a 5-week EMST protocol. Outcome measures included: maximum expiratory pressure (MEP), physiologic measures of swallow and cough, and Penetration-Aspiration Scale (PAS) scores. Results Of those participants who entered the active phase of the study (n=15), EMST was well tolerated and led to significant increases in MEPs and maximum hyoid displacement during swallowing post-EMST (P<0.05). No significant differences were observed for PAS scores or cough spirometry measures. Discussion EMST was feasible and well tolerated in this small cohort of ALS patients and led to improvements in expiratory force-generating pressures and swallow kinematics. Further investigation is warranted to confirm these preliminary findings. PMID:26599236

  13. Apoptosis signals in sporadic amyotrophic lateral sclerosis: an immunocytochemical study.

    PubMed

    Embacher, N; Kaufmann, W A; Beer, R; Maier, H; Jellinger, K A; Poewe, W; Ransmayr, G

    2001-11-01

    Sporadic amyotrophic lateral sclerosis (sALS) is a neurodegenerative disorder of unknown cause characterized by selective loss of both upper and lower motor neurons. Whether neuronal death in sALS is due to apoptosis has so far not been clarified. In this study, the expression and distribution patterns of pro- and anti-apoptotic bcl-2 family members as well as the executioner caspase-3 were investigated in post-mortem CNS tissue of eight sALS patients and seven age-matched controls. Sparse motor neurons were immunoreactive for bcl-2, bax, bak, and CM1 on serial sections through the spinal cord and motor cortex of individual sALS patients and controls. However, there was no obvious difference in the numbers of immunoreactive (IR) neurons between the two groups. The study did not find evidence for apoptosis as a major mechanism of motor neuronal cell death in sALS.

  14. REHABILITATION IN AMYOTROPHIC LATERAL SCLEROSIS: WHY IT MATTERS

    PubMed Central

    MAJMUDAR, SALONY; WU, JASON; PAGANONI, SABRINA

    2015-01-01

    Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that results in a constellation of problematic symptoms and a high patient and caregiver burden. Multidisciplinary care includes rehabilitation interventions that have the goal of assisting people to teach their fullest potential despite the presence of a disabling disease. Given the progressive nature of ALS, the clinician must be aware of the expected disease trajectory and apply appropriate interventions at each stage. This review will present rehabilitation strategies that can be utilized to maximize patient independence, function, safety, and quality of life, and to minimize disease-related symptoms. The role of bracing, exercise, assistive devices, and adaptive equipment will be discussed. At each disease stage, an experienced rehabilitation team is well positioned to make a significant impact on the life of ALS patients. PMID:24510737

  15. Current Therapy of Drugs in Amyotrophic Lateral Sclerosis.

    PubMed

    Lu, Haiyan; Le, Wei Dong; Xie, Ya-Ying; Wang, Xiao-Ping

    2016-01-01

    Amyotrophic lateral sclerosis (ALS), commonly termed as motor neuron disease (MND) in UK, is a chronically lethal disorder among the neurodegenerative diseases, meanwhile. ALS is basically irreversible and progressive deterioration of upper and lower motor neurons in the motor cortex, brain stem and medulla spinalis. Riluzole, used for the treatment of ALS, was demonstrated to slightly delay the initiation of respiratory dysfunction and extend the median survival of patients by a few months. In this study, the key biochemical defects were discussed, such as: mutant Cu/Zn superoxide dismutase, mitochondrial protectants, and anti-excitotoxic/ anti-oxidative / antiinflammatory/ anti-apoptotic agents, so the related drug candidates that have been studied in ALS models would possibly be further used in ALS patients.

  16. [A specific phobia of amyotrophic lateral sclerosis (ALS phobia)].

    PubMed

    Levitskiĭ, G N; Gilod, V M; Levin, O S

    2012-01-01

    A specific phobia of amyotrophic lateral sclerosis (ALS phobia) was not previously described in the literature. We examined 21 patients, 11 men and 10 women, aged 28-72 years, with symptoms of this phobia. Only 23% of patients had a history of the psychiatric disorder in the past. The duration of phobia symptoms was significantly higher in patients with moderate and severe phobia than in mild cases (1.5±0.6 and 5.0±1.1 months respectively; р<0.05). The severity of ALS phobia was correlated with its duration (r= -0.5; p=0.004). The primary character of phobia was established in 52.4% of patients basing on the regression of phobia symptoms assessed by the Hamilton anxiety scale after psychotherapy and pharmacotherapy (17±4 and 3±1 scores before and 3 months after treatment, respectively; p<0.05).

  17. Amyotrophic Lateral Sclerosis: What Nurses Need to Know.

    PubMed

    Bellomo, Tamara L; Cichminski, Lucille

    2017-03-01

    A MOTHER OF THREE teenage children, Mrs. S, 49, presented to her healthcare provider with bilateral leg twitching and weakness, difficulty swallowing, and fatigue that's worsened over the past few weeks. While she was on her daily morning walk, she tripped and fell. She experienced a small laceration to her leg, prompting her visit to the healthcare facility. Her husband said that she'd had periods of slurred speech over the past few months as well. She was alert and oriented, and her vital signs were all within normal limits.After an exam, her healthcare provider referred her to a neurologist who ordered magnetic resonance imaging (MRI), an electromyogram, and a full bloodwork panel. After multiple visits to rule out other causes, Mrs. S received the devastating diagnosis of amyotrophic lateral sclerosis (ALS).

  18. Venous thrombosis in subclavian, axillary, brachial veins with extension to internal jugular vein, right sigmoid sinus and simultaneous pulmonary embolism

    PubMed Central

    Tamizifar, Babak; Beigi, Arash; Rismankarzadeh, Maryam

    2013-01-01

    We present a rare case of Venous Thrombosis in Subclavian, Axillary, Brachial Veins with extension to Internal Jugular vein, right sigmoid sinus and simultaneous Pulmonary embolism during the treatment with low molecular weight heparin. PMID:23901341

  19. Neuron-specific antioxidant OXR1 extends survival of a mouse model of amyotrophic lateral sclerosis

    PubMed Central

    Liu, Kevin X.; Edwards, Benjamin; Lee, Sheena; Finelli, Mattéa J.; Davies, Ben

    2015-01-01

    Amyotrophic lateral sclerosis is a devastating neurodegenerative disorder characterized by the progressive loss of spinal motor neurons. While the aetiological mechanisms underlying the disease remain poorly understood, oxidative stress is a central component of amyotrophic lateral sclerosis and contributes to motor neuron injury. Recently, oxidation resistance 1 (OXR1) has emerged as a critical regulator of neuronal survival in response to oxidative stress, and is upregulated in the spinal cord of patients with amyotrophic lateral sclerosis. Here, we tested the hypothesis that OXR1 is a key neuroprotective factor during amyotrophic lateral sclerosis pathogenesis by crossing a new transgenic mouse line that overexpresses OXR1 in neurons with the SOD1G93A mouse model of amyotrophic lateral sclerosis. Interestingly, we report that overexpression of OXR1 significantly extends survival, improves motor deficits, and delays pathology in the spinal cord and in muscles of SOD1G93A mice. Furthermore, we find that overexpression of OXR1 in neurons significantly delays non-cell-autonomous neuroinflammatory response, classic complement system activation, and STAT3 activation through transcriptomic analysis of spinal cords of SOD1G93A mice. Taken together, these data identify OXR1 as the first neuron-specific antioxidant modulator of pathogenesis and disease progression in SOD1-mediated amyotrophic lateral sclerosis, and suggest that OXR1 may serve as a novel target for future therapeutic strategies. PMID:25753484

  20. Microstructural Correlates of Emotional Attribution Impairment in Non-Demented Patients with Amyotrophic Lateral Sclerosis

    PubMed Central

    Cerami, Chiara; Dodich, Alessandra; Canessa, Nicola; Iannaccone, Sandro; Corbo, Massimo; Lunetta, Christian; Falini, Andrea; Cappa, Stefano F.

    2016-01-01

    Impairments in the ability to recognize and attribute emotional states to others have been described in amyotrophic lateral sclerosis patients and linked to the dysfunction of key nodes of the emotional empathy network. Microstructural correlates of such disorders are still unexplored. We investigated the white-matter substrates of emotional attribution deficits in a sample of amyotrophic lateral sclerosis patients without cognitive decline. Thirteen individuals with either probable or definite amyotrophic lateral sclerosis and 14 healthy controls were enrolled in a Diffusion Tensor Imaging study and administered the Story-based Empathy Task, assessing the ability to attribute mental states to others (i.e., Intention and Emotion attribution conditions). As already reported, a significant global reduction of empathic skills, mainly driven by a failure in Emotion Attribution condition, was found in amyotrophic lateral sclerosis patients compared to healthy subjects. The severity of this deficit was significantly correlated with fractional anisotropy along the forceps minor, genu of corpus callosum, right uncinate and inferior fronto-occipital fasciculi. The involvement of frontal commissural fiber tracts and right ventral associative fronto-limbic pathways is the microstructural hallmark of the impairment of high-order processing of socio-emotional stimuli in amyotrophic lateral sclerosis. These results support the notion of the neurofunctional and neuroanatomical continuum between amyotrophic lateral sclerosis and frontotemporal dementia. PMID:27513746

  1. Microstructural Correlates of Emotional Attribution Impairment in Non-Demented Patients with Amyotrophic Lateral Sclerosis.

    PubMed

    Crespi, Chiara; Cerami, Chiara; Dodich, Alessandra; Canessa, Nicola; Iannaccone, Sandro; Corbo, Massimo; Lunetta, Christian; Falini, Andrea; Cappa, Stefano F

    2016-01-01

    Impairments in the ability to recognize and attribute emotional states to others have been described in amyotrophic lateral sclerosis patients and linked to the dysfunction of key nodes of the emotional empathy network. Microstructural correlates of such disorders are still unexplored. We investigated the white-matter substrates of emotional attribution deficits in a sample of amyotrophic lateral sclerosis patients without cognitive decline. Thirteen individuals with either probable or definite amyotrophic lateral sclerosis and 14 healthy controls were enrolled in a Diffusion Tensor Imaging study and administered the Story-based Empathy Task, assessing the ability to attribute mental states to others (i.e., Intention and Emotion attribution conditions). As already reported, a significant global reduction of empathic skills, mainly driven by a failure in Emotion Attribution condition, was found in amyotrophic lateral sclerosis patients compared to healthy subjects. The severity of this deficit was significantly correlated with fractional anisotropy along the forceps minor, genu of corpus callosum, right uncinate and inferior fronto-occipital fasciculi. The involvement of frontal commissural fiber tracts and right ventral associative fronto-limbic pathways is the microstructural hallmark of the impairment of high-order processing of socio-emotional stimuli in amyotrophic lateral sclerosis. These results support the notion of the neurofunctional and neuroanatomical continuum between amyotrophic lateral sclerosis and frontotemporal dementia.

  2. Neuron-specific antioxidant OXR1 extends survival of a mouse model of amyotrophic lateral sclerosis.

    PubMed

    Liu, Kevin X; Edwards, Benjamin; Lee, Sheena; Finelli, Mattéa J; Davies, Ben; Davies, Kay E; Oliver, Peter L

    2015-05-01

    Amyotrophic lateral sclerosis is a devastating neurodegenerative disorder characterized by the progressive loss of spinal motor neurons. While the aetiological mechanisms underlying the disease remain poorly understood, oxidative stress is a central component of amyotrophic lateral sclerosis and contributes to motor neuron injury. Recently, oxidation resistance 1 (OXR1) has emerged as a critical regulator of neuronal survival in response to oxidative stress, and is upregulated in the spinal cord of patients with amyotrophic lateral sclerosis. Here, we tested the hypothesis that OXR1 is a key neuroprotective factor during amyotrophic lateral sclerosis pathogenesis by crossing a new transgenic mouse line that overexpresses OXR1 in neurons with the SOD1(G93A) mouse model of amyotrophic lateral sclerosis. Interestingly, we report that overexpression of OXR1 significantly extends survival, improves motor deficits, and delays pathology in the spinal cord and in muscles of SOD1(G93A) mice. Furthermore, we find that overexpression of OXR1 in neurons significantly delays non-cell-autonomous neuroinflammatory response, classic complement system activation, and STAT3 activation through transcriptomic analysis of spinal cords of SOD1(G93A) mice. Taken together, these data identify OXR1 as the first neuron-specific antioxidant modulator of pathogenesis and disease progression in SOD1-mediated amyotrophic lateral sclerosis, and suggest that OXR1 may serve as a novel target for future therapeutic strategies.

  3. Comparison of Brachial Artery Vasoreactivity in Elite Power Athletes and Age-Matched Controls

    PubMed Central

    Welsch, Michael A.; Blalock, Paul; Credeur, Daniel P.; Parish, Tracie R.

    2013-01-01

    Elite endurance athletes typically have larger arteries contributing to greater skeletal muscle blood flow, oxygen and nutrient delivery and improved physical performance. Few studies have examined structural and functional properties of arteries in power athletes. Purpose To compare the size and vasoreactivity of the brachial artery of elite power athletes to age-matched controls. It was hypothesized brachial artery diameters of athletes would be larger, have less vasodilation in response to cuff occlusion, but more constriction after a cold pressor test than age-matched controls. Methods Eight elite power athletes (age = 23±2 years) and ten controls (age = 22±1 yrs) were studied. High-resolution ultrasonography was used to assess brachial artery diameters at rest and following 5 minutes of forearm occlusion (Brachial Artery Flow Mediated Dilation = BAFMD) and a cold pressor test (CPT). Basic fitness measures included a handgrip test and 3-minute step test. Results Brachial arteries of athletes were larger (Athletes 5.39±1.51 vs. Controls: 3.73±0.71 mm, p<0.05), had greater vasodilatory (BAFMD%: Athletes: 8.21±1.78 vs. Controls: 5.69±1.56%) and constrictor (CPT %: Athletes: -2.95±1.07 vs. Controls: −1.20±0.48%) responses, compared to controls. Vascular operating range (VOR = Peak dilation+Peak Constriction) was also greater in athletes (VOR: Athletes: 0.55±0.15 vs. Controls: 0.25±0.18 mm, p<0.05). Athletes had superior handgrip strength (Athletes: 55.92±17.06 vs. Controls: 36.77±17.06 kg, p<0.05) but similar heart rate responses at peak (Athletes: 123±16 vs. Controls: 130±25 bpm, p>0.05) and 1 minute recovery (Athletes: 88±21 vs. Controls: 98±26 bpm, p>0.05) following the step test. Conclusion Elite power athletes have larger brachial arteries, and greater vasoreactivity (greater vasodilatory and constrictor responses) than age-matched controls, contributing to a significantly greater VOR. These data extend the existence of an

  4. Brachial artery reactivity in patients with severe sepsis: an observational study

    PubMed Central

    2012-01-01

    Introduction Ultrasound measurements of brachial artery reactivity in response to stagnant ischemia provide estimates of microvascular function and conduit artery endothelial function. We hypothesized that brachial artery reactivity would independently predict severe sepsis and severe sepsis mortality. Methods This was a combined case-control and prospective cohort study. We measured brachial artery reactivity in 95 severe sepsis patients admitted to the medical and surgical intensive care units of an academic medical center and in 52 control subjects without acute illness. Measurements were compared in severe sepsis patients versus control subjects and in severe sepsis survivors versus nonsurvivors. Multivariable analyses were also conducted. Results Hyperemic velocity (centimeters per cardiac cycle) and flow-mediated dilation (percentage) were significantly lower in severe sepsis patients versus control subjects (hyperemic velocity: severe sepsis = 34 (25 to 48) versus controls = 63 (52 to 81), P < 0.001; flow-mediated dilation: severe sepsis = 2.65 (0.81 to 4.79) versus controls = 4.11 (3.06 to 6.78), P < 0.001; values expressed as median (interquartile range)). Hyperemic velocity, but not flow-mediated dilation, was significantly lower in hospital nonsurvivors versus survivors (hyperemic velocity: nonsurvivors = 25 (16 to 28) versus survivors = 39 (30 to 50), P < 0.001; flow-mediated dilation: nonsurvivors = 1.90 (0.68 to 3.41) versus survivors = 2.96 (0.91 to 4.86), P = 0.12). Lower hyperemic velocity was independently associated with hospital mortality in multivariable analysis (odds ratio = 1.11 (95% confidence interval = 1.04 to 1.19) per 1 cm/cardiac cycle decrease in hyperemic velocity; P = 0.003). Conclusions Brachial artery hyperemic blood velocity is a noninvasive index of microvascular function that independently predicts mortality in severe sepsis. In contrast, brachial artery flow-mediated dilation, reflecting conduit artery endothelial function

  5. Cubesat Proximity Operations Demonstration (CPOD)

    NASA Technical Reports Server (NTRS)

    Villa, Marco; Martinez, Andres; Petro, Andrew

    2015-01-01

    The CubeSat Proximity Operations Demonstration (CPOD) project will demonstrate rendezvous, proximity operations and docking (RPOD) using two 3-unit (3U) CubeSats. Each CubeSat is a satellite with the dimensions 4 inches x 4 inches x 13 inches (10 centimeters x 10 centimeters x 33 centimeters) and weighing approximately 11 pounds (5 kilograms). This flight demonstration will validate and characterize many new miniature low-power proximity operations technologies applicable to future missions. This mission will advance the state of the art in nanosatellite attitude determination,navigation and control systems, in addition to demonstrating relative navigation capabilities.The two CPOD satellites are scheduled to be launched together to low-Earth orbit no earlier than Dec. 1, 2015.

  6. Hereditary motor and sensory neuropathy (proximal dominant form, HMSN-P) among Brazilians of Japanese ancestry.

    PubMed

    Maeda, Kengo; Sugiura, Makoto; Kato, Hiroko; Sanada, Mitsuru; Kawai, Hiromichi; Yasuda, Hitoshi

    2007-11-01

    Hereditary motor and sensory neuropathy (proximal dominant form, HMSN-P) has been reported exclusively from Okinawa Prefecture in Japan. We herein report three brothers with HMSN-P who are among Brazilians of Japanese ancestry. They showed the typical clinical manifestations and were compatible with HMSN-P. Okinawa Prefecture has been a site of emigration to other countries, mainly in South America, since 1908. Although this is the first reported familial case of HMSN-P occurring outside Japan, it is estimated that there are 19 or 20 individuals with HMSN-P among these emigrants. Since HMSN-P might be misdiagnosed as familial amyotrophic lateral sclerosis or spinal muscular atrophy, neurologists in countries where individuals of Okinawan extraction reside should be aware of this hereditary neuropathy. HMSN-P should no longer be regarded as an endemic condition limited to Okinawa.

  7. Ultrasound guided therapeutic injections of the cervical spine and brachial plexus

    PubMed Central

    2015-01-01

    Abstract Introduction: Recent applications in ultrasound imaging include ultrasound assessment and ultrasound guided therapeutic injections of the spine and brachial plexus. Discussion: Ultrasound is an ideal modality for these regions as it allows accurate safe and quick injection of single or multiple sites. It has the added advantages of lack of ionising radiation, and can be done without requiring large expensive radiology equipment. Conclusion: Brachial plexus pathology may be present in patients presenting for shoulder symptoms where very little is found at imaging the shoulder. It is important to understand the anatomy and normal variants that may exist to be able to recognise when pathology is present. When pathology is demonstrated it is easy to do a trial of therapy with ultrasound guided injection of steroid around the nerve lesion. This review will outline the normal anatomy and variants and common pathology, which can be amenable to ultrasound guided injection of steroid. PMID:28191203

  8. Respiratory distress in a one-month-old child suffering brachial plexus palsy.

    PubMed

    Héritier, Odile; Vasseur Maurer, Sabine; Reinberg, Olivier; Cotting, Jacques; Perez, Marie-Hélène

    2013-01-01

    This paper describes a one-month-old girl presenting with respiratory and growth failure due to diaphragmatic paralysis associated with left brachial plexus palsy after forceps delivery. Despite continuous positive pressure ventilation and nasogastric feeding, the situation did not improve and a laparoscopic diaphragmatic plication had to be performed. When dealing with a child born with brachial plexus palsy, one must think of this possible association and if necessary proceed to the complementary radiological examinations. The treatment must avoid complications like feeding difficulties and failure to thrive, respiratory infections or atelectasis. It includes intensive support and a good evaluation of the prognosis of the lesion to decide the best moment for a surgical therapy.

  9. Medial antebrachial cutaneous nerve injury after brachial plexus block: two case reports.

    PubMed

    Jung, Mi Jin; Byun, Ha Young; Lee, Chang Hee; Moon, Seung Won; Oh, Min-Kyun; Shin, Heesuk

    2013-12-01

    Medial antebrachial cutaneous (MABC) nerve injury associated with iatrogenic causes has been rarely reported. Local anesthesia may be implicated in the etiology of such injury, but has not been reported. Two patients with numbness and painful paresthesia over the medial aspect of the unilateral forearm were referred for electrodiagnostic study, which revealed MABC nerve lesion in each case. The highly selective nature of the MABC nerve injuries strongly suggested that they were the result of direct nerve injury by an injection needle during previous brachial plexus block procedures. Electrodiagnostic studies can be helpful in evaluating cases of sensory disturbance after local anesthesia. To our knowledge, these are the first documented cases of isolated MABC nerve injury following ultrasound-guided axillary brachial plexus block.

  10. Mediastinal mass and brachial plexopathy caused by subclavian arterial aneurysm in Behçet's disease.

    PubMed

    Yoo, W H; Kim, H K; Park, J H; Park, T S; Baek, H S

    2000-01-01

    Vascular involvement in Behçet's disease is divided into venous and arterial thrombosis and arterial aneurysmal formation. Subclavian arterial aneurysm rarely occurs in Behçet's disease; however, when it does occur, it causes serious aneurysmal rupture and local complications such as nerve compression and arterial ischemia. We describe the case of a 39-year-old male who presented with neurologic symptoms and signs of brachial plexopathy and mediastinal mass caused by Behçet's subclavian arterial aneurysm. This case shows that the occurrence of brachial plexopathy should be considered a manifestation of Behçet's disease, and that Behçet's aneurysm should be considered in the differential diagnosis of upper mediastinal mass.

  11. Complete Brachial Artery Transection following closed Posterior Elbow Dislocation: A Rare Case Report

    PubMed Central

    C, JayanthKumar B; Sampath, Deepak; N, Hanumantha Reddy; Motukuru, Vishnu

    2015-01-01

    Introduction: Vascular injury associated withclosed posterior elbow dislocations is rare and it usually occurs along with open dislocation, anterior dislocation, penetrating injuries, dislocations associated with fracture. We report such a case of closed posterior elbow dislocation with complete brachial artery rupture. Case Report: A 58 years old lady sustained posterior dislocation of right elbow following a fall at home. She presented three days later with complaints of severe pain, swelling around the right elbow and numbness of fingers following a closed reduction done elsewhere. Computed graft angiography showed complete transection of brachialartery. Patient was treated with thrombectomy, right great saphenous vein graft interposition repair of brachial artery and forearm fasciotomy. Conclusion: Vascular injuries associated with posterior elbow dislocation are very rare, but high index of suspicion of arterial injury need to be thought off and repeated vascular examination during pre and post reduction stage should be done to prevent complications. PMID:27299092

  12. Effectiveness of primary conservative management for infants with obstetric brachial plexus palsy.

    PubMed

    Bialocerkowski, Andrea; Kurlowicz, Kirsty; Vladusic, Sharon; Grimmer, Karen

    2005-03-01

    Background  Obstetric brachial plexus palsy, a complication of childbirth, occurs in 1-3 per 1000 live births internationally. Traction and/or compression of the brachial plexus is thought to be the primary mechanism of injury and this may occur in utero, during the descent through the birth canal or during delivery. This results in a spectrum of injuries that vary in severity, extent of damage and functional use of the affected upper limb. Most infants receive treatment, such as conservative management (physiotherapy, occupational therapy) or surgery; however, there is controversy regarding the most appropriate form of management. To date, no synthesised evidence is available regarding the effectiveness of primary conservative management for obstetric brachial plexus palsy. Objectives  The objective of this review was to systematically assess the literature and present the best available evidence that investigated the effectiveness of primary conservative management for infants with obstetric brachial plexus palsy. Search strategy  A systematic literature search was performed using 14 databases: TRIP, MEDLINE, CINAHL, AMED, Web of Science, Proquest 5000, Evidence Based Medicine Reviews, Expanded Academic ASAP, Meditext, Science Direct, Physiotherapy Evidence Database, Proquest Digital Dissertations, Open Archives Initiative Search Engine, Australian Digital Thesis Program. Those studies that were reported in English and published over the last decade (July 1992 to June 2003) were included in this review. Selection criteria  Quantitative studies that investigated the effectiveness of primary conservative management for infants with obstetric brachial plexus palsy were eligible for inclusion in this review. This excluded studies that solely investigated the effect of primary surgery for these infants, management of secondary deformities and the investigation of the effects of pharmacological agents, such as botulinum toxin. Data collection and analysis

  13. Brachial vs. central systolic pressure and pulse wave transmission indicators: a critical analysis.

    PubMed

    Izzo, Joseph L

    2014-12-01

    This critique is intended to provide background for the reader to evaluate the relative clinical utilities of brachial cuff systolic blood pressure (SBP) and its derivatives, including pulse pressure, central systolic pressure, central augmentation index (AI), and pulse pressure amplification (PPA). The critical question is whether the newer indicators add sufficient information to justify replacing or augmenting brachial cuff blood pressure (BP) data in research and patient care. Historical context, pathophysiology of variations in pulse wave transmission and reflection, issues related to measurement and model errors, statistical limitations, and clinical correlations are presented, along with new comparative data. Based on this overview, there is no compelling scientific or practical reason to replace cuff SBP with any of the newer indicators in the vast majority of clinical situations. Supplemental value for central SBP may exist in defining patients with exaggerated PPA ("spurious systolic hypertension"), managing cardiac and aortic diseases, and in studies of cardiovascular drugs, but there are no current standards for these possibilities.

  14. Vibration sensation as an indicator of surgical anesthesia following brachial plexus block

    PubMed Central

    Jindal, Seema; Sidhu, Gurkaran Kaur; Sood, Dinesh; Grewal, Anju

    2016-01-01

    Background: Local anesthetic instillation in close vicinity to nerves anywhere in body blocks sensations in the same order as in central neuraxial blockade. The main purpose of this study was to evaluate the efficacy of vibration sense as criteria to determine the onset of surgical anesthesia following brachial plexus block and its correlation with loss of sensory and motor power. Materials and Methods: This prospective study included fifty patients of American Society of Anaesthesiologist physical status I and II, aged between 18 and 45 years, undergoing elective upper limb surgery under brachial plexus block by supraclavicular approach. The baseline values of vibration sense perception using 128 Hz Rydel–Seiffer tuning fork, motor power using formal motor power of wrist flexion and wrist extension, and sensory score by pinprick method were recorded preoperatively and every 5 min after giving block till the onset of complete surgical anesthesia. Results: The mean ± standard deviation of time (in minutes) for sensory, motor, and vibration block was 13.33 ± 3.26, 21.10 ± 3.26, and 25.50 ± 2.02, respectively (P < 0.05). Although all the patients achieved complete sensory and motor block after 25 min, 14% of the patients still had vibration sensations intact and 100% of the patients achieved complete sensory, motor, and vibration block after 30 min. Conclusions: Vibration sense serves as a reliable indicator for the onset of surgical anesthesia following brachial plexus block. Vibration sense testing with 128 Hz Rydel–Seiffer tuning fork along with motor power assessment should be used as an objective tool to assess the onset of surgical anesthesia following brachial plexus block. PMID:27833488

  15. Familial long thoracic nerve palsy: a manifestation of brachial plexus neuropathy.

    PubMed

    Phillips, L H

    1986-09-01

    Long thoracic nerve palsy causes weakness of the serratus anterior muscle and winging of the scapula. It is usually traumatic in origin. Isolated long thoracic nerve palsy has not been recognized as the major manifestation of familial brachial plexus neuropathy, but I have studied the syndrome in four members of three generations of one family. One individual suffered an episode of facial paresis. The inheritance pattern was autosomal dominant.

  16. Sensory Evaluation of the Hands in Children with Brachial Plexus Birth Injury

    ERIC Educational Resources Information Center

    Palmgren, Tove; Peltonen, Jari; Linder, Tove; Rautakorpi, Sanna; Nietosvaara, Yrjana

    2007-01-01

    The aim of this study was to examine sensory changes of the hand in brachial plexus birth injury (BPBI). Ninety-five patients (43 females, 52 males) comprising two age groups, 6 to 8 years (mean age 7y 6mo) and 12 to 14 years (mean age 13y 2mo), were included. Sixty-four had upper (cervical [C] 5-6), 19 upper and middle (C5-7), and 12 had total…

  17. Newborn brachial plexus injuries: The twisting and extension of the fetal head as contributing causes.

    PubMed

    Sandmire, H; Morrison, J; Racinet, C; Hankins, G; Pecorari, D; Gherman, R

    2008-02-01

    The exact mechanism of the causation of brachial plexus injury (BPI) has long been a matter of controversy. It is our opinion that the twisting and the extension of the fetal head, during the labour and delivery process, will increase the stretching of the neck, thus contributing to the labour forces as the cause of BPI. Our opinions are offered to other researchers and readers for their consideration of how the labour forces can cause BPI.

  18. Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons.

    PubMed

    Gopal, Pallavi P; Nirschl, Jeffrey J; Klinman, Eva; Holzbaur, Erika L F

    2017-03-21

    Ribonucleoprotein (RNP) granules are enriched in specific RNAs and RNA-binding proteins (RBPs) and mediate critical cellular processes. Purified RBPs form liquid droplets in vitro through liquid-liquid phase separation and liquid-like non-membrane-bound structures in cells. Mutations in the human RBPs TAR-DNA binding protein 43 (TDP-43) and RNA-binding protein FUS cause amyotrophic lateral sclerosis (ALS), but the biophysical properties of these proteins have not yet been studied in neurons. Here, we show that TDP-43 RNP granules in axons of rodent primary cortical neurons display liquid-like properties, including fusion with rapid relaxation to circular shape, shear stress-induced deformation, and rapid fluorescence recovery after photobleaching. RNP granules formed from wild-type TDP-43 show distinct biophysical properties depending on axonal location, suggesting maturation to a more stabilized structure is dependent on subcellular context, including local density and aging. Superresolution microscopy demonstrates that the stabilized population of TDP-43 RNP granules in the proximal axon is less circular and shows spiculated edges, whereas more distal granules are both more spherical and more dynamic. RNP granules formed by ALS-linked mutant TDP-43 are more viscous and exhibit disrupted transport dynamics. We propose these altered properties may confer toxic gain of function and reflect differential propensity for pathological transformation.

  19. Shifting the balance: cell-based therapeutics as modifiers of the amyotrophic lateral sclerosis-specific neuronal microenvironment.

    PubMed

    Riley, Jonathan; Sweeney, Walter; Boulis, Nicholas

    2008-01-01

    Recent advances in the laboratory have improved the current understanding of neurobiological mechanisms underlying the initiating events and pathological progression observed in amyotrophic lateral sclerosis (ALS). Whereas initial studies have revealed the late-stage intracellular cascades contributing to neuronal dysfunction and cell death, more recently collected data have begun to elucidate the presence and importance of a "non-cell autonomous" component indicating that affected glial cell subtypes may serve distinct and required roles. Pharmacological interventions for ALS have largely been disappointing likely in part because they have failed to address either the proximate events contributing to neuronal dysfunction and death or the deleterious contributions of non-neuronal cells within the local microenvironment. Alternatively, cell-based therapeutics offer the potential of a multifaceted approach oriented toward the dual ends of protecting remaining viable neurons and attempting to restore neuronal function lost as a manifestation of disease progression. The authors review the evolving knowledge of disease initiation and progression, with specific emphasis on the role of affected glia as crucial contributors to the observed ALS phenotype. This basis is used to underscore the potential roles of cell-based therapeutics as modifiers of the ALS-specific microenvironment.

  20. Comparison between Flail Arm Syndrome and Upper Limb Onset Amyotrophic Lateral Sclerosis: Clinical Features and Electromyographic Findings

    PubMed Central

    Yoon, Byung-Nam; Choi, Seong Hye; Rha, Joung-Ho; Kang, Sa-Yoon; Lee, Kwang-Woo

    2014-01-01

    Flail arm syndrome (FAS), an atypical presentation of amyotrophic lateral sclerosis (ALS), is characterized by progressive, predominantly proximal, weakness of upper limbs, without involvement of the lower limb, bulbar, or respiratory muscles. When encountering a patient who presents with this symptomatic profile, possible diagnoses include upper limb onset ALS (UL-ALS), and FAS. The lack of information regarding FAS may make differential diagnosis between FAS and UL-ALS difficult in clinical settings. The aim of this study was to compare clinical and electromyographic findings from patients diagnosed with FAS with those from patients diagnosed with UL-ALS. To accomplish this, 18 patients with FAS and 56 patients with UL-ALS were examined. Significant differences were observed between the 2 groups pertaining to the rate of fasciculation, patterns of predominantly affected muscles, and the Medical Research Council scale of the weakest muscle. The presence of upper motor neuron signs and lower motor neuron involvement evidenced through electromyography showed no significant between-group differences. PMID:25258573

  1. Driven shielding capacitive proximity sensor

    NASA Technical Reports Server (NTRS)

    Vranish, John M. (Inventor); McConnell, Robert L. (Inventor)

    2000-01-01

    A capacitive proximity sensing element, backed by a reflector driven at the same voltage as and in phase with the sensor, is used to reflect the field lines away from a grounded robot arm towards an intruding object, thus dramatically increasing the sensor's range and sensitivity.

  2. Proximity Sensors Make Robot Dexterous

    NASA Technical Reports Server (NTRS)

    Hess, Cliff; Li, Larry C. H.

    1990-01-01

    Control system enables robot hand to grasp objects of varied shapes. Key features of system: reflective proximity sensors furnishing data on position, orientation, and distance of object and software protocol controlling sequence of operations in approaching and grasping objects. Reflected-beam sensing concept applied to simple opposed-jaw industrial grippers as well as to dexterous robot hands.

  3. A Comparative Study in the Use of Brachial Photoplethysmography and the QRS Complex as Timing References in Determination of Pulse Transit Time

    DTIC Science & Technology

    2007-11-02

    splinted in each case to keep the brachial archery prominent. The probes were connected with flexible cable to the data acquisition unit. The subject was...pressure. A sample of data was taken during this period. A measurement of the distance from the detection point on the brachial archery to the...brachial archery , at the elbow, and produced a strong, if elusive, plethysmograph. The wider focus of this work is to explore a means for non

  4. Gross anatomy of the brachial plexus in the giant Anteater (Myrmecophaga tridactyla).

    PubMed

    Souza, P R; Cardoso, J R; Araujo, L B M; Moreira, P C; Cruz, V S; Araujo, E G

    2014-10-01

    Ten forelimbs of five Myrmecophaga tridactyla were examined to study the anatomy of the brachial plexus. The brachial plexuses of the M. tridactyla observed in the present study were formed by the ventral rami of the last four cervical spinal nerves, C5 through C8, and the first thoracic spinal nerve, T1. These primary roots joined to form two trunks: a cranial trunk comprising ventral rami from C5-C7 and a caudal trunk receiving ventral rami from C8-T1. The nerves originated from these trunks and their most constant arrangement were as follows: suprascapular (C5-C7), subscapular (C5-C7), cranial pectoral (C5-C8), caudal pectoral (C8-T1), axillary (C5-C7), musculocutaneous (C5-C7), radial (C5-T1), median (C5-T1), ulnar (C5-T1), thoracodorsal (C5-C8), lateral thoracic (C7-T1) and long thoracic (C6-C7). In general, the brachial plexus in the M. tridactyla is similar to the plexuses in mammals, but the number of rami contributing to the formation of each nerve in the M. tridactyla was found to be larger than those of most mammals. This feature may be related to the very distinctive anatomical specializations of the forelimb of the anteaters.

  5. [The value of brachial artery peak velocity variation during the Valsalva maneuver to predict fluid responsiveness].

    PubMed

    Sheng, L F; Yan, M; Zhang, F J; Ren, Q S; Yu, S H; Wu, M

    2017-02-14

    Objective: To evaluate whether brachial artery peak velocity variation(ΔVp) during a Valsalva maneuver(VM) could predict fluid responsiveness in spontaneously breathing patients. Methods: Ninety-six patients required radial artery catheter for elective surgery of Ningbo Yinzhou People's Hospital from December 2014 to June 2016 were enrolled. The brachial artery Doppler signal was recorded to measure the ΔVp while the VM was performed.Then doing the volume expansion (VE) , the cardiac output variation (ΔCO) before and after VE were measured.Pearson correlational analyses were conducted between ΔVp and ΔCO. Also the sensitivity and specificity of ΔVp were determined in predicting fluid responsiveness by the receiver operating characteristic (ROC) curve. Results: Patients were classified as group responders (n=24) and group non-responders (n=72). Responder was defined as cardiac output increased≥15% after VE.The ΔVp correlated well with ΔCO (r=0.792, P<0.01). The area under ROC curve was 0.903, with the ΔVp cut-off of 33%, the sensitivity of 87% and the specificity of 82%(P<0.01). Conclusion: Brachial artery peak velocity variation during a valsalva maneuver is a feasible method for predicting fluid responsiveness in spontaneously breathing patients.

  6. [Transradial percutaneous approach for cardiac catheterization in patients with previous brachial artery cutdown].

    PubMed

    Magariños, Eduardo; Solioz, Germán; Cermesoni, Gabriel; Koretzky, Martín; Carnevalini, Mariana; González, Daniel

    2013-01-01

    The percutaneous punction of the radial artery for catheterization procedures has gained acceptance lately. This was a consequence of achieving results similar to the femoral approach, with the benefits of a lower rate of complications and increased comfort for the patients post procedure. Recently it has gained an additional impulse with the better prognosis obtained in acute coronary syndromes. In this trial we have evaluated if the feasibility, results and advantages related with the use of the radial artery percutaneous approach to perform catheterization procedures, continues when used in patients who have had a previous brachial artery cutdown. Out of a total of 1356 percutaneous radial accesses, 53 were in patients with previous brachial artery cutdown. Through this access 71 catheterization procedures were performed, achieving access success in 96.2% (51/53) of the punctions. Once the access success was obtained, 93.6% (44/47) of the diagnostic procedures and 100% (24/24) of the therapeutics procedures were successful. During hospitalization, in this group of patients, no major adverse cardiac events occurred and there was a 1.4% (1/71) rate of minor events. At seven days follow up, no new complications were recorded. Although this is a small group, we believe that it is enough to show that percutaneous punctions of the radial artery to perform catheterization procedures, in patients with previous brachial artery cutdown, are feasible, allowing high access and procedure success rates, with a low frequency of complications.

  7. Surgical outcomes of the brachial plexus lesions caused by gunshot wounds in adults

    PubMed Central

    2009-01-01

    Background The management of brachial plexus injuries due to gunshot wounds is a surgical challenge. Better surgical strategies based on clinical and electrophysiological patterns are needed. The aim of this study is to clarify the factors which may influence the surgical technique and outcome of the brachial plexus lesions caused by gunshot injuries. Methods Two hundred and sixty five patients who had brachial plexus lesions caused by gunshot injuries were included in this study. All of them were male with a mean age of 22 years. Twenty-three patients were improved with conservative treatment while the others underwent surgical treatment. The patients were classified and managed according to the locations, clinical and electrophysiological findings, and coexisting lesions. Results The wounding agent was shrapnel in 106 patients and bullet in 159 patients. Surgical procedures were performed from 6 weeks to 10 months after the injury. The majority of the lesions were repaired within 4 months were improved successfully. Good results were obtained in upper trunk and lateral cord lesions. The outcome was satisfactory if the nerve was intact and only compressed by fibrosis or the nerve was in-contunuity with neuroma or fibrosis. Conclusion Appropriate surgical techniques help the recovery from the lesions, especially in patients with complete functional loss. Intraoperative nerve status and the type of surgery significantly affect the final clinical outcome of the patients. PMID:19627573

  8. Brachial Plexus in the Pampas Fox (Lycalopex gymnocercus): a Descriptive and Comparative Analysis.

    PubMed

    de Souza Junior, Paulo; da Cruz de Carvalho, Natan; de Mattos, Karine; Abidu Figueiredo, Marcelo; Luiz Quagliatto Santos, André

    2017-03-01

    Twenty thoracic limbs of ten Lycalopex gymnocercus were dissected to describe origin and distribution of the nerves forming brachial plexuses. The brachial plexus resulted from the connections between the ventral branches of the last three cervical nerves (C6, C7, and C8) and first thoracic nerve (T1). These branches connected the suprascapular, subscapular, axillary, musculocutaneous, radial, median and ulnar nerves to the intrinsic musculature and connected the brachiocephalic, thoracodorsal, lateral thoracic, long thoracic, cranial pectoral and caudal pectoral nerves to the extrinsic musculature. The C7 ventral branches contribute most to the formation of the nerves (62.7%), followed by C8 (58.8%), T1 (40.0%) and C6 (24.6%). Of the 260 nerves dissected, 69.2% resulted from a combination of two or three branches, while only 30.8% originated from a single branch. The origin and innervation area of the pampas fox brachial plexus, in comparison with other domestic and wild species, were most similar to the domestic dog and wild canids from the neotropics. The results of this study can serve as a base for comparative morphofunctional analysis involving this species and development of nerve block techniques. Anat Rec, 300:537-548, 2017. © 2016 Wiley Periodicals, Inc.

  9. Results of ulnar nerve neurotization to biceps brachii muscle in brachial plexus injury

    PubMed Central

    Rezende, Marcelo Rosa De; Rabelo, Neylor Teofilo Araújo; Silveira, Clóvis Castanho; Petersen, Pedro Araújo; Paula, Emygdio José Leomil De; Mattar, Rames

    2012-01-01

    OBJECTIVE: To evaluate the factors influencing the results of ulnar nerve neurotization at the motor branch of the brachii biceps muscle, aiming at the restoration of elbow flexion in patients with brachial plexus injury. METHODS: 19 patients, with 18 men and 1 woman, mean age 28.7 years. Eight patients had injury to roots C5-C6 and 11, to roots C5-C6-C7. The average time interval between injury and surgery was 7.5 months. Four patients had cervical fractures associated with brachial plexus injury. The postoperative follow-up was 15.7 months. RESULTS: Eight patients recovered elbow flexion strength MRC grade 4; two, MRC grade 3 and nine, MRC <3. There was no impairment of the previous ulnar nerve function. CONCLUSION: The surgical results of ulnar nerve neurotization at the motor branch of brachii biceps muscle are dependent on the interval between brachial plexus injury and surgical treatment, the presence of associated fractures of the cervical spine and occipital condyle, residual function of the C8-T1 roots after the injury and the involvement of the C7 root. Signs of reinnervation manifested up to 3 months after surgery showed better results in the long term. Level of Evidence: IV, Case Series. PMID:24453624

  10. Proximity and Anti-proximity effects in nanowires

    NASA Astrophysics Data System (ADS)

    Chan, Moses

    Near a superconductor/normal-metal interface, the leakage of Cooper pairs extends superconducting behavior into the metal. The spatial range of this proximity effect in a normal metal can be as long as 1 µm. However, when a ferromagnet is placed in contact with a superconductor, the Cooper pairs from the superconductor are not expected to survive beyond at most a few nanometers into the ferromagnet. Surprisingly we find when a cobalt nanowire as long as 600 nm is sandwiched between superconducting electrodes, it attains zero resistance at low temperature. For even longer wires, the transition to incomplete superconductivity via this (long range) proximity effect is foreshadowed by a large resistance peak (1). On the other hand when Zn nanowires of 40 nm diameter are contacted by superconducting electrodes, their superconductivity is unexpectedly suppressed (2). Worked supported by Penn State MRSEC.

  11. Oropharyngeal dysphagia in amyotrophic lateral sclerosis alters quality of life.

    PubMed

    Paris, G; Martinaud, O; Petit, A; Cuvelier, A; Hannequin, D; Roppeneck, P; Verin, E

    2013-03-01

    Dysphagia is one of the most important complications encountered in amyotrophic lateral sclerosis (ALS). Our aim was to determine whether oropharyngeal dysphagia impacted the quality of life (QoL) of patients with ALS. Thirty consecutive patients were recruited (31-82 years, 18 men). Swallowing function was evaluated using a standardised videofluoroscopic barium swallow. All the patients completed a specific questionnaire on quality of life in dysphagia (SWAL-QoL) immediately after the videofluoroscopy. The results of dysphagia outcome severity scale separated 14 patients with oropharyngeal dysphagia and 16 with normal swallowing function. There was no difference in the average age, weight and body mass index of the two groups (dysphagic patients: 68 ± 11 kg versus non-dysphagic patients: 69 ± 14 kg). Most of the dysphagic patients had a bulbar affection based on their Norris scores which determine the importance of cranial nerves illness (20 ± 8), significantly lower than those of the non-dysphagic patients (35 ± 5) (P < 0·0001). There was no difference in the neurological peripheral symptoms evaluated by Amyotrophic Lateral Sclerosis Functional Rating Scale scores (dysphagic patients: 26 ± 7 versus non-dysphagic patients: 27 ± 8) (ns). The swallowing quality of life questionnaire revealed that the dysphagic patients had significant burden (P < 0·001). They were affected by the necessity to applied a food selection (P < 0·01), by the increase in eating duration (P < 0·05) and described a decrease in eating desire (P < 0·05). They complained of fear regarding the risk of dysphagia (P < 0·05). They also described difficulties with oral communication (P < 0·001). All of those complained about dysphagia which impacted directly mental health (P < 0·05) and social life (P < 0·05). In conclusion, oropharyngeal dysphagia is a common symptom accompanying ALS, which alters the patient's QoL, especially social health.

  12. Central blood pressure estimation by using N-point moving average method in the brachial pulse wave.

    PubMed

    Sugawara, Rie; Horinaka, Shigeo; Yagi, Hiroshi; Ishimura, Kimihiko; Honda, Takeharu

    2015-05-01

    Recently, a method of estimating the central systolic blood pressure (C-SBP) using an N-point moving average method in the radial or brachial artery waveform has been reported. Then, we investigated the relationship between the C-SBP estimated from the brachial artery pressure waveform using the N-point moving average method and the C-SBP measured invasively using a catheter. C-SBP using a N/6 moving average method from the scaled right brachial artery pressure waveforms using VaSera VS-1500 was calculated. This estimated C-SBP was compared with the invasively measured C-SBP within a few minutes. In 41 patients who underwent cardiac catheterization (mean age: 65 years), invasively measured C-SBP was significantly lower than right cuff-based brachial BP (138.2 ± 26.3 vs 141.0 ± 24.9 mm Hg, difference -2.78 ± 1.36 mm Hg, P = 0.048). The cuff-based SBP was significantly higher than invasive measured C-SBP in subjects with younger than 60 years old. However, the estimated C-SBP using a N/6 moving average method from the scaled right brachial artery pressure waveforms and the invasively measured C-SBP did not significantly differ (137.8 ± 24.2 vs 138.2 ± 26.3 mm Hg, difference -0.49 ± 1.39, P = 0.73). N/6-point moving average method using the non-invasively acquired brachial artery waveform calibrated by the cuff-based brachial SBP was an accurate, convenient and useful method for estimating C-SBP. Thus, C-SBP can be estimated simply by applying a regular arm cuff, which is greatly feasible in the practical medicine.

  13. Riluzole exerts central and peripheral modulating effects in amyotrophic lateral sclerosis.

    PubMed

    Vucic, Steve; Lin, Cindy Shin-Yi; Cheah, Benjamin C; Murray, Jenna; Menon, Parvathi; Krishnan, Arun V; Kiernan, Matthew C

    2013-05-01

    Riluzole, a benzothiazole derivative, has been shown to be effective in prolonging survival in amyotrophic lateral sclerosis. The mechanisms by which riluzole exerts neuroprotective effects in amyotrophic lateral sclerosis remains to be fully elucidated, although inhibition of glutamatergic transmission and modulation of Na+ channel function have been proposed. In an attempt to determine the mechanisms by which riluzole exerts neuroprotective effects, in particular to dissect the relative contributions of inhibition of glutamatergic transmission and Na+ channel modulation, the present study utilized a combination of cortical and peripheral axonal excitability approaches to monitor changes in excitability and function in patients with amyotrophic lateral sclerosis. Cortical assessment was undertaken by utilising the threshold tracking transcranial magnetic stimulation (TMS) technique and combined with peripheral axonal excitability studies in 25 patients with amyotrophic lateral sclerosis. Studies were performed at baseline and repeated when patients were receiving riluzole 100 mg/day. At the time of second testing all patients were tolerating the medication well. Motor evoked potential and compound muscle action potential responses were recorded over the abductor pollicis brevis muscle. At baseline, features of cortical hyperexcitability were evident in patients with amyotrophic lateral sclerosis, indicated by marked reduction in short interval intracortical inhibition (P < 0.001) and cortical silent period duration (P < 0.001), as well as an increase in the motor evoked potential amplitude (P < 0.01). Riluzole therapy partially normalized cortical excitability by significantly increasing short interval intracortical inhibition (short interval intracortical inhibitionbaseline 0.5 ± 1.8%; short interval intracortical inhibitionON riluzole 7.9 ± 1.7%, P < 0.01). In contrast, riluzole did not exert any modulating effect on cortical silent period duration (P = 0

  14. Lockhart Clarke’s contribution to the description of amyotrophic lateral sclerosis

    PubMed Central

    Turner, Martin R.; Swash, Michael; Ebers, George C.

    2011-01-01

    The definition of the clinicopathological entity of amyotrophic lateral sclerosis evolved over half a century. Although the definitive term amyotrophic lateral sclerosis that acknowledged both upper and lower motor neuron involvement was attributed to Jean-Martin Charcot in 1874, his initial case was published nearly a decade earlier; and it is accepted that, from at least the 1830s, several others (including Charles Bell, François-Amilcar Aran and Jean Cruveilhier) had already recognized a progressive lower motor neuron-only syndrome within a broader, clinically-defined group of disorders, termed progressive muscular atrophy. Although William Gowers first grouped the three phenotypes of amyotrophic lateral sclerosis, progressive muscular atrophy and progressive bulbar palsy together as part of the same syndrome, the term motor neuron disease, as an over-arching label, was not suggested until nearly a century later by W. Russell Brain. Augustus Jacob Lockhart Clarke (1817–80) is best known for his descriptions of spinal cord anatomy. However, in two detailed case reports from the 1860s, he carried out rigorous post-mortem neuropathological studies of what appear to be classical cases of amyotrophic lateral sclerosis. Furthermore, he recognized the additional involvement of the corticospinal tracts that distinguished this from progressive muscular atrophy. Several aspects of the exquisite clinical histories documented as part of both studies, one by Charles Bland Radcliffe, resonate with contemporary debates concerning the evolution of disease in amyotrophic lateral sclerosis. These ‘past masters’ still have much to teach us. PMID:20576696

  15. Defective cholesterol metabolism in amyotrophic lateral sclerosis[S

    PubMed Central

    Abdel-Khalik, Jonas; Yutuc, Eylan; Crick, Peter J.; Gustafsson, Jan-Åke; Warner, Margaret; Roman, Gustavo; Talbot, Kevin; Gray, Elizabeth; Turner, Martin R.; Wang, Yuqin

    2017-01-01

    As neurons die, cholesterol is released in the central nervous system (CNS); hence, this sterol and its metabolites may represent a biomarker of neurodegeneration, including in amyotrophic lateral sclerosis (ALS), in which altered cholesterol levels have been linked to prognosis. More than 40 different sterols were quantified in serum and cerebrospinal fluid (CSF) from ALS patients and healthy controls. In CSF, the concentration of cholesterol was found to be elevated in ALS samples. When CSF metabolite levels were normalized to cholesterol, the cholesterol metabolite 3β,7α-dihydroxycholest-5-en-26-oic acid, along with its precursor 3β-hydroxycholest-5-en-26-oic acid and product 7α-hydroxy-3-oxocholest-4-en-26-oic acid, were reduced in concentration, whereas metabolites known to be imported from the circulation into the CNS were not found to differ in concentration between groups. Analysis of serum revealed that (25R)26-hydroxycholesterol, the immediate precursor of 3β-hydroxycholest-5-en-26-oic acid, was reduced in concentration in ALS patients compared with controls. We conclude that the acidic branch of bile acid biosynthesis, known to be operative in-part in the brain, is defective in ALS, leading to a failure of the CNS to remove excess cholesterol, which may be toxic to neuronal cells, compounded by a reduction in neuroprotective 3β,7α-dihydroxycholest-5-en-26-oic acid. PMID:27811233

  16. Widespread temporo-occipital lobe dysfunction in amyotrophic lateral sclerosis

    PubMed Central

    Loewe, Kristian; Machts, Judith; Kaufmann, Jörn; Petri, Susanne; Heinze, Hans-Jochen; Borgelt, Christian; Harris, Joseph Allen; Vielhaber, Stefan; Schoenfeld, Mircea Ariel

    2017-01-01

    Recent studies suggest that amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) lie on a single clinical continuum. However, previous neuroimaging studies have found only limited involvement of temporal lobe regions in ALS. To better delineate possible temporal lobe involvement in ALS, the present study aimed to examine changes in functional connectivity across the whole brain, particularly with regard to extra-motor regions, in a group of 64 non-demented ALS patients and 38 healthy controls. To assess between-group differences in connectivity, we computed edge-level statistics across subject-specific graphs derived from resting-state functional MRI data. In addition to expected ALS-related decreases in functional connectivity in motor-related areas, we observed extensive changes in connectivity across the temporo-occipital cortex. Although ALS patients with comorbid FTD were deliberately excluded from this study, the pattern of connectivity alterations closely resembles patterns of cerebral degeneration typically seen in FTD. This evidence for subclinical temporal dysfunction supports the idea of a common pathology in ALS and FTD. PMID:28067298

  17. Genetic correlation between amyotrophic lateral sclerosis and schizophrenia

    PubMed Central

    McLaughlin, Russell L.; Schijven, Dick; van Rheenen, Wouter; van Eijk, Kristel R.; O'Brien, Margaret; Kahn, René S.; Ophoff, Roel A.; Goris, An; Bradley, Daniel G.; Al-Chalabi, Ammar; van den Berg, Leonard H.; Luykx, Jurjen J.; Hardiman, Orla; Veldink, Jan H.; Shatunov, Aleksey; Dekker, Annelot M.; Diekstra, Frank P.; Pulit, Sara L.; van der Spek, Rick A. A.; van Doormaal, Perry T. C.; Sproviero, William; Jones, Ashley R.; Nicholson, Garth A.; Rowe, Dominic B.; Pamphlett, Roger; Kiernan, Matthew C.; Bauer, Denis; Kahlke, Tim; Williams, Kelly; Eftimov, Filip; Fogh, Isabella; Ticozzi, Nicola; Lin, Kuang; Millecamps, Stéphanie; Salachas, François; Meininger, Vincent; de Carvalho, Mamede; Pinto, Susana; Mora, Jesus S.; Rojas-García, Ricardo; Polak, Meraida; Chandran, Siddharthan; Colville, Shuna; Swingler, Robert; Morrison, Karen E.; Shaw, Pamela J.; Hardy, John; Orrell, Richard W.; Pittman, Alan; Sidle, Katie; Fratta, Pietro; Malaspina, Andrea; Petri, Susanne; Abdulla, Susanna; Drepper, Carsten; Sendtner, Michael; Meyer, Thomas; Wiedau-Pazos, Martina; Lomen-Hoerth, Catherine; Van Deerlin, Vivianna M.; Trojanowski, John Q.; Elman, Lauren; McCluskey, Leo; Basak, Nazli; Meitinger, Thomas; Lichtner, Peter; Blagojevic-Radivojkov, Milena; Andres, Christian R.; Maurel, Cindy; Bensimon, Gilbert; Landwehrmeyer, Bernhard; Brice, Alexis; Payan, Christine A. M.; Saker-Delye, Safa; Dürr, Alexandra; Wood, Nicholas; Tittmann, Lukas; Lieb, Wolfgang; Franke, Andre; Rietschel, Marcella; Cichon, Sven; Nöuthen, Markus M.; Amouyel, Philippe; Tzourio, Christophe; Dartigues, Jean- François; Uitterlinden, Andre G.; Rivadeneira, Fernando; Estrada, Karol; Hofman, Albert; Curtis, Charles; van der Kooi, Anneke J.; de Visser, Marianne; Weber, Markus; Shaw, Christopher E.; Smith, Bradley N.; Pansarasa, Orietta; Cereda, Cristina; Del Bo, Roberto; Comi, Giacomo P.; D'Alfonso, Sandra; Bertolin, Cinzia; Sorarù, Gianni; Mazzini, Letizia; Pensato, Viviana; Gellera, Cinzia; Tiloca, Cinzia; Ratti, Antonia; Calvo, Andrea; Moglia, Cristina; Brunetti, Maura; Arcuti, Simon; Capozzo, Rosa; Zecca, Chiara; Lunetta, Christian; Penco, Silvana; Riva, Nilo; Padovani, Alessandro; Filosto, Massimiliano; Blair, Ian; Leigh, P Nigel; Casale, Federico; Chio, Adriano; Beghi, Ettore; Pupillo, Elisabetta; Tortelli, Rosanna; Logroscino, Giancarlo; Powell, John; Ludolph, Albert C.; Weishaupt, Jochen H.; Robberecht, Wim; Van Damme, Philip; Brown, Robert H.; Glass, Jonathan; Landers, John E.; Andersen, Peter M.; Corcia, Philippe; Vourc'h, Patrick; Silani, Vincenzo; van Es, Michael A.; Pasterkamp, R Jeroen; Lewis, Cathryn M.; Breen, Gerome; Ripke, Stephan; Neale, Benjamin M.; Corvin, Aiden; Walters, James T. R.; Farh, Kai-How; Holmans, Peter A; Lee, Phil; Bulik-Sullivan, Brendan; Collier, David A.; Huang, Hailiang; Pers, Tune H.; Agartz, Ingrid; Agerbo, Esben; Albus, Margot; Alexander, Madeline; Amin, Farooq; Bacanu, Silviu A.; Begemann, Martin; Belliveau, Richard A.; Bene, Judit; Bergen, Sarah E.; Bevilacqua, Elizabeth; Bigdeli, Tim B.; Black, Donald W.; Bruggeman, Richard; Buccola, Nancy G.; Buckner, Randy L.; Byerley, William; Cahn, Wiepke; Cai, Guiqing; Campion, Dominique; Cantor, Rita M.; Carr, Vaughan J.; Carrera, Noa; Catts, Stanley V.; Chambert, Kimberley D.; Chan, Raymond C. K.; Chan, Ronald Y. L.; Chen, Eric Y. H.; Cheng, Wei; Cheung, Eric F. C.; Chong, Siow Ann; Cloninger, C Robert; Cohen, David; Cohen, Nadine; Cormican, Paul; Craddock, Nick; Crowley, James J.; Curtis, David; Davidson, Michael; Davis, Kenneth L.; Degenhardt, Franziska; Del Favero, Jurgen; Demontis, Ditte; Dikeos, Dimitris; Dinan, Timothy; Djurovic, Srdjan; Donohoe, Gary; Drapeau, Elodie; Duan, Jubao; Dudbridge, Frank; Durmishi, Naser; Eichhammer, Peter; Eriksson, Johan; Escott-Price, Valentina; Essioux, Laurent; Fanous, Ayman H.; Farrell, Martilias S.; Frank, Josef; Franke, Lude; Freedman, Robert; Freimer, Nelson B.; Friedl, Marion; Friedman, Joseph I.; Fromer, Menachem; Genovese, Giulio; Georgieva, Lyudmila; Giegling, Ina; Giusti-Rodríguez, Paola; Godard, Stephanie; Goldstein, Jacqueline I.; Golimbet, Vera; Gopal, Srihari; Gratten, Jacob; de Haan, Lieuwe; Hammer, Christian; Hamshere, Marian L.; Hansen, Mark; Hansen, Thomas; Haroutunian, Vahram; Hartmann, Annette M.; Henskens, Frans A.; Herms, Stefan; Hirschhorn, Joel N.; Hoffmann, Per; Hofman, Andrea; Hollegaard, Mads V.; Hougaard, David M.; Ikeda, Masashi; Joa, Inge; Julià, Antonio; Kalaydjieva, Luba; Karachanak-Yankova, Sena; Karjalainen, Juha; Kavanagh, David; Keller, Matthew C.; Kennedy, James L.; Khrunin, Andrey; Kim, Yunjung; Klovins, Janis; Knowles, James A.; Konte, Bettina; Kucinskas, Vaidutis; Kucinskiene, Zita Ausrele; Kuzelova-Ptackova, Hana; Kähler, Anna K.; Laurent, Claudine; Lee, Jimmy; Lee, S Hong; Legge, Sophie E.; Lerer, Bernard; Li, Miaoxin; Li, Tao; Liang, Kung-Yee; Lieberman, Jeffrey; Limborska, Svetlana; Loughland, Carmel M.; Lubinski, Jan; Lönnqvist, Jouko; Macek, Milan; Magnusson, Patrik K. E.; Maher, Brion S.; Maier, Wolfgang; Mallet, Jacques; Marsal, Sara; Mattheisen, Manuel; Mattingsdal, Morten; McCarley, Robert W.; McDonald, Colm; McIntosh, Andrew M.; Meier, Sandra; Meijer, Carin J.; Melegh, Bela; Melle, Ingrid; Mesholam-Gately, Raquelle I.; Metspalu, Andres; Michie, Patricia T.; Milani, Lili; Milanova, Vihra; Mokrab, Younes; Morris, Derek W.; Mors, Ole; Murphy, Kieran C.; Murray, Robin M.; Myin-Germeys, Inez; Müller-Myhsok, Bertram; Nelis, Mari; Nenadic, Igor; Nertney, Deborah A.; Nestadt, Gerald; Nicodemus, Kristin K.; Nikitina-Zake, Liene; Nisenbaum, Laura; Nordin, Annelie; O'Callaghan, Eadbhard; O'Dushlaine, Colm; O'Neill, F Anthony; Oh, Sang-Yun; Olincy, Ann; Olsen, Line; Van Os, Jim; Pantelis, Christos; Papadimitriou, George N.; Papiol, Sergi; Parkhomenko, Elena; Pato, Michele T.; Paunio, Tiina; Pejovic-Milovancevic, Milica; Perkins, Diana O.; Pietiläinen, Olli; Pimm, Jonathan; Pocklington, Andrew J.; Price, Alkes; Pulver, Ann E.; Purcell, Shaun M.; Quested, Digby; Rasmussen, Henrik B.; Reichenberg, Abraham; Reimers, Mark A.; Richards, Alexander L.; Roffman, Joshua L.; Roussos, Panos; Ruderfer, Douglas M.; Salomaa, Veikko; Sanders, Alan R.; Schall, Ulrich; Schubert, Christian R.; Schulze, Thomas G.; Schwab, Sibylle G.; Scolnick, Edward M.; Scott, Rodney J.; Seidman, Larry J.; Shi, Jianxin; Sigurdsson, Engilbert; Silagadze, Teimuraz; Silverman, Jeremy M.; Sim, Kang; Slominsky, Petr; Smoller, Jordan W.; So, Hon-Cheong; Spencer, Chris C. A.; Stahl, Eli A.; Stefansson, Hreinn; Steinberg, Stacy; Stogmann, Elisabeth; Straub, Richard E.; Strengman, Eric; Strohmaier, Jana; Stroup, T Scott; Subramaniam, Mythily; Suvisaari, Jaana; Svrakic, Dragan M.; Szatkiewicz, Jin P.; Söderman, Erik; Thirumalai, Srinivas; Toncheva, Draga; Tosato, Sarah; Veijola, Juha; Waddington, John; Walsh, Dermot; Wang, Dai; Wang, Qiang; Webb, Bradley T.; Weiser, Mark; Wildenauer, Dieter B.; Williams, Nigel M.; Williams, Stephanie; Witt, Stephanie H.; Wolen, Aaron R.; Wong, Emily H. M.; Wormley, Brandon K.; Xi, Hualin Simon; Zai, Clement C.; Zheng, Xuebin; Zimprich, Fritz; Wray, Naomi R.; Stefansson, Kari; Visscher, Peter M.; Adolfsson, Rolf; Andreassen, Ole A.; Blackwood, Douglas H. R.; Bramon, Elvira; Buxbaum, Joseph D.; Børglum, Anders D.; Darvasi, Ariel; Domenici, Enrico; Ehrenreich, Hannelore; Esko, Tõnu; Gejman, Pablo V.; Gill, Michael; Gurling, Hugh; Hultman, Christina M.; Iwata, Nakao; Jablensky, Assen V.; Jönsson, Erik G.; Kendler, Kenneth S.; Kirov, George; Knight, Jo; Lencz, Todd; Levinson, Douglas F.; Li, Qingqin S.; Liu, Jianjun; Malhotra, Anil K.; McCarroll, Steven A.; McQuillin, Andrew; Moran, Jennifer L.; Mortensen, Preben B.; Mowry, Bryan J.; Owen, Michael J.; Palotie, Aarno; Pato, Carlos N.; Petryshen, Tracey L.; Posthuma, Danielle; Riley, Brien P.; Rujescu, Dan; Sham, Pak C.; Sklar, Pamela; St Clair, David; Weinberger, Daniel R.; Wendland, Jens R.; Werge, Thomas; Daly, Mark J.; Sullivan, Patrick F.; O'Donovan, Michael C.

    2017-01-01

    We have previously shown higher-than-expected rates of schizophrenia in relatives of patients with amyotrophic lateral sclerosis (ALS), suggesting an aetiological relationship between the diseases. Here, we investigate the genetic relationship between ALS and schizophrenia using genome-wide association study data from over 100,000 unique individuals. Using linkage disequilibrium score regression, we estimate the genetic correlation between ALS and schizophrenia to be 14.3% (7.05–21.6; P=1 × 10−4) with schizophrenia polygenic risk scores explaining up to 0.12% of the variance in ALS (P=8.4 × 10−7). A modest increase in comorbidity of ALS and schizophrenia is expected given these findings (odds ratio 1.08–1.26) but this would require very large studies to observe epidemiologically. We identify five potential novel ALS-associated loci using conditional false discovery rate analysis. It is likely that shared neurobiological mechanisms between these two disorders will engender novel hypotheses in future preclinical and clinical studies. PMID:28322246

  18. Non-invasive ventilation in amyotrophic lateral sclerosis.

    PubMed

    Vrijsen, Bart; Testelmans, Dries; Belge, Catharina; Robberecht, Wim; Van Damme, Philip; Buyse, Bertien

    2013-03-01

    Abstract Non-invasive ventilation (NIV) is widely used to improve alveolar hypoventilation in amyotrophic lateral sclerosis. Several studies indicate a better survival when NIV is used, certainly in patients with none to moderate bulbar dysfunction. Data on quality of life (QoL) are rather disputable. Overall QoL is shown to be equivalent in patients with or without NIV, although health-related QoL is shown to be increased in patients with none to moderate bulbar dysfunction. NIV improves sleep quality, although patient-ventilator asynchronies are demonstrated. FVC < 50%, seated or supine, has been widely applied as threshold to initiate NIV. Today, measurements of respiratory muscle strength, nocturnal gas exchange and symptomatic complaints are used as indicators to start NIV. Being compliant with NIV therapy increases QoL and survival. Cough augmentation has an important role in appropriate NIV. Patients have today more technical options and patients with benefit from these advances are growing in number. Tracheal ventilation needs to be discussed when NIV seems impossible or becomes insufficient.

  19. INSPIRATIonAL--INSPIRAtory muscle training in amyotrophic lateral sclerosis.

    PubMed

    Cheah, Benjamin C; Boland, Robert A; Brodaty, Nina E; Zoing, Margie C; Jeffery, Sandra E; McKenzie, David K; Kiernan, Matthew C

    2009-01-01

    Respiratory impairment, due to respiratory muscle weakness, is a major cause of morbidity and mortality in patients with amyotrophic lateral sclerosis/motor neuron disease (ALS/MND). Threshold loading may strengthen the inspiratory muscles and thereby improve patient prognosis. A phase II, double-blind, randomized-controlled trial was undertaken to determine whether a 12-week inspiratory muscle training programme attenuated the decline in respiratory function and inspiratory muscle strength in patients with ALS/MND. Nine patients were randomized to inspiratory muscle training and 10 to sham training. Primary endpoints were respiratory function (forced vital capacity, vital capacity), lung volumes and inspiratory muscle strength. Patients were assessed before, during and immediately after a 12-week training period, and at eight weeks follow-up. While improvements in inspiratory muscle strength were observed in both treatment arms, there was a non-significant increase in maximum inspiratory pressure of 6.1% in the experimental group compared to controls (standard error of mean, 6.93%; 95% confidence interval -8.58 -20.79; p=0.39). The gains in inspiratory muscle strength were partially reversed during a period of training cessation. In conclusion, inspiratory muscle training may potentially strengthen the inspiratory muscles and slow the decline in respiratory function in patients with ALS/MND.

  20. CCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia

    PubMed Central

    Williams, Kelly L.; Topp, Simon; Yang, Shu; Smith, Bradley; Fifita, Jennifer A.; Warraich, Sadaf T.; Zhang, Katharine Y.; Farrawell, Natalie; Vance, Caroline; Hu, Xun; Chesi, Alessandra; Leblond, Claire S.; Lee, Albert; Rayner, Stephanie L.; Sundaramoorthy, Vinod; Dobson-Stone, Carol; Molloy, Mark P.; van Blitterswijk, Marka; Dickson, Dennis W.; Petersen, Ronald C.; Graff-Radford, Neill R.; Boeve, Bradley F.; Murray, Melissa E.; Pottier, Cyril; Don, Emily; Winnick, Claire; McCann, Emily P.; Hogan, Alison; Daoud, Hussein; Levert, Annie; Dion, Patrick A.; Mitsui, Jun; Ishiura, Hiroyuki; Takahashi, Yuji; Goto, Jun; Kost, Jason; Gellera, Cinzia; Gkazi, Athina Soragia; Miller, Jack; Stockton, Joanne; Brooks, William S.; Boundy, Karyn; Polak, Meraida; Muñoz-Blanco, José Luis; Esteban-Pérez, Jesús; Rábano, Alberto; Hardiman, Orla; Morrison, Karen E.; Ticozzi, Nicola; Silani, Vincenzo; de Belleroche, Jacqueline; Glass, Jonathan D.; Kwok, John B. J.; Guillemin, Gilles J.; Chung, Roger S.; Tsuji, Shoji; Brown, Robert H.; García-Redondo, Alberto; Rademakers, Rosa; Landers, John E.; Gitler, Aaron D.; Rouleau, Guy A.; Cole, Nicholas J.; Yerbury, Justin J.; Atkin, Julie D.; Shaw, Christopher E.; Nicholson, Garth A.; Blair, Ian P.

    2016-01-01

    Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are overlapping, fatal neurodegenerative disorders in which the molecular and pathogenic basis remains poorly understood. Ubiquitinated protein aggregates, of which TDP-43 is a major component, are a characteristic pathological feature of most ALS and FTD patients. Here we use genome-wide linkage analysis in a large ALS/FTD kindred to identify a novel disease locus on chromosome 16p13.3. Whole-exome sequencing identified a CCNF missense mutation at this locus. Interrogation of international cohorts identified additional novel CCNF variants in familial and sporadic ALS and FTD. Enrichment of rare protein-altering CCNF variants was evident in a large sporadic ALS replication cohort. CCNF encodes cyclin F, a component of an E3 ubiquitin–protein ligase complex (SCFCyclin F). Expression of mutant CCNF in neuronal cells caused abnormal ubiquitination and accumulation of ubiquitinated proteins, including TDP-43 and a SCFCyclin F substrate. This implicates common mechanisms, linked to protein homeostasis, underlying neuronal degeneration. PMID:27080313

  1. Genetic correlation between amyotrophic lateral sclerosis and schizophrenia.

    PubMed

    McLaughlin, Russell L; Schijven, Dick; van Rheenen, Wouter; van Eijk, Kristel R; O'Brien, Margaret; Kahn, René S; Ophoff, Roel A; Goris, An; Bradley, Daniel G; Al-Chalabi, Ammar; van den Berg, Leonard H; Luykx, Jurjen J; Hardiman, Orla; Veldink, Jan H

    2017-03-21

    We have previously shown higher-than-expected rates of schizophrenia in relatives of patients with amyotrophic lateral sclerosis (ALS), suggesting an aetiological relationship between the diseases. Here, we investigate the genetic relationship between ALS and schizophrenia using genome-wide association study data from over 100,000 unique individuals. Using linkage disequilibrium score regression, we estimate the genetic correlation between ALS and schizophrenia to be 14.3% (7.05-21.6; P=1 × 10(-4)) with schizophrenia polygenic risk scores explaining up to 0.12% of the variance in ALS (P=8.4 × 10(-7)). A modest increase in comorbidity of ALS and schizophrenia is expected given these findings (odds ratio 1.08-1.26) but this would require very large studies to observe epidemiologically. We identify five potential novel ALS-associated loci using conditional false discovery rate analysis. It is likely that shared neurobiological mechanisms between these two disorders will engender novel hypotheses in future preclinical and clinical studies.

  2. The split hand syndrome in amyotrophic lateral sclerosis.

    PubMed

    Eisen, Andrew; Kuwabara, Satoshi

    2012-04-01

    In amyotrophic lateral sclerosis (ALS), hand muscle wasting preferentially affects the 'thenar (lateral) hand', including the abductor pollicis brevis (APB) and first dorsal interosseous (FDI) muscles, with relative sparing of the hypothenar muscles (the abductor digiti minimi (ADM)). This peculiar pattern of dissociated atrophy of the intrinsic hand muscles is termed the 'split hand' and is rarely seen in diseases other than ALS. The muscles involved in the split hand are innervated through the same spinal segments (C8 and T1), and FDI and ADM, which are differentially affected, are both ulnar nerve innervated. The physiological mechanisms underlying the split hand in ALS are incompletely understood but both cortical and spinal/peripheral mechanisms are probably involved. Motor potentials evoked by magnetic stimulation are significantly smaller when recorded from the thenar complex, compared with the hypothenar muscles, supporting a cortical mechanism. But peripheral axonal excitability studies have suggested that APB/FDI motor axons have more prominent persistent sodium currents than ADM axons, leading to higher axonal excitability and thereby more ready degeneration. Pincer or precision grip is vital to human hand function, and frequent use of thenar complex muscles may lead to greater oxidative stress and metabolic demands at both upper and lower motoneurons innervating the APB and FDI. The split hand is a useful diagnostic sign in early ALS, and recent objective studies indicate that the sign has a high degree of specificity.

  3. Peripherin partially localizes in Bunina bodies in amyotrophic lateral sclerosis.

    PubMed

    Mizuno, Yuji; Fujita, Yukio; Takatama, Masamitsu; Okamoto, Koichi

    2011-03-15

    Peripherin is a type III intermediate filament protein expressed with low levels in spinal motor neurons. Amyotrophic lateral sclerosis (ALS) is characterized by the presence of Bunina bodies, skein-like inclusions, and Lewy body-like inclusions (LBLIs) in the remaining anterior horn cells, where the first and third structures are detected by Hematoxylin-Eosin (H & E) staining. We examined paraffin sections of lumbar spinal cords from six ALS patients, using H & E staining and immunostaining for human peripherin. The results demonstrated that there were a total of 73 anterior horn cells containing one or more Bunina bodies, and that twelve of these cells (approximately 16.4%) demonstrated peripherin-positive Bunina bodies. In fact, some part of chain-like Bunina bodies showed peripherin-positive reaction, although there were a much higher number of non-immunoreacitive Bunina bodies in each neuron. LBLIs were clearly immunostained for peripherin corresponding to the core, while some of them showed different types of immunoreactivities due to oblique cutting of inclusions. Our findings suggest that although the mechanisms underlying peripherin co-localization in Bunina bodies are unknown, peripherin could be involved in forming these inclusions. Furthermore, following cystatin C and transferrin, peripherin is the third most prevalent protein that partially localizes in Bunina bodies.

  4. A(a)LS: Ammonia-induced amyotrophic lateral sclerosis

    PubMed Central

    Parekh, Bhavin

    2015-01-01

    Amyotrophic lateral sclerosis (ALS) is a dreadful, devastating and incurable motor neuron disease. Aetiologically, it is a multigenic, multifactorial and multiorgan disease. Despite intense research, ALS pathology remains unexplained. Following extensive literature review, this paper posits a new integrative explanation. This framework proposes that ammonia neurotoxicity is a main player in ALS pathogenesis. According to this explanation, a combination of impaired ammonia removal— mainly because of impaired hepatic urea cycle dysfunction—and increased ammoniagenesis— mainly because of impaired glycolytic metabolism in fast twitch skeletal muscle—causes chronic hyperammonia in ALS. In the absence of neuroprotective calcium binding proteins (calbindin, calreticulin and parvalbumin), elevated ammonia—a neurotoxin—damages motor neurons. Ammonia-induced motor neuron damage occurs through multiple mechanisms such as macroautophagy-endolysosomal impairment, endoplasmic reticulum (ER) stress, CDK5 activation, oxidative/nitrosative stress, neuronal hyperexcitability and neuroinflammation. Furthermore, the regional pattern of calcium binding proteins’ loss, owing to either ER stress and/or impaired oxidative metabolism, determines clinical variability of ALS. Most importantly, this new framework can be generalised to explain other neurodegenerative disorders such as Huntington’s disease and Parkinsonism. PMID:27785351

  5. Identification of new mutations in familial amyotrophic lateral sclerosis

    SciTech Connect

    Siddique, T.; Deng, H.X.; Hentati, A.

    1994-09-01

    Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease due to motor neuron death in the cortex, brain stem and spinal cord. Ten percent of ALS cases are familial (FALS). Previously a subset of FALS families have been mapped to chromosome 21 and mutations in the Cu,Zn superoxide dismutase gene have been identified in those families. Nineteen different mutations at 16 distinct codons have been documented, of which 12 different mutations were identified in our 29 FALS families. These mutations account for about twenty percent of all FALS families screened. The mutations identified in our FALS families are A4V, A4T, G37R, G41D, H43R, G85R, G93A, E100G, L106V, I113T, L144F, and V148G. Mutation A4V is the most frequent one which occurred in 14 out of our 29 FALS families. In further screening of our FALS families, two new mutations, V14M and L84V, have been identified. Thus a total of 21 different mutations at 18 distinct codon sites have been identified in SOD1.

  6. Emerging molecular biomarker targets for amyotrophic lateral sclerosis.

    PubMed

    Costa, Júlia; de Carvalho, Mamede

    2016-04-01

    Amyotrophic lateral sclerosis is a rapidly progressive neurodegenerative disease that affects upper (UMN) and lower motor (LMN) neurons. It is associated with a short survival and there is no effective treatment, in spite of a large number of clinical trials. Strong efforts have been made to identify novel disease biomarkers to support diagnosis, provide information on prognosis, to measure disease progression in trials and increase our knowledge on disease pathogenesis. Electromyography by testing the function of the LMN can be used as a biomarker of its dysfunction. A number of electrophysiological and neuroimaging methods have been explored to identify a reliable marker of UMN degeneration. Recently, strong evidence from independent groups, large cohorts of patients and multicenter studies indicate that neurofilaments are very promising diagnostic biomarkers, in particular cerebrospinal fluid and blood levels of phosphoneurofilament heavy chain and neurofilament light chain. Furthermore, their increased levels are associated with poor prognosis. Additional studies have been performed aiming to identify other biomarkers, which alone or in combination with neurofilaments could increase the sensitivity and the specificity of the assays. Emerging molecular marker targets are being discovered, but more studies with standardized methods are required in larger cohorts of ALS patients.

  7. The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis.

    PubMed

    Loeffler, Jean-Philippe; Picchiarelli, Gina; Dupuis, Luc; Gonzalez De Aguilar, Jose-Luis

    2016-03-01

    Amyotrophic lateral sclerosis (ALS) is a fatal adult-onset disease primarily characterized by upper and lower motor neuron degeneration, muscle wasting and paralysis. It is increasingly accepted that the pathological process leading to ALS is the result of multiple disease mechanisms that operate within motor neurons and other cell types both inside and outside the central nervous system. The implication of skeletal muscle has been the subject of a number of studies conducted on patients and related animal models. In this review, we describe the features of ALS muscle pathology and discuss on the contribution of muscle to the pathological process. We also give an overview of the therapeutic strategies proposed to alleviate muscle pathology or to deliver curative agents to motor neurons. ALS muscle mainly suffers from oxidative stress, mitochondrial dysfunction and bioenergetic disturbances. However, the way by which the disease affects different types of myofibers depends on their contractile and metabolic features. Although the implication of muscle in nourishing the degenerative process is still debated, there is compelling evidence suggesting that it may play a critical role. Detailed understanding of the muscle pathology in ALS could, therefore, lead to the identification of new therapeutic targets.

  8. Ethical considerations in the management of amyotrophic lateral sclerosis.

    PubMed

    Eisen, Andrew; Krieger, Charles

    2013-11-01

    This article examines some of the ethical concerns relevant for the management of amyotrophic lateral sclerosis (ALS). We emphasize the importance for providing a competent assessment of the clinical deficit to correctly identify the disease and to avoid incorrect diagnoses. Conveying the diagnosis to the patient and their family requires empathy and it is important to remain supportive and positive, even in the face of this incurable disease. The essence of care in ALS is to permit the patient to have optimal function for their level of ability. This may require the use of gastrostomy and non-invasive or permanent ventilation. Employment of a multi-disciplinary team will permit optimization of patient care to achieve a good quality of life for as long as possible. The patient should also be informed of the risks associated with unproven therapies and the risks and potential benefits of therapeutic trials. The wishes of patients in regard to gastrostomy, long-term ventilation and end-of life decisions must be considered in an unbiased fashion. Recent advances in the genetics of familial ALS (FALS) have demonstrated some overlap between FALS, sporadic ALS and fronto-temporal lobar dementia (FTLD). The interpretation and dissemination of the results of genetic testing although important can induce confusion, considerable anxiety and guilt in patients and their families and proper counseling is imperative.

  9. Endocannabinoids in Multiple Sclerosis and Amyotrophic Lateral Sclerosis.

    PubMed

    Pryce, Gareth; Baker, David

    2015-01-01

    There are numerous reports that people with multiple sclerosis (MS) have for many years been self-medicating with illegal street cannabis or more recently medicinal cannabis to alleviate the symptoms associated with MS and also amyotrophic lateral sclerosis (ALS). These anecdotal reports have been confirmed by data from animal models and more recently clinical trials on the ability of cannabinoids to alleviate limb spasticity, a common feature of progressive MS (and also ALS) and neurodegeneration. Experimental studies into the biology of the endocannabinoid system have revealed that cannabinoids have efficacy, not only in symptom relief but also as neuroprotective agents which may slow disease progression and thus delay the onset of symptoms. This review discusses what we now know about the endocannabinoid system as it relates to MS and ALS and also the therapeutic potential of cannabinoid therapeutics as disease-modifying or symptom control agents, as well as future therapeutic strategies including the potential for slowing disease progression in MS and ALS.

  10. Intraspinal Stem Cell Transplantation for Amyotrophic Lateral Sclerosis

    PubMed Central

    Chen, Kevin S.; Sakowski, Stacey A.; Feldman, Eva L.

    2015-01-01

    Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder in which the loss of upper and lower motor neurons produces progressive weakness and eventually death. In the decades since the approval of riluzole, the only FDA approved medication to moderately slow progression of ALS, no new therapeutics have arisen to alter the course of the disease. This is partly due to our incomplete understanding of the complex pathogenesis of motor neuron degeneration. Stem cells have emerged as an attractive option in treating ALS since they come armed with equally complex cellular machinery and may modulate the local microenvironment in many ways to rescue diseased motor neurons. While various stem cell types are being evaluated in preclinical and early clinical applications, here we review the preclinical strategies and advances supporting the recent clinical translation of neural progenitor cell therapy for ALS. Specifically, we focus on the use of spinal cord neural progenitor cells and the pipeline starting from preclinical studies to the designs of the Phase I and IIa clinical trials involving direct intraspinal transplantation in humans. PMID:26696091

  11. Longitudinal changes in discourse production in amyotrophic lateral sclerosis.

    PubMed

    Roberts-South, Angela; Findlater, Kate; Strong, Michael J; Orange, J B

    2012-02-01

    Amyotrophic lateral sclerosis (ALS) is a multisystem disease that significantly impacts communication as a function of changes in motor speech, cognition, and language skills. Although discourse tasks have been used to assess language in a variety of acquired disorders, little work to date has been published on changes in discourse in ALS and even less work has evaluated these changes with disease progression. In the present study, discourse samples (gained from a picture description task) as well as standardized language test measures obtained from 16 individuals with ALS without dementia and 12 healthy controls (collected over a duration of 24 months). Discourse samples were analyzed for both productivity and content. Results indicate that there were no differences for ALS versus controls for any of the standardized language tests. However, findings suggest that discourse analysis methods may be more sensitive for identifying subtle language deficits in ALS. Overall, discourse productivity appears less impaired than discourse content for individuals with ALS. Although there was a general trend for decline in language performance over the study duration, there was the suggestion of subgroups of language performance among ALS participants. The results suggest that subtle cognitive language deficits that affect discourse emerge early in ALS and progress with disease progression.

  12. Mitochondrial Disorders May Mimic Amyotrophic Lateral Sclerosis at Onset

    PubMed Central

    Finsterer, Josef; Zarrouk-Mahjoub, Sinda

    2016-01-01

    Similarities between a mitochondrial disorder (MID) and amyotrophic lateral sclerosis (ALS) fade with disease progression and the development of mitochondrial multiple organ dysfunction syndrome (MIMODS). However, with mild MIMODS, a MID may still be misinterpreted as ALS. We report a 48-year-old male who presented to the Neurological Hospital Rosenhügel, Vienna, Austria, in February 2001 with slowly progressive weakness, wasting and left upper limb fasciculations which spread to the shoulder girdle and lower limbs. Additionally, he developed tetraspasticity and bulbar involvement. He had been diagnosed with ALS a year previously due to electrophysiological investigations indicative of a chronic neurogenic lesion. However, a muscle biopsy revealed morphological features of a MID and a combined complex-II/III defect. Nerve conduction studies were performed over subsequent years until February 2011. This case demonstrates that MIDs may mimic ALS at onset and begin as a mono-organ disorder but develop into a multi-organ disease with long-term progression. A combined complex II/III defect may manifest with bulbar involvement. PMID:26909222

  13. Current view and perspectives in amyotrophic lateral sclerosis

    PubMed Central

    Mathis, Stéphane; Couratier, Philippe; Julian, Adrien; Corcia, Philippe; Le Masson, Gwendal

    2017-01-01

    Amyotrophic lateral sclerosis (ALS), identified as a distinct clinical entity by Charcot since the end of the nineteenth century, is a devastating and fatal neurodegenerative disorder that affects motor neurons in the brain, brainstem and spinal cord. Survival of patients with ALS is associated with several factors such as clinical phenotype, age at onset, gender, early presence of respiratory failure, weight loss and treatment with Riluzole (the only disease-modifying drug approved for this disease). Nowadays, there is still no curative treatment for ALS: palliative care and symptomatic treatment are therefore essential components in the management of these patients. Nevertheless, the scientific knowledge in the field of ALS motor neuron degeneration is growing, with the prospect of new treatments. Based on this physiopathological knowledge, several new therapeutic targets are being studied, involving various mechanisms such as excitotoxicity, neuroinflammation, mitochondrial dysfunction, oxidative stress, RNA metabolism and other attractive concepts. Moreover, it is also important to identify reliable biomarkers that will be essential components for future therapeutic development and study design in ALS. In this review, we present the main recent advances and promising therapeutics and biomarkers in the field of ALS.

  14. The Puzzling Case of Hyperexcitability in Amyotrophic Lateral Sclerosis

    PubMed Central

    Bae, Jong Seok; Simon, Neil G.; Menon, Parvathi; Vucic, Steve

    2013-01-01

    The development of hyperexcitability in amyotrophic lateral sclerosis (ALS) is a well-known phenomenon. Despite controversy as to the underlying mechanisms, cortical hyperexcitability appears to be closely related to the interplay between excitatory corticomotoneurons and inhibitory interneurons. Hyperexcitability is not a static phenomenon but rather shows a pattern of progression in a spatiotemporal aspect. Cortical hyperexcitability may serve as a trigger to the development of anterior horn cell degeneration through a 'dying forward' process. Hyperexcitability appears to develop during the early disease stages and gradually disappears in the advanced stages of the disease, linked to the destruction of corticomotorneuronal pathways. As such, a more precise interpretation of these unique processes may provide new insight regarding the pathophysiology of ALS and its clinical features. Recently developed technologies such as threshold tracking transcranial magnetic stimulation and automated nerve excitability tests have provided some clues about underlying pathophysiological processes linked to hyperexcitability. Additionally, these novel techniques have enabled clinicians to use the specific finding of hyperexcitability as a useful diagnostic biomarker, enabling clarification of various ALS-mimic syndromes, and the prediction of disease development in pre-symptomatic carriers of familial ALS. In terms of nerve excitability tests for peripheral nerves, an increase in persistent Na+ conductances has been identified as a major determinant of peripheral hyperexcitability in ALS, inversely correlated with the survival in ALS. As such, the present Review will focus primarily on the puzzling theory of hyperexcitability in ALS and summarize clinical and pathophysiological implications for current and future ALS research. PMID:23626643

  15. The pre-clinical discovery of Amyotrophic Lateral Sclerosis Drugs

    PubMed Central

    Glicksman, Marcie A.

    2012-01-01

    Introduction Amyotrophic Lateral Sclerosis, also referred to as Lou Gehrig’s disease is characterized by the progressive loss of cells in the brain and spinal cord that leads to debilitation and death in 3–5 years. Only one therapeutic drug, Riluzole, has been approved for ALS and that drug improves survival by 2–3 months. The need for new therapeutics, either that can postpone or slow the progression of the motor deficits and prolong survival, is still a strong unmet medical need. Areas Covered Although there are a number of drugs currently in clinical trials for ALS, this review provides an overview of the most promising biological targets and preclinical strategies that are currently being developed and deployed. The list of targets for ALS was compiled from a variety of websites including: individual companies that have ALS programs, and the author’s experience. Expert Opinion Progress is being made in the identification of possible new therapeutics for ALS with recent efforts in: understanding the genetic causes of the disease, susceptibility factors, and the development of additional preclinical animal models. However, many challenges remain in the identification of new ALS therapeutics including: the use of relevant biomarkers, the need for earlier diagnosis of the disease, and additional animal models. Multiple strategies need to be tested, in the clinic, in order to determine what will be effective in patients. PMID:22646982

  16. Glycosphingolipids are modulators of disease pathogenesis in amyotrophic lateral sclerosis

    PubMed Central

    Dodge, James C.; Treleaven, Christopher M.; Pacheco, Joshua; Cooper, Samantha; Bao, Channa; Abraham, Marissa; Cromwell, Mandy; Sardi, S. Pablo; Chuang, Wei-Lien; Sidman, Richard L.; Cheng, Seng H.; Shihabuddin, Lamya S.

    2015-01-01

    Recent genetic evidence suggests that aberrant glycosphingolipid metabolism plays an important role in several neuromuscular diseases including hereditary spastic paraplegia, hereditary sensory neuropathy type 1, and non-5q spinal muscular atrophy. Here, we investigated whether altered glycosphingolipid metabolism is a modulator of disease course in amyotrophic lateral sclerosis (ALS). Levels of ceramide, glucosylceramide, galactocerebroside, lactosylceramide, globotriaosylceramide, and the gangliosides GM3 and GM1 were significantly elevated in spinal cords of ALS patients. Moreover, enzyme activities (glucocerebrosidase-1, glucocerebrosidase-2, hexosaminidase, galactosylceramidase, α-galactosidase, and β-galactosidase) mediating glycosphingolipid hydrolysis were also elevated up to threefold. Increased ceramide, glucosylceramide, GM3, and hexosaminidase activity were also found in SOD1G93A mice, a familial model of ALS. Inhibition of glucosylceramide synthesis accelerated disease course in SOD1G93A mice, whereas infusion of exogenous GM3 significantly slowed the onset of paralysis and increased survival. Our results suggest that glycosphingolipids are likely important participants in pathogenesis of ALS and merit further analysis as potential drug targets. PMID:26056266

  17. Canine degenerative myelopathy: a model of human amyotrophic lateral sclerosis.

    PubMed

    Nardone, Raffaele; Höller, Yvonne; Taylor, Alexandra C; Lochner, Piergiorgio; Tezzon, Frediano; Golaszewski, Stefan; Brigo, Francesco; Trinka, Eugen

    2016-02-01

    Canine degenerative myelopathy (CDM) represents a unique naturally occurring animal model for human amyotrophic lateral sclerosis (ALS) because of similar clinical signs, neuropathologic findings, and involvement of the superoxide dismutase 1 (SOD1) mutation. A definitive diagnosis can only be made postmortem through microscopic detection of axonal degeneration, demyelination and astroglial proliferation, which is more severe in the dorsal columns of the thoracic spinal cord and in the dorsal portion of the lateral funiculus. Interestingly, the muscle acetylcholine receptor complexes are intact in CDM prior to functional impairment, thus suggesting that muscle atrophy in CDM does not result from physical denervation. Moreover, since sensory involvement seems to play an important role in CDM progression, a more careful investigation of the sensory pathology in ALS is also warranted. The importance of SOD1 expression remains unclear, while oxidative stress and denatured ubiquinated proteins appear to play a crucial role in the pathogenesis of CDM. In this updated narrative review we performed a systematic search of the published studies on CDM that may shed light on the pathophysiological mechanisms of human ALS. A better understanding of the factors that determine the disease progression in CDM may be beneficial for the development of effective treatments for ALS.

  18. Percutaneous nocturnal oximetry in amyotrophic lateral sclerosis: periodic desaturation.

    PubMed

    de Carvalho, Mamede; Costa, João; Pinto, Susana; Pinto, Anabela

    2009-06-01

    Percutaneous nocturnal oximetry (PNO) is useful to screen respiratory function in amyotrophic lateral sclerosis (ALS). PNO recordings of some patients disclose a periodical pattern of O(2) desaturation (PP), whose significance is unknown. We aimed to characterize PP pattern, and we used a prospective study enrolling 261 consecutive ALS patients. Clinical, pulmonary and neurophysiological tests performed included: ALS functional rating scale, forced vital capacity (FVC), maximal inspiratory pressure (PImax), mouth occlusion pressure (MOP), phrenic nerve motor response, needle electromyography of the diaphragm, PNO, and sleep study. A total of 837 PNO recordings were analysed (3.2 recordings/patient) and 45 patients showed typical PP (17.2%). Four were excluded, 13 had normal diaphragm (group 1, G1), and in 28 the diaphragm was abnormal (G2). The two groups were comparable, apart from respiratory score, FVC and PImax which were lower in G2. In G1, REM sleep was absent and hypoventilation occurred at slow-wave sleep. Five patients in G1 were very spastic, had low MOP/FVC and a short survival. This study identified a subgroup of ALS patients (G1) with marked signs of upper motor neuron lesion, strong respiratory muscles, PP, low MOP/FVC ratio and poor prognosis. We speculate that they have a central respiratory dysfunction and deserve special care.

  19. Roles of Vascular Endothelial Growth Factor in Amyotrophic Lateral Sclerosis

    PubMed Central

    Pronto-Laborinho, Ana Catarina; Pinto, Susana; de Carvalho, Mamede

    2014-01-01

    Amyotrophic lateral sclerosis (ALS) is a fatal devastating neurodegenerative disorder, involving progressive degeneration of motor neurons in spinal cord, brainstem, and motor cortex. Riluzole is the only drug approved in ALS but it only confers a modest improvement in survival. In spite of a high number of clinical trials no other drug has proved effectiveness. Recent studies support that vascular endothelial growth factor (VEGF), originally described as a key angiogenic factor, also plays a key role in the nervous system, including neurogenesis, neuronal survival, neuronal migration, and axon guidance. VEGF has been used in exploratory clinical studies with promising results in ALS and other neurological disorders. Although VEGF is a very promising compound, translating the basic science breakthroughs into clinical practice is the major challenge ahead. VEGF-B, presenting a single safety profile, protects motor neurons from degeneration in ALS animal models and, therefore, it will be particularly interesting to test its effects in ALS patients. In the present paper the authors make a brief description of the molecular properties of VEGF and its receptors and review its different features and therapeutic potential in the nervous system/neurodegenerative disease, particularly in ALS. PMID:24987705

  20. Stratified gene expression analysis identifies major amyotrophic lateral sclerosis genes.

    PubMed

    Jones, Ashley R; Troakes, Claire; King, Andrew; Sahni, Vibhu; De Jong, Simone; Bossers, Koen; Papouli, Efterpi; Mirza, Muddassar; Al-Sarraj, Safa; Shaw, Christopher E; Shaw, Pamela J; Kirby, Janine; Veldink, Jan H; Macklis, Jeffrey D; Powell, John F; Al-Chalabi, Ammar

    2015-05-01

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of motor neurons resulting in progressive paralysis. Gene expression studies of ALS only rarely identify the same gene pathways as gene association studies. We hypothesized that analyzing tissues by matching on degree of disease severity would identify different patterns of gene expression from a traditional case-control comparison. We analyzed gene expression changes in 4 postmortem central nervous system regions, stratified by severity of motor neuron loss. An overall comparison of cases (n = 6) and controls (n = 3) identified known ALS gene, SOX5, as showing differential expression (log2 fold change = 0.09, p = 5.5 × 10(-5)). Analyses stratified by disease severity identified expression changes in C9orf72 (p = 2.77 × 10(-3)), MATR3 (p = 3.46 × 10(-3)), and VEGFA (p = 8.21 × 10(-4)), all implicated in ALS through genetic studies, and changes in other genes in pathways involving RNA processing and immune response. These findings suggest that analysis of gene expression stratified by disease severity can identify major ALS genes and may be more efficient than traditional case-control comparison.

  1. Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis.

    PubMed

    Manfredi, Giovanni; Kawamata, Hibiki

    2016-06-01

    Physical and functional interactions between mitochondria and the endoplasmic reticulum (ER) are crucial for cell life. These two organelles are intimately connected and collaborate to essential processes, such as calcium homeostasis and phospholipid biosynthesis. The connections between mitochondria and endoplasmic reticulum occur through structures named mitochondria associated membranes (MAMs), which contain lipid rafts and a large number of proteins, many of which serve multiple functions at different cellular sites. Growing evidence strongly suggests that alterations of ER-mitochondria interactions are involved in neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS), a devastating and rapidly fatal motor neuron disease. Mutations in proteins that participate in ER-mitochondria interactions and MAM functions are increasingly being associated with genetic forms of ALS and other neurodegenerative diseases. This evidence strongly suggests that, rather than considering the two organelles separately, a better understanding of the disease process can derive from studying the alterations in their crosstalk. In this review we discuss normal and pathological ER-mitochondria interactions and the evidence that link them to ALS.

  2. A neurophysiological analysis of working memory in amyotrophic lateral sclerosis.

    PubMed

    Hammer, Anke; Vielhaber, Stefan; Rodriguez-Fornells, Antoni; Mohammadi, Bahram; Münte, Thomas F

    2011-11-03

    Frontal lobe functions, in particular working memory (WM) and verbal fluency, have been found to be deficient in amyotrophic lateral sclerosis (ALS). To study the neural correlates of WM-impairment, ALS patients and healthy age-matched controls were subjected to two working memory tasks following the 2-back paradigm, one requiring the storage of figural information, the other storage of spatial information. A significant proportion of ALS patients were unable to perform the WM-tasks. Those who could showed worse performance in the spatial task than the controls. Event-related brain potentials recorded during the task revealed a topographical change of the working memory effect in the ALS patients. Thus, behavioral and electrophysiological data suggest an alteration of working memory, in particular for spatial information, in ALS. Additionally, the patients also took part in two Go/Nogo tasks (spatial, figural) using the same stimulus material but defining targets prior to the experiment instead of a working memory manipulation. Here, an anteriorization of the nogo-P3 was found which has been established as an index of impaired inhibitory functions.

  3. Abnormalities of cortical inhibitory neurons in amyotrophic lateral sclerosis.

    PubMed

    Enterzari-Taher, M; Eisen, A; Stewart, H; Nakajima, M

    1997-01-01

    We have used peristimulus time histograms to study how paired, transcranial magnetic stimulation alters the firing of single motor units and the magnitude of unitary excitatory postsynaptic potentials (EPSPs) recorded from the extensor digitorum communis muscle. With stimulus intensity at threshold and an interstimulus interval of 30 ms, normal subjects (n = 20) demonstrated marked inhibition with a mean test/conditioning EPSP ratio of 13.8% (range 0-51%) and in 7 subjects the ratio was 0 (100% inhibition). In amyotrophic lateral sclerosis (ALS) the ratio was 133% (range 64-267%), P < 0.001. Fifty percent of patients had a test/conditioning EPSP ratio greater than 100% (0 inhibition). The abnormalities were independent of disease severity, bulbar versus spinal ALS, more prominent upper versus lower motor neuron findings, and disease duration. Normal inhibition occurred in 3 individuals, 1 each with multiple sclerosis, Kennedy's syndrome, and monomelic amyotrophy. We speculate that the marked loss of inhibition seen in all patients with ALS, which may be unique to this disorder, reflects loss of inhibitory modulation of the corticomotoneuron and could result in their chronic excitatory drive and eventual demise.

  4. Genetic correlation between amyotrophic lateral sclerosis and schizophrenia

    NASA Astrophysics Data System (ADS)

    McLaughlin, Russell L.; Schijven, Dick; van Rheenen, Wouter; van Eijk, Kristel R.; O'Brien, Margaret; Kahn, René S.; Ophoff, Roel A.; Goris, An; Bradley, Daniel G.; Al-Chalabi, Ammar; van den Berg, Leonard H.; Luykx, Jurjen J.; Hardiman, Orla; Veldink, Jan H.; Shatunov, Aleksey; Dekker, Annelot M.; Diekstra, Frank P.; Pulit, Sara L.; van der Spek, Rick A. A.; van Doormaal, Perry T. C.; Sproviero, William; Jones, Ashley R.; Nicholson, Garth A.; Rowe, Dominic B.; Pamphlett, Roger; Kiernan, Matthew C.; Bauer, Denis; Kahlke, Tim; Williams, Kelly; Eftimov, Filip; Fogh, Isabella; Ticozzi, Nicola; Lin, Kuang; Millecamps, Stéphanie; Salachas, François; Meininger, Vincent; de Carvalho, Mamede; Pinto, Susana; Mora, Jesus S.; Rojas-García, Ricardo; Polak, Meraida; Chandran, Siddharthan; Colville, Shuna; Swingler, Robert; Morrison, Karen E.; Shaw, Pamela J.; Hardy, John; Orrell, Richard W.; Pittman, Alan; Sidle, Katie; Fratta, Pietro; Malaspina, Andrea; Petri, Susanne; Abdulla, Susanna; Drepper, Carsten; Sendtner, Michael; Meyer, Thomas; Wiedau-Pazos, Martina; Lomen-Hoerth, Catherine; van Deerlin, Vivianna M.; Trojanowski, John Q.; Elman, Lauren; McCluskey, Leo; Basak, Nazli; Meitinger, Thomas; Lichtner, Peter; Blagojevic-Radivojkov, Milena; Andres, Christian R.; Maurel, Cindy; Bensimon, Gilbert; Landwehrmeyer, Bernhard; Brice, Alexis; Payan, Christine A. M.; Saker-Delye, Safa; Dürr, Alexandra; Wood, Nicholas; Tittmann, Lukas; Lieb, Wolfgang; Franke, Andre; Rietschel, Marcella; Cichon, Sven; Nöuthen, Markus M.; Amouyel, Philippe; Tzourio, Christophe; Dartigues, Jean-François; Uitterlinden, Andre G.; Rivadeneira, Fernando; Estrada, Karol; Hofman, Albert; Curtis, Charles; van der Kooi, Anneke J.; de Visser, Marianne; Weber, Markus; Shaw, Christopher E.; Smith, Bradley N.; Pansarasa, Orietta; Cereda, Cristina; Del Bo, Roberto; Comi, Giacomo P.; D'Alfonso, Sandra; Bertolin, Cinzia; Sorarù, Gianni; Mazzini, Letizia; Pensato, Viviana; Gellera, Cinzia; Tiloca, Cinzia; Ratti, Antonia; Calvo, Andrea; Moglia, Cristina; Brunetti, Maura; Arcuti, Simon; Capozzo, Rosa; Zecca, Chiara; Lunetta, Christian; Penco, Silvana; Riva, Nilo; Padovani, Alessandro; Filosto, Massimiliano; Blair, Ian; Leigh, P. Nigel; Casale, Federico; Chio, Adriano; Beghi, Ettore; Pupillo, Elisabetta; Tortelli, Rosanna; Logroscino, Giancarlo; Powell, John; Ludolph, Albert C.; Weishaupt, Jochen H.; Robberecht, Wim; van Damme, Philip; Brown, Robert H.; Glass, Jonathan; Landers, John E.; Andersen, Peter M.; Corcia, Philippe; Vourc'h, Patrick; Silani, Vincenzo; van Es, Michael A.; Pasterkamp, R. Jeroen; Lewis, Cathryn M.; Breen, Gerome; Ripke, Stephan; Neale, Benjamin M.; Corvin, Aiden; Walters, James T. R.; Farh, Kai-How; Holmans, Peter A.; Lee, Phil; Bulik-Sullivan, Brendan; Collier, David A.; Huang, Hailiang; Pers, Tune H.; Agartz, Ingrid; Agerbo, Esben; Albus, Margot; Alexander, Madeline; Amin, Farooq; Bacanu, Silviu A.; Begemann, Martin; Belliveau, Richard A.; Bene, Judit; Bergen, Sarah E.; Bevilacqua, Elizabeth; Bigdeli, Tim B.; Black, Donald W.; Bruggeman, Richard; Buccola, Nancy G.; Buckner, Randy L.; Byerley, William; Cahn, Wiepke; Cai, Guiqing; Campion, Dominique; Cantor, Rita M.; Carr, Vaughan J.; Carrera, Noa; Catts, Stanley V.; Chambert, Kimberley D.; Chan, Raymond C. K.; Chan, Ronald Y. L.; Chen, Eric Y. H.; Cheng, Wei; Cheung, Eric F. C.; Chong, Siow Ann; Cloninger, C. Robert; Cohen, David; Cohen, Nadine; Cormican, Paul; Craddock, Nick; Crowley, James J.; Curtis, David; Davidson, Michael; Davis, Kenneth L.; Degenhardt, Franziska; Del Favero, Jurgen; Demontis, Ditte; Dikeos, Dimitris; Dinan, Timothy; Djurovic, Srdjan; Donohoe, Gary; Drapeau, Elodie; Duan, Jubao; Dudbridge, Frank; Durmishi, Naser; Eichhammer, Peter; Eriksson, Johan; Escott-Price, Valentina; Essioux, Laurent; Fanous, Ayman H.; Farrell, Martilias S.; Frank, Josef; Franke, Lude; Freedman, Robert; Freimer, Nelson B.; Friedl, Marion; Friedman, Joseph I.; Fromer, Menachem; Genovese, Giulio; Georgieva, Lyudmila; Giegling, Ina; Giusti-Rodríguez, Paola; Godard, Stephanie; Goldstein, Jacqueline I.; Golimbet, Vera; Gopal, Srihari; Gratten, Jacob; de Haan, Lieuwe; Hammer, Christian; Hamshere, Marian L.; Hansen, Mark; Hansen, Thomas; Haroutunian, Vahram; Hartmann, Annette M.; Henskens, Frans A.; Herms, Stefan; Hirschhorn, Joel N.; Hoffmann, Per; Hofman, Andrea; Hollegaard, Mads V.; Hougaard, David M.; Ikeda, Masashi; Joa, Inge; Julià, Antonio; Kalaydjieva, Luba; Karachanak-Yankova, Sena; Karjalainen, Juha; Kavanagh, David; Keller, Matthew C.; Kennedy, James L.; Khrunin, Andrey; Kim, Yunjung; Klovins, Janis; Knowles, James A.; Konte, Bettina; Kucinskas, Vaidutis; Kucinskiene, Zita Ausrele; Kuzelova-Ptackova, Hana; Kähler, Anna K.; Laurent, Claudine; Lee, Jimmy; Lee, S. Hong; Legge, Sophie E.; Lerer, Bernard; Li, Miaoxin; Li, Tao; Liang, Kung-Yee; Lieberman, Jeffrey; Limborska, Svetlana; Loughland, Carmel M.; Lubinski, Jan; Lönnqvist, Jouko; Macek, Milan; Magnusson, Patrik K. E.; Maher, Brion S.; Maier, Wolfgang; Mallet, Jacques; Marsal, Sara; Mattheisen, Manuel; Mattingsdal, Morten; McCarley, Robert W.; McDonald, Colm; McIntosh, Andrew M.; Meier, Sandra; Meijer, Carin J.; Melegh, Bela; Melle, Ingrid; Mesholam-Gately, Raquelle I.; Metspalu, Andres; Michie, Patricia T.; Milani, Lili; Milanova, Vihra; Mokrab, Younes; Morris, Derek W.; Mors, Ole; Murphy, Kieran C.; Murray, Robin M.; Myin-Germeys, Inez; Müller-Myhsok, Bertram; Nelis, Mari; Nenadic, Igor; Nertney, Deborah A.; Nestadt, Gerald; Nicodemus, Kristin K.; Nikitina-Zake, Liene; Nisenbaum, Laura; Nordin, Annelie; O'Callaghan, Eadbhard; O'Dushlaine, Colm; O'Neill, F. Anthony; Oh, Sang-Yun; Olincy, Ann; Olsen, Line; van Os, Jim; Pantelis, Christos; Papadimitriou, George N.; Papiol, Sergi; Parkhomenko, Elena; Pato, Michele T.; Paunio, Tiina; Pejovic-Milovancevic, Milica; Perkins, Diana O.; Pietiläinen, Olli; Pimm, Jonathan; Pocklington, Andrew J.; Price, Alkes; Pulver, Ann E.; Purcell, Shaun M.; Quested, Digby; Rasmussen, Henrik B.; Reichenberg, Abraham; Reimers, Mark A.; Richards, Alexander L.; Roffman, Joshua L.; Roussos, Panos; Ruderfer, Douglas M.; Salomaa, Veikko; Sanders, Alan R.; Schall, Ulrich; Schubert, Christian R.; Schulze, Thomas G.; Schwab, Sibylle G.; Scolnick, Edward M.; Scott, Rodney J.; Seidman, Larry J.; Shi, Jianxin; Sigurdsson, Engilbert; Silagadze, Teimuraz; Silverman, Jeremy M.; Sim, Kang; Slominsky, Petr; Smoller, Jordan W.; So, Hon-Cheong; Spencer, Chris C. A.; Stahl, Eli A.; Stefansson, Hreinn; Steinberg, Stacy; Stogmann, Elisabeth; Straub, Richard E.; Strengman, Eric; Strohmaier, Jana; Stroup, T. Scott; Subramaniam, Mythily; Suvisaari, Jaana; Svrakic, Dragan M.; Szatkiewicz, Jin P.; Söderman, Erik; Thirumalai, Srinivas; Toncheva, Draga; Tosato, Sarah; Veijola, Juha; Waddington, John; Walsh, Dermot; Wang, Dai; Wang, Qiang; Webb, Bradley T.; Weiser, Mark; Wildenauer, Dieter B.; Williams, Nigel M.; Williams, Stephanie; Witt, Stephanie H.; Wolen, Aaron R.; Wong, Emily H. M.; Wormley, Brandon K.; Xi, Hualin Simon; Zai, Clement C.; Zheng, Xuebin; Zimprich, Fritz; Wray, Naomi R.; Stefansson, Kari; Visscher, Peter M.; Adolfsson, Rolf; Andreassen, Ole A.; Blackwood, Douglas H. R.; Bramon, Elvira; Buxbaum, Joseph D.; Børglum, Anders D.; Darvasi, Ariel; Domenici, Enrico; Ehrenreich, Hannelore; Esko, Tõnu; Gejman, Pablo V.; Gill, Michael; Gurling, Hugh; Hultman, Christina M.; Iwata, Nakao; Jablensky, Assen V.; Jönsson, Erik G.; Kendler, Kenneth S.; Kirov, George; Knight, Jo; Lencz, Todd; Levinson, Douglas F.; Li, Qingqin S.; Liu, Jianjun; Malhotra, Anil K.; McCarroll, Steven A.; McQuillin, Andrew; Moran, Jennifer L.; Mortensen, Preben B.; Mowry, Bryan J.; Owen, Michael J.; Palotie, Aarno; Pato, Carlos N.; Petryshen, Tracey L.; Posthuma, Danielle; Riley, Brien P.; Rujescu, Dan; Sham, Pak C.; Sklar, Pamela; St Clair, David; Weinberger, Daniel R.; Wendland, Jens R.; Werge, Thomas; Daly, Mark J.; Sullivan, Patrick F.; O'Donovan, Michael C.

    2017-03-01

    We have previously shown higher-than-expected rates of schizophrenia in relatives of patients with amyotrophic lateral sclerosis (ALS), suggesting an aetiological relationship between the diseases. Here, we investigate the genetic relationship between ALS and schizophrenia using genome-wide association study data from over 100,000 unique individuals. Using linkage disequilibrium score regression, we estimate the genetic correlation between ALS and schizophrenia to be 14.3% (7.05-21.6 P=1 × 10-4) with schizophrenia polygenic risk scores explaining up to 0.12% of the variance in ALS (P=8.4 × 10-7). A modest increase in comorbidity of ALS and schizophrenia is expected given these findings (odds ratio 1.08-1.26) but this would require very large studies to observe epidemiologically. We identify five potential novel ALS-associated loci using conditional false discovery rate analysis. It is likely that shared neurobiological mechanisms between these two disorders will engender novel hypotheses in future preclinical and clinical studies.

  5. Widespread temporo-occipital lobe dysfunction in amyotrophic lateral sclerosis

    NASA Astrophysics Data System (ADS)

    Loewe, Kristian; Machts, Judith; Kaufmann, Jörn; Petri, Susanne; Heinze, Hans-Jochen; Borgelt, Christian; Harris, Joseph Allen; Vielhaber, Stefan; Schoenfeld, Mircea Ariel

    2017-01-01

    Recent studies suggest that amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) lie on a single clinical continuum. However, previous neuroimaging studies have found only limited involvement of temporal lobe regions in ALS. To better delineate possible temporal lobe involvement in ALS, the present study aimed to examine changes in functional connectivity across the whole brain, particularly with regard to extra-motor regions, in a group of 64 non-demented ALS patients and 38 healthy controls. To assess between-group differences in connectivity, we computed edge-level statistics across subject-specific graphs derived from resting-state functional MRI data. In addition to expected ALS-related decreases in functional connectivity in motor-related areas, we observed extensive changes in connectivity across the temporo-occipital cortex. Although ALS patients with comorbid FTD were deliberately excluded from this study, the pattern of connectivity alterations closely resembles patterns of cerebral degeneration typically seen in FTD. This evidence for subclinical temporal dysfunction supports the idea of a common pathology in ALS and FTD.

  6. Dynamic markers of altered gait rhythm in amyotrophic lateral sclerosis

    NASA Technical Reports Server (NTRS)

    Hausdorff, J. M.; Lertratanakul, A.; Cudkowicz, M. E.; Peterson, A. L.; Kaliton, D.; Goldberger, A. L.

    2000-01-01

    Amyotrophic lateral sclerosis (ALS) is a disorder marked by loss of motoneurons. We hypothesized that subjects with ALS would have an altered gait rhythm, with an increase in both the magnitude of the stride-to-stride fluctuations and perturbations in the fluctuation dynamics. To test for this locomotor instability, we quantitatively compared the gait rhythm of subjects with ALS with that of normal controls and with that of subjects with Parkinson's disease (PD) and Huntington's disease (HD), pathologies of the basal ganglia. Subjects walked for 5 min at their usual pace wearing an ankle-worn recorder that enabled determination of the duration of each stride and of stride-to-stride fluctuations. We found that the gait of patients with ALS is less steady and more temporally disorganized compared with that of healthy controls. In addition, advanced ALS, HD, and PD were associated with certain common, as well as apparently distinct, features of altered stride dynamics. Thus stride-to-stride control of gait rhythm is apparently compromised with ALS. Moreover, a matrix of markers based on gait dynamics may be useful in characterizing certain pathologies of motor control and, possibly, in quantitatively monitoring disease progression and evaluating therapeutic interventions.

  7. Acceptance of brain-computer interfaces in amyotrophic lateral sclerosis.

    PubMed

    Geronimo, Andrew; Stephens, Helen E; Schiff, Steven J; Simmons, Zachary

    2015-06-01

    Brain-computer interfaces (BCI) have the potential to permit patients with amyotrophic lateral sclerosis (ALS) to communicate even when locked in. Although as many as half of patients with ALS develop cognitive or behavioral dysfunction, the impact of these factors on acceptance of and ability to use a BCI has not been studied. We surveyed patients with ALS and their caregivers about BCIs used as assistive communication tools. The survey focused on the features of a BCI system, the desired end-use functions, and requirements. Functional, cognitive, and behavioral data were collected from patients and analyzed for their influence over decisions about BCI device use. Results showed that behavioral impairment was associated with decreased receptivity to the use of BCI technology. In addition, the operation of a BCI system during a pilot study altered patients' opinions of the utility of the system, generally in line with their perceived performance at controlling the device. In conclusion, these two findings have implications for the engineering design and clinical care phases of assistive device deployment.

  8. Gene discovery in amyotrophic lateral sclerosis: implications for clinical management.

    PubMed

    Al-Chalabi, Ammar; van den Berg, Leonard H; Veldink, Jan

    2017-02-01

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease predominantly affecting upper and lower motor neurons. The disease leads to relentlessly progressive weakness of voluntary muscles, with death typically resulting from diaphragmatic failure within 2-5 years. Since the discovery of mutations in SOD1, which account for ∼2% of ALS cases, increasing efforts have been made to understand the genetic component of ALS risk, with the expectation that this insight will not only aid diagnosis and classification, but also guide personalized treatment and reveal the mechanisms that cause motor neuron death. In this Review, we outline previous and current efforts to characterize genes that are associated with ALS, describe current knowledge about the genetic architecture of ALS - including the relevance of family history - and the probable nature of future gene discoveries, and explore how our understanding of ALS genetics affects present and future clinical decisions. We observe that many gene variants associated with ALS have effect sizes between those of mutations that greatly increase risk and those of common variants that have a small effect on risk, and combine this observation with insights from next-generation sequencing to explore the implications for genetic counselling.

  9. S-nitrosothiol depletion in amyotrophic lateral sclerosis.

    PubMed

    Schonhoff, Christopher M; Matsuoka, Masaaki; Tummala, Hemachand; Johnson, Michael A; Estevéz, Alvaro G; Wu, Rui; Kamaid, Andrés; Ricart, Karina C; Hashimoto, Yuichi; Gaston, Benjamin; Macdonald, Timothy L; Xu, Zuoshang; Mannick, Joan B

    2006-02-14

    Recent data suggest that either excessive or deficient levels of protein S-nitrosylation may contribute to disease. Disruption of S-nitrosothiol (SNO) homeostasis may result not only from altered nitric oxide (NO) synthase activity but also from alterations in the activity of denitrosylases that remove NO groups. A subset of patients with familial amyotrophic lateral sclerosis (ALS) have mutations in superoxide dismutase 1 (SOD1) that increase the denitrosylase activity of SOD1. Here, we show that the increased denitrosylase activity of SOD1 mutants leads to an aberrant decrease in intracellular protein and peptide S-nitrosylation in cell and animal models of ALS. Deficient S-nitrosylation is particularly prominent in the mitochondria of cells expressing SOD1 mutants. Our results suggest that SNO depletion disrupts the function and/or subcellular localization of proteins that are regulated by S-nitrosylation such as glyceraldehyde-3-phosphate dehydrogenase (GAPDH) and thereby contributes to ALS pathogenesis. Repletion of intracellular SNO levels with SNO donor compounds rescues cells from mutant SOD1-induced death. These results suggest that aberrant depletion of intracellular SNOs contributes to motor neuron death in ALS, and raises the possibility that deficient S-nitrosylation is a general mechanism of disease pathogenesis. SNO donor compounds may provide new therapeutic options for diseases such as ALS that are associated with deficient S-nitrosylation.

  10. Action verb comprehension in amyotrophic lateral sclerosis and Parkinson's disease.

    PubMed

    York, Collin; Olm, Christopher; Boller, Ashley; McCluskey, Leo; Elman, Lauren; Haley, Jenna; Seltzer, Emily; Chahine, Lama; Woo, John; Rascovsky, Katya; McMillan, Corey; Grossman, Murray

    2014-06-01

    Patients with amyotrophic lateral sclerosis (ALS) have a motor disorder and cognitive difficulties, including difficulty with action verbs. However, the basis for the action verb impairment is unknown. Thirty-six participants with ALS and 22 with Parkinson's disease (PD) were assessed on a simple, two-alternative forced-choice associativity judgment task, where performance was untimed and did not depend on motor functioning. We probed 120 frequency-matched action verbs, cognition verbs, concrete nouns and abstract nouns. Performance was related to T1 MRI imaging of gray matter atrophy. Patients with ALS were significantly impaired relative to healthy senior control participants only for action verbs. Patients with PD did not differ from controls for all word categories. Regression analyses related action verb performance in ALS to motor-associated cortices, but action verb judgments in PD were not related to cortical atrophy. These findings are consistent with the hypothesis that action verb difficulty in ALS is related in part to the degradation of action-related conceptual knowledge represented in motor-associated cortex.

  11. AMPK Signalling and Defective Energy Metabolism in Amyotrophic Lateral Sclerosis.

    PubMed

    Perera, Nirma D; Turner, Bradley J

    2016-03-01

    Amyotrophic lateral sclerosis (ALS) is caused by selective loss of upper and lower motor neurons by complex mechanisms that are incompletely understood. Motor neurons are large, highly polarised and excitable cells with unusually high energetic demands to maintain resting membrane potential and propagate action potentials. This leads to higher ATP consumption and mitochondrial metabolism in motor neurons relative to other cells. Here, we review increasing evidence that defective energy metabolism and homeostasis contributes to selective vulnerability and degeneration of motor neurons in ALS. Firstly, we provide a brief overview of major energetic pathways in the CNS, including glycolysis, oxidative phosphorylation and the AMP-activated protein kinase (AMPK) signalling pathway, while highlighting critical metabolic interactions between neurons and astrocytes. Next, we review evidence from ALS patients and transgenic mutant SOD1 mice for weight loss, hypermetabolism, hyperlipidemia and mitochondrial dysfunction in disease onset and progression. Genetic and therapeutic modifiers of energy metabolism in mutant SOD1 mice will also be summarised. We also present evidence that additional ALS-linked proteins, TDP-43 and FUS, lead to energy disruption and mitochondrial defects in motor neurons. Lastly, we review emerging evidence including our own that dysregulation of the AMPK signalling cascade in motor neurons is an early and common event in ALS pathogenesis. We suggest that an imbalance in energy metabolism should be considered an important factor in both progression and potential treatment of ALS.

  12. [Amyotrophic lateral sclerosis: is the astrocyte the cell primarily involved?].

    PubMed

    Sica, Roberto E

    2013-01-01

    So far, amyotrophic lateral sclerosis (ALS) is thought as due to a primary insult of the motor neurons. None of its pathogenic processes proved to be the cause of the illness, nor can be blamed environmental agents. Motor neurons die by apoptosis, leaving the possibility that their death might be due to an unfriendly environment, unable to sustain their health, rather than being directly targeted themselves. These reasons justify an examination of the astrocytes, because they have the most important role controlling the neurons' environment. It is known that astrocytes are plastic, enslaving their functions to the requirements of the neurons to which they are related. Each population of astrocytes is unique, and if it were affected the consequences would reach the neurons that it normally sustains. In regard to the motor neurons, this situation would lead to a disturbed production and release of astrocytic neurotransmitters and transporters, impairing nutritional and trophic support as well. For explaining the spreading of muscle symptoms in ALS, correlated with the type of spreading observed at the cortical and spinal motor neurons pools, the present hypotheses suggests that the illness-causing process is spreading among astrocytes, through their gap junctions, depriving the motor neurons of their support. Also it is postulated that a normal astrocytic protein becomes misfolded and infectious, inducing the misfolding of its wild type, travelling from one protoplasmatic astrocyte to another and to the fibrous astrocytes encircling the pyramidal pathway which joints the upper and lower motoneurones.

  13. Amyotrophic lateral sclerosis: mechanisms and therapeutics in the epigenomic era.

    PubMed

    Paez-Colasante, Ximena; Figueroa-Romero, Claudia; Sakowski, Stacey A; Goutman, Stephen A; Feldman, Eva L

    2015-05-01

    Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of the motor neurons, which results in weakness and atrophy of voluntary skeletal muscles. Treatments do not modify the disease trajectory effectively, and only modestly improve survival. A complex interaction between genes, environmental exposure and impaired molecular pathways contributes to pathology in patients with ALS. Epigenetic mechanisms control the hereditary and reversible regulation of gene expression without altering the basic genetic code. Aberrant epigenetic patterns-including abnormal microRNA (miRNA) biogenesis and function, DNA modifications, histone remodeling, and RNA editing-are acquired throughout life and are influenced by environmental factors. Thus, understanding the molecular processes that lead to epigenetic dysregulation in patients with ALS might facilitate the discovery of novel therapeutic targets and biomarkers that could reduce diagnostic delay. These achievements could prove crucial for successful disease modification in patients with ALS. We review the latest findings regarding the role of miRNA modifications and other epigenetic mechanisms in ALS, and discuss their potential as therapeutic targets.

  14. Regionality of disease progression predicts prognosis in amyotrophic lateral sclerosis.

    PubMed

    van der Kleij, Lisa A; Jones, Ashley R; Steen, I Nick; Young, Carolyn A; Shaw, Pamela J; Shaw, Christopher E; Leigh, P Nigel; Turner, Martin R; Al-Chalabi, Ammar

    2015-01-01

    Amyotrophic lateral sclerosis (ALS) is a devastating neurological syndrome in which motor neurons degenerate relentlessly. Although the site of onset and the rate of spread have been studied extensively, little is known about whether focal as opposed to diffuse disease affects prognosis. We therefore tested the hypothesis that regionality of disease burden is a prognostic factor in ALS. We analysed clinical data from two large multicentre, longitudinal trials. Regionality was defined as the difference in progression rates in three domains as measured by the revised ALS Functional Rating Scale, omitting the respiratory domain from analysis. We used death by trial end as the outcome variable and tested this by logistic regression against predictor variables including regionality and overall rate of disease progression. There were 561 patients. Regionality of disease was independently associated with significantly higher chance of death by study end (odds ratio most diffuse against most focal category 0.354 (0.191, 0.657), p = 0.001), with a direct relationship between degree of regionality and odds of death. We have shown using clinical trial data that focal disease is associated with a worse prognosis in ALS. Measures of regionality warrant further independent consideration in the development of future prognostic models.

  15. Dissociated lower limb muscle involvement in amyotrophic lateral sclerosis.

    PubMed

    Simon, Neil G; Lee, Michael; Bae, Jong Seok; Mioshi, Eneida; Lin, Cindy S-Y; Pfluger, Casey M; Henderson, Robert D; Vucic, Steve; Swash, Michael; Burke, David; Kiernan, Matthew C

    2015-06-01

    It has been suggested that corticomotoneuronal drive to ankle dorsiflexors is greater than to ankle plantar flexor muscles, despite the finding that plantar flexors are no less active than TA during walking and standing. The present study was undertaken to determine whether there was differential involvement of distal lower limb muscles in amyotrophic lateral sclerosis (ALS), to elucidate pathophysiological mechanisms of selective muscle involvement. Prospective studies were undertaken in 52 ALS patients, including clinical assessment, disease staging (revised ALS functional rating scale), Medical Research Council sum score, and a scale of upper motor neurone (UMN) dysfunction. Motor unit number estimates (MUNE) and compound muscle action potentials (CMAP) from ankle dorsiflexors and plantar flexors were used to provide objective measures. A novel 'split leg index' was calculated as follows: SLI = CMAPDF ÷ CMAPPF. In ALS, there was significantly greater reduction of MUNE and CMAP amplitude recorded from plantar flexors when compared to dorsiflexors, suggesting preferential involvement of plantar flexor muscles, underpinning a 'split leg' appearance. The SLI correlated with clinical plantar flexor strength (R= -0.56, p < 0.001). In no patient did the SLI suggest preferential dorsiflexor involvement. In subgroup analyses, mean SLI was greatest in lower limb-onset ALS. In conclusion, the present study has established dissociated involvement of muscles acting around the ankle in ALS. We suggest this reflects underlying differences in cortical, descending or local spinal modulation of these muscles.

  16. Epidemiology of amyotrophic lateral sclerosis: A review of literature.

    PubMed

    Couratier, P; Corcia, P; Lautrette, G; Nicol, M; Preux, P-M; Marin, B

    2016-01-01

    Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of motor neurons, resulting in worsening weakness of voluntary muscles until death occurs from respiratory failure. The incidence of ALS in European populations is two to three people per year per 100,000 of the general population. In Europe, crude prevalences range from 1.1/100,000 population in Yugoslavia to 8.2/100,000 in the Faroe Islands. Major advances have been made in our understanding of the genetic causes of ALS, whereas the contribution of environmental factors has been more difficult to assess and large-scale studies have not yet revealed a replicable, definitive environmental risk factor. The only established risk factors to date are older age, male gender and a family history of ALS. Median survival time from onset to death is usually 3 years from the first appearance of symptoms. Older age and bulbar onset are consistently reported to have poorer outcomes. However, there are conflicting data regarding gender, diagnostic delay and El Escorial criteria. The rate of symptom progression has been revealed to be an independent prognostic factor. Psychosocial factors and impaired cognitive function are negatively related to ALS outcome, while nutritional status and respiratory function are also related to ALS prognosis. The effect of enteral nutrition on survival is still unclear, although noninvasive positive pressure ventilation (NIPPV) has been found to improve survival. These findings have relevant implications for the design of future trials.

  17. Smads as muscle biomarkers in amyotrophic lateral sclerosis

    PubMed Central

    Si, Ying; Cui, Xianqin; Kim, Soojin; Wians, Robert; Sorge, Robert; Oh, Shin J; Kwan, Thaddeus; AlSharabati, Mohammad; Lu, Liang; Claussen, Gwen; Anderson, Tina; Yu, Shaohua; Morgan, Dylan; Kazamel, Mohamed; King, Peter H

    2014-01-01

    Objective To identify molecular signatures in muscle from patients with amyotrophic lateral sclerosis (ALS) that could provide insight into the disease process and serve as biomarkers. Methods RNA sequencing was performed on ALS and control muscle samples to identify Smad family members as potential markers of disease. Validation studies were performed in a cohort of 27 ALS patients and 33 controls. The markers were assessed in the G93A superoxide dismutase (SOD)1 mouse at different stages of disease and in a model of sciatic nerve injury. Results Smad8, and to a lesser extent Smad1 and 5, mRNAs were significantly elevated in human ALS muscle samples. The markers displayed a remarkably similar pattern in the G93A SOD1 mouse model of ALS with increases detected at preclinical stages. Expression at the RNA and protein levels as well as protein activation (phosphorylation) significantly increased with disease progression in the mouse. The markers were also elevated to a lesser degree in gastrocnemius muscle following sciatic nerve injury, but then reverted to baseline during the muscle reinnervation phase. Interpretation These data indicate that Smad1, 5, 8 mRNA and protein levels, as well as Smad phosphorylation, are elevated in ALS muscle and could potentially serve as markers of disease progression or regression. PMID:25493269

  18. Meta-analysis of social cognition in amyotrophic lateral sclerosis.

    PubMed

    Bora, Emre

    2017-03-01

    Amyotrophic lateral sclerosis (ALS) is associated with executive dysfunction and behavioural impairment. Recent studies suggested that social cognitive deficits might also be a prominent feature of ALS. Current meta-analysis aimed to summarize available evidence for deficits in social cognition including theory of mind (ToM) and emotion recognition in ALS. In this meta-analysis of 15 studies, facial emotion recognition and ToM performances of 389 patients with ALS and 471 healthy controls were compared. ALS was associated with significant impairments with medium effect sizes in ToM (d = .65) and facial emotion recognition (d = .69). Among individual emotions recognition of disgust and surprise were particularly impaired. Deficits in perspective taking (d = .73) aspects of ToM (ToM-PT) was more pronounced in comparison to decoding (d = .28) aspects of ToM (ToM-decoding). The severity of social cognitive impairment was similar to level of executive dysfunction and there was a significant relationship between social cognition and executive dysfunction. Deficits in social cognition are part of the cognitive phenotype of ALS.

  19. Serum microRNAs in patients with genetic amyotrophic lateral sclerosis and pre-manifest mutation carriers.

    PubMed

    Freischmidt, Axel; Müller, Kathrin; Zondler, Lisa; Weydt, Patrick; Volk, Alexander E; Božič, Anže Lošdorfer; Walter, Michael; Bonin, Michael; Mayer, Benjamin; von Arnim, Christine A F; Otto, Markus; Dieterich, Christoph; Holzmann, Karlheinz; Andersen, Peter M; Ludolph, Albert C; Danzer, Karin M; Weishaupt, Jochen H

    2014-11-01

    Knowledge about the nature of pathomolecular alterations preceding onset of symptoms in amyotrophic lateral sclerosis is largely lacking. It could not only pave the way for the discovery of valuable therapeutic targets but might also govern future concepts of pre-manifest disease modifying treatments. MicroRNAs are central regulators of transcriptome plasticity and participate in pathogenic cascades and/or mirror cellular adaptation to insults. We obtained comprehensive expression profiles of microRNAs in the serum of patients with familial amyotrophic lateral sclerosis, asymptomatic mutation carriers and healthy control subjects. We observed a strikingly homogenous microRNA profile in patients with familial amyotrophic lateral sclerosis that was largely independent from the underlying disease gene. Moreover, we identified 24 significantly downregulated microRNAs in pre-manifest amyotrophic lateral sclerosis mutation carriers up to two decades or more before the estimated time window of disease onset; 91.7% of the downregulated microRNAs in mutation carriers overlapped with the patients with familial amyotrophic lateral sclerosis. Bioinformatic analysis revealed a consensus sequence motif present in the vast majority of downregulated microRNAs identified in this study. Our data thus suggest specific common denominators regarding molecular pathogenesis of different amyotrophic lateral sclerosis genes. We describe the earliest pathomolecular alterations in amyotrophic lateral sclerosis mutation carriers known to date, which provide a basis for the discovery of novel therapeutic targets and strongly argue for studies evaluating presymptomatic disease-modifying treatment in amyotrophic lateral sclerosis.

  20. Micropower RF material proximity sensor

    DOEpatents

    McEwan, T.E.

    1998-11-10

    A level detector or proximity detector for materials capable of sensing through plastic container walls or encapsulating materials is disclosed. Thus, it can be used in corrosive environments, as well as in a wide variety of applications. An antenna has a characteristic impedance which depends on the materials in proximity to the antenna. An RF oscillator, which includes the antenna and is based on a single transistor in a Colpitt`s configuration, produces an oscillating signal. A detector is coupled to the oscillator which signals changes in the oscillating signal caused by changes in the materials in proximity to the antenna. The oscillator is turned on and off at a pulse repetition frequency with a low duty cycle to conserve power. The antenna consists of a straight monopole about one-quarter wavelength long at the nominal frequency of the oscillator. The antenna may be horizontally disposed on a container and very accurately detects the fill level within the container as the material inside the container reaches the level of the antenna. 5 figs.

  1. Micropower RF material proximity sensor

    DOEpatents

    McEwan, Thomas E.

    1998-01-01

    A level detector or proximity detector for materials capable of sensing through plastic container walls or encapsulating materials is of the sensor. Thus, it can be used in corrosive environments, as well as in a wide variety of applications. An antenna has a characteristic impedance which depends on the materials in proximity to the antenna. An RF oscillator, which includes the antenna and is based on a single transistor in a Colpitt's configuration, produces an oscillating signal. A detector is coupled to the oscillator which signals changes in the oscillating signal caused by changes in the materials in proximity to the antenna. The oscillator is turned on and off at a pulse repetition frequency with a low duty cycle to conserve power. The antenna consists of a straight monopole about one-quarter wavelength long at the nominal frequency of the oscillator. The antenna may be horizontally disposed on a container and very accurately detects the fill level within the container as the material inside the container reaches the level of the antenna.

  2. Accurate quantitative measurements of brachial artery cross-sectional vascular area and vascular volume elastic modulus using automated oscillometric measurements: comparison with brachial artery ultrasound

    PubMed Central

    Tomiyama, Yuuki; Yoshinaga, Keiichiro; Fujii, Satoshi; Ochi, Noriki; Inoue, Mamiko; Nishida, Mutumi; Aziki, Kumi; Horie, Tatsunori; Katoh, Chietsugu; Tamaki, Nagara

    2015-01-01

    Increasing vascular diameter and attenuated vascular elasticity may be reliable markers for atherosclerotic risk assessment. However, previous measurements have been complex, operator-dependent or invasive. Recently, we developed a new automated oscillometric method to measure a brachial artery's estimated area (eA) and volume elastic modulus (VE). The aim of this study was to investigate the reliability of new automated oscillometric measurement of eA and VE. Rest eA and VE were measured using the recently developed automated detector with the oscillometric method. eA was estimated using pressure/volume curves and VE was defined as follows (VE=Δ pressure/ (100 × Δ area/area) mm Hg/%). Sixteen volunteers (age 35.2±13.1 years) underwent the oscillometric measurements and brachial ultrasound at rest and under nitroglycerin (NTG) administration. Oscillometric measurement was performed twice on different days. The rest eA correlated with ultrasound-measured brachial artery area (r=0.77, P<0.001). Rest eA and VE measurement showed good reproducibility (eA: intraclass correlation coefficient (ICC)=0.88, VE: ICC=0.78). Under NTG stress, eA was significantly increased (12.3±3.0 vs. 17.1±4.6 mm2, P<0.001), and this was similar to the case with ultrasound evaluation (4.46±0.72 vs. 4.73±0.75 mm, P<0.001). VE was also decreased (0.81±0.16 vs. 0.65±0.11 mm Hg/%, P<0.001) after NTG. Cross-sectional vascular area calculated using this automated oscillometric measurement correlated with ultrasound measurement and showed good reproducibility. Therefore, this is a reliable approach and this modality may have practical application to automatically assess muscular artery diameter and elasticity in clinical or epidemiological settings. PMID:25693851

  3. Blockade of the brachial plexus abolishes activation of specific brain regions by electroacupuncture at LI4: a functional MRI study

    PubMed Central

    Gu, Weidong; Jiang, Wei; He, Jingwei; Liu, Songbin; Wang, Zhaoxin

    2015-01-01

    Objective Our aim was to test the hypothesis that electroacupuncture (EA) at acupuncture point LI4 activates specific brain regions by nerve stimulation that is mediatied through a pathway involving the brachial plexus. Methods Twelve acupuncture naive right-handed volunteers were allocated to receive three sessions of EA at LI4 in a random different order (crossover): (1) EA alone (EA); EA after injection of local anaesthetics into the deltoid muscle (EA+LA); and (3) EA after blockade of the brachial plexus (EA+NB). During each session, participants were imaged in a 3 T MRI scanner. Brain regions showing change in blood oxygen level-dependent (BOLD) signal (activation) were identified. Subjective acupuncture sensation was quantified after functional MRI scanning was completed. Results were compared between the three sessions for each individual, and averaged. Results Blockade of the brachial plexus inhibited acupuncture sensation during EA. EA and EA+LA activated the bilateral thalamus, basal ganglia, cerebellum and left putamen, whilst no significant activation was observed during EA+NB. The BOLD signal of the thalamus correlated significantly with acupuncture sensation score during EA. Conclusions Blockade of the brachial plexus completely abolishes patterns of brain activation induced by EA at LI4. The results suggest that EA activates specific brain regions through stimulation of the local nerves supplying the tissues at LI4, which transmit sensory information via the brachial plexus. Trial registration number ChiCTR-OO-13003389. PMID:26464415

  4. Acquired Brachial Cutaneous Dyschromatosis in a 60-Year-Old Male: A Case Report and Review of the Literature

    PubMed Central

    Foering, Kristen

    2014-01-01

    Acquired brachial cutaneous dyschromatosis is an acquired pigmentary disorder that has been described in only 20 patients but likely affects many more. This case of a man with acquired brachial cutaneous dyschromatosis is unique as most reports are in women. We report the case of a 60-year-old male who presents with an asymptomatic eruption characterized by hyperpigmented and telangiectatic macules coalescing into patches on the bilateral extensor aspects of the forearms which is consistent clinically and histopathologically with acquired brachial cutaneous dyschromatosis. Given its presence in patients with clinical evidence of chronic sun exposure and its histopathological finding of solar elastosis, acquired brachial cutaneous dyschromatosis is likely a disorder caused by cumulative UV damage. However, a possible association between angiotensin-converting enzyme inhibitors and acquired brachial cutaneous dyschromatosis exists. Further investigation is needed to elucidate both the pathogenesis of the disorder and forms of effective management. Treatment of the disorder should begin with current established treatments for disorders of dyspigmentation. PMID:25610668

  5. Proximate and polyphenolic characterization of cranberry pomace

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The proximate composition and identification and quantification of polyphenolic compounds in dried cranberry pomace were determined. Proximate analysis was conducted based on AOAC methods for moisture, protein, fat, and ash. Total carbohydrates were determined by the difference method. Polyphenolic ...

  6. Amyotrophic Lateral Sclerosis 2-Deficiency Leads to Neuronal Degeneration in Amyotrophic Lateral Sclerosis through Altered AMPA Receptor Trafficking

    PubMed Central

    Lai, Chen; Xie, Chengsong; McCormack, Stefanie G.; Chiang, Hsueh-Cheng; Michalak, Marta K.; Lin, Xian; Chandran, Jayanth; Shim, Hoon; Shimoji, Mika; Cookson, Mark R.; Huganir, Richard L.; Rothstein, Jeffrey D.; Price, Donald L.; Wong, Philip C.; Martin, Lee J.; Zhu, J. Julius; Cai, Huaibin

    2008-01-01

    Amyotrophic lateral sclerosis (ALS), the most common adult-onset motor neuron disease is caused by a selective loss of motor neurons. One form of juvenile onset autosomal recessive ALS (ALS2) has been linked to the loss of function of the ALS2 gene. The pathogenic mechanism of ALS2-deficiency, however, remains unclear. To further understand the function of alsin that is encoded by the full-length ALS2 gene, we screened proteins interacting with alsin. Here, we report that alsin interacted with glutamate receptor interacting protein 1 (GRIP1) both in vitro and in vivo, and colocalized with GRIP1 in neurons. In support of the physiological interaction between alsin and GRIP1, the subcellular distribution of GRIP1 was altered in ALS2-/- spinal motor neurons, which correlates with a significant reduction of AMPA-type glutamate receptor subunit 2 (GluR2) at the synaptic/cell surface of ALS2-/- neurons. The decrease of calcium-impermeable GluR2-containing AMPA receptors at the cell/synaptic surface rendered ALS2-/- neurons more susceptible to glutamate receptor-mediated neurotoxicity. Our findings reveal a novel function of alsin in AMPA receptor trafficking and provide a novel pathogenic link between ALS2-deficiency and motor neuron degeneration, suggesting a protective role of alsin in maintaining the survival of motor neurons. PMID:17093100

  7. Active Targets For Capacitive Proximity Sensors

    NASA Technical Reports Server (NTRS)

    Jenstrom, Del T.; Mcconnell, Robert L.

    1994-01-01

    Lightweight, low-power active targets devised for use with improved capacitive proximity sensors described in "Capacitive Proximity Sensor Has Longer Range" (GSC-13377), and "Capacitive Proximity Sensors With Additional Driven Shields" (GSC-13475). Active targets are short-distance electrostatic beacons; they generate known alternating electro-static fields used for alignment and/or to measure distances.

  8. Dietary BMAA Exposure in an Amyotrophic Lateral Sclerosis Cluster from Southern France

    PubMed Central

    Masseret, Estelle; Banack, Sandra; Boumédiène, Farid; Abadie, Eric; Brient, Luc; Pernet, Fabrice; Juntas-Morales, Raoul; Pageot, Nicolas; Metcalf, James; Cox, Paul; Camu, William

    2013-01-01

    Background Dietary exposure to the cyanotoxin BMAA is suspected to be the cause of amyotrophic lateral sclerosis in the Western Pacific Islands. In Europe and North America, this toxin has been identified in the marine environment of amyotrophic lateral sclerosis clusters but, to date, only few dietary exposures have been described. Objectives We aimed at identifying cluster(s) of amyotrophic lateral sclerosis in the Hérault district, a coastal district from Southern France, and to search, in the identified area(s), for the existence of a potential dietary source of BMAA. Methods A spatio-temporal cluster analysis was performed in the district, considering all incident amyotrophic lateral sclerosis cases identified from 1994 to 2009 by our expert center. We investigated the cluster area with serial collections of oysters and mussels that were subsequently analyzed blind for BMAA concentrations. Results We found one significant amyotrophic lateral sclerosis cluster (p = 0.0024), surrounding the Thau lagoon, the most important area of shellfish production and consumption along the French Mediterranean coast. BMAA was identified in mussels (1.8 µg/g to 6.0 µg/g) and oysters (0.6 µg/g to 1.6 µg/g). The highest concentrations of BMAA were measured during summer when the highest picocyanobacteria abundances were recorded. Conclusions While it is not possible to ascertain a direct link between shellfish consumption and the existence of this ALS cluster, these results add new data to the potential association of BMAA with sporadic amyotrophic lateral sclerosis, one of the most severe neurodegenerative disorder. PMID:24349504

  9. The extracellular domain of neurotrophin receptor p75 as a candidate biomarker for amyotrophic lateral sclerosis.

    PubMed

    Shepheard, Stephanie R; Chataway, Tim; Schultz, David W; Rush, Robert A; Rogers, Mary-Louise

    2014-01-01

    Objective biomarkers for amyotrophic lateral sclerosis would facilitate the discovery of new treatments. The common neurotrophin receptor p75 is up regulated and the extracellular domain cleaved from injured neurons and peripheral glia in amyotrophic lateral sclerosis. We have tested the hypothesis that urinary levels of extracellular neurotrophin receptor p75 serve as a biomarker for both human motor amyotrophic lateral sclerosis and the SOD1(G93A) mouse model of the disease. The extracellular domain of neurotrophin receptor p75 was identified in the urine of amyotrophic lateral sclerosis patients by an immuno-precipitation/western blot procedure and confirmed by mass spectrometry. An ELISA was established to measure urinary extracellular neurotrophin receptor p75. The mean value for urinary extracellular neurotrophin receptor p75 from 28 amyotrophic lateral sclerosis patients measured by ELISA was 7.9±0.5 ng/mg creatinine and this was significantly higher (p<0.001) than 12 controls (2.6±0.2 ng/mg creatinine) and 19 patients with other neurological disease (Parkinson's disease and Multiple Sclerosis; 4.1±0.2 ng/mg creatinine). Pilot data of disease progression rates in 14 MND patients indicates that p75NTR(ECD) levels were significantly higher (p = 0.0041) in 7 rapidly progressing patients as compared to 7 with slowly progressing disease. Extracellular neurotrophin receptor p75 was also readily detected in SOD1(G93A) mice by immuno-precipitation/western blot before the onset of clinical symptoms. These findings indicate a significant relation between urinary extracellular neurotrophin receptor p75 levels and disease progression and suggests that it may be a useful marker of disease activity and progression in amyotrophic lateral sclerosis.

  10. Motor nerve conduction study in cauda equina with high-voltage electrical stimulation in multifocal motor neuropathy and amyotrophic lateral sclerosis.

    PubMed

    Akaza, Miho; Kanouchi, Tadashi; Inaba, Akira; Numasawa, Yoshiyuki; Irioka, Takashi; Mizusawa, Hidehiro; Yokota, Takanori

    2011-02-01

    In this study we aim to establish a motor nerve conduction study (NCS) for the cauda equina and examine its usefulness in multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS). NCS of the tibial nerve proximal to the knee was performed with an optimized high-voltage electrical stimulation (HV-ES) method in 21 normal subjects, 5 with MMN, and 11 with ALS. HV-ES, but not magnetic stimulation, could supramaximally stimulate the cauda equina. Cauda equina motor conduction time determined by HV-ES, but not that with F-waves, correlated well with cauda equina length on magnetic resonance imaging. HV-ES revealed proximal lesions in 4 MMN patients but in none of the ALS patients. Importantly, 1 patient with "MMN without conduction block (CB)" had a CB in the cauda equina. Cauda equina motor conduction is better evaluated by HV-ES than with F-wave study or magnetic stimulation. HV-ES can help to distinguish MMN and "MMN without CB" from ALS.

  11. Characteristics of fasciculations in amyotrophic lateral sclerosis and the benign fasciculation syndrome.

    PubMed

    Mills, Kerry R

    2010-11-01

    The aim of this study was to determine first, if benign fasciculations and those in amyotrophic lateral sclerosis can de distinguished on the basis of their waveforms or firing characteristics, and second to determine how fasciculation parameters evolved with progression of amyotrophic lateral sclerosis. Fasciculation potentials recorded from 63 muscles of 28 patients with definite amyotrophic lateral sclerosis were compared with those from 21 muscles of 11 patients with the benign fasciculation syndrome. In each muscle, at a single site, up to 15 identifiable fasciculation potentials could be recognized. Thus the characteristics of 430 fasciculations from patients with amyotrophic lateral sclerosis and 191 benign fasciculations were analysed. Fasciculation potential amplitude, area, turns, duration, firing interval, indices of waveform variability, evidence of axonal conduction block, evidence of axonal conduction variability and propensity to produce double fasciculations were measured. The waveforms of fasciculations in amyotrophic lateral sclerosis were on average of shorter duration and had a greater number of turns than benign fasciculations, but, although irregular in both conditions, the firing rate in amyotrophic lateral sclerosis was significantly higher. In both conditions, there was evidence of multifocal distal generation of fasciculations, axonal conduction block in the motor unit arborization and of variable axonal conduction. When severe weakness and marked chronic neurogenic change were present on electromyography, the firing rate of fasciculations in amyotrophic lateral sclerosis was higher but fasciculation potential amplitude, area and indices of waveform variability were little changed. Double fasciculations in which the waveforms of the two potentials were the same occurred in both conditions. The intervals were in two bands: an early band with 4-10 ms intervals showed identical waveforms of the two potentials, indicating the region of

  12. Long term follow-up results of dorsal root entry zone lesions for intractable pain after brachial plexus avulsion injuries.

    PubMed

    Chen, H J; Tu, Y K

    2006-01-01

    Brachial plexus avulsion injury is one of the major complications after traffic, especially motorcycle accidents and machine injuries. Intractable pain and paralysis of the affected limbs are the major neurological deficits. During the past 18 years, we have encountered and treated more than 500 cases with brachial plexus avulsion injuries. Dorsal root entry zone lesions (DREZ) made by thermocoagulation were performed for intractable pain in 60 cases. Forty cases were under regular follow-up for 5-18 years. In early postoperative stage, the pain relief rate was excellent or good in 32 cases (80%). The pain relief rate dropped to 60% in 5 year follow-up period and only 9 cases (50%) had excellent or good result in 10 year follow-up. Reconstructive procedures were performed in almost all patients in the last 10 years. Dorsal root entry zone lesion is an effective procedure for pain control after brachial plexus avulsion injuries.

  13. The HTV Proximity Communication System

    NASA Astrophysics Data System (ADS)

    Harada, Motoyuki; Takahashi, Tetsuo; Tanaka, Tetsuo

    2002-01-01

    National Space Development Agency of Japan (NASDA) is developing the H-II Transfer Vehicle (HTV) as an unmanned logistic support vehicle for the International Space Station (ISS). The HTV, which is launched by the H-IIA rocket, transports both pressurized and un-pressurized cargoes to the ISS, reloads disposal items from the ISS and performs destructive reentry over ocean area. NASDA plans the first flight of HTV in 2005 for demonstration. The HTV will contribute the ISS assembly and logistic re-supply operations with international commonality. For unmanned vehicle operation, communication link is critical with commanding and control, especially in proximity region to the ISS. As for the HTV operation, NASDA is developing dedicated communication system installed on the Japanese Experiment Module (JEM) of the ISS, which is called the Proximity Communication System (PROX). The HTV receives commands and transmits telemetry data through the PROX in its proximity operation to the ISS. To achieve this, the PROX can communicate with the HTV on its nominal trajectory within 23km of the ISS. Especially within 3km of the ISS, the PROX has capability to perform omni-directional communication to the HTV. The PROX also has GPS receiver and send GPS data to the HTV to support the HTV navigation for relative GPS navigation used in "far" range (500m away from the ISS). In addition to the above fundamental functions, the PROX has a capability of range and range-rate measurement between the ISS and the HTV by the pseudo noise (PN) code epoch and the Doppler shift frequency. This provides a reference data independent of "main" navigation methods (rendezvous sensor navigation or GPS navigation) of the HTV. The PROX also assists the ISS crew with its commanding device, called the Hardware Command Panel (HCP), to issue time-dependent safety-related critical commands for HTV berthing/un-berthing operation. When a failure occurs somewhere on "communication path" from the PROX Base Band

  14. Proximal Biceps in Overhead Athletes.

    PubMed

    Chalmers, Peter N; Verma, Nikhil N

    2016-01-01

    The proximal long head of the biceps tendon and its attachment at the superior glenoid tubercle and labrum are subject to a spectrum of disorders in overhead athletes. Biceps disorders are commonly characterized by intermittent anterior or deep-seated shoulder pain exacerbated by activity. Diagnosis is reached via various physical examination maneuvers; MRI can be uncertain. Nonsteroidal anti-inflammatory medications, targeted ultrasound-guided corticosteroid injections, and supervised physical therapy are the mainstays of nonoperative treatment. Operative treatment, which remains controversial, provides reliable pain relief, restoration of function for activities of daily living, and low complication rates, but return to play can be unpredictable.

  15. Estimation of central aortic pressure waveform features derived from the brachial cuff volume displacement waveform.

    PubMed

    Butlin, Mark; Qasem, Ahmad; Avolio, Alberto P

    2012-01-01

    There is increasing interest in non-invasive estimation of central aortic waveform parameters in the clinical setting. However, controversy has arisen around radial tonometric based systems due to the requirement of a trained operator or lack of ease of use, especially in the clinical environment. A recently developed device utilizes a novel algorithm for brachial cuff based assessment of aortic pressure values and waveform (SphygmoCor XCEL, AtCor Medical). The cuff was inflated to 10 mmHg below an individual's diastolic blood pressure and the brachial volume displacement waveform recorded. The aortic waveform was derived using proprietary digital signal processing and transfer function applied to the recorded waveform. The aortic waveform was also estimated using a validated technique (radial tonometry based assessment, SphygmoCor, AtCor Medical). Measurements were taken in triplicate with each device in 30 people (17 female) aged 22 to 79 years of age. An average for each device for each individual was calculated, and the results from the two devices were compared using regression and Bland-Altman analysis. A high correlation was found between the devices for measures of aortic systolic (R(2)=0.99) and diastolic (R(2)=0.98) pressure. Augmentation index and subendocardial viability ratio both had a between device R(2) value of 0.82. The difference between devices for measured aortic systolic pressure was 0.5±1.8 mmHg, and for augmentation index, 1.8±7.0%. The brachial cuff based approach, with an individualized sub-diastolic cuff pressure, provides an operator independent method of assessing not only systolic pressure, but also aortic waveform features, comparable to existing validated tonometric-based methods.

  16. Brachial arterial pressure to assess cardiovascular structural damage: an overview and lessons from clinical trials.

    PubMed

    London, Gérald M

    2008-01-01

    Epidemiological studies have emphasized the relationship between blood pressure (BP) and the incidence of cardiovascular diseases. Severity of hypertension was in the past judged on the basis of diastolic BP. More recent epidemiological studies have directed attention to systolic pressure as a better guide to cardiovascular and all-cause mortality. Traditionally, hypertension was appreciated by measures of BP recorded in peripheral arteries, usually brachial artery which was assumed to reflect pressures in all parts of arterial system. All these studies neglected that peripheral systolic BP differs from pressure recorded in the aorta and central arteries. While mean and diastolic pressures are almost constant along the arterial tree, due to the stiffness and geometric heterogeneity of large arteries and the timing and magnitude of wave reflections systolic BP and pulse pressure are amplified from the aorta to peripheral arteries, and brachial systolic BP only indirectly reflects the systolic BP in the aorta and central arteries. Several recent studies have shown that the effects of antihypertensive drugs are not the same in peripheral and central arteries, fact which could account for different effects of various drugs on end-organ damage, such as regression of left ventricular hypertrophy. Moreover, it has been shown that aortic and central artery pressure (or their determinants) are stronger predictors of end-organ damage and cardiovascular outcome than conventionally measured brachial pressure. These studies have focused the attention on the physical properties of large arteries and on the way they influence the level of systolic and pulse pressures along the arterial tree.

  17. Décompression chirurgicale du syndrome de défilé thoraco-brachial

    PubMed Central

    Lukulunga, Loubet Unyendje; Moussa, Abdou Kadri; Mahfoud, Mustapha; Ismael, Farid; Berrada, Mohamed Saleh; El Yaacoubi, Moradh

    2014-01-01

    Le syndrome de défilé thoraco-brachial est une pathologie souvent méconnue à cause de diagnostic difficile par manque des signes pathognomoniques conduisant souvent à des errances. Les manifestations cliniques dépendent selon qu'il s'agit d'une compression nerveuse, vasculaire ou vasculo-nerveuse. Le but de cette étude est de décrire certains aspects cliniques particuliers et évaluer le résultat fonctionnel après la décompression chirurgicale du paquet vasculo-nerveux. Notre étude rétrospective a porté sur l'analyse des données cliniques, radiologiques, IRM et EMG sur les patients opérés entre janvier 2010 et juillet 2013 du syndrome de défilé thoraco-brachial dans le service de traumatologie orthopédie de l'hôpital Ibn Sina de Rabat. 15 cas ont été colligés: 12 cas post traumatiques (fracture de la clavicule) et 3 cas d'origines congénitales, dont l’âge moyen était 35 ans (20 à 50 ans) avec 9 femmes et 6 hommes. A la fin du traitement, le score de Dash est passé de 109 (46% Normal=0) à 70 (20%), et le stress test de Roos était de 70/100 à 80/100. Le résultat était excellent dans 12 cas soit (80%) et moins bon dans dans 3 cas (20%). En définitive, la résection de malformations osseuses, l'excision des brides et la neurolyse du plexus brachial suivie de la rééducation a donné une bonne évolution fonctionnelle. PMID:25709735

  18. [Research in amyotrophic lateral sclerosis: what is new in 2009?].

    PubMed

    Pradat, P-F; Attarian, S; Camdessanché, J-P; Carluer, L; Cintas, P; Corcia, P; Echaniz-Laguna, A; Gonzalez-Bermejo, J; Guy, N; Nicolas, G; Perez, T; Soriani, M-H; Vandenberghe, N; Verschueren, A

    2010-01-01

    This paper, written by French amyotrophic lateral sclerosis (ALS) center experts, presents an update of recent advances in fundamental, epidemiological and clinical research in ALS based on a review of the literature between September 2008 and November 2009. Among other pathophysiological mechanisms, the role of stress of the endoplasmic reticulum and the importance of energetic metabolic disturbances have been underscored. In the field of genetics, research has been advanced through the identification of mutations of the gene FUsed in Sarcoma/Translated in LipoSarcoma (FUS/TLS) in individuals with familial and sporadic ALS. This gene is involved in the regulation of transcription, splicing and RNA transport, and has functional homology to another ALS gene, TARDBP, which suggests that a common mechanism may underlie motor neuron degeneration. A report showed that mice expressing a mutant form of human TDP-43 develop a progressive and fatal neurodegenerative disease reminiscent of both ALS and frontotemporal lobar degeneration with ubiquitin aggregates (FTLD-U), providing a new animal model that may help to better understand the pathophysiology and test new therapeutics. Beside genetic studies, several epidemiologic studies have investigated the role of environmental factors. A recent study suggests that smoking is a risk factor for developing ALS and it is hypothesized that this could occur through lipid peroxidation via formaldehyde exposure. From a neuroprotective perspective, trials with IGF-1, sodium valproate, coenzyme Q or glatiramer acetate have failed to demonstrate any beneficial effect. A study published in 2008 argued that lithium may have a neuroprotective effect in ALS mice and also in patients. However, two preclinical studies failed to replicate the neuroprotective effect of lithium in ALS mice. Therapeutic trials have been performed or are currently ongoing in Europe and North America. Their results have not yet been published.

  19. Identification of Epigenetically Altered Genes in Sporadic Amyotrophic Lateral Sclerosis

    PubMed Central

    Bender, Diane E.; Delaney, Colin E.; Cataldo, Michael D.; Smith, Andrea L.; Yung, Raymond; Ruden, Douglas M.; Callaghan, Brian C.; Feldman, Eva L.

    2012-01-01

    Amyotrophic lateral sclerosis (ALS) is a terminal disease involving the progressive degeneration of motor neurons within the motor cortex, brainstem and spinal cord. Most cases are sporadic (sALS) with unknown causes suggesting that the etiology of sALS may not be limited to the genotype of patients, but may be influenced by exposure to environmental factors. Alterations in epigenetic modifications are likely to play a role in disease onset and progression in ALS, as aberrant epigenetic patterns may be acquired throughout life. The aim of this study was to identify epigenetic marks associated with sALS. We hypothesize that epigenetic modifications may alter the expression of pathogenesis-related genes leading to the onset and progression of sALS. Using ELISA assays, we observed alterations in global methylation (5 mC) and hydroxymethylation (5 HmC) in postmortem sALS spinal cord but not in whole blood. Loci-specific differentially methylated and expressed genes in sALS spinal cord were identified by genome-wide 5mC and expression profiling using high-throughput microarrays. Concordant direction, hyper- or hypo-5mC with parallel changes in gene expression (under- or over-expression), was observed in 112 genes highly associated with biological functions related to immune and inflammation response. Furthermore, literature-based analysis identified potential associations among the epigenes. Integration of methylomics and transcriptomics data successfully revealed methylation changes in sALS spinal cord. This study represents an initial identification of epigenetic regulatory mechanisms in sALS which may improve our understanding of sALS pathogenesis for the identification of biomarkers and new therapeutic targets. PMID:23300739

  20. Systemic inflammatory response and neuromuscular involvement in amyotrophic lateral sclerosis

    PubMed Central

    Lu, Ching-Hua; Allen, Kezia; Oei, Felicia; Leoni, Emanuela; Kuhle, Jens; Tree, Timothy; Fratta, Pietro; Sharma, Nikhil; Sidle, Katie; Howard, Robin; Orrell, Richard; Fish, Mark; Greensmith, Linda; Pearce, Neil; Gallo, Valentina

    2016-01-01

    Objective: To evaluate the combined blood expression of neuromuscular and inflammatory biomarkers as predictors of disease progression and prognosis in amyotrophic lateral sclerosis (ALS). Methods: Logistic regression adjusted for markers of the systemic inflammatory state and principal component analysis were carried out on plasma levels of creatine kinase (CK), ferritin, and 11 cytokines measured in 95 patients with ALS and 88 healthy controls. Levels of circulating biomarkers were used to study survival by Cox regression analysis and correlated with disease progression and neurofilament light chain (NfL) levels available from a previous study. Cytokines expression was also tested in blood samples longitudinally collected for up to 4 years from 59 patients with ALS. Results: Significantly higher levels of CK, ferritin, tumor necrosis factor (TNF)–α, and interleukin (IL)–1β, IL-2, IL-8, IL-12p70, IL-4, IL-5, IL-10, and IL-13 and lower levels of interferon (IFN)–γ were found in plasma samples from patients with ALS compared to controls. IL-6, TNF-α, and IFN-γ were the most highly regulated markers when all explanatory variables were jointly analyzed. High ferritin and IL-2 levels were predictors of poor survival. IL-5 levels were positively correlated with CK, as was TNF-α with NfL. IL-6 was strongly associated with CRP levels and was the only marker showing increasing expression towards end-stage disease in the longitudinal analysis. Conclusions: Neuromuscular pathology in ALS involves the systemic regulation of inflammatory markers mostly active on T-cell immune responses. Disease stratification based on the prognostic value of circulating inflammatory markers could improve clinical trials design in ALS. PMID:27308305

  1. Apelin Deficiency Accelerates the Progression of Amyotrophic Lateral Sclerosis

    PubMed Central

    Kasai, Atsushi; Kinjo, Toshihiko; Ishihara, Rie; Sakai, Ikumi; Ishimaru, Yuki; Yoshioka, Yasuhiro; Yamamuro, Akiko; Ishige, Kumiko; Ito, Yoshihisa; Maeda, Sadaaki

    2011-01-01

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the selective loss of motor neurons. Recent studies have implicated that chronic hypoxia and insufficient vascular endothelial growth factor (VEGF)-dependent neuroprotection may lead to the degeneration of motor neurons in ALS. Expression of apelin, an endogenous ligand for the G protein-coupled receptor APJ, is regulated by hypoxia. In addition, recent reports suggest that apelin protects neurons against glutamate-induced excitotoxicity. Here, we examined whether apelin is an endogenous neuroprotective factor using SOD1G93A mouse model of ALS. In mouse CNS tissues, the highest expressions of both apelin and APJ mRNAs were detected in spinal cord. APJ immunoreactivity was observed in neuronal cell bodies located in gray matter of spinal cord. Although apelin mRNA expression in the spinal cord of wild-type mice was not changed from 4 to 18 weeks age, that of SOD1G93A mice was reduced along with the paralytic phenotype. In addition, double mutant apelin-deficient and SOD1G93A displayed the disease phenotypes earlier than SOD1G93A littermates. Immunohistochemical observation revealed that the number of motor neurons was decreased and microglia were activated in the spinal cord of the double mutant mice, indicating that apelin deficiency pathologically accelerated the progression of ALS. Furthermore, we showed that apelin enhanced the protective effect of VEGF on H2O2-induced neuronal death in primary neurons. These results suggest that apelin/APJ system in the spinal cord has a neuroprotective effect against the pathogenesis of ALS. PMID:21887354

  2. Amyotrophic lateral sclerosis among cross-country skiers in Sweden.

    PubMed

    Fang, Fang; Hållmarker, Ulf; James, Stefan; Ingre, Caroline; Michaëlsson, Karl; Ahlbom, Anders; Feychting, Maria

    2016-03-01

    A highly increased risk of amyotrophic lateral sclerosis (ALS) has been suggested among professional athletes. We aimed to examine whether long distance cross-country skiers have also a higher risk of ALS and whether the increased risk was modified by skiing performance. We followed 212,246 cross-country skiers in the Swedish Vasaloppet cohort and a random selection of 508,176 general Swedes not participating in the Vasaloppet during 1989-2010. The associations between cross-country skiing as well as skiing performance (i.e., type of race, finishing time and number of races) and the consequent risk of ALS were estimated through hazard ratios (HRs) derived from Cox model. During the study, 39 cases of ALS were ascertained among the skiers. The fastest skiers (100-150% of winner time) had more than fourfold risk of ALS (HR 4.31, 95% confidence interval [CI] 1.78-10.4), as compared to skiers that finished at >180% of winner time. Skiers who participated >4 races during this period had also a higher risk (HR 3.13, 95% CI 1.37-7.17) than those participated only one race. When compared to the non-skiers, the fastest skiers still had a higher risk (HR 2.08, 95% CI 1.12-3.84), as skiers who had >4 races (HR 1.88, 95% CI 1.05-3.35), but those finishing at >180% of winner time had a lower risk (HR 0.46, 95% CI 0.24-0.87). In conclusion, long distance cross-country skiing is associated with a higher risk of ALS, but only among the best skiers; recreational skiers appear to have a largely reduced risk.

  3. [Respiratory domain of revised amyotrophic lateral sclerosis. Functional Rating Scale].

    PubMed

    Lima, Sandra E; Pessolano, Fernando A; Monteiro, Sergio G; De Vito, Eduardo L

    2009-01-01

    Virtually all patients with amyotrophic lateral sclerosis will complain of dyspnea, which is perhaps the most distressing symptom of this devastating disease. The objective was to correlate respiratory domain of ALSFRS-R with forced vital capacity and maximal static pressures in the mouth. We designed a prospective study in 20 consecutive patients without dyspnea during 24 months. The global decline of ALSFRS-R was from 34.3 +/- 10.3 to 22.1 +/- 8.0 (p = 0.0325), the contribution of respiratory domain was irrelevant. Those who referred dyspnea (n: 12), forced vital capacity fell 41 +/- 21% of the initial value but with similar value of fall (46 +/- 23%) 8 patients did not referred dyspnea. Total score of ALSFRS-R correlated with forced vital capacity (litres), r: 0.73, p = 0.0016 and maximal inspiratory pressure (cm H2O), r: 0.84, p = 0.0038, but the fall of the forced vital capacity (%) did not correlate with dyspnea (r(s): 0.23, p = 0.1400). There was a moderate correlation between dyspnea and maximal inspiratory pressure (%), r(s): 0.58, p = 0.0300 and between dyspnea and maximal expiratory pressure (%), r(s): 0.49, p = 0.0400. We concluded that the respiratory functional deterioration could not be predicted using respiratory domain of ALSFRS-R. This suggests that respiratory domain of this scale does not replace to respiratory function testing measurements and, due to the respiratory insufficiency could not be clinically evident; performing pulmonary function tests provides an objective view and permit to make anticipatory actions.

  4. Alzheimer disease and amyotrophic lateral sclerosis: An etiopathogenic connection

    PubMed Central

    Wang, Xiaochuan; Blanchard, Julie; Grundke-Iqbal, Inge; Wegiel, Jerzy; Deng, Han-Xiang; Siddique, Teepu; Iqbal, Khalid

    2013-01-01

    The etiopathogenesis of neither the sporadic form of Alzheimer disease (AD) nor of amyotrophic lateral sclerosis (ALS) are well understood. The activity of protein phosphatase-2A (PP2A), which regulates the phosphorylation of tau and neurofilaments, is negatively regulated by the myeloid leukemia-associated protein SET, also known as inhibitor-2 of PP2A, I2PP2A. In AD brain PP2A activity is compromised, probably because I2PP2A is overexpressed and is selectively cleaved at asparagine 175 into an N-terminal fragment, I2NTF, and a C-terminal fragment, I2CTF, and both fragments inhibit PP2A. Here we analyzed the spinal cords from ALS and control cases for I2PP2A cleavage and PP2A activity. As observed in AD brain, we found a selective increase in the cleavage of I2PP2A into I2NTF and I2CTF and inhibition of the activity and not the expression of PP2A in the spinal cords of ALS cases. To test the hypothesis that both AD and ALS could be triggered by I2CTF, a cleavage product of I2PP2A, we transduced by intracerebroventricular injections newborn rats with adeno-associated virus serotype 1 (AAV1) containing human I2CTF. AAV1- I2CTF produced reference memory impairment and tau pathology, and intraneuronal accumulation of Aβ by 5–8 months, and motor deficit and hyperphosphorylation and proliferation of neurofilaments, tau and TDP-43 pathologies, degeneration and loss of motor neurons and axons in the spinal cord by 10–14 months in rats. These findings suggest a previously undiscovered etiopathogenic relationship between sporadic forms of AD and ALS that is linked to I2PP2A and the potential of I2PP2A-based therapeutics for these diseases. PMID:24136402

  5. Mutant SOD1 mediated pathogenesis of Amyotrophic Lateral Sclerosis.

    PubMed

    Kaur, Simran J; McKeown, Stephanie R; Rashid, Shazia

    2016-02-15

    Amyotrophic lateral sclerosis (ALS) is a neural disorder that causes death of the motor neurons in the brain and spinal cord; this affects the voluntary muscles and gradually leads to paralysis of the whole body. Most ALS cases are sporadic, though about 5-10% are familial. ALS is caused by multiple factors including mutation in any one of a number of specific genes, one of the most frequently affected is superoxide dismutase (SOD) 1. Alterations in SOD 1 have been linked with several variants of familial ALS. SOD 1 is a powerful antioxidant enzyme that protects cells from the damaging effects of superoxide radicals. The enzyme binds both copper and zinc ions that are directly involved in the deactivation of toxic superoxide radicals. Mutated SOD1 gene can acquire both gain and loss of function mutations. The most commonly identified mutations in SOD1 that affect protein activity are D90A, A4V and G93A. Deleterious mutations have been shown to modify SOD1 activity, which leads to the accumulation of highly toxic hydroxyl radicals. Accumulation of these free radicals causes degradation of both nuclear and mitochondrial DNA and protein misfolding, features which can be used as pathological indicators associated with ALS. Numerous clinical trials have been carried out over last few years with limited success. In some patients advanced techniques like gene and stem cell therapy have been trialed. However no definitive treatment option can provide a cure and currently ALS is managed by drugs and other supportive therapies. Consequently there is a need to identify new approaches for treatment of this ultimately fatal disease.

  6. Prospective study of chemical exposures and amyotrophic lateral sclerosis

    PubMed Central

    Weisskopf, MG; Morozova, N; O'Reilly, EJ; McCullough, ML; Calle, EE; Thun, MJ; Ascherio, A

    2009-01-01

    Background Although environmental toxins, including pesticides, are suspected of contributing to the risk of amyotrophic lateral sclerosis (ALS), no data exist from large prospective investigations. This study assessed the association between exposure to chemicals and risk of ALS in a prospective cohort study. Methods We prospectively assessed the relation between self-report of regular exposure to 11 different chemical classes or X-rays and ALS mortality among over 1 million participants in the American Cancer Society's Cancer Prevention Study II. Follow-up from 1989 through 2004 identified 617 deaths from ALS among men and 539 among women. We calculated adjusted rate ratios (RR) using Cox proportional hazards. Results The RR for ALS mortality among individuals exposed to pesticides/herbicides compared to the unexposed was 1.07 (95% confidence interval (CI): 0.79-1.44), but somewhat higher after excluding those with missing duration of pesticides exposure (RR: 1.44; 95% CI: 0.89-2.31; p=0.14). A non-significant increase in ALS mortality was found among individuals who reported exposure to formaldehyde (RR: 1.34; 95% CI: 0.93-1.92). Excluding those with missing duration of formaldehyde exposure, the RR was 2.47 (95% CI: 1.58-3.86), and there was a strongly significant dose-response relation with increasing years of exposure (p-trend=0.0004). Conclusions We found little evidence for an association between pesticides/herbicide exposure and ALS. In contrast, we found evidence suggesting an increased risk of ALS with formaldehyde exposure. Because of the longitudinal design, this result is unlikely to be due to bias, but it should nevertheless be interpreted cautiously and needs to be independently verified. PMID:19372290

  7. PHOSPHORYLATED TAU: CANDIDATE BIOMARKER FOR AMYOTROPHIC LATERAL SCLEROSIS

    PubMed Central

    Grossman, Murray; Elman, Lauren; McCluskey, Leo; McMillan, Corey T.; Boller, Ashley; Powers, John; Rascovsky, Katya; Hu, William; Shaw, Les; Irwin, David J.; Lee, Virginia M.-Y.; Trojanowski, John Q.

    2014-01-01

    IMPORTANCE An increasingly varied clinical spectrum of cases with amyotrophic lateral sclerosis (ALS) has been identified, and objective criteria for clinical trial eligibility is necessary. OBJECTIVE We sought to develop a cerebrospinal fluid (CSF) biomarker sensitive and specific for the diagnosis of ALS. DESIGN Case-control study. SETTING Academic medical center. PARTICIPANTS 51 individuals with ALS and 23 individuals with a disorder associated with a four-repeat tauopathy (4R-tau). MAIN OUTCOME MEASURE CSF level of tau phosophorylated at threonine 181 (ptau), and ratio of ptau to total tau (ttau). RESULTS Using a cross-validation prediction procedure, we found significantly reduced CSF levels of ptau and ptau:ttau in ALS relative to 4R-tau and to controls. In the validation cohort, the receiver operating characteristic area under the curve for the ptau:ttau ratio was 0.916, and the comparison of ALS to 4R-tau showed sensitivity=92% and specificity=91.7%. Correct classification based on low CSF ptau:ttau was confirmed in 18 (85.7%) of 21 cases with autopsy-proven or genetically-determined disease. In patients with available measures, ptau:ttau in ALS correlated with clinical measures of disease severity such as Mini Mental State Exam (n=51) and ALS Functional Rating Scale-Revised (n=42), and regression analyses related ptau:ttau to MRI (n=10) evidence of disease in the corticospinal tract and white matter projections involving prefrontal cortex. CONCLUSIONS AND RELEVANCE CSF ptau:ttau may be a candidate biomarker to provide objective support for the diagnosis of ALS. PMID:24492862

  8. Laryngeal Sensitivity in Patients with Amyotrophic Lateral Sclerosis

    PubMed Central

    Ruoppolo, Giovanni; Onesti, Emanuela; Gori, Maria Cristina; Schettino, Ilenia; Frasca, Vittorio; Biasiotta, Antonella; Giordano, Carla; Ceccanti, Marco; Cambieri, Chiara; Greco, Antonio; Buonopane, Costantino Eugenio; Cruccu, Giorgio; De Vincentiis, Marco; Inghilleri, Maurizio

    2016-01-01

    Recent studies have shown the involvement of the sensory nervous system in patients with amyotrophic lateral sclerosis (ALS). The aim of our study was to investigate the correlation between the laryngeal sensitivity deficit and the type of ALS onset (bulbar or spinal) in a large series of 114 consecutive ALS patients. Participants were subdivided into two groups, bulbar and spinal ALS, according to the clinical onset of disease and submitted to a clinical and instrumental evaluation of swallowing, including a fiber-optic endoscopic evaluation of swallowing with sensory testing. Dysphagia severity was scored using the Penetration–Aspiration Scale (PAS) and the Pooling score (P-score). In addition, three patients with laryngeal sensitivity deficit were submitted to a laryngeal biopsy to assess the status of the sensory innervation. All patients showed a normal glottal closure during phonation and volitional cough. Fifty-six subjects (49%), 14 spinal- and 42 bulbar-onset ALS, showed dysphagia at the first clinical observation (PAS score >1; P-score >5). Dysphagia resulted more frequently in bulbar-onset ALS (P < 0.01). Thirty-eight (33%) patients had a sensory deficit of the larynx. The sensory deficit of the larynx was significantly more frequent in bulbar-onset ALS (P < 0.01). The sensory deficit of the larynx among dysphagic patients was also significantly more frequent in bulbar-onset ALS (P = 0.02). Several abnormalities were found in all three subjects who underwent a laryngeal biopsy: in one patient, no intraepidermal fiber was found; in the other two, the fibers showed morphological changes. Our observations are important to consider for assessment and management of dysphagia in patients with ALS. PMID:27965622

  9. Alzheimer disease and amyotrophic lateral sclerosis: an etiopathogenic connection.

    PubMed

    Wang, Xiaochuan; Blanchard, Julie; Grundke-Iqbal, Inge; Wegiel, Jerzy; Deng, Han-Xiang; Siddique, Teepu; Iqbal, Khalid

    2014-02-01

    The etiopathogenesis of neither the sporadic form of Alzheimer disease (AD) nor of amyotrophic lateral sclerosis (ALS) is well understood. The activity of protein phosphatase-2A (PP2A), which regulates the phosphorylation of tau and neurofilaments, is negatively regulated by the myeloid leukemia-associated protein SET, also known as inhibitor-2 of PP2A, I2(PP2A). In AD brain, PP2A activity is compromised, probably because I2(PP2A) is overexpressed and is selectively cleaved at asparagine 175 into an N-terminal fragment, I2NTF, and a C-terminal fragment, I2CTF, and both fragments inhibit PP2A. Here, we analyzed the spinal cords from ALS and control cases for I2(PP2A) cleavage and PP2A activity. As observed in AD brain, we found a selective increase in the cleavage of I2(PP2A) into I2NTF and I2CTF and inhibition of the activity and not the expression of PP2A in the spinal cords of ALS cases. To test the hypothesis that both AD and ALS could be triggered by I2CTF, a cleavage product of I2(PP2A), we transduced by intracerebroventricular injections newborn rats with adeno-associated virus serotype 1 (AAV1) containing human I2CTF. AAV1-I2CTF produced reference memory impairment and tau pathology, and intraneuronal accumulation of Aβ by 5-8 months, and motor deficit and hyperphosphorylation and proliferation of neurofilaments, tau and TDP-43 pathologies, degeneration and loss of motor neurons and axons in the spinal cord by 10-14 months in rats. These findings suggest a previously undiscovered etiopathogenic relationship between sporadic forms of AD and ALS that is linked to I2(PP2A) and the potential of I2(PP2A)-based therapeutics for these diseases.

  10. Noninvasive ventilation reduces energy expenditure in amyotrophic lateral sclerosis

    PubMed Central

    2014-01-01

    Background Amyotrophic lateral sclerosis (ALS) leads to chronic respiratory failure. Diaphragmatic dysfunction, a major driver of dyspnea and mortality, is associated with a shift of the burden of ventilation to extradiaphragmatic inspiratory muscles, including neck muscles. Besides, energy expenditure is often abnormally high in ALS, and this is associated with a negative prognostic value. We hypothesized that noninvasive ventilation (NIV) would relieve inspiratory neck muscles and reduce resting energy expenditure (REE). Methods Using indirect calorimetry, we measured REE during spontaneous breathing (REESB) and NIV (REENIV) in 16 ALS patients with diaphragmatic dysfunction, during the first 3 months of NIV. Measured values were compared with predicted REE (REEpred)(Harris-Benedict equation). Results NIV abolished inspiratory neck muscle activity. Even though our patients were not hypermetabolic, on the contrary, with a REESB that was lower than REEpred (average 11%), NIV did reduce energy expenditure. Indeed, median REENIV, in this population with a mean body mass index of 21.4 kg.m-2, was 1149 kcal/24 h [interquartile 970-1309], lower than REESB (1197 kcal/24 h, 1054-1402; mean difference 7%; p = 0.03, Wilcoxon). REESB and REENIV were correlated with forced vital capacity and maximal inspiratory pressure. Conclusions NIV can reduce energy expenditure in ALS patients probably by alleviating the ventilatory burden imposed on inspiratory neck muscles to compensate diaphragm weakness. It remains to be elucidated whether or not, in which population, and to what extent, NIV can be beneficial in ALS through the corresponding reduction in energy expenditure. PMID:24507664

  11. Glial activation colocalizes with structural abnormalities in amyotrophic lateral sclerosis

    PubMed Central

    Alshikho, Mohamad J.; Zürcher, Nicole R.; Loggia, Marco L.; Cernasov, Paul; Chonde, Daniel B.; Izquierdo Garcia, David; Yasek, Julia E.; Akeju, Oluwaseun; Catana, Ciprian; Rosen, Bruce R.; Cudkowicz, Merit E.

    2016-01-01

    Objective: In this cross-sectional study, we aimed to evaluate brain structural abnormalities in relation to glial activation in the same cohort of participants. Methods: Ten individuals with amyotrophic lateral sclerosis (ALS) and 10 matched healthy controls underwent brain imaging using integrated MR/PET and the radioligand [11C]-PBR28. Diagnosis history and clinical assessments including Upper Motor Neuron Burden Scale (UMNB) were obtained from patients with ALS. Diffusion tensor imaging (DTI) analyses including tract-based spatial statistics and tractography were applied. DTI metrics including fractional anisotropy (FA) and diffusivities (mean, axial, and radial) were measured in regions of interest. Cortical thickness was assessed using surface-based analysis. The locations of structural changes, measured by DTI and the areas of cortical thinning, were compared to regional glial activation measured by relative [11C]-PBR28 uptake. Results: In this cohort of individuals with ALS, reduced FA and cortical thinning colocalized with regions demonstrating higher radioligand binding. [11C]-PBR28 binding in the left motor cortex was correlated with FA (r = −0.68, p < 0.05) and cortical thickness (r = −0.75, p < 0.05). UMNB was correlated with glial activation (r = +0.75, p < 0.05), FA (r = −0.77, p < 0.05), and cortical thickness (r = −0.75, p < 0.05) in the motor cortex. Conclusions: Increased uptake of the glial marker [11C]-PBR28 colocalizes with changes in FA and cortical thinning. This suggests a link between disease mechanisms (gliosis and inflammation) and structural changes (cortical thinning and white and gray matter changes). In this multimodal neuroimaging work, we provide an in vivo model to investigate the pathogenesis of ALS. PMID:27837005

  12. Is amyotrophic lateral sclerosis a primary astrocytic disease?

    PubMed

    Sica, Roberto E

    2012-12-01

    Amyotrophic lateral sclerosis (ALS) is thought to be due to primary involvement of motor neurons. Pathogenic mechanisms underlying its appearance are relatively well known and include inflammation, excitotoxicity, oxidative stress, endoplasmic reticulum stress, protein damage, genetic abnormalities and type of neuronal death. Although these processes have been investigated in detail in the past two decades none of them appear to be the cause of the illness. In addition several possible environmental agents have been investigated but the results, in every case, were conflicting and therefore inconclusive. However, since the motor neurons display the features of apoptosis in this illness, the possibility remains that the motor neurons die because of a hostile environment, one that is unable to sustain their health, rather than being directly targeted themselves. The above considerations lead to an examination of astrocytes, for these cells play a key role in controlling the environment of neurons. It is known that astrocytes are exquisitely plastic, adapting their metabolism and behaviour to the needs of the neurons they contact. Each population of astrocytes is therefore unique and, were one to be adversely affected at the start of a disease process, the consequences would extend to the neurons that it normally chaperoned. The disturbed relationship might involve inappropriate production and secretion of astrocytic neurotransmitters, defective transport of glutamate and impaired trophic and metabolic support of the motor neurons. In order to explain the spread of weakness and pyramidal signs in ALS patients, which is very often from one group of muscles to a neighbouring one, it is postulated that, within the spinal cord, the brainstem and the motor cortex, the disease-causing process is also spreading-in this case, from one group of astrocytes to its neighbours. A misfolded protein, possibly a prion-like protein, would be a candidate for this type of transmission.

  13. Successful management of complex regional pain syndrome type 1 using single injection interscalene brachial plexus block

    PubMed Central

    Fallatah, Summayah M.A.

    2014-01-01

    Complex regional pain syndrome (CRPS) type 1 of the upper limb is a painful and debilitating condition. Interscalene brachial plexus block (ISB) in conjugation with other modalities was shown to be a feasible therapy with variable success. We reported a case of CRPS type 1 as diagnosed by International Association for the Study of Pain criteria in which pharmacological approaches failed to achieve adequate pain relief and even were associated with progressive dysfunction of the upper extremity. Single injection ISB, in combination with physical therapy and botulinum toxin injection, was successful to alleviate pain with functional restoration. PMID:25422619

  14. Reactivity to low-flow as a potential determinant for brachial artery flow-mediated vasodilatation.

    PubMed

    Aizawa, Kunihiko; Elyas, Salim; Adingupu, Damilola D; Casanova, Francesco; Gooding, Kim M; Strain, W David; Shore, Angela C; Gates, Phillip E

    2016-06-01

    Previous studies have reported a vasoconstrictor response in the radial artery during a cuff-induced low-flow condition, but a similar low-flow condition in the brachial artery results in nonuniform reactivity. This variable reactivity to low-flow influences the subsequent flow-mediated dilatation (FMD) response following cuff-release. However, it is uncertain whether reactivity to low-flow is important in data interpretation in clinical populations and older adults. This study aimed to determine the influence of reactivity to low-flow on the magnitude of brachial artery FMD response in middle-aged and older individuals with diverse cardiovascular risk profiles. Data were analyzed from 165 individuals, divided into increased cardiovascular risk (CVR: n = 115, 85M, 67.0 ± 8.8 years) and healthy control (CTRL: n = 50, 30M, 63.2 ± 7.2 years) groups. Brachial artery diameter and blood velocity data obtained from Doppler ultrasound were used to calculate FMD, reactivity to low-flow and estimated shear rate (SR) using semiautomated edge-detection software. There was a significant association between reactivity to low-flow and FMD in overall (r = 0.261), CTRL (r = 0.410) and CVR (r = 0.189, all P < 0.05) groups. Multivariate regression analysis found that reactivity to low-flow, peak SR, and baseline diameter independently contributed to FMD along with sex, the presence of diabetes, and smoking (total R(2) = 0.450). There was a significant association between reactivity to low-flow and the subsequent FMD response in the overall dataset, and reactivity to low-flow independently contributed to FMD These findings suggest that reactivity to low-flow plays a key role in the subsequent brachial artery FMD response and is important in the interpretation of FMD data.

  15. Brachial artery injury due to closed posterior elbow dislocation: case report☆

    PubMed Central

    Miyazaki, Alberto Naoki; Fregoneze, Marcelo; Santos, Pedro Doneux; do Val Sella, Guilherme; Checchia, Caio Santos; Checchia, Sergio Luiz

    2016-01-01

    An association between closed posterior elbow dislocation and traumatic brachial artery injury is rare. Absence of radial pulse on palpation is an important warning sign and arteriography is the gold-standard diagnostic test. Early diagnosis is essential for appropriate treatment to be provided. This consists of joint reduction and immobilization, along with urgent surgical restoration of arterial flow. Here, a case (novel to the Brazilian literature) of an association between these injuries (and the treatment implemented) in a 27-year-old male patient is reported. These injuries were sustained through physical assault. PMID:27069896

  16. Brachial artery injury due to closed posterior elbow dislocation: case report.

    PubMed

    Miyazaki, Alberto Naoki; Fregoneze, Marcelo; Santos, Pedro Doneux; do Val Sella, Guilherme; Checchia, Caio Santos; Checchia, Sergio Luiz

    2016-01-01

    An association between closed posterior elbow dislocation and traumatic brachial artery injury is rare. Absence of radial pulse on palpation is an important warning sign and arteriography is the gold-standard diagnostic test. Early diagnosis is essential for appropriate treatment to be provided. This consists of joint reduction and immobilization, along with urgent surgical restoration of arterial flow. Here, a case (novel to the Brazilian literature) of an association between these injuries (and the treatment implemented) in a 27-year-old male patient is reported. These injuries were sustained through physical assault.

  17. [Current concepts in perinatal brachial plexus palsy. Part 2: late phase. Shoulder deformities].

    PubMed

    Dogliotti, Andrés Alejandro

    2011-10-01

    The incidence of obstetric brachial palsy is high and their sequelaes are frequent. Physiotherapy, microsurgical nerve reconstruction and secondary corrections are used together to improve the shoulder function. The most common posture is shoulder in internal rotation and adduction, because of the antagonist weakness. The muscle forces imbalance over the osteoarticular system, will result in a progressive glenohumeral joint deformity which can be recognized with a magnetic resonance image. Tendon transfers of the internal rotators towards the external abductor/rotator muscles, has good results, but has to be combined with antero-inferior soft-tissue releases, if passive range of motion is limited.

  18. Recurrent upper limb ischaemia due to a crutch-induced brachial artery aneurysm.

    PubMed

    Furukawa, Kouji; Hayase, Takahiro; Yano, Mitsuhiro

    2013-07-01

    An 83-year old man who had used bilateral axillary crutches for 67 years was referred to our hospital for acute left upper limb ischaemia. He underwent successful recanalization through emergent catheter thromboembolectomy. However, a crutch-induced left brachial artery aneurysm was subsequently detected by computed tomography. Therefore, we performed aneurysm exclusion and subsequent saphenous vein bypass grafting. When a crutch user presents with upper limb ischaemia, a high index of suspicion and early identification of the crutch induced vascular injury are mandatory for appropriate treatment.

  19. Nerve transfers in brachial plexus birth palsies: indications, techniques, and outcomes.

    PubMed

    Kozin, Scott H

    2008-11-01

    The advent of nerve transfers has greatly increased surgical options for children who have brachial plexus birth palsies. Nerve transfers have considerable advantages, including easier surgical techniques, avoidance of neuroma resection, and direct motor and sensory reinnervation. Therefore, any functioning nerve fibers within the neuroma are preserved. Furthermore, a carefully selected donor nerve results in little or no clinical deficit. However, some disadvantages and unanswered questions remain. Because of a lack of head-to-head comparison between nerve transfers and nerve grafting, the window of opportunity for nerve grafting may be missed, which may degrade the ultimate outcome. Time will tell the ultimate role of nerve transfer or nerve grafting.

  20. Heralding Extramedullary Blast Crisis: Horner's Syndrome with Brachial Plexopathy in a Patient with Chronic Myelogenous Leukemia

    PubMed Central

    Patil, Sadanand I.

    2016-01-01

    Chronic myelogenous leukemia (CML) blast crisis is an ominous clinical event that is challenging to treat. This can develop at extramedullary sites rarely and is defined as the infiltration of blasts outside the bone marrow irrespective of proliferation of blasts within the bone marrow. We aim to report an unusual clinical presentation characterized by Horner's syndrome, ipsilateral arm weakness, and cervical lymphadenopathy as the first signs of extramedullary blast crisis in a CML patient. To the best of our knowledge, the extramedullary locations involving the brachial plexus along with cervicothoracic paraspinal chloroma have not been previously reported in the literature. PMID:28096817

  1. Effect of salt intake and potassium supplementation on brachial-ankle pulse wave velocity in Chinese subjects: an interventional study

    PubMed Central

    Wang, Y.; Mu, J.J.; Geng, L.K.; Wang, D.; Ren, K.Y.; Guo, T.S.; Chu, C.; Xie, B.Q.; Liu, F.Q.; Yuan, Z.Y.

    2014-01-01

    Accumulating evidence has suggested that high salt and potassium might be associated with vascular function. The aim of this study was to investigate the effect of salt intake and potassium supplementation on brachial-ankle pulse wave velocity (PWV) in Chinese subjects. Forty-nine subjects (28-65 years of age) were selected from a rural community of northern China. All subjects were sequentially maintained on a low-salt diet for 7 days (3.0 g/day NaCl), a high-salt diet for an additional 7 days (18.0 g/day NaCl), and a high-salt diet with potassium supplementation for a final 7 days (18.0 g/day NaCl+4.5 g/day KCl). Brachial-ankle PWV was measured at baseline and on the last day of each intervention. Blood pressure levels were significantly increased from the low-salt to high-salt diet, and decreased from the high-salt diet to high-salt plus potassium supplementation. Baseline brachial-ankle PWV in salt-sensitive subjects was significantly higher than in salt-resistant subjects. There was no significant change in brachial-ankle PWV among the 3 intervention periods in salt-sensitive, salt-resistant, or total subjects. No significant correlations were found between brachial-ankle PWV and 24-h sodium and potassium excretions. Our study indicates that dietary salt intake and potassium supplementation, at least in the short term, had no significant effect on brachial-ankle PWV in Chinese subjects. PMID:25493387

  2. Brachial Plexopathy in Apical Non-Small Cell Lung Cancer Treated With Definitive Radiation: Dosimetric Analysis and Clinical Implications

    SciTech Connect

    Eblan, Michael J.; Corradetti, Michael N.; Lukens, J. Nicholas; Xanthopoulos, Eric; Mitra, Nandita; Christodouleas, John P.; Grover, Surbhi; Fernandes, Annemarie T.; Langer, Corey J.; Evans, Tracey L.; Stevenson, James; Rengan, Ramesh; Apisarnthanarax, Smith

    2013-01-01

    Purpose: Data are limited on the clinical significance of brachial plexopathy in patients with apical non-small cell lung cancers (NSCLC) treated with definitive radiation therapy. We report the rates of radiation-induced brachial plexopathy (RIBP) and tumor-related brachial plexopathy (TRBP) and associated dosimetric parameters in apical NSCLC patients. Methods and Materials: Charts of NSCLC patients with primary upper lobe or superiorly located nodal disease who received {>=}50 Gy of definitive conventionally fractionated radiation or chemoradiation were retrospectively reviewed for evidence of brachial plexopathy and categorized as RIBP, TRBP, or trauma-related. Dosimetric data were gathered on ipsilateral brachial plexuses (IBP) contoured according to Radiation Therapy Oncology Group atlas guidelines. Results: Eighty patients were identified with a median follow-up and survival time of 17.2 and 17.7 months, respectively. The median prescribed dose was 66.6 Gy (range, 50.4-84.0), and 71% of patients received concurrent chemotherapy. RIBP occurred in 5 patients with an estimated 3-year rate of 12% when accounting for competing risk of death. Seven patients developed TRBP (estimated 3-year rate of 13%), comprising 24% of patients who developed locoregional failures. Grade 3 brachial plexopathy was more common in patients who experienced TRBP than RIBP (57% vs 20%). No patient who received {<=}78 Gy to the IBP developed RIBP. On multivariable competing risk analysis, IBP V76 receiving {>=}1 cc, and primary tumor failure had the highest hazard ratios for developing RIBP and TRBP, respectively. Conclusions: RIBP is a relatively uncommon complication in patients with apical NSCLC tumors receiving definitive doses of radiation, while patients who develop primary tumor failures are at high risk for developing morbid TRBP. These findings suggest that the importance of primary tumor control with adequate doses of radiation outweigh the risk of RIBP in this population of

  3. Thrombin Injection for Treatment of Brachial Artery Pseudoaneurysm at the Site of a Hemodialysis Fistula: Report of Two Patients

    SciTech Connect

    Clark, Timothy W.I.; Abraham, Robert J.

    2000-09-15

    We report two patients with arteriovenous hemodialysis fistulas that were complicated by brachial artery pseudoaneurysms. Each pseudoanerysm was percutaneously thrombosed with an injection of thrombin, using techniques to prevent escape of thrombin into the native brachial artery. In one patient, an angioplasty balloon was inflated across the neck of the aneurysm during thrombin injection. In the second patient, thrombin was injected during ultrasound-guided compression of the neck of the pseudoaneurysm. Complete thrombosis of each pseudoaneurysm was achieved within 30 sec. No ischemic or embolic events occurred. This technique may be useful in treating pseudoaneurysms of smaller peripheral arteries.

  4. Brachial artery transection associated with open elbow dislocation in a 12-year-old: a case report.

    PubMed

    Nazli, Yunus; Colak, Necmettin; Uras, Ismail; Komurcu, Mahmut; Cakir, Omer

    2013-02-01

    Although acute elbow dislocations are common orthopedic injuries, concomitant neurovascular injury is rare. Brachial artery transection can result from open elbow dislocation and responds well to vascular repair. Rapid evaluation and a high level of suspicion are essential to facilitate immediate treatment. Delay to identify vascular injury after elbow dislocation or reduction can potentially lead to limb ischemia, and potential loss of limb. We present a case of relatively rare transection of the brachial artery, with an accompanying traumatic open elbow dislocation in a 12-year-old boy.

  5. Brachial blood pressure-independent relations between radial late systolic shoulder-derived aortic pressures and target organ changes.

    PubMed

    Norton, Gavin R; Majane, Olebogeng H I; Maseko, Muzi J; Libhaber, Carlos; Redelinghuys, Michelle; Kruger, Deirdre; Veller, Martin; Sareli, Pinhas; Woodiwiss, Angela J

    2012-04-01

    Central aortic blood pressure (BP; BPc) predicts outcomes beyond brachial BP. In this regard, the application of a generalized transfer function (GTF) to radial pulse waves for the derivation of BPc is an easy and reproducible measurement technique. However, the use of the GTF may not be appropriate in all circumstances. Although the peak of the second shoulder of the radial waveform (P2) is closely associated with BPc, and, hence, BPc may be assessed without the need for a GTF, whether P2-derived BPc is associated with adverse cardiovascular changes independent of brachial BP is uncertain. Thus, P2- and GTF-derived aortic BPs were assessed using applanation tonometry and SphygmoCor software. Left ventricular mass was indexed for height(1.7) (n=678) and carotid intima-media thickness (IMT; n=462) was determined using echocardiography and vascular ultrasound. With adjustments for nurse-derived brachial pulse pressure (PP), P2-derived central PP was independently associated with left ventricular mass indexed for height(1.7) (partial r=0.18; P<0.0001) and IMT (partial r=0.40; P<0.0001). These relations were similar to nurse-derived brachial PP-independent relations between GTF-derived central PP and target organ changes (left ventricular mass indexed for height(1.7): partial r=0.17, P<0.0001; IMT: partial r=0.37, P<0.0001). In contrast, with adjustments for central PP, nurse-derived brachial PP-target organ relations were eliminated (partial r=-0.21 to 0.05). Twenty-four-hour, day, and night PP-target organ relations did not survive adjustments for nurse-derived brachial BP. In conclusion, central PP derived from P2, which does not require a GTF, is associated with cardiovascular target organ changes independent of brachial BP. Thus, when assessing adverse cardiovascular effects of aortic BP independent of brachial BP, P2-derived measures may complement GTF-derived measures of aortic BP.

  6. Protein Neighbors and Proximity Proteomics.

    PubMed

    Rees, Johanna S; Li, Xue-Wen; Perrett, Sarah; Lilley, Kathryn S; Jackson, Antony P

    2015-11-01

    Within cells, proteins can co-assemble into functionally integrated and spatially restricted multicomponent complexes. Often, the affinities between individual proteins are relatively weak, and proteins within such clusters may interact only indirectly with many of their other protein neighbors. This makes proteomic characterization difficult using methods such as immunoprecipitation or cross-linking. Recently, several groups have described the use of enzyme-catalyzed proximity labeling reagents that covalently tag the neighbors of a targeted protein with a small molecule such as fluorescein or biotin. The modified proteins can then be isolated by standard pulldown methods and identified by mass spectrometry. Here we will describe the techniques as well as their similarities and differences. We discuss their applications both to study protein assemblies and to provide a new way for characterizing organelle proteomes. We stress the importance of proteomic quantitation and independent target validation in such experiments. Furthermore, we suggest that there are biophysical and cell-biological principles that dictate the appropriateness of enzyme-catalyzed proximity labeling methods to address particular biological questions of interest.

  7. Fibrosis: ultimate and proximate causes

    PubMed Central

    Thannickal, Victor J.; Zhou, Yong; Gaggar, Amit; Duncan, Steven R.

    2014-01-01

    Fibrotic disorders account for an increasing burden of disease-associated morbidity and mortality worldwide. Although numerous risk factors have been recognized, the etiologies of many of these clinical syndromes have not been identified, and they are often termed idiopathic or cryptogenic. Here, we provide an evolutionary perspective on fibrosis aimed at elucidating its etiopathogenesis. By asking the ultimate question of “why” this process evolved in multicellular organisms, we hope to uncover proximate explanations for “how” it causes disease in humans. We posit that physiological fibrosis-like reactions evolved as an essential process in host defense against pathogens and in normal wound healing. Based on this premise, we reason that pathological fibrosis is related to one or more of the following: unidentified infectious or noninfectious antigens, autoimmunity, impaired regenerative responses, and the antagonistically pleiotropic action of genes involved in wound healing or development. The importance of genetic susceptibility, epigenetics, aging, and the modern-day environment are highlighted. Consideration of both ultimate and proximate causation goes beyond philosophical cogitations, as it will better inform pathobiological mechanisms of disease and aid in the prevention and treatment of fibrotic diseases. PMID:25365073

  8. Ash Aggregates in Proximal Settings

    NASA Astrophysics Data System (ADS)

    Porritt, L. A.; Russell, K.

    2012-12-01

    Ash aggregates are thought to have formed within and been deposited by the eruption column and plume and dilute density currents and their associated ash clouds. Moist, turbulent ash clouds are considered critical to ash aggregate formation by facilitating both collision and adhesion of particles. Consequently, they are most commonly found in distal deposits. Proximal deposits containing ash aggregates are less commonly observed but do occur. Here we describe two occurrences of vent proximal ash aggregate-rich deposits; the first within a kimberlite pipe where coated ash pellets and accretionary lapilli are found within the intra-vent sequence; and the second in a glaciovolcanic setting where cored pellets (armoured lapilli) occur within <1 km of the vent. The deposits within the A418 pipe, Diavik Diamond Mine, Canada, are the residual deposits within the conduit and vent of the volcano and are characterised by an abundance of ash aggregates. Coated ash pellets are dominant but are followed in abundance by ash pellets, accretionary lapilli and rare cored pellets. The coated ash pellets typically range from 1 - 5 mm in diameter and have core to rim ratios of approximately 10:1. The formation and preservation of these aggregates elucidates the style and nature of the explosive phase of kimberlite eruption at A418 (and other pipes?). First, these pyroclasts dictate the intensity of the kimberlite eruption; it must be energetic enough to cause intense fragmentation of the kimberlite to produce a substantial volume of very fine ash (<62 μm). Secondly, the ash aggregates indicate the involvement of moisture coupled with the presence of dilute expanded eruption clouds. The structure and distribution of these deposits throughout the kimberlite conduit demand that aggregation and deposition operate entirely within the confines of the vent; this indicates that aggregation is a rapid process. Ash aggregates within glaciovolcanic sequences are also rarely documented. The

  9. High and Low Contrast Visual Acuity Are Not Affected in Amyotrophic Lateral Sclerosis.

    PubMed

    Moss, Heather E; Samelson, Monica; Mohan, Girish; Jiang, Qin Li

    2016-01-01

    The afferent visual system may be affected by neuro-degeneration in amyotrophic lateral sclerosis (ALS) based on observations of visual function impairment and retinal inclusions on histopathology in ALS patients. To test the hypothesis that visual acuity is impaired in ALS, we compared three measures of visual acuity in ALS patients (n = 25) attending a multidisciplinary ALS clinic and age matched control subjects (n = 25). Bilateral monocular and binocular visual acuities were assessed using high contrast (black letters on white background) and low contrast (2.5%, 1.25% grey letters on white background) visual acuity charts under controlled lighting conditions following refraction. Binocular summation was calculated as the difference between binocular and best monocular acuity scores. There were no associations between binocular or monocular high contrast visual acuity or low contrast visual acuity and amyotrophic lateral sclerosis diagnosis (generalized estimating equation models accounting for age). Binocular summation was similar in both amyotrophic lateral sclerosis and control subjects. There was a small magnitude association between increased duration of ALS symptoms and reduced 1.25% low contrast visual acuity. This study does not confirm prior observations of impaired visual acuity in patients with amyotrophic lateral sclerosis and does not support this particular measure of visual function for use in broad scale assessment of visual pathway involvement in ALS patients.

  10. High and Low Contrast Visual Acuity Are Not Affected in Amyotrophic Lateral Sclerosis

    PubMed Central

    Samelson, Monica; Mohan, Girish; Jiang, Qin Li

    2016-01-01

    The afferent visual system may be affected by neuro-degeneration in amyotrophic lateral sclerosis (ALS) based on observations of visual function impairment and retinal inclusions on histopathology in ALS patients. To test the hypothesis that visual acuity is impaired in ALS, we compared three measures of visual acuity in ALS patients (n = 25) attending a multidisciplinary ALS clinic and age matched control subjects (n = 25). Bilateral monocular and binocular visual acuities were assessed using high contrast (black letters on white background) and low contrast (2.5%, 1.25% grey letters on white background) visual acuity charts under controlled lighting conditions following refraction. Binocular summation was calculated as the difference between binocular and best monocular acuity scores. There were no associations between binocular or monocular high contrast visual acuity or low contrast visual acuity and amyotrophic lateral sclerosis diagnosis (generalized estimating equation models accounting for age). Binocular summation was similar in both amyotrophic lateral sclerosis and control subjects. There was a small magnitude association between increased duration of ALS symptoms and reduced 1.25% low contrast visual acuity. This study does not confirm prior observations of impaired visual acuity in patients with amyotrophic lateral sclerosis and does not support this particular measure of visual function for use in broad scale assessment of visual pathway involvement in ALS patients. PMID:28033389

  11. Methods of Communication at End of Life for the Person with Amyotrophic Lateral Sclerosis

    ERIC Educational Resources Information Center

    Brownlee, Alisa; Bruening, Lisa M.

    2012-01-01

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that results in loss of most motor functions by the time of death. Most persons with ALS experience a dysarthria that eventually renders oral/vocal communication unintelligible. This article reviews the communication needs of persons with ALS and the range of communication…

  12. 38 CFR 3.318 - Presumptive service connection for amyotrophic lateral sclerosis.

    Code of Federal Regulations, 2010 CFR

    2010-07-01

    ... under this section: (1) If there is affirmative evidence that amyotrophic lateral sclerosis was not incurred during or aggravated by active military, naval, or air service; (2) If there is affirmative... sclerosis manifested at any time after discharge or release from active military, naval, or air service...

  13. 78 FR 72573 - Specially Adapted Housing Eligibility for Amyotrophic Lateral Sclerosis Beneficiaries

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-12-03

    ... AFFAIRS 38 CFR Part 3 RIN 2900-AO84 Specially Adapted Housing Eligibility for Amyotrophic Lateral... housing (SAH). The amendment authorizes automatic issuance of a certificate of eligibility for SAH to all... indicate that they are submitted in response to ``RIN 2900-AO84--Specially Adapted Housing Eligibility...

  14. Ethnic and demographic incidence of amyotrophic lateral sclerosis (ALS) in Brazil: A population based study.

    PubMed

    Moura, Mirian Conceicao; Casulari, Luiz Augusto; Carvalho Garbi Novaes, Maria Rita

    2016-01-01

    Our objectives were to examine demographic and ethnic factors associated with amyotrophic lateral sclerosis in Brazil. The method used was a retrospective study of death certificates performed in June 2015, identifying the incidence of amyotrophic lateral sclerosis over 10 years, from January 2004 to December 2013, related to gender, age and race. Results revealed 8942 death certificates with 8152 as the underlying cause and 790 as a secondary cause. The average age was 62.7 ± 13.2 years, with a predominance of males (1·3:1). The adjusted mortality rate over 20 years was 0.61 to 0.89/100,000 person-years, and over 45 years was 1.77 to 2.3/100,000 person-years. There was a predominance of amyotrophic lateral sclerosis in Caucasians compared to the general population above 20 years (2010 Census), with an odds ratio (OR) of 2.92 (95% CI 2.78-3.07). The OR in blacks was 0.04 (95% CI: 0.03-0.04), in mestizos was 0.05 (0.04-0.07), and in Indians was 0.02 (0.01-0.04). The mean age was lower than in European populations (48.5 ± 12.3 years) (p < 0.0001). In conclusion, the incidence of amyotrophic lateral sclerosis in Brazil is close to other Latin American populations, with a lower age at death and clear predominance in Caucasians.

  15. Is There a Role for Exercise in the Management of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis?

    ERIC Educational Resources Information Center

    Plowman, Emily K.

    2015-01-01

    Purpose: The role of exercise in the management of people with amyotrophic lateral sclerosis (PALS) is controversial and currently unclear. The purpose of this review article is to review literature examining the impact of limb, respiratory, and oral motor exercise on function, disease progression, and survival in PALS and the transgenic ALS…

  16. Characteristics of Speaking Rate in the Dysarthria Associated with Amyotrophic Lateral Sclerosis.

    ERIC Educational Resources Information Center

    Turner, Greg S.; Weismer, Gary

    1993-01-01

    The ability to alter speaking rate was studied in nine adult subjects with amyotrophic lateral sclerosis and nine control subjects. Results suggest that the relationship between speaking rate, articulation rate, pause duration, and pause frequency remained largely intact for the dysarthric speakers. Data showed greater dependence on pausing by the…

  17. Against All Odds: Positive Life Experiences of People with Advanced Amyotrophic Lateral Sclerosis.

    ERIC Educational Resources Information Center

    Young, Jenny M.; McNicoll, Paule

    1998-01-01

    Describes the nature of positive life experiences of 13 people coping exceptionally well while living with advanced amyotrophic lateral sclerosis (ALS), or Lou Gehrig's, disease and the resulting significant physical disabilities. Emerging themes were the use of cognitive reappraisal, reframing, and intellectual stimulation as coping mechanisms;…

  18. Speech Intelligibility and Marital Communication in Amyotrophic Lateral Sclerosis: An Exploratory Study

    ERIC Educational Resources Information Center

    Joubert, Karin; Bornman, Juan; Alant, Erna

    2011-01-01

    Amyotrophic lateral sclerosis (ALS), a rapidly progressive neuromuscular disease, has a devastating impact not only on individuals diagnosed with ALS but also their spouses. Speech intelligibility, often compromised as a result of dysarthria, affects the couple's ability to maintain effective, intimate communication. The purpose of this…

  19. Novel cases of amyotrophic lateral sclerosis after treatment of cerebral arteriovenous malformationss.

    PubMed

    Linnebank, Michael; McDougall, Cameron G; Krueger, Stefanie; Biskup, Saskia; Neumann, Manuela; Weller, Michael; Valavanis, Antonios; Prudlo, Johannes

    2016-01-01

    Previous case studies reported nine patients with cerebral arteriovenous malformations (AVM) who developed amyotrophic lateral sclerosis (ALS) after AVM embolisation. Here, we describe three novel cases of ALS which developed 13-34 years after treatment, including embolisation, of cerebral AVM. This study provides further arguments supporting the thesis that embolisation of cerebral AVM might influence the risk of later ALS development.

  20. Variations of the origin of collateral branches emerging from the posterior aspect of the brachial plexus

    PubMed Central

    2007-01-01

    Background The frequency of variation found in the arrangement and distribution of the branches in the brachial plexus, make this anatomical region extremely complicated. The medical concerns involved with these variations include anesthetic blocks, surgical approaches, interpreting tumor or traumatic nervous compressions having unexplained clinical symptoms (sensory loss, pain, wakefulness and paresis), and the possibility of these structures becoming compromised. The clinical importance of these variations is discussed in the light of their differential origins. Methods The anatomy of brachial plexus structures from 46 male and 11 female cadaverous specimens were studied. The 40–80 year-old specimens were obtained from the Universidad Industrial de Santander's Medical Faculty's Anatomy Department (dissection laboratory). Parametric measures were used for calculating results. Results Almost half (47.1%) of the evaluated plexuses had collateral variations. Subscapular nerves were the most varied structure, including the presence of a novel accessory nerve. Long thoracic nerve variations were present, as were the absence of C5 or C7 involvement, and late C7 union with C5–C6. Conclusion Further studies are needed to confirm the existence of these variations in a larger sample of cadaver specimens. PMID:17587464

  1. Shoulder tendon transfer options for adult patients with brachial plexus injury.

    PubMed

    Elhassan, Bassem; Bishop, Alan; Shin, Alexander; Spinner, Robert

    2010-07-01

    Enhancement of upper-extremity function, specifically shoulder function, after brachial plexus injury requires a good understanding of nerve repair and transfer, with their expected outcome, as well as shoulder anatomy and biomechanics enabling the treating surgeon to use available functioning muscles around the shoulder for transfer, to improve shoulder function. Surgical treatment should address painful shoulder subluxation in addition to improvement of function. The literature focuses on improving shoulder abduction, but improving shoulder external rotation should take priority because this function, even if isolated, will allow patients to position their hand in front of their body. With a functional elbow and hand, patients will be able to do most activities of daily living. The lower trapezius has been shown to be a good transfer to restore external rotation of the shoulder. Other parts of the trapezius, levator scapulae, rhomboids, and, when available, the latissimus dorsi, pectoralis major, teres major, biceps, triceps, and serratus anterior muscles can all be used to replace the rotator cuff and deltoid muscle function. To optimize the results, a close working relationship is required between surgeons reconstructing brachial plexus injury and shoulder specialists.

  2. Tick paralysis with atypical presentation: isolated, reversible involvement of the upper trunk of brachial plexus.

    PubMed

    Engin, A; Elaldi, N; Bolayir, E; Dokmetas, I; Bakir, M

    2006-07-01

    Tick paralysis is a disease that occurs worldwide. It is a relatively rare but potentially fatal condition. The only way to establish the diagnosis is to carefully search for the tick paralysis. It is caused by a neurotoxin secreted by engorged female ticks. Tick paralysis generally begins in the lower extremities and ascends symmetrically to involve the trunk, upper extremities and head within a few hours. Although early-onset prominent bulbar palsy and isolated facial weakness without generalised paralysis are rare, there is no report in the English literature concerning isolated, reversible involvement of the upper trunk of brachial plexus caused by tick bite. We report a case of isolated, reversible involvement of the upper trunk of brachial plexus as a variant of tick paralysis. Diagnosis was confirmed with needle electromyography and nerve conduction examination. Within 2 weeks, the patient was fully recovered. The purpose of presenting this case is to remind clinicians that tick paralysis should be considered even in cases with atypical neurological findings admitted to the emergency department.

  3. The Role of Ankle-Brachial Index for Predicting Peripheral Arterial Disease

    PubMed Central

    RAC-ALBU, Marius; ILIUTA, Luminita; GUBERNA, Suzana Maria; SINESCU, Crina

    2014-01-01

    The presence of peripheral arterial disease (PAD) is associated with higher cardiovascular morbidity and mortality, regardless of gender or its clinical form of presentation (symptomatic or asymptomatic). PAD is considered an independent predictor for cardiovascular mortality, more important for survival than clinical history of coronary artery disease. The ankle brachial index (ABI) is a sensitive and cost-effective screening tool for PAD. ABI is valuable for screening of peripheral artery disease in patients at risk and for diagnosing the disease in patients who present with lower-extremity symptoms. Compared to other diagnostic methods, ABI is superior because it is s a simple, noninvasive test, which could be routinely determined in all patients. Normal cut-off values for ABI are between 0.9 and 1.4. An abnormal ankle-brachial index- below 0.9-is a powerful independent marker of cardiovascular risk. There is an inverse correlation between ABI values, non-fatal cardiac events (myocardial infarction, stroke and heart failure exacerbation) and mortality (cardiovascular and global), the relation being nonlinear, patients with very low ABI (<0.3) having a significantly higher additional risk. Also, ABI values over 1.3-1.4 correlate with major adverse cardiovascular events. Therefore, ABI can be considered a generalized atherosclerotic predictor, identifying patients at high risk for developing cardio- or cerebrovascular events and should be incorporated into routine clinical practice. PMID:25705296

  4. Brachial Artery Conductance During Reactive Hyperemia is Increased in Women with Polycystic Ovary Syndrome

    PubMed Central

    Raja-Khan, Nazia; Shuja, Showieb A.; Kunselman, Allen R.; Hogeman, Cynthia S.; Demers, Laurence M.; Gnatuk, Carol L.; Legro, Richard S.

    2010-01-01

    Objective To examine changes in brachial artery conductance (BAC) during reactive hyperemia in women with polycystic ovary syndrome (PCOS) compared to controls. Study Design This is a pilot case-control study performed at a single academic medical center. Changes in BAC during reactive hyperemia were evaluated in 31 women with PCOS and 11 healthy control women. Fasting glucose, insulin, lipids and androgen levels were also determined. A mixed-effects model was used to compare the PCOS curve to the control curve for change in BAC from baseline during reactive hyperemia. Results Body mass index (BMI) and testosterone levels were significantly increased in the PCOS group compared to controls (P < 0.05). In addition, the PCOS group had higher total and LDL cholesterol levels (P = 0.05 and 0.09, respectively). Change in BAC from baseline during reactive hyperemia was significantly increased in the PCOS group compared to controls even after adjusting for age, BMI and LDL cholesterol levels (P < 0.0001). There were no significant differences between the two groups in age, blood pressure, or fasting glucose or insulin levels. Conclusions Brachial artery conductance during reactive hyperemia is significantly increased in women with PCOS compared to controls and may be a novel early indicator of increased cardiovascular risk in women with PCOS. PMID:21112136

  5. Variations of Cords of Brachial Plexus and Branching Pattern of Nerves Emanating From Them.

    PubMed

    Singh, Rajani

    2017-03-01

    Brachial plexus is complex network of nerves, formed by joining and splitting of ventral rami of spinal nerves C5, C6, C7, C8, and T1 forming trunks, divisions, and cords. The nerves emerging from trunks and cords innervate the upper limb and to some extent pectoral region. Scanty literature describes the variations in the formation of cords and nerves emanating from them. Moreover, the variations of cords of brachial plexus and nerves emanating from them have iatrogenic implications in the upper limb and pectoral region. Hence study has been carried out. Twenty-eight upper limbs and posterior triangles from 14 cadavers fixed in formalin were dissected and rare and new variations of cords were observed. Most common variation consisted of formation of posterior cord by fusion of posterior division of upper and middle trunk and lower trunk continued as medial cord followed by originating of 2 pectoral nerves from anterior divisions of upper and middle trunk. Other variations include anterior division of upper trunk continued as lateral cord and pierced the coracobrachialis, upper and middle trunk fused to form common cord which divided into lateral and posterior cords, upper trunk gave suprascapular nerve and abnormal lateral pectoral nerve and formation of median nerve by 3 roots. These variations were analyzed for diagnostic and clinical significance making the study relevant for surgeons, radiologists in arresting failure patients and anatomists academically in medical education.

  6. Pan-brachial plexus neuropraxia following lightning: A rare case report

    PubMed Central

    Patnaik, Ashis; Mahapatra, Ashok Kumar; Jha, Menka

    2015-01-01

    Background: Neurological complications following lightning are rare and occur in form of temporary neurological deficits of central origin. Involvement of peripheral nervous system is extremely rare and only a few cases have been described in the literature. Isolated unilateral pan-brachial plexus neuropraxia has never been reported in the literature. Steroids have long been used for treatment of neuropraxia. However, their use in lightning neural injury is unique and requires special mention. Case Description: We report a rare case of lightning-induced unilateral complete flaccid paralysis along with sensory loss in a young patient. Lightning typically causes central nervous involvement in various types of motor and sensory deficit. Surprisingly, the nerve conduction study showed the involvement of peripheral nervous system involvement. Steroids were administered and there was significant improvement in neurological functions within a short span of days. Patients’ functions in the affected limb were normal in one month. Conclusion: Our case was interesting since it is the first such case in the literature where lightning has caused such a rare instance of unilateral pan-brachial plexus lesion. Such cases when seen, raises the possibility of more common central nervous system pathology rather than peripheral involvement. However, such lesions can be purely benign forms of peripheral nerve neuropraxia, which can be managed by steroid treatment without leaving any long-term neurological deficits. PMID:25883854

  7. [Cervico-omo-brachial pain and disability in a person of advanced age].

    PubMed

    Usui, M

    1997-07-01

    A person of advanced age usually has degenerative changes of bone, joint and ligament, which can be causes of cervico-omo-brachial pain and disability. He or she may also suffer from metastatic bone tumor of cervical spine or upper extremity. This article described pathology, signs and symptoms and recent treatment of these diseases. Cervical myelopathy and radiculopathy, which are most common causes of cervico-omo-brachial symptoms, are sometimes accompanied by peripheral entrapment neuropathy such as cubital tunnel syndrome or carpal tunnel syndrome (double crush syndrome). In this complicated situation, decompression of neural tissue in both cervical spine and carpal tunnel are necessary. In treatment for carpal tunnel syndrome, release of transverse carpal ligament under an arthroscope has proven to be useful and has been becoming popular. This minimally invasive surgery is also useful in shoulder surgery such as subacromial decompression in aged patients with rotator cuff tear and removal of calcium deposit in the shoulder joint. Osteoarthritis of the elbow also cause pain or disability of the elbow and the hand. Some metastatic bone tumors are treated by tumor resection and reconstruction with instruments, prosthesis or composite grafts, which are attempted not to cure the disease but to maintain or improve the quality of life of the patient.

  8. Tick paralysis with atypical presentation: isolated, reversible involvement of the upper trunk of brachial plexus

    PubMed Central

    Engin, A; Elaldi, N; Bolayir, E; Dokmetas, I; Bakir, M

    2006-01-01

    Tick paralysis is a disease that occurs worldwide. It is a relatively rare but potentially fatal condition. The only way to establish the diagnosis is to carefully search for the tick paralysis. It is caused by a neurotoxin secreted by engorged female ticks. Tick paralysis generally begins in the lower extremities and ascends symmetrically to involve the trunk, upper extremities and head within a few hours. Although early‐onset prominent bulbar palsy and isolated facial weakness without generalised paralysis are rare, there is no report in the English literature concerning isolated, reversible involvement of the upper trunk of brachial plexus caused by tick bite. We report a case of isolated, reversible involvement of the upper trunk of brachial plexus as a variant of tick paralysis. Diagnosis was confirmed with needle electromyography and nerve conduction examination. Within 2 weeks, the patient was fully recovered. The purpose of presenting this case is to remind clinicians that tick paralysis should be considered even in cases with atypical neurological findings admitted to the emergency department. PMID:16794084

  9. Brachial artery Doppler flux parameters before and after hot flush in Mexican postmenopausal women: preliminary report

    PubMed Central

    Rodríguez, Karina Vázquez; Ortiz, Sergio Rosales

    2016-01-01

    Objective To analyse brachial artery flux parameters in postmenopausal women before and after hot flush. Material and methods Two groups of postmenopausal women were studied: Group I, without vasomotor symptoms (n = 10) and Group II, with vasomotor symptoms (n = 10). In all them a brachial artery Doppler ultrasound was done, measuring before and after hyperaemic stimulus of the arterial diameter (AD), the pulsatility index (PI), and the resistive index (RI). In Group I, measurements were done at baseline and five minutes after. In Group II, measurements were at baseline, and one and five minutes after the hot-flush. Comparison between the groups was done with Mann-Whitney U test, and within the groups with Wilcoxon test. Results No differences were found among the groups in Doppler parameters. When comparing each group separately, in Group I, at baseline and at five minutes measurements, the AD was greater after the hyperaemic stimulus than before it. In group II at baseline, the PI was significantly greater after the hyperaemic stimulus than before to it. At the first and fifth minute, the AD was significantly greater after the hyperaemic stimulus than before to it. Conclusions No differences were found between those who did not have and those who had hot flushes. PMID:27095957

  10. Perineural administration of dexmedetomidine in combination with ropivacaine prolongs axillary brachial plexus block.

    PubMed

    Zhang, Yu; Wang, Chang-Song; Shi, Jing-Hui; Sun, Bo; Liu, Shu-Jie; Li, Peng; Li, En-You

    2014-01-01

    To evaluate the hypothesis that adding dexmedetomidine to ropivacaine prolongs axillary brachial plexus block. Forty-five patients of ASA I~II and aged 25-60 yr who were scheduled for elective forearm and hand surgery were randomly divided into 3 equal groups and received 40 ml of 0.33% ropivacaine + 1 ml dexmedetomidine (50 μg) (Group DR1), 40 ml of 0.33% ropivacaine + 1 ml dexmedetomidine (100 μg) (group DR2) or 40 ml of 0.33% ropivacaine + 1 ml saline (group R) in a double-blind fashion. The onset and duration of sensory and motor blocks and side effects were recorded. The demographic data and surgical characteristics were similar in each group. Sensory and motor block onset times were the same in the three groups. Sensory and motor blockade durations were longer in group DR2 than in group R (P < 0.05). There was no significant difference in the sensory blockade duration between group DR1 and group R. Bradycardia, hypertension and hypotension were not observed in group R and occurred more often in group DR2 than in group DR1. Dexmedetomidine added to ropivacaine for an axillary brachial plexus block prolongs the duration of the block. However, dexmedetomidine may also lead to side effects such as bradycardia, hypertension, and hypotension.

  11. Pressure-specified sensory device versus electrodiagnostic testing in brachial plexus upper trunk injury.

    PubMed

    Nath, Rahul Kumar; Bowen, Margaret Elaine; Eichhorn, Mitchell George

    2010-05-01

    Brachial plexus upper trunk injury is associated with winged scapula owing to the close anatomical course of the long thoracic nerve and upper trunk. Needle electromyography is a common diagnostic test for this injury; however, it does not detect injury in most patients with upper trunk damage. The pressure-specified sensory device may be an alternative to needle electromyography. Thirty patients with winged scapula and upper trunk injury were evaluated with needle electromyography (EMG) and pressure-specified sensory device (PSSD) tests. EMG testing of the biceps muscle was compared with PSSD testing of the dorsal hand skin (C6 damage), and EMG testing of the deltoid and spinati muscles was compared with PSSD testing of the deltoid skin (C5 damage). PSSD pressure values were significantly higher on the affected arm. On the basis of published and calculated threshold values the PSSD was found to be significantly more sensitive than EMG. The PSSD tests consistently identified injuries that were not detected by needle EMG tests. These findings provide strong evidence that the PSSD is more effective than needle EMG in the detection of brachial plexus upper trunk injury.

  12. Protein disulphide isomerase protects against protein aggregation and is S-nitrosylated in amyotrophic lateral sclerosis.

    PubMed

    Walker, Adam K; Farg, Manal A; Bye, Chris R; McLean, Catriona A; Horne, Malcolm K; Atkin, Julie D

    2010-01-01

    Amyotrophic lateral sclerosis is a rapidly progressing fatal neurodegenerative disease characterized by the presence of protein inclusions within affected motor neurons. Endoplasmic reticulum stress leading to apoptosis was recently recognized to be an important process in the pathogenesis of sporadic human amyotrophic lateral sclerosis as well as in transgenic models of mutant superoxide dismutase 1-linked familial amyotrophic lateral sclerosis. Endoplasmic reticulum stress occurs early in disease, indicating a critical role in pathogenesis, and involves upregulation of an important endoplasmic reticulum chaperone, protein disulphide isomerase. We aimed to investigate the involvement of protein disulphide isomerase in endoplasmic reticulum stress induction, protein aggregation, inclusion formation and toxicity in amyotrophic lateral sclerosis. Motor neuron-like NSC-34 cell lines were transfected with superoxide dismutase 1 and protein disulphide isomerase encoding vectors and small interfering RNA, and examined by immunocytochemistry and immunoblotting. Expression of mutant superoxide dismutase 1 induced endoplasmic reticulum stress, predominantly in cells bearing mutant superoxide dismutase 1 inclusions but also in a proportion of cells expressing mutant superoxide dismutase 1 without visible inclusions. Over-expression of protein disulphide isomerase decreased mutant superoxide dismutase 1 aggregation, inclusion formation, endoplasmic reticulum stress induction and toxicity, whereas small interfering RNA targeting protein disulphide isomerase increased mutant superoxide dismutase 1 inclusion formation, indicating a protective role for protein disulphide isomerase against superoxide dismutase 1 misfolding. Aberrant modification of protein disulphide isomerase by S-nitrosylation of active site cysteine residues has previously been shown as an important process in neurodegeneration in Parkinson's and Alzheimer's disease brain tissue, but has not been described in

  13. Anti-Ganglioside Antibodies in Amyotrophic Lateral Sclerosis Revisited

    PubMed Central

    Kollewe, Katja; Wurster, Ulrich; Sinzenich, Thomas; Körner, Sonja; Dengler, Reinhard; Mohammadi, Bahram; Petri, Susanne

    2015-01-01

    Background Amyotrophic Lateral Sclerosis (ALS) is a devastating neurodegenerative disorder with typical onset in the 5th- 6th decade of life. The hypothesis of an autoimmune origin of ALS receives less attention today, but immunological phenomena still seem to be involved and mechanisms such as protective autoimmunity may be important. Detection of antibodies against a variety of gangliosides has been repeatedly described in ALS-patients by several authors, but widely differing frequencies and titres have been reported. Therefore, we investigated the presence of six common antibodies with a commercially available test panel for GA1, GM1, GM2, GD1a, GD1b and GQ1b in a large group of clinically well-characterized ALS patients and compared them to a collective of 200 healthy blood donors. Methods IgG and IgM antibodies to the six gangliosides asialoGM1 (GA1), GM1, GM2, GD1a, GD1b, GQ1b were determined by GanglioCombi ELISA in sera of 84 ALS patients. Results were expressed as a %-ratio of a highly positive control and categorized as negative (<30%), borderline (30–50%), moderately (50–100%) and strongly positive (>100%). The values obtained from 200 Swiss blood donors served as a reference group. Results In twenty-two (26.2%) ALS-patients elevated anti-ganglioside antibodies could be detected: Taking all subspecific antibodies together, IgG antibodies were found in 9/84 (10.7%) and IgM in 15/84 (17.9%) patients. There was no correlation between age, gender, site of onset or survival and anti-ganglioside-positive/-negative titres in ALS-patients. No statistically significant difference in the frequency of anti-ganglioside antibodies compared to the group of healthy blood donors was found. Conclusion Even with this more comprehensive approach, anti-ganglioside antibody frequencies and patterns in our ALS cohort closely resembled the values measured in healthy controls. In accordance with other studies, we did not observe any association of a distinct ALS phenotype

  14. Amyotrophic lateral sclerosis and occupational exposure to electromagnetic fields

    SciTech Connect

    Davanipour, Z.; Sobel, E.; Bowman, J.D.; Qian, Z.; Will, A.D.

    1997-03-01

    In an hypothesis-generating case-control study of amyotrophic lateral sclerosis, lifetime occupational histories were obtained. The patients (n = 28) were clinic based. The occupational exposure of interest in this report is electromagnetic fields (EMFs). This is the first and so far the only exposure analyzed in this study. Occupational exposure up to 2 years prior to estimated disease symptom onset was used for construction of exposure indices for cases. Controls (n = 32) were blood and nonblood relatives of cases. Occupational exposure for controls was through the same age as exposure for the corresponding cases. Twenty (71%) cases and 28 (88%) controls had at least 20 years of work experience covering the exposure period. The occupational history and task data were used to classify blindly each occupation for each subject as having high, medium/high, medium, medium/low, or low EMF exposure, based primarily on data from an earlier and unrelated study designed to obtain occupational EMF exposure information on workers in ``electrical`` and ``nonelectrical`` jobs. By using the length of time each subject spent in each occupation through the exposure period, two indices of exposure were constructed: total occupational exposure (E{sub 1}) and average occupational exposure (E{sub 2}). For cases and controls with at least 20 years of work experience, the odds ratio (OR) for exposure at the 75th percentile of the E{sub 1} case exposure data relative to minimum exposure was 7.5 (P < 0.02; 95% CI, 1.4--38.1) and the corresponding OR for E{sub 2} was 5.5 (P < 0.02; 95% CI, 1.3--22.5). For all cases and controls, the ORs were 2.5 (P < 0.1; 95% CI, 0.9--8.1) for E{sub 1} and 2.3 (P = 0.12; 95% CI, 0.8--6.6) for E{sub 2}. This study should be considered an hypothesis-generating study. Larger studies, using incident cases and improved exposure assessment, should be undertaken.

  15. Deformed proximity potential for heavy ion reactions

    SciTech Connect

    Baltz, A.J.

    1989-09-01

    The proximity potential is discussed for the inelastic scattering of a spherical nucleus on a deformed nucleus or the mutual interaction of two deformed nuclei. It is shown that the proximity potential is, in general, geometrically more correct than the usual centerline prescription used in inelastic scattering analyses. For the cases where the proximity potential is inadequate a folding model approach is advocated. Techniques to facilitate the coupled channels analysis are presented. 11 refs., 6 figs.

  16. Cutting Balloon-Assisted Angioplasty of an Anastomotic Carotid-Brachial Bypass Graft Stenosis: A Case Report

    SciTech Connect

    Prabhudesai, Vikramaditya; Orme, Richard Fox, Anthony D.

    2004-08-15

    Neointimal hyperplasia leads to anastomotic stenosis in bypass grafts. These stenoses are often resistant to conventional balloon dilatation. We present a case of a carotid-brachial bypass graft stenosis, which was treated by a 5-mm cutting balloon angioplasty with a good angiographic and clinical result.

  17. Comparison Between Ultrasound-Guided Supraclavicular and Interscalene Brachial Plexus Blocks in Patients Undergoing Arthroscopic Shoulder Surgery

    PubMed Central

    Ryu, Taeha; Kil, Byung Tae; Kim, Jong Hae

    2015-01-01

    Abstract Although supraclavicular brachial plexus block (SCBPB) was repopularized by the introduction of ultrasound, its usefulness in shoulder surgery has not been widely reported. The objective of this study was to compare motor and sensory blockades, the incidence of side effects, and intraoperative opioid analgesic requirements between SCBPB and interscalene brachial plexus block (ISBPB) in patients undergoing arthroscopic shoulder surgery. Patients were randomly assigned to 1 of 2 groups (ISBPB group: n = 47; SCBPB group: n = 46). The side effects of the brachial plexus block (Horner's syndrome, hoarseness, and subjective dyspnea), the sensory block score (graded from 0 [no cold sensation] to 100 [intact sensation] using an alcohol swab) for each of the 5 dermatomes (C5–C8 and T1), and the motor block score (graded from 0 [complete paralysis] to 6 [normal muscle force]) for muscle forces corresponding to the radial, ulnar, median, and musculocutaneous nerves were evaluated 20 min after the brachial plexus block. Fentanyl was administered in 50 μg increments when the patients complained of pain that was not relieved by the brachial plexus block. There were no conversions to general anesthesia due to a failed brachial plexus block. The sensory block scores for the C5 to C8 dermatomes were significantly lower in the ISBPB group. However, the percentage of patients who received fentanyl was comparable between the 2 groups (27.7% [ISBPB group] and 30.4% [SCBPB group], P = 0.77). SCBPB produced significantly lower motor block scores for the radial, ulnar, and median nerves than did ISBPB. A significantly higher incidence of Horner's syndrome was observed in the ISBPB group (59.6% [ISBPB group] and 19.6% [SCBPB group], P < 0.001). No patient complained of subjective dyspnea. Despite the weaker degree of sensory blockade provided by SCBPB in comparison to ISBPB, opioid analgesic requirements are similar during arthroscopic shoulder surgery under

  18. Brachial Plexus-Associated Neuropathy After High-Dose Radiation Therapy for Head-and-Neck Cancer

    SciTech Connect

    Chen, Allen M.; Hall, William H.; Li, Judy; Beckett, Laurel; Farwell, D. Gregory; Lau, Derick H.; Purdy, James A.

    2012-09-01

    Purpose: To identify clinical and treatment-related predictors of brachial plexus-associated neuropathies after radiation therapy for head-and-neck cancer. Methods and Materials: Three hundred thirty patients who had previously completed radiation therapy for head-and-neck cancer were prospectively screened using a standardized instrument for symptoms of neuropathy thought to be related to brachial plexus injury. All patients were disease-free at the time of screening. The median time from completion of radiation therapy was 56 months (range, 6-135 months). One-hundred fifty-five patients (47%) were treated by definitive radiation therapy, and 175 (53%) were treated postoperatively. Radiation doses ranged from 50 to 74 Gy (median, 66 Gy). Intensity-modulated radiation therapy was used in 62% of cases, and 133 patients (40%) received concurrent chemotherapy. Results: Forty patients (12%) reported neuropathic symptoms, with the most common being ipsilateral pain (50%), numbness/tingling (40%), motor weakness, and/or muscle atrophy (25%). When patients with <5 years of follow-up were excluded, the rate of positive symptoms increased to 22%. On univariate analysis, the following factors were significantly associated with brachial plexus symptoms: prior neck dissection (p = 0.01), concurrent chemotherapy (p = 0.01), and radiation maximum dose (p < 0.001). Cox regression analysis confirmed that both neck dissection (p < 0.001) and radiation maximum dose (p < 0.001) were independently predictive of symptoms. Conclusion: The incidence of brachial plexus-associated neuropathies after radiation therapy for head-and-neck cancer may be underreported. In view of the dose-response relationship identified, limiting radiation dose to the brachial plexus should be considered when possible.

  19. [A case of subacute necrotizing lymphadenitis complicated with brachial plexus neuritis].

    PubMed

    Sugiyama, A; Araki, E; Arakawa, K; Kikuchi, H; Iwaki, T; Yamada, T; Kira, J

    1998-01-01

    A 22-year-old female noted a low grade fever and swelling of the cervical lymph nodes in May 1997, and later developed a dry cough. She was diagnosed to have interstitial pneumonitis, and then administration of corticosteroids alleviated her symptoms. On February 6, 1998, however, a high fever recurred and her swollen cervical lymph node on the right side was biopsied on February 9, 1998. A histological examination revealed an increased number of histiocytes and karyorrhexis of the lymphocytes in the paracortical areas, and she was therefore diagnosed to have histiocytic necrotizing lymphadenitis. She could not fully elevate her arm on February 16, 1998. On admission, her cervical lymph node was swollen on the left side. A neurological examination revealed a marked weakness of the right deltoid muscle, moderate weakness of the right latissimus dorsi, triceps and brachioradialis muscles and also a mild weakness of the serratus anterior, supra- and infra-spinatus, and biceps brachii muscles. The muscle power of the other muscles were normal and no muscle atrophy was evident. Winging of the right scapula was observed. The deep tendon reflexes were normal in all four limbs, and her sensation was also normal. No cerebellar sign was found. The Jackson, Spurling, Allen, Morley and Adson tests were all negative. ESR was mildly elevated to 18 mm/hr, but CRP was negative. RF, ANA and anti-SS-A and SS-B antibodies were positive, whereas LE-test, direct and indirect Coombs tests and other autoantibodies were negative. Needle EMG disclosed fasciculation potentials in the right triceps muscle and polyphasic waves in the right deltoid muscle. MRI showed gadolinium-enhancement of the right brachial plexus. Although an abnormal accumulation of gallium was detected in the right parotid and bilateral submandibular glands, no sicca symptoms were found and the Schirmer test findings were normal. Oral prednisolone (50 mg/day with gradual tapering) alleviated both her symptoms and the

  20. Range gated strip proximity sensor

    DOEpatents

    McEwan, T.E.

    1996-12-03

    A range gated strip proximity sensor uses one set of sensor electronics and a distributed antenna or strip which extends along the perimeter to be sensed. A micro-power RF transmitter is coupled to the first end of the strip and transmits a sequence of RF pulses on the strip to produce a sensor field along the strip. A receiver is coupled to the second end of the strip, and generates a field reference signal in response to the sequence of pulse on the line combined with received electromagnetic energy from reflections in the field. The sensor signals comprise pulses of radio frequency signals having a duration of less than 10 nanoseconds, and a pulse repetition rate on the order of 1 to 10 MegaHertz or less. The duration of the radio frequency pulses is adjusted to control the range of the sensor. An RF detector feeds a filter capacitor in response to received pulses on the strip line to produce a field reference signal representing the average amplitude of the received pulses. When a received pulse is mixed with a received echo, the mixing causes a fluctuation in the amplitude of the field reference signal, providing a range-limited Doppler type signature of a field disturbance. 6 figs.

  1. Proximal Priority Laser Therapy: PPLT

    NASA Astrophysics Data System (ADS)

    Ohshiro, Toshio

    2004-09-01

    The author has, in the past, classified treatment methods for pain geometrically as point, line, two-dimensional, three-dimensional treatment and has used these over the years. However as a practitioner of western medicine, the author originally treated pain only directed at the painful site, and encountered cases where local treatment did not suffice. The author proved with SPECT and the Rand Phantom that treating the neck which is the midpoint of the brain, the center of the nervous system and the heart, the center of circulation, increased cerebral blood flow and also that laser emitted to neck will reach the spinal chord no matter from where on the neck the laser is emitted. From such research and 25 years of clinical experience, the author has created an anatomy based, systemic treatment method called the Proximal Priority Laser Therapy (PPLT) where not only the cerebral cortex, spinal chord and peripheral nerves are treated but also the tracts of blood vessels and lymph ducts are treated as well. Treatment method and cases are presented herein.

  2. Range gated strip proximity sensor

    DOEpatents

    McEwan, Thomas E.

    1996-01-01

    A range gated strip proximity sensor uses one set of sensor electronics and a distributed antenna or strip which extends along the perimeter to be sensed. A micro-power RF transmitter is coupled to the first end of the strip and transmits a sequence of RF pulses on the strip to produce a sensor field along the strip. A receiver is coupled to the second end of the strip, and generates a field reference signal in response to the sequence of pulse on the line combined with received electromagnetic energy from reflections in the field. The sensor signals comprise pulses of radio frequency signals having a duration of less than 10 nanoseconds, and a pulse repetition rate on the order of 1 to 10 MegaHertz or less. The duration of the radio frequency pulses is adjusted to control the range of the sensor. An RF detector feeds a filter capacitor in response to received pulses on the strip line to produce a field reference signal representing the average amplitude of the received pulses. When a received pulse is mixed with a received echo, the mixing causes a fluctuation in the amplitude of the field reference signal, providing a range-limited Doppler type signature of a field disturbance.

  3. Grouping by proximity in haptic contour detection.

    PubMed

    Overvliet, Krista E; Krampe, Ralf Th; Wagemans, Johan

    2013-01-01

    We investigated the applicability of the Gestalt principle of perceptual grouping by proximity in the haptic modality. To do so, we investigated the influence of element proximity on haptic contour detection. In the course of four sessions ten participants performed a haptic contour detection task in which they freely explored a haptic random dot display that contained a contour in 50% of the trials. A contour was defined by a higher density of elements (raised dots), relative to the background surface. Proximity of the contour elements as well as the average proximity of background elements was systematically varied. We hypothesized that if proximity of contour elements influences haptic contour detection, detection will be more likely when contour elements are in closer proximity. This should be irrespective of the ratio with the proximity of the background elements. Results showed indeed that the closer the contour elements were, the higher the detection rates. Moreover, this was the case independent of the contour/background ratio. We conclude that the Gestalt law of proximity applies to haptic contour detection.

  4. Sugar proximity and human grip strength.

    PubMed

    Quintanar, A F; Hill, T V

    1988-12-01

    In a double-blind experiment with 90 undergraduate students the hypothesis was tested that proximity to sugar reduces human grip strength. An earlier study indicated that sugar proximity does indeed significantly reduce human grip strength. When additional controls were added to this design to reduce random sampling error, the hypothesis was not supported.

  5. Grouping by Proximity in Haptic Contour Detection

    PubMed Central

    Overvliet, Krista E.; Krampe, Ralf Th.; Wagemans, Johan

    2013-01-01

    We investigated the applicability of the Gestalt principle of perceptual grouping by proximity in the haptic modality. To do so, we investigated the influence of element proximity on haptic contour detection. In the course of four sessions ten participants performed a haptic contour detection task in which they freely explored a haptic random dot display that contained a contour in 50% of the trials. A contour was defined by a higher density of elements (raised dots), relative to the background surface. Proximity of the contour elements as well as the average proximity of background elements was systematically varied. We hypothesized that if proximity of contour elements influences haptic contour detection, detection will be more likely when contour elements are in closer proximity. This should be irrespective of the ratio with the proximity of the background elements. Results showed indeed that the closer the contour elements were, the higher the detection rates. Moreover, this was the case independent of the contour/background ratio. We conclude that the Gestalt law of proximity applies to haptic contour detection. PMID:23762364

  6. Proximal Participation: A Pathway into Work

    ERIC Educational Resources Information Center

    Chan, Selena

    2013-01-01

    In a longitudinal case study of apprentices, the term proximal participation was coined to describe the entry process of young people, with unclear career destinations, into the trade of baking. This article unravels the significance of proximal participation in the decision-making processes of young people who enter a trade through initial…

  7. Information-seeking Behavior and Information Needs in Patients With Amyotrophic Lateral Sclerosis: Analyzing an Online Patient Community.

    PubMed

    Oh, Juyeon; Kim, Jung A

    2017-02-22

    A few studies have examined the specific informational needs of the population with amyotrophic lateral sclerosis. The aims of this study were to describe the information-seeking behavior and information needs of patients with amyotrophic lateral sclerosis and their families in Korea by analyzing messages from an online patient community. A total of 1047 messages from the question and answer forum of the "Lou Gehrig's Disease Network" (http://cafe.daum.net/alsfree) from January 2010 to September 2015 were collected. The word frequency, main questions, and asker of the messages were analyzed and coded. Terms such as "hospital," "mother," "father," "gastrostomy," and "ALS" were most frequently identified. The most commonly mentioned main topic was about disease-specific information, while the most frequent subcategory was symptoms or management of symptoms. Other prominent categories concerned information about treatment, rehabilitation, and the medical system. The people who wrote the questions were mostly the son/daughter of patients with amyotrophic lateral sclerosis. Patients with amyotrophic lateral sclerosis and their family members commonly obtained information by posting their inquiries online and have a variety of questions regarding amyotrophic lateral sclerosis in this study. The findings of this study can be used as a base of information for developing educational programs and resources for patients with amyotrophic lateral sclerosis and their families.

  8. The ratio of N-acetyl aspartate to glutamate correlates with disease duration of amyotrophic lateral sclerosis.

    PubMed

    Sako, Wataru; Abe, Takashi; Izumi, Yuishin; Harada, Masafumi; Kaji, Ryuji

    2016-05-01

    Glutamate (Glu)-induced excitotoxicity has been implicated in the neuronal loss of amyotrophic lateral sclerosis. To test the hypothesis that Glu in the primary motor cortex contributes to disease severity and/or duration, the Glu level was investigated using MR spectroscopy. Seventeen patients with amyotrophic lateral sclerosis were diagnosed according to the El Escorial criteria for suspected, possible, probable or definite amyotrophic lateral sclerosis, and enrolled in this cross-sectional study. We measured metabolite concentrations, including N-acetyl aspartate (NAA), creatine, choline, inositol, Glu and glutamine, and performed partial correlation between each metabolite concentration or NAA/Glu ratio and disease severity or duration using age as a covariate. Considering our hypothesis that Glu is associated with neuronal cell death in amyotrophic lateral sclerosis, we investigated the ratio of NAA to Glu, and found a significant correlation between NAA/Glu and disease duration (r=-0.574, p=0.02). The "suspected" amyotrophic lateral sclerosis patients showed the same tendency as possible, probable and definite amyotrophic lateral sclerosis patients in regard to correlation of NAA/Glu ratio with disease duration. The other metabolites showed no significant correlation. Our findings suggested that glutamatergic neurons are less vulnerable compared to other neurons and this may be because inhibitory receptors are mainly located presynaptically, which supports the notion of Glu-induced excitotoxicity.

  9. Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43

    PubMed Central

    Uchida, Azusa; Sasaguri, Hiroki; Kimura, Nobuyuki; Tajiri, Mio; Ohkubo, Takuya; Ono, Fumiko; Sakaue, Fumika; Kanai, Kazuaki; Hirai, Takashi; Sano, Tatsuhiko; Shibuya, Kazumoto; Kobayashi, Masaki; Yamamoto, Mariko; Yokota, Shigefumi; Kubodera, Takayuki; Tomori, Masaki; Sakaki, Kyohei; Enomoto, Mitsuhiro; Hirai, Yukihiko; Kumagai, Jiro; Yasutomi, Yasuhiro; Mochizuki, Hideki; Kuwabara, Satoshi; Uchihara, Toshiki; Mizusawa, Hidehiro

    2012-01-01

    Amyotrophic lateral sclerosis is a fatal neurodegenerative disease characterized by progressive motoneuron loss. Redistribution of transactive response deoxyribonucleic acid-binding protein 43 from the nucleus to the cytoplasm and the presence of cystatin C-positive Bunina bodies are considered pathological hallmarks of amyotrophic lateral sclerosis, but their significance has not been fully elucidated. Since all reported rodent transgenic models using wild-type transactive response deoxyribonucleic acid-binding protein 43 failed to recapitulate these features, we expected a species difference and aimed to make a non-human primate model of amyotrophic lateral sclerosis. We overexpressed wild-type human transactive response deoxyribonucleic acid-binding protein 43 in spinal cords of cynomolgus monkeys and rats by injecting adeno-associated virus vector into the cervical cord, and examined the phenotype using behavioural, electrophysiological, neuropathological and biochemical analyses. These monkeys developed progressive motor weakness and muscle atrophy with fasciculation in distal hand muscles first. They also showed regional cytoplasmic transactive response deoxyribonucleic acid-binding protein 43 mislocalization with loss of nuclear transactive response deoxyribonucleic acid-binding protein 43 staining in the lateral nuclear group of spinal cord innervating distal hand muscles and cystatin C-positive cytoplasmic aggregates, reminiscent of the spinal cord pathology of patients with amyotrophic lateral sclerosis. Transactive response deoxyribonucleic acid-binding protein 43 mislocalization was an early or presymptomatic event and was later associated with neuron loss. These findings suggest that the transactive response deoxyribonucleic acid-binding protein 43 mislocalization leads to α-motoneuron degeneration. Furthermore, truncation of transactive response deoxyribonucleic acid-binding protein 43 was not a prerequisite for motoneuronal degeneration, and

  10. Analysis of amyotrophic lateral sclerosis as a multistep process: a population-based modelling study

    PubMed Central

    Al-Chalabi, Ammar; Calvo, Andrea; Chio, Adriano; Colville, Shuna; Ellis, Cathy M; Hardiman, Orla; Heverin, Mark; Howard, Robin S; Huisman, Mark H B; Keren, Noa; Leigh, P Nigel; Mazzini, Letizia; Mora, Gabriele; Orrell, Richard W; Rooney, James; Scott, Kirsten M; Scotton, William J; Seelen, Meinie; Shaw, Christopher E; Sidle, Katie S; Swingler, Robert; Tsuda, Miho; Veldink, Jan H; Visser, Anne E; van den Berg, Leonard H; Pearce, Neil

    2014-01-01

    Summary Background Amyotrophic lateral sclerosis shares characteristics with some cancers, such as onset being more common in later life, progression usually being rapid, the disease affecting a particular cell type, and showing complex inheritance. We used a model originally applied to cancer epidemiology to investigate the hypothesis that amyotrophic lateral sclerosis is a multistep process. Methods We generated incidence data by age and sex from amyotrophic lateral sclerosis population registers in Ireland (registration dates 1995–2012), the Netherlands (2006–12), Italy (1995–2004), Scotland (1989–98), and England (2002–09), and calculated age and sex-adjusted incidences for each register. We regressed the log of age-specific incidence against the log of age with least squares regression. We did the analyses within each register, and also did a combined analysis, adjusting for register. Findings We identified 6274 cases of amyotrophic lateral sclerosis from a catchment population of about 34 million people. We noted a linear relationship between log incidence and log age in all five registers: England r2=0·95, Ireland r2=0·99, Italy r2=0·95, the Netherlands r2=0·99, and Scotland r2=0·97; overall r2=0·99. All five registers gave similar estimates of the linear slope ranging from 4·5 to 5·1, with overlapping confidence intervals. The combination of all five registers gave an overall slope of 4·8 (95% CI 4·5–5·0), with similar estimates for men (4·6, 4·3–4·9) and women (5·0, 4·5–5·5). Interpretation A linear relationship between the log incidence and log age of onset of amyotrophic lateral sclerosis is consistent with a multistage model of disease. The slope estimate suggests that amyotrophic lateral sclerosis is a six-step process. Identification of these steps could lead to preventive and therapeutic avenues. Funding UK Medical Research Council; UK Economic and Social Research Council; Ireland Health Research Board; The

  11. MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis.

    PubMed

    MacNair, Laura; Xiao, Shangxi; Miletic, Denise; Ghani, Mahdi; Julien, Jean-Pierre; Keith, Julia; Zinman, Lorne; Rogaeva, Ekaterina; Robertson, Janice

    2016-01-01

    Tar DNA-binding protein 43 (TDP-43) is an RNA-binding protein normally localized to the nucleus of cells, where it elicits functions related to RNA metabolism such as transcriptional regulation and alternative splicing. In amyotrophic lateral sclerosis, TDP-43 is mislocalized from the nucleus to the cytoplasm of diseased motor neurons, forming ubiquitinated inclusions. Although mutations in the gene encoding TDP-43, TARDBP, are found in amyotrophic lateral sclerosis, these are rare. However, TDP-43 pathology is common to over 95% of amyotrophic lateral sclerosis cases, suggesting that abnormalities of TDP-43 play an active role in disease pathogenesis. It is our hypothesis that a loss of TDP-43 from the nucleus of affected motor neurons in amyotrophic lateral sclerosis will lead to changes in RNA processing and expression. Identifying these changes could uncover molecular pathways that underpin motor neuron degeneration. Here we have used translating ribosome affinity purification coupled with microarray analysis to identify the mRNAs being actively translated in motor neurons of mutant TDP-43(A315T) mice compared to age-matched non-transgenic littermates. No significant changes were found at 5 months (presymptomatic) of age, but at 10 months (symptomatic) the translational profile revealed significant changes in genes involved in RNA metabolic process, immune response and cell cycle regulation. Of 28 differentially expressed genes, seven had a ≥ 2-fold change; four were validated by immunofluorescence labelling of motor neurons in TDP-43(A315T) mice, and two of these were confirmed by immunohistochemistry in amyotrophic lateral sclerosis cases. Both of these identified genes, DDX58 and MTHFSD, are RNA-binding proteins, and we show that TDP-43 binds to their respective mRNAs and we identify MTHFSD as a novel component of stress granules. This discovery-based approach has for the first time revealed translational changes in motor neurons of a TDP-43 mouse model

  12. Executive deficits, not processing speed relates to abnormalities in distinct prefrontal tracts in amyotrophic lateral sclerosis.

    PubMed

    Pettit, Lewis D; Bastin, Mark E; Smith, Colin; Bak, Thomas H; Gillingwater, Thomas H; Abrahams, Sharon

    2013-11-01

    Cognitive impairment in amyotrophic lateral sclerosis is characterized by deficits on tests of executive function; however, the contribution of abnormal processing speed is unknown. Methods are confounded by tasks that depend on motor speed in patients with physical disability. Structural and functional magnetic resonance imaging studies have revealed multi-system cerebral involvement, with evidence of reduced white matter volume and integrity in predominant frontotemporal regions. The current study has two aims. First, to investigate whether cognitive impairments in amyotrophic lateral sclerosis are due to executive dysfunction or slowed processing speed using methodology that accommodates motor disability. This is achieved using a dual-task paradigm and tasks that manipulate stimulus presentation times and do not rely on response motor speed. Second, to identify relationships between specific cognitive impairments and the integrity of distinct white matter tracts. Thirty patients with amyotrophic lateral sclerosis and 30 age- and education-matched control subjects were administered an experimental dual-task procedure that combined a visual inspection time task and digit recall. In addition, measures of executive function (including letter fluency) and processing speed (visual inspection time and rapid serial letter identification) were administered. Integrity of white matter tracts was determined using region of interest analyses of diffusion tensor magnetic resonance imaging data. Patients with amyotrophic lateral sclerosis did not show impairments on tests of processing speed, but executive deficits were revealed once visual inspection time was combined with digit recall (dual-task) and in letter fluency. In addition to the corticospinal tracts, significant differences in fractional anisotropy and mean diffusivity were found between groups in a number of prefrontal and temporal white matter tracts including the anterior cingulate, anterior thalamic radiation

  13. Widespread grey matter pathology dominates the longitudinal cerebral MRI and clinical landscape of amyotrophic lateral sclerosis.

    PubMed

    Menke, Ricarda A L; Körner, Sonja; Filippini, Nicola; Douaud, Gwenaëlle; Knight, Steven; Talbot, Kevin; Turner, Martin R

    2014-09-01

    Diagnosis, stratification and monitoring of disease progression in amyotrophic lateral sclerosis currently rely on clinical history and examination. The phenotypic heterogeneity of amyotrophic lateral sclerosis, including extramotor cognitive impairments is now well recognized. Candidate biomarkers have shown variable sensitivity and specificity, and studies have been mainly undertaken only cross-sectionally. Sixty patients with sporadic amyotrophic lateral sclerosis (without a family history of amyotrophic lateral sclerosis or dementia) underwent baseline multimodal magnetic resonance imaging at 3 T. Grey matter pathology was identified through analysis of T1-weighted images using voxel-based morphometry. White matter pathology was assessed using tract-based spatial statistics analysis of indices derived from diffusion tensor imaging. Cross-sectional analyses included group comparison with a group of healthy controls (n = 36) and correlations with clinical features, including regional disability, clinical upper motor neuron signs and cognitive impairment. Patients were offered 6-monthly follow-up MRI, and the last available scan was used for a separate longitudinal analysis (n = 27). In cross-sectional study, the core signature of white matter pathology was confirmed within the corticospinal tract and callosal body, and linked strongly to clinical upper motor neuron burden, but also to limb disability subscore and progression rate. Localized grey matter abnormalities were detected in a topographically appropriate region of the left motor cortex in relation to bulbar disability, and in Broca's area and its homologue in relation to verbal fluency. Longitudinal analysis revealed progressive and widespread changes in the grey matter, notably including the basal ganglia. In contrast there was limited white matter pathology progression, in keeping with a previously unrecognized limited change in individual clinical upper motor neuron scores, despite advancing disability

  14. Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43.

    PubMed

    Uchida, Azusa; Sasaguri, Hiroki; Kimura, Nobuyuki; Tajiri, Mio; Ohkubo, Takuya; Ono, Fumiko; Sakaue, Fumika; Kanai, Kazuaki; Hirai, Takashi; Sano, Tatsuhiko; Shibuya, Kazumoto; Kobayashi, Masaki; Yamamoto, Mariko; Yokota, Shigefumi; Kubodera, Takayuki; Tomori, Masaki; Sakaki, Kyohei; Enomoto, Mitsuhiro; Hirai, Yukihiko; Kumagai, Jiro; Yasutomi, Yasuhiro; Mochizuki, Hideki; Kuwabara, Satoshi; Uchihara, Toshiki; Mizusawa, Hidehiro; Yokota, Takanori

    2012-03-01

    Amyotrophic lateral sclerosis is a fatal neurodegenerative disease characterized by progressive motoneuron loss. Redistribution of transactive response deoxyribonucleic acid-binding protein 43 from the nucleus to the cytoplasm and the presence of cystatin C-positive Bunina bodies are considered pathological hallmarks of amyotrophic lateral sclerosis, but their significance has not been fully elucidated. Since all reported rodent transgenic models using wild-type transactive response deoxyribonucleic acid-binding protein 43 failed to recapitulate these features, we expected a species difference and aimed to make a non-human primate model of amyotrophic lateral sclerosis. We overexpressed wild-type human transactive response deoxyribonucleic acid-binding protein 43 in spinal cords of cynomolgus monkeys and rats by injecting adeno-associated virus vector into the cervical cord, and examined the phenotype using behavioural, electrophysiological, neuropathological and biochemical analyses. These monkeys developed progressive motor weakness and muscle atrophy with fasciculation in distal hand muscles first. They also showed regional cytoplasmic transactive response deoxyribonucleic acid-binding protein 43 mislocalization with loss of nuclear transactive response deoxyribonucleic acid-binding protein 43 staining in the lateral nuclear group of spinal cord innervating distal hand muscles and cystatin C-positive cytoplasmic aggregates, reminiscent of the spinal cord pathology of patients with amyotrophic lateral sclerosis. Transactive response deoxyribonucleic acid-binding protein 43 mislocalization was an early or presymptomatic event and was later associated with neuron loss. These findings suggest that the transactive response deoxyribonucleic acid-binding protein 43 mislocalization leads to α-motoneuron degeneration. Furthermore, truncation of transactive response deoxyribonucleic acid-binding protein 43 was not a prerequisite for motoneuronal degeneration, and

  15. Brachial neuritis or Parsonage-Turner syndrome: A problem of liability. A presentation of 3 cases.

    PubMed

    Rodríguez-Hornillo, M; de la Riva, M C; Ojeda, R

    2016-01-01

    Neuralgic amyotrophy, brachial neuritis or Parsonage-Turner syndrome is a rare neuromuscular involvement of unknown aetiology. When it onsets in connection with a health care act, such as childbirth or surgery, a malpractice argument is often used as a cause of adverse outcome, usually due to an incorrect position of the patient on the operating table, a circumstance which directly involves the anesthesia area. Three cases are presented of Parsonage-Turner syndrome following very different surgery, with different results as regards prognosis. A review and discussion of bibliography is presented on the possibility that such circumstances are the subject of malpractice claims. Special emphasis is placed on the most currently accepted aetiopathogenic theories, and the relationship of this syndrome with the surgical act as a determining medico-legal aspect. Valuation parameters are proposed.

  16. Clinical Assessment of the Infant and Child Following Perinatal Brachial Plexus Injury

    PubMed Central

    Duff, Susan V.; DeMatteo, Carol

    2015-01-01

    STUDY DESIGN Literature review INTRODUCTION After perinatal brachial plexus injury (PBPI), clinicians play an important role in injury classification as well as the assessment of recovery and secondary conditions. Early assessment guides the initial plan of care and influences follow-up and long-term outcome. PURPOSE To review methods used to assess, classify and monitor the extent and influence of PBPI with an emphasis on guidelines for clinicians. METHODS We use The International Classification of Functioning, Disability, and Health (ICF) model to provide a guide to assessment after PBPI for rehabilitation clinicians. DISCUSSION With information gained from targeted assessments, clinicians can design interventions to increase the opportunities infants and children have for optimal recovery and to attain skills that allow participation in areas of interest. PMID:25840493

  17. Correlation between ankle brachial index and coronary artery disease severity in elderly Egyptians.

    PubMed

    Amer, Moatasem S; Tawfik, Heba Mohamed; Elmoteleb, Ayman M Abd; Maamoun, Manar M A

    2014-11-01

    We investigated the association between ankle brachial index (ABI) and coronary heart disease (CHD) severity in elderly Egyptians using different measures. We conducted a case-control study from November 2010 to June 2012 including 200 male and female patients with ischemia≥60 years who were divided into 100 cases and 100 controls according to ABI and redivided according to age. They underwent coronary angiography followed by ABI measurement using a hand-held Doppler. The CHD severity was estimated using the SYNTAX and Jeopardy scores and number of diseased vessels, which increased significantly in patients with peripheral artery disease (P<.001) for all. All 3 measures had strong negative correlation with ABI (P≤.001 for Jeopardy, <.001 for SYNTAX scores, and .004 for number of diseased vessels) and were correlated with each other. We concluded that ABI can reflect CHD severity in elderly Egyptians.

  18. [Brachial plexus compression from supraclavicular encapsulated fat necrosis. A case report].

    PubMed

    Domínguez-Páez, Miguel; de Miguel-Pueyo, Luis; Marín-Salido, Esteban José; Carrasco-Brenes, Antonio; Martín-Gallego, Alvaro; Arráez-Sánchez, Miguel Ángel

    2014-01-01

    We report the case of a 44-year-old male, lacking clinical history of previous illness, who had surgery at our hospital to treat a mass in the supraclavicular space. The patient presented with a 1-month progressive distal paresis of the left arm. The histo-pathological examination of the mass revealed an encapsulated fat necrosis. Fat necrosis is characterised by cystic architecture, encapsulation with fat necrosis within, and inflammatory infiltration of its walls. Neural structure compression secondary to this tumour mass is very rare. Fat necrosis is more frequent in the lower limbs, in areas exposed to trauma. This article is the first report of brachial plexus compression due to supraclavicular fat necrosis.

  19. Hypotensive bradycardic events during shoulder arthroscopic surgery under interscalene brachial plexus blocks

    PubMed Central

    Song, Seok Young

    2012-01-01

    Sudden, profound hypotensive and bradycardic events (HBEs) have been reported in more than 20% of patients undergoing shoulder arthroscopy in the sitting position. Although HBEs may be associated with the adverse effects of interscalene brachial plexus block (ISBPB) in the sitting position, the underlying mechanisms responsible for HBEs during the course of shoulder surgery are not well understood. The basic mechanisms of HBEs may be associated with the underlying mechanisms responsible for vasovagal syncope, carotid sinus hypersensitivity or orthostatic syncope. In this review, we discussed the possible mechanisms of HBEs during shoulder arthroscopic surgery, in the sitting position, under ISBPB. In particular, we focused on the relationship between HBEs and various types of syncopal reactions, the relationship between HBEs and the Bezold-Jarisch reflex, and the new contributing factors for the occurrence of HBEs, such as stellate ganglion block or the intraoperative administration of intravenous fentanyl. PMID:22474545

  20. Unrecognized high brachial artery bifurcation is associated with higher rate of dialysis access failure.

    PubMed

    Kirksey, Lee

    2011-01-01

    A thorough consideration of all factors contributing to successful dialysis access creation is necessary to achieve optimal outcomes. A high bifurcation of the brachial artery (brachioradial variant) occurs in greater than 20% of patients. Dialysis access was created in 22 limbs with this variant--15 fistula, and 7 prosthetic grafts. Nonmaturation occurred in 33% of fistula. Early thromboses occurred in 29% of prosthetic bridge grafts. In this experience, the brachioradial variant is associated with a relatively higher rate of fistula nonmaturation and prosthetic graft thromboses. These findings reinforce the critical role of preoperative imaging studies in dialysis access creation. A sound algorithm for the surgical management of the brachioradial variation facilitates decision making and will improve dialysis access outcomes.

  1. Radiological Imaging Findings of a Case with Vertebral Osteoid Osteoma Leading to Brachial Neuralgia

    PubMed Central

    Gokce, Erkan; Ayan, Erdoğan; Çelikyay, Fatih; Acu, Berat

    2013-01-01

    Osteoid osteoma is a small, benign osteoblastic tumor consisting of a highly vascularized nidus of connective tissue surrounded by sclerotic bone. Three-quarters of osteoid osteomas are located in the long bones, and only 7-12% in the vertebral column. The classical clinical presentation of spinal osteoid osteoma is that of painful scoliosis. Other clinical features include nerve root irritation and night pain. Osteoid osteoma has characteristic computed tomography (CT) findings. Because magnetic resonance imaging (MRI) findings of the osteoid osteomas causing intense perinidal edema can be confusing, these patients should be evaluated with clinical findings and other imaging techniques. In this study, we present X-ray, CT, and MRI findings of a case with osteoid osteoma located in thoracic 1 vertebra left lamina and transverse process junction leading to brachial neuralgia symptoms. PMID:24404413

  2. Multimodality imaging of peripheral neuropathies of the upper limb and brachial plexus.

    PubMed

    Linda, Dorota Dominika; Harish, Srinivasan; Stewart, Brian G; Finlay, Karen; Parasu, Naveen; Rebello, Ryan Paul

    2010-09-01

    The peripheral nerves of the upper limb are affected by a number of entrapment and compression neuropathies. These discrete syndromes involve the brachial plexus as well as the musculocutaneous, axillary, suprascapular, ulnar, radial, and median nerves. Clinical examination and electrophysiologic studies are the traditional mainstay of diagnostic work-up; however, ultrasonography and magnetic resonance imaging provide spatial information regarding the affected nerve and its surroundings, often assisting in narrowing the differential diagnosis and guiding treatment. Imaging is particularly valuable in complex cases with discrepant nerve function test results. Familiarity with the clinical features of various peripheral neuropathies of the upper extremity, the relevant anatomy, and the most common sites and causes of nerve entrapment assists in diagnosis and treatment.

  3. Attenuation of brain grey matter volume in brachial plexus injury patients.

    PubMed

    Lu, Yechen; Liu, Hanqiu; Hua, Xuyun; Xu, Jian-Guang; Gu, Yu-Dong; Shen, Yundong

    2016-01-01

    Brachial plexus injury (BPI) causes functional changes in the brain, but the structural changes resulting from BPI remain unknown. In this study, we compared grey matter volume between nine BPI patients and ten healthy controls by means of voxel-based morphometry. This was the first study of cortical morphology in BPI. We found that brain regions including the cerebellum, anterior cingulate cortex, bilateral inferior, medial, superior frontal lobe, and bilateral insula had less grey matter in BPI patients. Most of the affected brain regions of BPI patients are closely related to motor function. We speculate that the loss of grey matter in multiple regions might be the neural basis of the difficulties in the motor rehabilitation of BPI patients. The mapping result might provide new target regions for interventions of motor rehabilitation.

  4. Postoperative monitoring in free muscle transfers for reconstruction in brachial plexus injuries.

    PubMed

    Dodakundi, Chaitanya; Doi, Kazuteru; Hattori, Yasunori; Sakamoto, Soutetsu; Yonemura, Hiroshi; Fujihara, Yuki

    2012-03-01

    Free gracilis transfers are done for reanimation of the upper limb in traumatic total brachial plexus palsy. Because of buried nature of the free muscle and monitoring skin flap in the axillary or infraclavicular region, it is always a tricky situation for continuous and repeated monitoring to assess vascular status. Critical ischemia times vary between the muscle and monitoring skin flap because of which signs of ischemic changes in the monitoring skin flap are always delayed with respect to the muscle. We describe a novel method that uses the principle of evoked potentials from the muscle to assess the vascular status of the free muscle and detects vascular compromise early before the skin changes are apparent.

  5. Anatomic Variation of Subclavian Artery Visualized on Ultrasound-Guided Supraclavicular Brachial Plexus Block

    PubMed Central

    Prasad, Arunima; Banerjee, Sumantra Sarathi

    2014-01-01

    Use of ultrasonography for performance of nerve and plexus blocks has made the process simpler and safer. However, at times, variant anatomy of the visualized structures can lead to failure of blocks or complications such as intravascular injections. This is especially true in case of novice operators. We report a case of a variant branch of subclavian artery, possibly the dorsal scapular artery passing through the brachial plexus nerve bundles in the supraclavicular area. Since this variation in anatomy was visualized in the scout scan prior to the performance of the block, it was possible to avoid any accidental puncture. Hence, a thorough knowledge of the ultrasound anatomy is important in order to identify various aberrations and variations. It is also prudent to perform a preliminary scan, prior to performance of the block to localize the target area and avoid any inadvertent complications. PMID:25143765

  6. Radiological imaging findings of a case with vertebral osteoid osteoma leading to brachial neuralgia.

    PubMed

    Gokce, Erkan; Ayan, Erdoğan; Celikyay, Fatih; Acu, Berat

    2013-01-01

    Osteoid osteoma is a small, benign osteoblastic tumor consisting of a highly vascularized nidus of connective tissue surrounded by sclerotic bone. Three-quarters of osteoid osteomas are located in the long bones, and only 7-12% in the vertebral column. The classical clinical presentation of spinal osteoid osteoma is that of painful scoliosis. Other clinical features include nerve root irritation and night pain. Osteoid osteoma has characteristic computed tomography (CT) findings. Because magnetic resonance imaging (MRI) findings of the osteoid osteomas causing intense perinidal edema can be confusing, these patients should be evaluated with clinical findings and other imaging techniques. In this study, we present X-ray, CT, and MRI findings of a case with osteoid osteoma located in thoracic 1 vertebra left lamina and transverse process junction leading to brachial neuralgia symptoms.

  7. Extended Long-Term (5 Years) Outcomes of Triangle Tilt Surgery in Obstetric Brachial Plexus Injury

    PubMed Central

    Nath, Rahul K; Somasundaram, Chandra

    2013-01-01

    Objective: We evaluated the "extended" long-term (5 years) functional outcomes in obstetric brachial plexus injury (OBPI) patients, who underwent triangle tilt surgery between February 2005 and January 2008. Methods: Twenty two children (9 girls and 13 boys, mean age at surgery was 5.8 years; ranging 2.1-11.8 years old), who initially presented with medial rotation contracture and scapula deformity secondary to obstetric brachial plexus injury were included in this study. Functional movements were evaluated pre-operatively, and 5 years following triangle tilt surgery by modified Mallet scale. Results: Here, we report long-term (5 years) follow-up of triangle tilt surgery for 22 OBPI patients. Upper extremity functional movements such as, external rotation (2.5±0.6 to 4.1±0.8, p<0.0001), hand-to-spine (2.6±0.6 to 3.4±1.1, p<0.005), hand-to-neck (2.7±0.7 to 4.3±0.7, p<0.0001), hand-to-mouth (2.3±0.9 (92º±33) to 4.2±0.5 (21º±16), p<0.0001), and supination (2.6±1.1 (-8.2º ±51) to 4.1±0.7 (61±32)) were significantly improved (p<0.0001), and maintained over the extended long-term (5 years). Total modified Mallet functional score was also shown to improve from 14.1±2.7 to 20.3±2.5. Conclusions: The triangle tilt surgery improved all shoulder functions significantly, and maintained over the extended long-term (5 years) in these patients. PMID:23730369

  8. Brachial artery vasomotion and transducer pressure effect on measurements by active contour segmentation on ultrasound

    SciTech Connect

    Cary, Theodore W.; Sultan, Laith R.; Sehgal, Chandra M.; Reamer, Courtney B.; Mohler, Emile R.

    2014-02-15

    Purpose: To use feed-forward active contours (snakes) to track and measure brachial artery vasomotion on ultrasound images recorded in both transverse and longitudinal views; and to compare the algorithm's performance in each view. Methods: Longitudinal and transverse view ultrasound image sequences of 45 brachial arteries were segmented by feed-forward active contour (FFAC). The segmented regions were used to measure vasomotion artery diameter, cross-sectional area, and distention both as peak-to-peak diameter and as area. ECG waveforms were also simultaneously extracted frame-by-frame by thresholding a running finite-difference image between consecutive images. The arterial and ECG waveforms were compared as they traced each phase of the cardiac cycle. Results: FFAC successfully segmented arteries in longitudinal and transverse views in all 45 cases. The automated analysis took significantly less time than manual tracing, but produced superior, well-behaved arterial waveforms. Automated arterial measurements also had lower interobserver variability as measured by correlation, difference in mean values, and coefficient of variation. Although FFAC successfully segmented both the longitudinal and transverse images, transverse measurements were less variable. The cross-sectional area computed from the longitudinal images was 27% lower than the area measured from transverse images, possibly due to the compression of the artery along the image depth by transducer pressure. Conclusions: FFAC is a robust and sensitive vasomotion segmentation algorithm in both transverse and longitudinal views. Transverse imaging may offer advantages over longitudinal imaging: transverse measurements are more consistent, possibly because the method is less sensitive to variations in transducer pressure during imaging.

  9. Associations of Depressive Symptoms and Brachial Artery Reactivity among Police Officers

    PubMed Central

    Charles, Luenda E; Gu, Ja K; Burchfiel, Cecil M; Andrew, Michael E; Joseph, Parveen N; Dorn, Joan M

    2013-01-01

    Objectives Mental health has been shown to be linked with certain underlying physiological mechanisms. The objective of this cross sectional study was to investigate the relationship between depressive symptoms and brachial artery reactivity (BAR) in an understudied population: police officers. Methods Participants were 351 police officers who were clinically examined in the Buffalo Cardio-Metabolic Police Stress (BCOPS) study. BAR was performed using standard B-Mode ultrasound procedures. Depressive symptoms were measured using the Center for Epidemiological Studies Depression (CES-D) scale. Mean values of the difference between the baseline and maximum diameters of the brachial artery were determined across three categories of CES-D score using the analysis of variance and the analysis of covariance. p-values for linear trends were obtained from linear regression models. Results The mean age (± standard deviation) of all officers was 40.9 ± 7.2 years. Women had a slightly higher mean CES-D score than men (8.9 ± 8.9 vs. 7.4 ± 6.4) and a slightly higher percentage increase of BAR than men (6.90 vs. 5.26%). Smoking status significantly modified the associations between depressive symptoms and BAR. Among current smokers, mean absolute values of BAR significantly decreased as depressive symptoms increased after adjustment for age, gender, race/ethnicity, hypertension, and diabetes; the multivariate-adjusted p-values were 0.033 (absolute) and 0.040 (%). Associations between depressive symptoms and BAR were not statistically significant among former smokers or never smokers. Conclusion Depressive symptoms were inversely associated with BAR among police officers who were current smokers and together may be considered a risk factor for cardiovascular disease among police officers. Further prospective research is warranted. PMID:23516114

  10. Characterizing Methods of Measuring Flow-Mediated Dilation in the Brachial Artery

    NASA Technical Reports Server (NTRS)

    Callender, Ariane R.

    2010-01-01

    Regulation of vascular tone is one of the many important functions of the vascular endothelium. Endothelial dysfunction is a critical early event in the pathogenesis of atherosclerosis and occurs in the absence of angiographic disease. Flow-Mediated Dilation (FMD) is a noninvasive technique commonly used to evaluate endothelium-dependent vasodilation in humans and gauge the health of the cardiovascular system. Reductions in brachial artery FMD have been strongly correlated with disease progression and are predictive of future cardiac events. The flow stimulus for brachial artery FMD occurs as a result of the increased shear stress following deflation of an occlusion cuff around the upper arm. Using 2-dimensional ultrasound, changes in arterial diameter up to 5-minutes following cuff deflation are calculated from baseline image measurements. Along with pulsed Doppler measures of flow velocity through the artery, flow-mediated, endothelium-dependent vasodilation can be assessed. There is debate among investigators, however, about the proper positioning of the occlusion cuff during FMD testing. It is thought that placement of the cuff around the upper arm may not accurately reflect the impact of nitric oxide, a critically important molecule released as a result of the increased shear stress created by the FMD technique. Data suggest that the production of other endogenous metabolites may also contribute to FMD-related changes when positioning the cuff around the upper arm. To overcome the potential influence of such molecules, researchers now suggest that the occlusion cuff be placed below the elbow allowing a more precise estimate of nitric oxide mediated dilation. The purpose of this study is to compare the differences in FMD between the two methodologies of occlusion cuff placement. In addition, this study will determine the method that is easier for ultrasound technicians to perform and will produce a low coefficient of variance between technicians. Ultimately

  11. Comparative evaluation of ropivacaine and ropivacaine with dexamethasone in supraclavicular brachial plexus block for postoperative analgesia

    PubMed Central

    Kumar, Santosh; Palaria, Urmila; Sinha, Ajay K.; Punera, D. C.; Pandey, Vijita

    2014-01-01

    Background: Mixing of various adjuvants has been tried with local anesthetics in an attempt to prolong anesthesia from peripheral nerve blocks but have met with inconclusive success. More recent studies indicate that 8 mg dexamethasone added to perineural local anesthetic injections augment the duration of peripheral nerve block analgesia. Aims: Evaluating the hypothesis that adding dexamethasone to ropivacaine significantly prolongs the duration of analgesia in supraclavicular brachial plexus block compared with ropivacaine alone. Patients and Methods: It was a randomized, prospective, and double-blind clinical trial. Eighty patients of ASA I and II of either sex, aged 16-60 years, undergoing elective upper limb surgeries were equally divided into two groups and given supraclavicular nerve block. Group R patients (n = 40) received 30 ml of 0.5% ropivacaine with distilled water (2 ml)-control group whereas Group D patients (n = 40) received 30 ml of 0.5% ropivacaine with 8 mg dexamethasone (2 ml)-study group. The primary outcome was measured as duration of analgesia that was defined as the interval between the onset of sensory block and the first request for analgesia by the patient. The secondary outcome included maximum visual analogue scale (VAS), total analgesia consumption, surgeon satisfaction, and side effects. Results: Group R patients required first rescue analgesia earlier (557 ± 58.99 min) than those of Group D patients (1179.4 ± 108.60 min), which was found statistically significant in Group D (P < 0.000). The total dose of rescue analgesia was higher in Group R as compared to Group D, which was statistically significant (P < 0.00). Conclusion: Addition of dexamethasone (8 mg) to ropivacaine in supraclavicular brachial plexus approach significantly and safely prolongs motor blockade and postoperative analgesia (sensory) that lasted much longer than that produced by local anesthetic alone. PMID:25886227

  12. Brachial artery peak velocity variation to predict fluid responsiveness in mechanically ventilated patients

    PubMed Central

    2009-01-01

    Introduction Although several parameters have been proposed to predict the hemodynamic response to fluid expansion in critically ill patients, most of them are invasive or require the use of special monitoring devices. The aim of this study is to determine whether noninvasive evaluation of respiratory variation of brachial artery peak velocity flow measured using Doppler ultrasound could predict fluid responsiveness in mechanically ventilated patients. Methods We conducted a prospective clinical research in a 17-bed multidisciplinary ICU and included 38 mechanically ventilated patients for whom fluid administration was planned due to the presence of acute circulatory failure. Volume expansion (VE) was performed with 500 mL of a synthetic colloid. Patients were classified as responders if stroke volume index (SVi) increased ≥ 15% after VE. The respiratory variation in Vpeakbrach (ΔVpeakbrach) was calculated as the difference between maximum and minimum values of Vpeakbrach over a single respiratory cycle, divided by the mean of the two values and expressed as a percentage. Radial arterial pressure variation (ΔPPrad) and stroke volume variation measured using the FloTrac/Vigileo system (ΔSVVigileo), were also calculated. Results VE increased SVi by ≥ 15% in 19 patients (responders). At baseline, ΔVpeakbrach, ΔPPrad and ΔSVVigileo were significantly higher in responder than nonresponder patients [14 vs 8%; 18 vs. 5%; 13 vs 8%; P < 0.0001, respectively). A ΔVpeakbrach value >10% predicted fluid responsiveness with a sensitivity of 74% and a specificity of 95%. A ΔPPrad value >10% and a ΔSVVigileo >11% predicted volume responsiveness with a sensitivity of 95% and 79%, and a specificity of 95% and 89%, respectively. Conclusions Respiratory variations in brachial artery peak velocity could be a feasible tool for the noninvasive assessment of fluid responsiveness in patients with mechanical ventilatory support and acute circulatory failure. Trial Registration

  13. Impaired handgrip exercise-induced brachial artery flow-mediated dilation in young obese males.

    PubMed

    Slattery, David J; Stuckless, Troy J R; King, Trevor J; Pyke, Kyra E

    2016-05-01

    Flow mediated dilation (FMD) stimulated by different shear stress stimulus profiles may recruit distinct transduction mechanisms, and provide distinct information regarding endothelial function. The purpose of this study was to determine whether obesity influences brachial artery FMD differently depending on the shear stress profile used for FMD assessment. The FMD response to a brief, intermediate, and sustained shear stress profile was assessed in obese (n = 9) and lean (n = 19) young men as follows: brief stimulus, standard reactive hyperemia (RH) following a 5 min forearm occlusion (5 min RH); intermediate stimulus, RH following a 15 min forearm occlusion (15 min RH); sustained stimulus, 10 min of handgrip exercise (HGEX). Brachial artery diameter and mean shear stress were assessed using echo and Doppler ultrasound, respectively, during each FMD test. There was no group difference in HGEX shear stress (p = 0.390); however, the obese group had a lower HGEX-FMD (5.2 ± 3.0% versus 11.5 ± 4.4%, p < 0.001). There was no group difference in 5 min RH-FMD (p = 0.466) or 15 min RH-FMD (p = 0.181); however, the shear stress stimulus was larger in the obese group. After normalization to the stimulus the 15 min RH-FMD (p = 0.002), but not the 5 min RH-FMD (p = 0.118) was lower in the obese group. These data suggest that obesity may have a more pronounced impact on the endothelium's ability to respond to prolonged increases in shear stress.

  14. Clinicopathologic variability of the GRN A9D mutation, including amyotrophic lateral sclerosis

    PubMed Central

    Cannon, Ashley; Fujioka, Shinsuke; Rutherford, Nicola J.; Ferman, Tanis J.; Broderick, Daniel F.; Boylan, Kevin B.; Graff-Radford, Neill R.; Uitti, Ryan J.; Rademakers, Rosa; Wszolek, Zbigniew K.

    2013-01-01

    Objective: We examined the clinical and pathologic phenotypes of GRN mutation carriers with the pathogenic A9D (g.26C>A) missense mutation. Methods: Three patients with GRN A9D mutations were evaluated clinically and came to autopsy with subsequent neuropathologic examination. Results: The clinical diagnoses of patients with GRN A9D mutations were amyotrophic lateral sclerosis, atypical extrapyramidal disorder, and behavioral variant frontotemporal dementia. Immunohistochemistry for TAR DNA-binding protein 43 (TDP-43) revealed variability in morphology and distribution of pathology. One patient had notable involvement of motor neurons in the spinal cord as well as type B TDP-43, whereas 2 other patients had type A TDP-43. Conclusions: The clinical presentation of the GRN A9D missense mutation is not restricted to behavioral variant frontotemporal dementia and may include aphasia, extrapyramidal features, and, notably, amyotrophic lateral sclerosis. PMID:23596077

  15. [Diagnostic Criteria for Amyotrophic Lateral Sclerosis/Parkinsonism-Dementia Complex in the Kii Peninsula, Japan].

    PubMed

    Kokubo, Yasumasa

    2015-07-01

    The diagnostic criteria for amyotrophic lateral sclerosis/parkinsonism-dementia complex in the Kii peninsula of Japan (Kii ALS/PDC) have been proposed. Muro disease has been considered an endemic neurodegenerative disease in the southern part of the Kii peninsula. Recent intensive and comprehensive research has revealed it to be a complex and genetically heterogeneous disease. At present, there are four subtypes of Muro disease: sporadic amyotrophic lateral sclerosis (ALS), Kii ALS/PDC with tauopathy, ALS with C9orf72 gene mutation, and ALS with optineurin gene mutation. The present criteria are applicable to only one of these subtypes, Kii ALS/PDC with tauopathy, which is quite similar to ALS/PDC in Guam.

  16. A journey of suffering: living with amyotrophic lateral sclerosis in South Korea.

    PubMed

    Oh, Hyunjin; Schepp, Karen G; McGrath, Barbara B

    2014-06-01

    Amyotrophic lateral sclerosis presents significant challenges for patients because of the devastating disease characteristics and the fact that there is no treatment available. In this article, we explored the illness experiences from the perspectives of patients with amyotrophic lateral sclerosis in the sociocultural context of South Korea. Fifteen patients were observed and interviewed between September 2009 and July 2010 in the metropolitan area of South Korea. We used an ethnographic approach for data collection and analysis. The meta-theme generated was "a journey of suffering," and three themes emerged: (a) off the course, (b) drifting, and (c) on a new boat. Participants experienced multidimensional suffering as the disease progressed. Healthcare professionals should understand that, for many patients, this disease is a process or a series of experiences rather than a single diagnosis. This knowledge would allow healthcare providers to help patients prepare for those needs that arise as the disease worsens.

  17. Dose–Volume Modeling of Brachial Plexus-Associated Neuropathy After Radiation Therapy for Head-and-Neck Cancer: Findings From a Prospective Screening Protocol

    SciTech Connect

    Chen, Allen M.; Wang, Pin-Chieh; Daly, Megan E.; Cui, Jing; Hall, William H.; Vijayakumar, Srinivasan; Phillips, Theodore L.; Farwell, D. Gregory; Purdy, James A.

    2014-03-15

    Purpose: Data from a prospective screening protocol administered for patients previously irradiated for head-and-neck cancer was analyzed to identify dosimetric predictors of brachial plexus-associated neuropathy. Methods and Materials: Three hundred fifty-two patients who had previously completed radiation therapy for squamous cell carcinoma of the head and neck were prospectively screened from August 2007 to April 2013 using a standardized self-administered instrument for symptoms of neuropathy thought to be related to brachial plexus injury. All patients were disease-free at the time of screening. The median time from radiation therapy was 40 months (range, 6-111 months). A total of 177 patients (50%) underwent neck dissection. Two hundred twenty-one patients (63%) received concurrent chemotherapy. Results: Fifty-one patients (14%) reported brachial plexus-related neuropathic symptoms with the most common being ipsilateral pain (50%), numbness/tingling (40%), and motor weakness and/or muscle atrophy (25%). The 3- and 5-year estimates of freedom from brachial plexus-associated neuropathy were 86% and 81%, respectively. Clinical/pathological N3 disease (P<.001) and maximum radiation dose to the ipsilateral brachial plexus (P=.01) were significantly associated with neuropathic symptoms. Cox regression analysis revealed significant dose–volume effects for brachial plexus-associated neuropathy. The volume of the ipsilateral brachial plexus receiving >70 Gy (V70) predicted for symptoms, with the incidence increasing with V70 >10% (P<.001). A correlation was also observed for the volume receiving >74 Gy (V74) among patients treated without neck dissection, with a cutoff of 4% predictive of symptoms (P=.038). Conclusions: Dose–volume guidelines were developed for radiation planning that may limit brachial plexus-related neuropathies.

  18. Targeted Riluzole Delivery by Antioxidant Nanovectors for Treating Amyotrophic Lateral Sclerosis

    DTIC Science & Technology

    2015-06-01

    murine model of amyotrophic lateral sclerosis. HCCs were produced by Dr. James Tour of Rice University and provided to Dr. Grill to assess in his... Rice University. The goal of this proposal was to determine whether functionalized PEG-HCCs could alone, or in combination with riluzole, enhance...Homozygous subjects were grafted with osmotic minipumps filled with either PEG-HCCs (condition A) created and provided by the Tour lab of Rice

  19. Psychological wellbeing and quality of life in amyotrophic lateral sclerosis: a review.

    PubMed

    Pagnini, Francesco

    2013-01-01

    Amyotrophic lateral sclerosis is a fatal neurodegenerative disease with a progressive and rapid course that, so far, cannot be stopped or reversed. The psychological impact of the disease is huge, on both patients and caregivers. This review summarizes studies that have investigated quality of life, depression, anxiety, pain, spiritual and existential issues, hope, and hopelessness in the ALS field, with attention to both patients and their caregivers. Psychological support and the possible role of psychologists in the ALS field are also discussed.

  20. Amyotrophic lateral sclerosis and Alzheimer's disease--clinical and neuropathological considerations in two cases.

    PubMed

    Rusina, R; Sheardová, K; Rektorová, I; Ridzon, P; Kulist'ák, P; Matej, R

    2007-07-01

    Amyotrophic lateral sclerosis (ALS) may be accompanied by cognitive impairment; when present, it is mainly in the form of frontotemporal impairment. We report on two cases with clinically defined ALS that subsequently developed dementia. Neuropathological examination showed not only the typical neuropathological hallmarks characteristic of ALS but, surprisingly, also showed neurofibrillary tangles and neuritic plaques in sufficient numbers to fulfill the diagnostic criteria of definite Alzheimer's disease.

  1. A mitochondrial origin for frontotemporal dementia and amyotrophic lateral sclerosis through CHCHD10 involvement.

    PubMed

    Bannwarth, Sylvie; Ait-El-Mkadem, Samira; Chaussenot, Annabelle; Genin, Emmanuelle C; Lacas-Gervais, Sandra; Fragaki, Konstantina; Berg-Alonso, Laetitia; Kageyama, Yusuke; Serre, Valérie; Moore, David G; Verschueren, Annie; Rouzier, Cécile; Le Ber, Isabelle; Augé, Gaëlle; Cochaud, Charlotte; Lespinasse, Françoise; N'Guyen, Karine; de Septenville, Anne; Brice, Alexis; Yu-Wai-Man, Patrick; Sesaki, Hiromi; Pouget, Jean; Paquis-Flucklinger, Véronique

    2014-08-01

    Mitochondrial DNA instability disorders are responsible for a large clinical spectrum, among which amyotrophic lateral sclerosis-like symptoms and frontotemporal dementia are extremely rare. We report a large family with a late-onset phenotype including motor neuron disease, cognitive decline resembling frontotemporal dementia, cerebellar ataxia and myopathy. In all patients, muscle biopsy showed ragged-red and cytochrome c oxidase-negative fibres with combined respiratory chain deficiency and abnormal assembly of complex V. The multiple mitochondrial DNA deletions found in skeletal muscle revealed a mitochondrial DNA instability disorder. Patient fibroblasts present with respiratory chain deficiency, mitochondrial ultrastructural alterations and fragmentation of the mitochondrial network. Interestingly, expression of matrix-targeted photoactivatable GFP showed that mitochondrial fusion was not inhibited in patient fibroblasts. Using whole-exome sequencing we identified a missense mutation (c.176C>T; p.Ser59Leu) in the CHCHD10 gene that encodes a coiled-coil helix coiled-coil helix protein, whose function is unknown. We show that CHCHD10 is a mitochondrial protein located in the intermembrane space and enriched at cristae junctions. Overexpression of a CHCHD10 mutant allele in HeLa cells led to fragmentation of the mitochondrial network and ultrastructural major abnormalities including loss, disorganization and dilatation of cristae. The observation of a frontotemporal dementia-amyotrophic lateral sclerosis phenotype in a mitochondrial disease led us to analyse CHCHD10 in a cohort of 21 families with pathologically proven frontotemporal dementia-amyotrophic lateral sclerosis. We identified the same missense p.Ser59Leu mutation in one of these families. This work opens a novel field to explore the pathogenesis of the frontotemporal dementia-amyotrophic lateral sclerosis clinical spectrum by showing that mitochondrial disease may be at the origin of some of these

  2. Efficacy of peptide nucleic acid and selected conjugates against specific cellular pathologies of amyotrophic lateral sclerosis.

    PubMed

    Browne, Elisse C; Parakh, Sonam; Duncan, Luke F; Langford, Steven J; Atkin, Julie D; Abbott, Belinda M

    2016-04-01

    Cellular studies have been undertaken on a nonamer peptide nucleic acid (PNA) sequence, which binds to mRNA encoding superoxide dismutase 1, and a series of peptide nucleic acids conjugated to synthetic lipophilic vitamin analogs including a recently prepared menadione (vitamin K) analog. Reduction of both mutant superoxide dismutase 1 inclusion formation and endoplasmic reticulum stress, two of the key cellular pathological hallmarks in amyotrophic lateral sclerosis, by two of the prepared PNA oligomers is reported for the first time.

  3. Masticatory muscle pain and progressive mouth opening limitation caused by amyotrophic lateral sclerosis: a case report.

    PubMed

    Pang, Kang Mi; Park, Ji Woon

    2015-01-01

    This article reports a case of masticatory muscle pain and progressive limited mouth opening secondary to amyotrophic lateral sclerosis (ALS), popularly known as Lou Gehrig's disease. The symptoms were first mistaken as those of temporomandibular disorders, before fatty degeneration of all masticatory muscles were discovered on magnetic resonance imaging (MRI). ALS should be considered in the differential diagnosis process when the patient presents with longstanding progressive mouth opening limitation associated with pain. MRI could facilitate the diagnostic process.

  4. Novel Neuroprotective Multicomponent Therapy for Amyotrophic Lateral Sclerosis Designed by Networked Systems.

    PubMed

    Herrando-Grabulosa, Mireia; Mulet, Roger; Pujol, Albert; Mas, José Manuel; Navarro, Xavier; Aloy, Patrick; Coma, Mireia; Casas, Caty

    2016-01-01

    Amyotrophic Lateral Sclerosis is a fatal, progressive neurodegenerative disease characterized by loss of motor neuron function for which there is no effective treatment. One of the main difficulties in developing new therapies lies on the multiple events that contribute to motor neuron death in amyotrophic lateral sclerosis. Several pathological mechanisms have been identified as underlying events of the disease process, including excitotoxicity, mitochondrial dysfunction, oxidative stress, altered axonal transport, proteasome dysfunction, synaptic deficits, glial cell contribution, and disrupted clearance of misfolded proteins. Our approach in this study was based on a holistic vision of these mechanisms and the use of computational tools to identify polypharmacology for targeting multiple etiopathogenic pathways. By using a repositioning analysis based on systems biology approach (TPMS technology), we identified and validated the neuroprotective potential of two new drug combinations: Aliretinoin and Pranlukast, and Aliretinoin and Mefloquine. In addition, we estimated their molecular mechanisms of action in silico and validated some of these results in a well-established in vitro model of amyotrophic lateral sclerosis based on cultured spinal cord slices. The results verified that Aliretinoin and Pranlukast, and Aliretinoin and Mefloquine promote neuroprotection of motor neurons and reduce microgliosis.

  5. Novel Neuroprotective Multicomponent Therapy for Amyotrophic Lateral Sclerosis Designed by Networked Systems

    PubMed Central

    Herrando-Grabulosa, Mireia; Mulet, Roger; Pujol, Albert; Mas, José Manuel; Navarro, Xavier; Aloy, Patrick; Coma, Mireia; Casas, Caty

    2016-01-01

    Amyotrophic Lateral Sclerosis is a fatal, progressive neurodegenerative disease characterized by loss of motor neuron function for which there is no effective treatment. One of the main difficulties in developing new therapies lies on the multiple events that contribute to motor neuron death in amyotrophic lateral sclerosis. Several pathological mechanisms have been identified as underlying events of the disease process, including excitotoxicity, mitochondrial dysfunction, oxidative stress, altered axonal transport, proteasome dysfunction, synaptic deficits, glial cell contribution, and disrupted clearance of misfolded proteins. Our approach in this study was based on a holistic vision of these mechanisms and the use of computational tools to identify polypharmacology for targeting multiple etiopathogenic pathways. By using a repositioning analysis based on systems biology approach (TPMS technology), we identified and validated the neuroprotective potential of two new drug combinations: Aliretinoin and Pranlukast, and Aliretinoin and Mefloquine. In addition, we estimated their molecular mechanisms of action in silico and validated some of these results in a well-established in vitro model of amyotrophic lateral sclerosis based on cultured spinal cord slices. The results verified that Aliretinoin and Pranlukast, and Aliretinoin and Mefloquine promote neuroprotection of motor neurons and reduce microgliosis. PMID:26807587

  6. The brachial plexus branches to the pectoral muscles in adult rats: morphological aspects and morphometric normative data.

    PubMed

    Riva, Nilo; Domi, Teuta; Lopez, Ignazio Diego; Triolo, Daniela; Fossaghi, Andrea; Dina, Giorgia; Podini, Paola; Comi, Giancarlo; Quattrini, Angelo

    2012-01-01

    Animal models provide an important tool to investigate the pathogenesis of neuromuscular disorders. In the present study, we analyze fiber composition of the brachial plexus branches to the pectoral muscles: the medial anterior thoracic nerve (MATN) and the lateral anterior thoracic nerve (LATN). The morphological and morphometric characteristics and the percentage of motor fibers within each nerve are here reported, adding information to microscopic anatomy knowledge of the rat brachial plexus. As control, we employed the quadriceps nerve, commonly used for the evaluation of motor fibers at hindlimbs. We demonstrated that the MATN and the LATN are predominantly composed of large motor fibers and therefore could be employed to evaluate the peripheral nervous system (PNS) involvement at forelimbs in neurological diseases models, predominantly affecting the motor fiber compartment.

  7. The brachial plexus branches to the pectoral muscles in adult rats: morphological aspects and morphometric normative data

    PubMed Central

    Riva, Nilo; Domi, Teuta; Lopez, Ignazio Diego; Triolo, Daniela; Fossaghi, Andrea; Dina, Giorgia; Podini, Paola; Comi, Giancarlo; Quattrini, Angelo

    2012-01-01

    Animal models provide an important tool to investigate the pathogenesis of neuromuscular disorders. In the present study, we analyze fiber composition of the brachial plexus branches to the pectoral muscles: the medial anterior thoracic nerve (MATN) and the lateral anterior thoracic nerve (LATN). The morphological and morphometric characteristics and the percentage of motor fibers within each nerve are here reported, adding information to microscopic anatomy knowledge of the rat brachial plexus. As control, we employed the quadriceps nerve, commonly used for the evaluation of motor fibers at hindlimbs. We demonstrated that the MATN and the LATN are predominantly composed of large motor fibers and therefore could be employed to evaluate the peripheral nervous system (PNS) involvement at forelimbs in neurological diseases models, predominantly affecting the motor fiber compartment. PMID:23087618

  8. Bilateral variations of brachial plexus involving the median nerve and lateral cord: An anatomical case study with clinical implications.

    PubMed

    Butz, James J; Shiwlochan, Devina G; Brown, Kevin C; Prasad, Alathady M; Murlimanju, Bukkambudhi V; Viswanath, Srikanteswara

    2014-01-01

    During the routine dissection of upper limbs of a Caucasian male cadaver, variations were observed in the brachial plexus. In the right extremity, the lateral cord was piercing the coracobrachialis muscle. The musculocutaneous nerve and lateral root of the median nerve were observed to be branching inferior to the lower attachment of coracobrachialis muscle. The left extremity exhibited the passage of the median nerve through the flat tendon of the coracobrachialis muscle near its distal insertion into the medial surface of the body of humerus. A variation in the course and branching of the nerve might lead to variant or dual innervation of a muscle and, if inappropriately compressed, could result in a distal neuropathy. Identification of these variants of brachial plexus plays an especially important role in both clinical diagnosis and surgical practice.

  9. Promoting proximal formative assessment with relational discourse

    NASA Astrophysics Data System (ADS)

    Scherr, Rachel E.; Close, Hunter G.; McKagan, Sarah B.

    2012-02-01

    The practice of proximal formative assessment - the continual, responsive attention to students' developing understanding as it is expressed in real time - depends on students' sharing their ideas with instructors and on teachers' attending to them. Rogerian psychology presents an account of the conditions under which proximal formative assessment may be promoted or inhibited: (1) Normal classroom conditions, characterized by evaluation and attention to learning targets, may present threats to students' sense of their own competence and value, causing them to conceal their ideas and reducing the potential for proximal formative assessment. (2) In contrast, discourse patterns characterized by positive anticipation and attention to learner ideas increase the potential for proximal formative assessment and promote self-directed learning. We present an analysis methodology based on these principles and demonstrate its utility for understanding episodes of university physics instruction.

  10. A Brief Index for Proximity Searching

    NASA Astrophysics Data System (ADS)

    Téllez, Eric Sadit; Chávez, Edgar; Camarena-Ibarrola, Antonio

    Many pattern recognition tasks can be modeled as proximity searching. Here the common task is to quickly find all the elements close to a given query without sequentially scanning a very large database.

  11. Elective cesarean section to prevent anal incontinence and brachial plexus injuries associated with macrosomia--a decision analysis.

    PubMed

    Culligan, Patrick J; Myers, John A; Goldberg, Roger P; Blackwell, Linda; Gohmann, Stephan F; Abell, Troy D

    2005-01-01

    Our aim was to determine the cost-effectiveness of a policy of elective C-section for macrosomic infants to prevent maternal anal incontinence, urinary incontinence, and newborn brachial plexus injuries. We used a decision analytic model to compare the standard of care with a policy whereby all primigravid patients in the United States would undergo an ultrasound at 39 weeks gestation, followed by an elective C-section for any fetus estimated at > or =4500 g. The following clinical consequences were considered crucial to the analysis: brachial plexus injury to the newborn; maternal anal and urinary incontinence; emergency hysterectomy; hemorrhage requiring blood transfusion; and maternal mortality. Our outcome measures included (1) number of brachial plexus injuries or cases of incontinence averted, (2) incremental monetary cost per 100,000 deliveries, (3) expected quality of life of the mother and her child, and (4) "quality-adjusted life years" (QALY) associated with the two policies. For every 100,000 deliveries, the policy of elective C-section resulted in 16.6 fewer permanent brachial plexus injuries, 185.7 fewer cases of anal incontinence, and cost savings of $3,211,000. Therefore, this policy would prevent one case of anal incontinence for every 539 elective C-sections performed. The expected quality of life associated with the elective C-section policy was also greater (quality of life score 0.923 vs 0.917 on a scale from 0.0 to 1.0 and 53.6 QALY vs 53.2). A policy whereby primigravid patients in the United States have a 39 week ultrasound-estimated fetal weight followed by C-section for any fetuses > or =4500 g appears cost effective. However, the monetary costs in our analysis were sensitive to the probability estimates of urinary incontinence following C-section and vaginal delivery and the cost estimates for urinary incontinence, vaginal delivery, and C-section.

  12. Polarization proximity effect in isolator crystal pairs.

    PubMed

    Linzon, Y; Ferrera, M; Razzari, L; Pignolet, A; Morandotti, R

    2008-12-01

    We experimentally study the polarization dynamics (orientation and ellipticity) of near-infrared light transmitted through magneto-optical yttrium iron garnet isolator crystal pairs using a modified balanced detection scheme. When the pair separation is in the submillimeter range, we observed a proximity effect in which the saturation field is reduced by up to 20%. One-dimensional calculations suggest that the proximity effect originates from magnetostatic interactions between the dipole moments of the isolator crystals.

  13. Proximity sensor system development. CRADA final report

    SciTech Connect

    Haley, D.C.; Pigoski, T.M.

    1998-01-01

    Lockheed Martin Energy Research Corporation (LMERC) and Merritt Systems, Inc. (MSI) entered into a Cooperative Research and Development Agreement (CRADA) for the development and demonstration of a compact, modular proximity sensing system suitable for application to a wide class of manipulator systems operated in support of environmental restoration and waste management activities. In teleoperated modes, proximity sensing provides the manipulator operator continuous information regarding the proximity of the manipulator to objects in the workspace. In teleoperated and robotic modes, proximity sensing provides added safety through the implementation of active whole arm collision avoidance capabilities. Oak Ridge National Laboratory (ORNL), managed by LMERC for the United States Department of Energy (DOE), has developed an application specific integrated circuit (ASIC) design for the electronics required to support a modular whole arm proximity sensing system based on the use of capacitive sensors developed at Sandia National Laboratories. The use of ASIC technology greatly reduces the size of the electronics required to support the selected sensor types allowing deployment of many small sensor nodes over a large area of the manipulator surface to provide maximum sensor coverage. The ASIC design also provides a communication interface to support sensor commands from and sensor data transmission to a distributed processing system which allows modular implementation and operation of the sensor system. MSI is a commercial small business specializing in proximity sensing systems based upon infrared and acoustic sensors.

  14. Proximity operations considerations affecting spacecraft design

    NASA Technical Reports Server (NTRS)

    Staas, Steven K.

    1991-01-01

    Experience from several recent spacecraft development programs, such as Space Station Freedom (SSF) and the Orbital Maneuvering Vehicle (OMV) has shown the need for factoring proximity operations considerations into the vehicle design process. Proximity operations, those orbital maneuvers and procedures which involve operation of two or more spacecraft at ranges of less than one nautical mile, are essential to the construction, servicing, and operation of complex spacecraft. Typical proximity operations considerations which drive spacecraft design may be broken into two broad categories; flight profile characteristics and concerns, and use of various spacecraft systems during proximity operations. Proximity operations flight profile concerns include the following: (1) relative approach/separation line; (2) relative orientation of the vehicles; (3) relative translational and rotational rates; (4) vehicle interaction, in the form of thruster plume impingement, mating or demating operations, or uncontrolled contact/collision; and (5) active vehicle piloting. Spacecraft systems used during proximity operations include the following: (1) sensors, such as radar, laser ranging devices, or optical ranging systems; (2) effector hardware, such as thrusters; (3) flight control software; and (4) mating hardware, needed for docking or berthing operations. A discussion of how these factors affect vehicle design follows, addressing both active and passive/cooperative vehicles.

  15. Magnetic resonance imaging characteristics of peripheral nerve sheath tumors of the canine brachial plexus in 18 dogs.

    PubMed

    Kraft, Susan; Ehrhart, E J; Gall, David; Klopp, Lisa; Gavin, Patrick; Tucker, Russ; Bagley, Rod; Kippenes, Hege; DeHaan, Constance; Pedroia, Vince; Partington, Beth; Olby, Natasha

    2007-01-01

    Magnetic resonance imaging (MRI) examinations from 18 dogs with a histologically confirmed peripheral nerve sheath tumor (PNST) of the brachial plexus were assessed retrospectively. Almost half (8/18) had a diffuse thickening of the brachial plexus nerve(s), six of which extended into the vertebral canal. The other 10/18 dogs had a nodule or mass in the axilla (1.2-338 cm3). Seven of those 10 masses also had diffuse nerve sheath thickening, three of which extended into the vertebral canal. The majority of tumors were hyperintense to muscle on T2-weighted images and isointense on T1-weighted images. Eight of 18 PNSTs had only minimal to mild contrast enhancement and many (13/18) enhanced heterogeneously following gadolinium DTPA administration. Transverse plane images with a large enough field of view (FOV) to include both axillae and the vertebral canal were essential, allowing in-slice comparison to detect lesions by asymmetry of structures. Higher resolution, smaller FOV, multiplanar examination of the cervicothoracic spine was important for appreciating nerve root and foraminal involvement. Short tau inversion recovery, T2-weighted, pre and postcontrast T1-weighted pulse sequences were all useful. Contrast enhancement was critical to detecting subtle diffuse nerve sheath involvement or small isointense nodules, and for accurately identifying the full extent of disease. Some canine brachial plexus tumors can be challenging to detect, requiring a rigorous multiplanar multi-pulse sequence MRI examination.

  16. Unusual and Unique Variant Branches of Lateral Cord of Brachial Plexus and its Clinical Implications- A Cadaveric Study

    PubMed Central

    Padur, Ashwini Aithal; Shanthakumar, Swamy Ravindra; Shetty, Surekha Devadas; Prabhu, Gayathri Sharath; Patil, Jyothsna

    2016-01-01

    Introduction Adequate knowledge on variant morphology of brachial plexus and its branches are important in clinical applications pertaining to trauma and surgical procedures of the upper extremity. Aim Current study was aimed to report variations of the branches of the lateral cord of brachial plexus in the axilla and their possible clinical complications. Materials and Methods Total number of 82 upper limbs from 41 formalin embalmed cadavers was dissected. Careful observation was made to note the formation and branching pattern of lateral cord. Meticulous inspection for absence of branches, presence of additional or variant branches and presence of abnormal communications between its branches or with branches of other cords was carried out. Results In the present study, we noted varied branching pattern of lateral cord in 6 out of 82 limbs (7%). In one of the limb, the median nerve was formed by three roots; two from lateral cord and one from medial cord. Two limbs had absence of lateral pectoral nerve supplemented by medial pectoral nerves. One of which had an atypical ansa pectoralis. In 2 upper limbs, musculocutaneous nerve was absent and in both cases it was supplemented by median nerve. In one of the limb, coracobrachialis had dual nerve supply by musculocutaneous nerve and by an additional branch from the lateral cord. Conclusion Variations of brachial plexus and its branches could pose both intraoperative and postoperative complications which eventually affect the normal sensory and motor functions of the upper limb. PMID:27190783

  17. System xC- is a mediator of microglial function and its deletion slows symptoms in amyotrophic lateral sclerosis mice.

    PubMed

    Mesci, Pinar; Zaïdi, Sakina; Lobsiger, Christian S; Millecamps, Stéphanie; Escartin, Carole; Seilhean, Danielle; Sato, Hideyo; Mallat, Michel; Boillée, Séverine

    2015-01-01

    Amyotrophic lateral sclerosis is the most common adult-onset motor neuron disease and evidence from mice expressing amyotrophic lateral sclerosis-causing SOD1 mutations suggest that neurodegeneration is a non-cell autonomous process where microglial cells influence disease progression. However, microglial-derived neurotoxic factors still remain largely unidentified in amyotrophic lateral sclerosis. With excitotoxicity being a major mechanism proposed to cause motor neuron death in amyotrophic lateral sclerosis, our hypothesis was that excessive glutamate release by activated microglia through their system [Formula: see text] (a cystine/glutamate antiporter with the specific subunit xCT/Slc7a11) could contribute to neurodegeneration. Here we show that xCT expression is enriched in microglia compared to total mouse spinal cord and absent from motor neurons. Activated microglia induced xCT expression and during disease, xCT levels were increased in both spinal cord and isolated microglia from mutant SOD1 amyotrophic lateral sclerosis mice. Expression of xCT was also detectable in spinal cord post-mortem tissues of patients with amyotrophic lateral sclerosis and correlated with increased inflammation. Genetic deletion of xCT in mice demonstrated that activated microglia released glutamate mainly through system [Formula: see text]. Interestingly, xCT deletion also led to decreased production of specific microglial pro-inflammatory/neurotoxic factors including nitric oxide, TNFa and IL6, whereas expression of anti-inflammatory/neuroprotective markers such as Ym1/Chil3 were increased, indicating that xCT regulates microglial functions. In amyotrophic lateral sclerosis mice, xCT deletion surprisingly led to earlier symptom onset but, importantly, this was followed by a significantly slowed progressive disease phase, which resulted in more surviving motor neurons. These results are consistent with a deleterious contribution of microglial-derived glutamate during symptomatic

  18. [In search of unknown aetiopathogenesis: from Henry Louis ("Lou") Gehrig to Gianluca Signorini: history of the link between amyotrophic lateral sclerosis and sport].

    PubMed

    Cristani, Alessandro; Romagnoli, Elisa

    2006-01-01

    From "Lou" Gehirg to Gianluca Signorini: the history of almost 70 years of link between amyotrophic lateral sclerosis and sport. From first intuitions and assumptions to late epidemiological evidences supporting the possible relation between Italian soccer and amyotrophic lateral sclerosis; comparison among old and new aetiopathogenetic hypothesis.

  19. Numerical validation of a new method to assess aortic pulse wave velocity from a single recording of a brachial artery waveform with an occluding cuff.

    PubMed

    Trachet, B; Reymond, P; Kips, J; Swillens, A; De Buyzere, M; Suys, B; Stergiopulos, N; Segers, P

    2010-03-01

    Recently a new method has been proposed as a tool to measure arterial pulse wave velocity (PWV), a measure of the stiffness of the large arteries and an emerging parameter used as indicator of clinical cardiovascular risk. The method is based on measurement of brachial blood pressure during supra-systolic pressure inflation of a simple brachial cuff [the device is known as the Arteriograph (Tensiomed, Budapest, Hungary)]. This occlusion yields pronounced first and secondary peaks in the pressure waveform, the latter ascribed to a reflection from the aortic bifurcation, and PWV is calculated as the ratio of twice the jugulum-symphysis distance and the time difference between the two peaks. To test the validity of this working principle, we used a numerical model of the arterial tree to simulate pressures and flows in the normal configuration, and in a configuration with an occluded brachial artery. A pronounced secondary peak was indeed found in the brachial pressure signal of the occluded model, but its timing was only related to brachial stiffness and not to aortic stiffness. We also compared PWV's calculated with three different methods: PWVATG (approximately Arteriograph principle), PWVcar-fem (approximately carotid-femoral PWV, the current clinical gold standard method), and PWVtheor (approximately Bramwell-Hill equation). Both PWVATG (R2=0.94) and PWVcar-fem (R2=0.95) correlated well with PWVtheor, but their numerical values were lower (by 2.17+/-0.42 and 1.08+/-0.70 m/s for PWVATG and PWVcar-fem, respectively). In conclusion, our simulations question the working principle of the Arteriograph. Our data indicate that the method picks up wave reflection phenomena confined to the brachial artery, and derived values of PWV rather reflect the stiffness of the brachial arteries.

  20. Imaging assessment of glenohumeral dysplasia secondary to brachial plexus birth palsy*

    PubMed Central

    Chagas-Neto, Francisco Abaete; Dalto, Vitor Faeda; Crema, Michel Daoud; Waters, Peter M.; Gregio-Junior, Everaldo; Mazzer, Nilton; Nogueira-Barbosa, Marcello Henrique

    2016-01-01

    Objective To assess imaging parameters related to the morphology of the glenohumeral joint in children with unilateral brachial plexus birth palsy (BPBP), in comparison with those obtained for healthy shoulders. Materials and Methods We conducted a retrospective search for cases of unilateral BPBP diagnosed at our facility. Only patients with a clinical diagnosis of unilateral BPBP were included, and the final study sample consisted of 10 consecutive patients who were assessed with cross-sectional imaging. The glenoid version, the translation of the humeral head, and the degrees of glenohumeral dysplasia were assessed. Results The mean diameter of the affected humeral heads was 1.93 cm, compared with 2.33 cm for those of the normal limbs. In two cases, there was no significant posterior displacement of the humeral head, five cases showed posterior subluxation of the humeral head, and the remaining three cases showed total luxation of the humeral head. The mean glenoid version angle of the affected limbs (90-α) was -9.6º, versus +1.6º for the normal, contralateral limbs. Conclusion The main deformities found in this study were BPBP-associated retroversion of the glenoid cavity, developmental delay of the humeral head, and posterior translation of the humeral head. PMID:27403013

  1. Balance Impairments after Brachial Plexus Injury as Assessed through Clinical and Posturographic Evaluation

    PubMed Central

    Souza, Lidiane; Lemos, Thiago; Silva, Débora C.; de Oliveira, José M.; Guedes Corrêa, José F.; Tavares, Paulo L.; Oliveira, Laura A.; Rodrigues, Erika C.; Vargas, Claudia D.

    2016-01-01

    Objective: To investigate whether a sensorimotor deficit of the upper limb following a brachial plexus injury (BPI) affects the upright balance. Design: Eleven patients with a unilateral BPI and 11 healthy subjects were recruited. The balance assessment included the Berg Balance Scale (BBS), the number of feet touches on the ground while performing a 60 s single-leg stance and posturographic assessment (eyes open and feet placed hip-width apart during a single 60 s trial). The body weight distribution (BWD) between the legs was estimated from the center of pressure (COP) lateral position. The COP variability was quantified in the anterior-posterior and lateral directions. Results: BPI patients presented lower BBS scores (p = 0.048) and a higher frequency of feet touches during the single-leg stance (p = 0.042) compared with those of the healthy subjects. An asymmetric BWD toward the side opposite the affected arm was shown by 73% of BPI patients. Finally, higher COP variability was observed in BPI patients compared with healthy subjects for anterior-posterior (p = 0.020), but not for lateral direction (p = 0.818). Conclusions: This study demonstrates that upper limb sensorimotor deficits following BPI affect body balance, serving as a warning for the clinical community about the need to prevent and treat the secondary outcomes of this condition. PMID:26834610

  2. Electrostimulation with or without ultrasound-guidance in interscalene brachial plexus block for shoulder surgery.

    PubMed

    Salem, Mohamed H; Winckelmann, Jörg; Geiger, Peter; Mehrkens, Hans-Hinrich; Salem, Khaled H

    2012-08-01

    In a prospective controlled trial to compare conventional interscalene brachial plexus block (ISBPB) using anatomic landmarks and electro-stimulation with a combined technique of ultrasound guidance followed by nerve stimulation, 60 patients were randomized into 2 matched equal groups: Group A using nerve stimulation (NS) alone and Group B using the combination of ultrasound and NS. The time to detect the plexus (3.9 ± 4 min in Group A and 3.3 ± 1.4 min in Group B) was not significantly different. We needed to reposition the needle once (n = 13) or twice (n = 4) in Group B. First-shot motor response was achieved in all but one patient in Group A; here we were only able to locate the plexus by use of ultrasound. None of the patients needed general anaesthesia. There were no significant differences between postoperative pain, motor power, or patient's satisfaction. ISBPB seems similarly effective using electro-stimulation and ultrasound if performed by experienced anesthesiologists.

  3. Perineural versus intravenous dexamethasone as adjuncts to local anaesthetic brachial plexus block for shoulder surgery.

    PubMed

    Rosenfeld, D M; Ivancic, M G; Hattrup, S J; Renfree, K J; Watkins, A R; Hentz, J G; Gorlin, A W; Spiro, J A; Trentman, T L

    2016-04-01

    This randomised, double-blind, placebo-controlled study compared the effect of perineural with intravenous dexamethasone, both administered concomitantly with interscalene brachial plexus block for shoulder surgery. Patients received 8 mg dexamethasone mixed with ropivacaine in the block injection (n = 42), 8 mg dexamethasone intravenously at the time of the block (n = 37), or intravenous saline (n = 41) at the time of the block. Perineural and intravenous dexamethasone resulted in prolonged mean (SD) duration of block to 16.9 (5.2) h and 18.2 (6.4) h, respectively, compared with 13.8 (3.8) h for saline (p = 0.001). Mean (SD) opioid consumption (morphine equivalents) during the first 24 h after postanaesthesia recovery arrival was 12.2 (9.3) mg in the perineural dexamethasone, 17.1 (15.9) mg in the intravenous dexamethasone and 24.1 (14.3) mg in the saline groups (p = 0.001). Dexamethasone via either route reduced anti-emetic use (p = 0.046). There was no effect on patient satisfaction. These results suggest that both perineural and intravenous dexamethasone are useful adjuncts to ropivacaine interscalene block, with the intravenous route preferred as this avoids the possibility of neural toxicity of dexamethasone.

  4. Automated oscillometric determination of the ankle-brachial index provides accuracy necessary for office practice.

    PubMed

    Beckman, Joshua A; Higgins, Caitlin O; Gerhard-Herman, Marie

    2006-01-01

    Peripheral arterial disease (PAD) remains underdiagnosed by primary care and cardiovascular physicians. The office-based assessment of PAD is limited by the need for specialized equipment and the time required for performance of the ankle-brachial index (ABI). We explored whether the accuracy of automated ABI measurement by oscillometry compared favorably with the gold-standard method using continuous-wave Doppler ultrasound. Consecutive patients referred to our university hospital noninvasive vascular laboratory for ABI measurement were invited for participation. Of 205 patients, 201 participated, including 55 with PAD. The ABI was measured by automated oscillometry and Doppler ultrasound. The test of trends revealed a correlation coefficient of 0.78 in the left leg and 0.78 in the right leg (P<0.01 for both). The mean ABI difference between methods was 0.04+/-0.01 and 0.06+/-0.01, respectively, in the left and right legs. The differences between the methods followed a normal distribution. Oscillometric determination of the ABI provides an accurate determination of the ABI in an outpatient population. Our findings show automated oscillometry to be a reliable and easier method of ABI measurement, lowering the barrier to incorporation of this diagnostic test into clinical practice.

  5. Ultrasound-guided supraclavicular approach for regional anesthesia of the brachial plexus.

    PubMed

    Kapral, S; Krafft, P; Eibenberger, K; Fitzgerald, R; Gosch, M; Weinstabl, C

    1994-03-01

    We prospectively studied 40 patients (ASA grades I-III) undergoing surgery of the forearm and hand, to investigate the use of ultrasonic cannula guidance for supraclavicular brachial plexus block and its effect on success rate and frequency of complications. Patients were randomized into Group S (supraclavicular paravascular approach; n = 20) and Group A (axillary approach; n = 20). Ultrasonographic study of the plexus sheath was done. After visualization of the anatomy, the plexus sheath was penetrated using a 24-gauge cannula. Plexus block was performed using 30 mL bupivacaine 0.5%. Onset of sensory and motor block of the radial, ulnar, and median nerves was recorded in 10-min intervals for 1 h. Satisfactory surgical anesthesia was attained in 95% of both groups. In Group A, 25% showed an incomplete sensory block of the musculocutaneous nerve, whereas all patients in Group S had a block of this nerve. Complete sensory block of the radial, median, and ulnar nerves was attained after an average of 40 min without a significant difference between the two groups. Because of the direct ultrasonic view of the cervical pleura, we had no cases of pneumothorax. An accidental puncture of subclavian or axillary vessels, as well as neurologic damage, was avoided in all cases. An ultrasonography-guided approach for supraclavicular block combines the safety of axillary block with the larger extent of block of the supraclavicular approach.

  6. [Percutaneous exclusion of traumatic abdominal aortic pseudoaneurysm from a brachial approach].

    PubMed

    Gamboa, Ricardo; Ríos-Méndez, Raúl E; Solernó, Raúl; Giachello, Federico; Videla-Lynch, Ángeles; Sarmiento, Ricardo A

    2012-01-01

    Abdominal aortic pseudoaneurysm (AAP) is a rare lesion, although traumatic aortic injury is described as one of the main causes; both the rupture as the surgical treatment of the defect has high morbidity and mortality. Therefore, endovascular treatment either by chemical embolization or exclusion of defect with devices has emerged as an alternative treatment. However, there are risks such as occlusion of visceral vessels near the neck of the defect, embolization material or aortic rupture. Therefore, the choice of material and method of approach should be planned carefully in each case. We report a patient who ten years after abdominal wound firearm was diagnosed with AAP 17 x 13 cm, with short neck originated close to the ostium of the celiac trunk at an acute angle with the aortic axis. We perform the exclusion of the defect with a device designed for closing atrial septal defect from the left brachial access due to the angulation of the neck defect. There were no complications. At 72 hours was granted discharge. A month later, CT scan control showed the false aneurysm of equal size and no residual flow. The monitoring to date is five months and the patient remained asymptomatic.

  7. Brain Reorganization in Patients with Brachial Plexus Injury: A Longitudinal Functional MRI Study

    PubMed Central

    Yoshikawa, Takeharu; Hayashi, Naoto; Tajiri, Yasuhito; Satake, Yoshirou; Ohtomo, Kuni

    2012-01-01

    The aim of this study is to assess plastic changes of the sensorimotor cortex (SMC) in patients with traumatic brachial plexus injury (BPI) using functional magnetic resonance imaging (fMRI). Twenty patients with traumatic BPI underwent fMRI using blood oxygen level-dependent technique with echo-planar imaging before the operation. Sixteen patients underwent their second fMRI at approximately one year after injury. The subjects performed two tasks: a flexion-extension task of the affected elbow and a task of the unaffected elbow. After activation, maps were generated, the number of significantly activated voxels in SMC contralateral to the elbow movement in the affected elbow task study (Naf) and that in the unaffected task study (Nunaf) were counted. An asymmetry index (AI) was calculated, where AI = (Naf − Nunaf)/(Naf + Nunaf). Ten healthy volunteers were also included in this fMRI study. The AI of the first fMRI of the patients with BPI was significantly lower than that of the healthy subjects (P = 0.035). The AI of the second fMRI significantly decreased compared with that of the first fMRI (P = 0.045). Brain reorganization associates with peripheral nervous changes after BPI and after operation for functional reconstruction. PMID:22623904

  8. Numerical validation of a suprasystolic brachial cuff-based method for estimating aortic pressure.

    PubMed

    Liang, Fuyou

    2014-01-01

    Central aortic pressures are better predictors of cardiovascular events than peripheral pressures. However, central aortic blood pressures cannot be measured noninvasively; for this reason, estimating aortic pressures from noninvasive measurements of peripheral pressures has been the subject of numerous studies. In the present study, a novel method was proposed to noninvasively estimate aortic pressures from the oscillometric wave of a suprasystolic brachial cuff. The errors of estimation were evaluated in relation to various cardiovascular properties using an integrated cardiovascular-cuff model. Obtained results demonstrated that the estimation errors are affected mainly by aortic stiffness. The estimation errors for aortic systolic pressure, diastolic pressure, pulse pressure and wave shape under the assumed cardiovascular conditions were 5.84 ± 1.58 mmHg, -0.28 ± 0.41 mmHg, 6.12 ± 1.42 mmHg and 1.72 ± 0.57 mmHg, respectively, all of which fell within the error ranges established by existing devices. Since the method is easy to be automated and bases the estimation fully on patient-specific information, its clinical application is promising, although further clinical studies are awaited to validate the method in vivo.

  9. [A case of brachial plexus neuropathy who presented with acute paralysis of the hand after sleep].

    PubMed

    Iijima, Makiko; Okuma, Yasuyuki; Ohizumi, Hideki; Fujishima, Kenji; Goto, Keigo; Mizuno, Yoshikuni

    2002-09-01

    We report a 46-year-old woman who presented with acute paresis of the right hand and arm. She was well until when she noted a paresis and dysesthesia in her right hand in the morning. Neurological examination revealed weakness in the muscles which were supplied by lower cervical segments, with increased deep tendon reflexes in the right arm. Allen's test and Wright's test were positive. The nerve conduction studies disclosed a reduced CMAPs more severely by right median than ulnar nerve stimulation. The frequency and amplitude of the F waves was also reduced. Needle electromyogram showed a mild neurogenic pattern in the right hand muscles. Digital subtraction angiography revealed a tapering of the subclavian artery when the right arm was abducted. She underwent decompression surgery. A remarkable improvement of the symptoms was observed after surgery. Our patient suggests that brachial plexus neuropathy should be considered in the acute paresis of the hand after sleep, and that surgical procedure would lead to a successful outcome.

  10. Reduced functional connectivity within the primary motor cortex of patients with brachial plexus injury.

    PubMed

    Fraiman, D; Miranda, M F; Erthal, F; Buur, P F; Elschot, M; Souza, L; Rombouts, S A R B; Schimmelpenninck, C A; Norris, D G; Malessy, M J A; Galves, A; Vargas, C D

    2016-01-01

    This study aims at the effects of traumatic brachial plexus lesion with root avulsions (BPA) upon the organization of the primary motor cortex (M1). Nine right-handed patients with a right BPA in whom an intercostal to musculocutaneous (ICN-MC) nerve transfer was performed had post-operative resting state fMRI scanning. The analysis of empirical functional correlations between neighboring voxels revealed faster correlation decay as a function of distance in the M1 region corresponding to the arm in BPA patients as compared to the control group. No differences between the two groups were found in the face area. We also investigated whether such larger decay in patients could be attributed to a gray matter diminution in M1. Structural imaging analysis showed no difference in gray matter density between groups. Our findings suggest that the faster decay in neighboring functional correlations without significant gray matter diminution in BPA patients could be related to a reduced activity in intrinsic horizontal connections in M1 responsible for upper limb motor synergies.

  11. Evaluation and Management of Proximal Humerus Fractures

    PubMed Central

    Khmelnitskaya, Ekaterina; Lamont, Lauren E.; Taylor, Samuel A.; Lorich, Dean G.; Dines, David M.; Dines, Joshua S.

    2012-01-01

    Proximal humerus fractures are common injuries, especially among older osteoporotic women. Restoration of function requires a thorough understanding of the neurovascular, musculotendinous, and bony anatomy. This paper addresses the relevant anatomy and highlights various management options, including indication for arthroplasty. In the vast majority of cases, proximal humerus fractures may be treated nonoperatively. In the case of displaced fractures, when surgical intervention may be pursued, numerous constructs have been investigated. Of these, the proximal humerus locking plate is the most widely used. Arthroplasty is generally reserved for comminuted 4-part fractures, head-split fractures, or fractures with significant underlying arthritic changes. Reverse total shoulder arthroplasty is reserved for patients with a deficient rotator cuff, or highly comminuted tuberosities. PMID:23316376

  12. Prognostic Factors in Amyotrophic Lateral Sclerosis: A Population-Based Study

    PubMed Central

    Moura, Mirian Conceicao; Novaes, Maria Rita Carvalho Garbi; Eduardo, Emanoel Junio; Zago, Yuri S. S. P.; Freitas, Ricardo Del Negro Barroso; Casulari, Luiz Augusto

    2015-01-01

    Objective To determine the prognostic factors associated with survival in amyotrophic lateral sclerosis at diagnosis. Methods This retrospective population-based study evaluated 218 patients treated with riluzole between 2005 and 2014 and described their clinical and demographic profiles after the analysis of clinical data and records from the mortality information system in the Federal District, Brazil. Cox multivariate regression analysis was conducted for the parameters found. Results The study sample consisted of 132 men and 86 women with a mean age at disease onset of 57.2±12.3 years; 77.6% of them were Caucasian. The mean periods between disease onset and diagnosis were 22.7 months among men and 23.5 months among women, and the mean survival periods were 45.7±47.0 months among men and 39.3±29.8 months among women. In addition, 80.3% patients presented non-bulbar-onset amyotrophic lateral sclerosis, and 19.7% presented bulbar-onset. Cox regression analysis indicated worse prognosis for body mass index (BMI) <25 kg/m2 (relative risk [RR]: 3.56, 95% confidence interval [CI]: 1.44–8.86), age >75 years (RR: 12.47, 95% CI: 3.51–44.26), and bulbar-onset (RR: 4.56, 95% CI: 2.06–10.12). Electromyography did not confirm the diagnosis in 55.6% of the suspected cases and in 27.9% of the bulbar-onset cases. Conclusions The factors associated with lower survival in amyotrophic lateral sclerosis were age >75 years, BMI <25 kg/m2, and bulbar-onset. PMID:26517122

  13. Two-Dimensional Optical Proximity Effects

    NASA Astrophysics Data System (ADS)

    Flanner, Philip D.; Subramanian, Shankar; Neureuther, Andrew R.

    1986-08-01

    In projection printing the proximity effects between adjacent two-dimensional features such as concentric elbows can be the limiting factor in designing layout rules. An aerial image simulation code based on the imaging algorithms in SAMPLE has been developed and used to investigate these proximity effects. The program accepts arbitrary polygonal shapes constructed of rectangular and triangular patches. The image is calculated using Hopkins transmission cross coefficient formulation and uses rapid integral evaluation techniques. The cpu time for this FORTRAN F77 program depends on the size of the mask and the partial coherence factor as 0.25[(1 + σ) 2A(NA/λ)2]2 seconds on a DEC VAX 11/780 using double precision, where A is the mask area, σ the coherence factor, NA the numerical aperture and λ the wavelength. The output intensity can be displayed with graphics tools such as UNIGRAFIX or cross-sectioned for input to SAMPLE development simulation along critical paths. Proximity effects in critical regions between features such as nested elbows, contacts near contacts and lines, and lines near large pads are studied. For small contacts studies show that a contact hole can be placed as close as 0.5λ/NA microns to another contact hole. For nested elbows the critical effect is the variation in intensity in the straight regions just adjacent to the corner. This undesirable variation is primarily due to the intrafeature intensity interactions and is not greatly influenced by the proximity of another nested elbow. For general feature shapes the proximity effects are reduced by increasing the partial coherence factor to 0.5 or higher but at the cost of reducing contrast and peak intensity. For contact masks a partial coherence of 0.3 is recommended for higher edge slope and peak intensities. Proximity effects of small defects are also illustrated.

  14. Strategy for photostable proximity bioassays using lanthanides

    PubMed Central

    Haushalter, Jeanne P.; Faris, Gregory W.

    2011-01-01

    We report initial findings for research aimed at creating photostable lanthanide chelate reporters for proximity assays. These reporters take advantage of the nanometer scale distance dependence of fluorescence enhancement for molecules in the vicinity of noble metal nanoparticles and also capitalize on some unique properties of lanthanide chelates. This approach promises to lead to proximity assays that do not suffer from photobleaching and offer very high on/off enhancement ratios. Results for lanthanide chelates on silver island films and in colloidal suspensions are reported. Enhancement factors range from 1 to 2 orders of magnitude, with larger enhancements for strongly quenched lanthanides. PMID:17356638

  15. Palliative Care Issues in Amyotrophic Lateral Sclerosis: An Evidenced-Based Review.

    PubMed

    Karam, Chafic Y; Paganoni, Sabrina; Joyce, Nanette; Carter, Gregory T; Bedlack, Richard

    2016-02-01

    As palliative care physicians become increasingly involved in the care of patients with amyotrophic lateral sclerosis (ALS), they will be asked to provide guidance regarding the use of supplements, diet, exercise, and other common preventive medicine interventions. Moreover, palliative care physicians have a crucial role assisting patients with ALS in addressing health care decisions to maximize quality of life and cope with a rapidly disabling disease. It is therefore important for palliative care physicians to be familiar with commonly encountered palliative care issues in ALS. This article provides an evidenced-based review of palliative care options not usually addressed in national and international ALS guidelines.

  16. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.

    PubMed

    Neumann, Manuela; Sampathu, Deepak M; Kwong, Linda K; Truax, Adam C; Micsenyi, Matthew C; Chou, Thomas T; Bruce, Jennifer; Schuck, Theresa; Grossman, Murray; Clark, Christopher M; McCluskey, Leo F; Miller, Bruce L; Masliah, Eliezer; Mackenzie, Ian R; Feldman, Howard; Feiden, Wolfgang; Kretzschmar, Hans A; Trojanowski, John Q; Lee, Virginia M-Y

    2006-10-06

    Ubiquitin-positive, tau- and alpha-synuclein-negative inclusions are hallmarks of frontotemporal lobar degeneration with ubiquitin-positive inclusions and amyotrophic lateral sclerosis. Although the identity of the ubiquitinated protein specific to either disorder was unknown, we showed that TDP-43 is the major disease protein in both disorders. Pathologic TDP-43 was hyper-phosphorylated, ubiquitinated, and cleaved to generate C-terminal fragments and was recovered only from affected central nervous system regions, including hippocampus, neocortex, and spinal cord. TDP-43 represents the common pathologic substrate linking these neurodegenerative disorders.

  17. Juvenile amyotrophic lateral sclerosis: Classical wine glass sign on magnetic resonance imaging

    PubMed Central

    Kumar, Saurabh; Aga, Pallavi; Gupta, Aakansha; Kohli, Neera

    2016-01-01

    Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig disease, is a chronic degenerative neurologic disease and is characterized by the selective involvement of the motor system. Usually, patients present with upper motor neuron (UMN) and lower motor neuron compromise. Degeneration of the UMN in the cerebral cortex is one of the main pathologic changes in ALS. These changes usually affect corticospinal tracts leading to degeneration of the fibers which show characteristic hyperintensities along the tracts leading to the “wine glass sign.” Patients with ALS usually present in the sixth decade of life; presentation in pediatric age in the form of juvenile ALS being rare. PMID:27195035

  18. [Structure of peripheral blood platelets surface in patients with amyotrophic lateral sclerosis and multiple sclerosis].

    PubMed

    Kiktenko, A I; Zlobina, G P; Brusov, O S; Zakharova, M N

    2005-01-01

    Using scanning electronic microscopy of peripheral blood platelets in 32 patients with amyotrophic lateral sclerosis (ALS) and 6 patients with multiple sclerosis (MS), platelets activation, emerging in pseudopodiums formation, aggregation and lysis, was found. Platelets activation was more pronounced in ALS than in MS. It was most markedly seen for quantitative evaluation of the above traits. The features of platelet activation found in the study support the evidence for considering ALS as a systemic disorder being characterized by a broad spectrum of alterations of biological processes.

  19. Amyotrophic Lateral Sclerosis and Organ Donation: Is There Risk of Disease Transmission?

    PubMed Central

    Holmes, Brandon B.; Diamond, Marc I.

    2013-01-01

    A new protocol suggests that patients with amyotrophic lateral sclerosis (ALS) are a viable source of tissue for organ transplantation. However, multiple lines of evidence suggest that many neurodegenerative diseases, including ALS, might progress due to transcellular propagation of protein aggregation among neurons. Transmission of the disease state from donor to host thus may be possible under the permissive circumstances of graft transplantation. We argue for careful patient selection and close longitudinal follow-up of recipients when harvesting organs from individuals with neurodegenerative disease, especially dominantly inherited forms. PMID:23280834

  20. Depression and disease progression in amyotrophic lateral sclerosis: A comprehensive meta-regression analysis.

    PubMed

    Pagnini, Francesco; Manzoni, Gian Mauro; Tagliaferri, Aurora; Gibbons, Chris J

    2015-08-01

    Depression in people with amyotrophic lateral sclerosis, a fatal and progressive neurodegenerative disorder, is a serious issue with important clinical consequences. However, physical impairment may confound the diagnosis when using generic questionnaires. We conducted a comprehensive review of literature. Mean scores from depression questionnaires were meta-regressed on study-level mean time since onset of symptoms. Data from 103 studies (3190 subjects) indicate that the Beck Depression Inventory and, to a lesser degree, the Hospital Anxiety and Depression Scale are influenced by the time since symptom onset, strongly related to physical impairment. Our results suggest that widely used depression scales overestimate depression due to confounding with physical symptoms.

  1. Evidence for a dopaminergic deficit in sporadic amyotrophic lateral sclerosis on positron emission scanning

    SciTech Connect

    Takahashi, Hirohide; Snow, B.J.; Bhatt, M.H.; Peppard, R.; Eisen, A.; Calne, D.B. )

    1993-10-23

    Although rare, the chronic neurodegenerative disorders amyotrophic lateral sclerosis (ALS) and idiopathic parkinsonism coexist to a greater degree than expected by chance. This suggests that patients with ALS may have subclinical lesions of the nigrostriatal dopaminergic pathway. To study this hypothesis, the authors did positron emission tomography with 6-fluorodopa on 16 patients with sporadic ALS and without extrapyramidal disease, and compared the results with age-matched controls. They found a significant progressive fall in 6-fluorodopa uptake with time since diagnosis, and reduced dopaminergic function in 3 patients with ALS of long duration. This supports the hypothesis that ALS and IP may share pathogenesis, and, perhaps, etiology.

  2. Intraneuronal aluminum accumulation in amyotrophic lateral sclerosis and Parkinsonism-dementia of Guam

    SciTech Connect

    Perl, D.P.; Gajdusek, D.C.; Garruto, R.M.; Yanagihara, R.T.; Gibbs, C.J.

    1982-09-10

    Scanning electron microscopy with energy-dispersive x-ray spectrometry was used to analyze the elemental content of neurofibrillary tangle (NFT)-bearing and NFT-free neurons within the Sommer's sector (H1 region) of the hippocampus in Guamanian Chamorros with amyotrophic lateral sclerosis and parkinsonism-dementia and in neurologically normal controls. Preliminary data indicate prominent accumulation of aluminum within the nuclear region and perikaryal cytoplasm of NFT-bearing hippocampal neurons, regardless of the underlying neurological diagnosis. These findings further extend the association between intraneuronal aluminum and NFT formation and support the hypothesis that environmental factors are related to the neurodegenerative changes seen in the Chamorro population.

  3. Autoimmune-like hepatitis during masitinib therapy in an amyotrophic lateral sclerosis patient

    PubMed Central

    Salvado, Maria; Vargas, Victor; Vidal, Marta; Simon-Talero, Macarena; Camacho, Jessica; Gamez, Josep

    2015-01-01

    We report a case of acute severe hepatitis resulting from masitinib in a young amyotrophic lateral sclerosis patient. Hepatotoxicity induced by masitinib, a tyrosine kinase inhibitor, is usually transient with mild elevation of transaminases, although acute hepatitis has been not reported to date. The hepatitis was resolved after masitinib was discontinued and a combination of prednisone and azathioprine was started. The transaminases returned to baseline normal values five months later. This is the first case in the hepatitis literature associated with masitinib. The autoimmune role of this drug-induced liver injury is discussed. Physicians should be aware of this potential complication. PMID:26420975

  4. Application of botulinum toxin to treat sialorrhea in amyotrophic lateral sclerosis patients: a literature review.

    PubMed

    Oliveira, Ademar Francisco de; Silva, Gêssyca Adryene de Menezes; Almeida, Débora Milenna Xavier

    2016-01-01

    Amyotrophic lateral sclerosis is a progressive and fatal neurodegenerative disease characterized by the degeneration of motor neurons, which are the central nervous system cells that control voluntary muscle movements. The excessive salivation (sialorrhea) is present in approximately 50% of amyotrophic lateral sclerosis cases. Thus, some alternative therapeutic methods are sought, such as anticholinergic drugs and surgery. Recently the use of botulinum toxin applied at a midpoint of the salivary glands, often guided by ultrasound, have demonstrated positive results. The objective was to review the literature to demonstrate an alternative method to treatments of sialorrhea in patients with amyotrophic lateral sclerosis. In recent studies, the efficacy of botulinum toxin is confirmed, although new applications are required. Since the side effects are negligible, this is an alternative to treat amyotrophic lateral sclerosis, and other patients with diseases that present sialorrhea. RESUMO Esclerose lateral amiotrófica é uma doença neurodegenerativa progressiva e fatal, caracterizada pela degeneração dos neurônios motores, as células do sistema nervoso central que controlam os movimentos voluntários dos músculos. A salivação excessiva (sialorreia) está presente em cerca de 50% dos casos de esclerose lateral amiotrófica. Dessa forma, surgem medidas terapêuticas alternativas como drogas anticolinérgicas e cirurgia, e recentemente, o uso da toxina botulínica, aplicada em um ponto central das glândulas salivares, muitas vezes guiado por ultrassonografia, demostrou resultados positivos. Objetivou-se revisar a literatura no intuito de demonstrar um método alternativo aos tratamentos de sialorreia em pacientes com esclerose lateral amiotrófica. Em estudos recentes, a eficácia do tratamento com toxina botulínica foi confirmada e, mesmo requerendo novas aplicações, os efeitos colaterais são ínfimos. Ela surge então como alternativa não só ao

  5. Palliative Care Issues in Amyotrophic Lateral Sclerosis: An Evidenced-Based Review

    PubMed Central

    Karam, Chafic Y.; Paganoni, Sabrina; Joyce, Nanette; Carter, Gregory T.; Bedlack, Richard

    2015-01-01

    As palliative care physicians become increasingly involved in the care of patients with amyotrophic lateral sclerosis (ALS), they will be asked to provide guidance regarding the use of supplements, diet, exercise, and other common preventive medicine interventions. Moreover, palliative care physicians have a crucial role assisting patients with ALS in addressing health care decisions to maximize quality of life and cope with a rapidly disabling disease. It is therefore important for palliative care physicians to be familiar with commonly encountered palliative care issues in ALS. This article provides an evidenced-based review of palliative care options not usually addressed in national and international ALS guidelines. PMID:25202033

  6. The expanding syndrome of amyotrophic lateral sclerosis: a clinical and molecular odyssey

    PubMed Central

    Turner, Martin R; Swash, Michael

    2015-01-01

    Recent advances in understanding amyotrophic lateral sclerosis (ALS) have delivered new questions. Disappointingly, the initial enthusiasm for transgenic mouse models of the disease has not been followed by rapid advances in therapy or prevention. Monogenic models may have inadvertently masked the true complexity of the human disease. ALS has evolved into a multisystem disorder, involving a final common pathway accessible via multiple upstream aetiological tributaries. Nonetheless, there is a common clinical core to ALS, as clear today as it was to Charcot and others. We stress the continuing relevance of clinical observations amid the increasing molecular complexity of ALS. PMID:25644224

  7. Cronobacter sakazakii DNA Detection in Cerebrospinal Fluid of a Patient with Amyotrophic Lateral Sclerosis Mimic Syndrome

    PubMed Central

    Piombo, Marianna; Chiarello, Daniela; Corbetto, Marzia; Di Pino, Giovanni; Dicuonzo, Giordano; Angeletti, Silvia; Riva, Elisabetta; De Florio, Lucia; Capone, Fioravante; Di Lazzaro, Vincenzo

    2015-01-01

    A 45-year-old male noticed progressive weakness of the right lower limb with gait disturbance. Over the following months, motor deficits worsened, spreading to the right upper limb. Electromyography showed active denervation in the upper and lower limb muscles. A diagnosis of amyotrophic lateral sclerosis (ALS) was made. About 2 years after symptom onset, gradual improvement occurred. Cerebrospinal fluid analysis performed about 3 years after the beginning of symptoms identified Cronobacter sakazakii. Since no other possible causes were identified, we suggest that an almost completely reversible ALS-like syndrome had been triggered by Cronobacter infection in our immunocompetent patient. PMID:26955334

  8. Motor unit changes in thoracic paraspinal muscles in amyotrophic lateral sclerosis.

    PubMed

    de Carvalho, Mamede; Pinto, Susana; Swash, Michael

    2009-01-01

    In 38 amyotrophic lateral sclerosis (ALS) patients and 28 controls, we performed motor unit potential (MUP) analysis in the C-6 and T-5 paraspinal and biceps muscles. In ALS cases, we found similar abnormalities in MUPs in paraspinal and limb muscles. Fasciculation potentials (FPs) were more frequent in biceps than in paraspinal muscles, but fibrillation potentials and positive sharp waves (fibs-sw) were equally frequent in all three muscles. These results confirm the value of paraspinal MUP analysis in the diagnosis of ALS.

  9. A Systematic Review of Outcomes of Contralateral C7 for the Treatment of Traumatic Brachial Plexus Injury: Part 1-Overall outcomes of contralateral C7 transfer for traumatic brachial plexus injury

    PubMed Central

    Yang, Guang; Chang, Kate W.-C.; Chung, Kevin C.

    2015-01-01

    Background Contralateral C7 (CC7) transfer has been used for treating traumatic brachial plexus injury. However, the effectiveness of CC7 transfer remains a subject of debate. We performed a systematic review to study the overall outcomes of CC7 transfer to different recipient nerves in traumatic brachial plexus injuries. Methods A literature search was conducted using PubMed and EMBASE databases to identify original articles related to CC7 transfer for traumatic brachial plexus injury. The data extracted were study/ patient characteristics, and objective outcomes of CC7 transfer to the recipient nerves. We normalized modifications of MRC and other outcome measures into an MRC-based outcome scale for comparisons. Results Thirty-nine studies were identified. The outcomes were categorized based on the three major recipient nerves: median, musculocutaneous, and radial/triceps nerves. Regarding overall functional recovery, 11% of patients achieved MRC grade M4 wrist flexion and 38% achieved M3. Grade M4 finger flexion was achieved by 7% of patients whereas 36% achieved M3. Finally, 56% of patients achieved ≥S3 sensory recovery in the median nerve territories. In the musculocutaneous nerve group, 38% of patients regained elbow flexor strength to M4 and 37% regained to M3. In the radial/triceps nerve group, 25% regained elbow or wrist extension strength to an MRC grade M4 and 25% regained to M3. Conclusions Outcome measures in the included studies were not consistently reported to uncover true patient-related benefits from the CC7 transfer. Reliable and validated outcome instruments should be applied to critically evaluate patients undergoing CC7 transfer. PMID:26397253

  10. [Proximity and breastfeeding at the maternity hospital].

    PubMed

    Fradin-Charrier, Anne-Claire

    2015-01-01

    The establishment of breastfeeding, as well as its duration, are facilitated through the proximity of the mother with her new baby. However, in maternity hospitals, breastfeeding mothers very often leave their baby in the nursery at night time. A study carried out in 2014 in several maternity hospitals put forward suggestions and highlighted areas to improve in everyday practice.

  11. Vortices in normal part of proximity system

    DOE PAGES

    Kogan, V. G.

    2015-05-26

    It is shown that the order parameter Δ induced in the normal part of superconductor-normal-superconductor proximity system is modulated in the magnetic field differently from vortices in bulk superconductors. Whereas Δ turns zero at vortex centers, the magnetic structure of these vortices differs from that of Abrikosov's.

  12. Current status of proximal gastric vagotomy.

    PubMed Central

    Schirmer, B D

    1989-01-01

    Proximal gastric vagotomy is nearing its twentieth year in clinical use as an operation for peptic ulcer disease. No other acid-reducing operation has undergone as much scrutiny or study. At this time, the evidence of such studies and long-term follow-up strongly supports the use of proximal gastric vagotomy as the treatment of choice for chronic duodenal ulcer in patients who have failed medical therapy. Its application in treating the complications of peptic ulcer disease, which recently have come to represent an increasingly greater percentage of all operations done for peptic ulcer disease, is well-tested. However, initial series suggest that it should probably occupy a prominent role in treating some of these complications, particularly in selected patients, in the future. The operation has the well-documented ability to reduce gastric acid production, not inhibit gastric bicarbonate production, and also minimally inhibit gastric motility. The combination of these physiologic results after proximal gastric vagotomy, along with preservation of the normal antropyloroduodenal mechanism of gastrointestinal control, serve to allow patients with proximal gastric vagotomy the improved benefits of significantly fewer severe gastrointestinal side effects than are seen after other operations for peptic ulcer disease. PMID:2644897

  13. Encoding Direction when Interpreting Proximal Terms

    ERIC Educational Resources Information Center

    Ashley, Aaron; Carlson, Laura A.

    2007-01-01

    The location of an object is often described by spatially relating it to a known landmark. The spatial terms used in such descriptions can provide various types of information. For example, projective terms such as "above" indicate direction but not distance, whereas proximal terms such as "near" indicate distance but not direction. Previous…

  14. Object detection system using SPAD proximity detectors

    NASA Astrophysics Data System (ADS)

    Stark, Laurence; Raynor, Jeffrey M.; Henderson, Robert K.

    2011-10-01

    This paper presents an object detection system based upon the use of multiple single photon avalanche diode (SPAD) proximity sensors operating upon the time-of-flight (ToF) principle, whereby the co-ordinates of a target object in a coordinate system relative to the assembly are calculated. The system is similar to a touch screen system in form and operation except that the lack of requirement of a physical sensing surface provides a novel advantage over most existing touch screen technologies. The sensors are controlled by FPGA-based firmware and each proximity sensor in the system measures the range from the sensor to the target object. A software algorithm is implemented to calculate the x-y coordinates of the target object based on the distance measurements from at least two separate sensors and the known relative positions of these sensors. Existing proximity sensors were capable of determining the distance to an object with centimetric accuracy and were modified to obtain a wide field of view in the x-y axes with low beam angle in z in order to provide a detection area as large as possible. Design and implementation of the firmware, electronic hardware, mechanics and optics are covered in the paper. Possible future work would include characterisation with alternative designs of proximity sensors, as this is the component which determines the highest achievable accur1acy of the system.

  15. Laparoscopic Proximal Gastrectomy With Gastric Tube Reconstruction

    PubMed Central

    Shiraishi, Norio; Toujigamori, Manabu; Shiroshita, Hidefumi; Etoh, Tsuyoshi; Inomata, Masafumi

    2016-01-01

    Background and Objectives: There is no standardized method of reconstruction in laparoscopic proximal gastrectomy (LPG). We present a novel technique of reconstruction with a long, narrow gastric tube in LPG for early gastric cancer (EGC). Methods: During the laparoscopic procedure, the upper part of the stomach is fully mobilized with perigastric and suprapancreatic lymphadenectomy, and then the abdominal esophagus is transected. After a minilaparotomy is created, the entire stomach is pulled outside. A long, narrow gastric tube (20 cm long, 3 cm wide) is created with a linear stapler. The proximal part of the gastric tube is formed into a cobra head shape for esophagogastric tube anastomosis, which is then performed with a 45-mm linear stapler under laparoscopic view. The end of the esophagus is fixed on the gastric tube to prevent postoperative esophageal reflux. Results: Thirteen patients with early proximal gastric cancer underwent the procedure. The mean operative time was 283 min, and median blood loss was 63 ml. There were no conversions to open surgery, and no intraoperative complications. Conclusion: This new technique of reconstruction after LPG is simple and feasible. The procedure has the potential of becoming a standard reconstruction technique after LPG for proximal EGC. PMID:27547027

  16. Goal-Proximity Decision-Making

    ERIC Educational Resources Information Center

    Veksler, Vladislav D.; Gray, Wayne D.; Schoelles, Michael J.

    2013-01-01

    Reinforcement learning (RL) models of decision-making cannot account for human decisions in the absence of prior reward or punishment. We propose a mechanism for choosing among available options based on goal-option association strengths, where association strengths between objects represent previously experienced object proximity. The proposed…

  17. Proximity correction for electron beam lithography

    NASA Astrophysics Data System (ADS)

    Marrian, Christie R.; Chang, Steven; Peckerar, Martin C.

    1996-09-01

    As the critical dimensions required in mask making and direct write by electron beam lithography become ever smaller, correction for proximity effects becomes increasingly important. Furthermore, the problem is beset by the fact that only a positive energy dose can be applied with an electron beam. We discuss techniques such as chopping and dose shifting, which have been proposed to meet the positivity requirement. An alternative approach is to treat proximity correction as an optimization problem. Two such methods, local area dose correction and optimization using a regularizer proportional to the informational entropy of the solution, are compared. A notable feature of the regularized proximity correction is the ability to correct for forward scattering by the generation of a 'firewall' set back from the edge of a feature. As the forward scattering width increases, the firewall is set back farther from the feature edge. The regularized optimization algorithm is computationally time consuming using conventional techniques. However, the algorithm lends itself to a microelectronics integrated circuit coprocessor implementation, which could perform the optimization faster than even the fastest work stations. Scaling the circuit to larger number of pixels is best approached with a hybrid serial/parallel digital architecture that would correct for proximity effects over 108 pixels in about 1 h. This time can be reduced by simply adding additional coprocessors.

  18. Proximity correction for e-beam lithography

    NASA Astrophysics Data System (ADS)

    Marrian, Christie R.; Chang, Steven; Peckerar, Martin C.

    1995-12-01

    As the critical dimensions required for masks and e-beam direct write become ever smaller, the correction of proximity effects becomes more necessary. Furthermore, the problem is beset by the fact that only a positive energy dose can be applied with the e-beam. We discuss here approaches such as chopping and dose shifting which have been proposed to meet the positivity requirement. An alternative approach is to treat proximity correction as an optimization problem. Two such methods, local area dose correction and optimization using a regularizer proportional to the informational entropy of the solution, are compared. A notable feature of the regularized proximity correction is the ability to correct for forward scattering by the generation of a 'firewall' set back from the edge of a feature. As the forward scattering width increases, the firewall is set back further from the feature edge. The regularized optimization algorithm is computationally time consuming using conventional techniques. However, the algorithm lends itself to a microelectronics integrated circuit coprocessor implementation which could perform the optimization much faster than even the fastest work stations. Scaling the circuit to larger number of pixels is best approached with a hybrid serial/parallel digital architecture which would correct for proximity effects over 108 pixels about one hour. This time can be reduced by simply adding additional coprocessors.

  19. Anatomical architecture of the brachial plexus in the common hippopotamus (Hippopotamus amphibius) with special reference to the derivation and course of its unique branches.

    PubMed

    Yoshitomi, S; Kawashima, T; Murakami, K; Takayanagi, M; Inoue, Y; Aoyagi, R; Sato, F

    2012-08-01

    The anatomy of the brachial plexus in the common hippopotamus (Hippopotamus amphibius), which has not been previously reported, was first examined bilaterally in a newborn hippopotamus. Our observations clarified the following: (1) the brachial plexus comprises the fifth cervical (C5) to first thoracic (T1) nerves. These formed two trunks, C5-C6 and C7-T1; in addition, the axillary artery passed in between C6 and C7, (2) unique branches to the brachialis muscle and those of the lateral cutaneous antebrachii nerves ramified from the median nerve, (3) nerve fibre analysis revealed that these unique nerve branches from the median nerve were closely related and structurally similar to the musculocutaneous (MC) nerve; however, they had changed course from the MC to the median nerve, and (4) this unique branching pattern is likely to be a common morphological feature of the brachial plexus in amphibians, reptiles and certain mammals.

  20. Degree of Contracture Related to Residual Muscle Shoulder Strength in Children with Obstetric Brachial Plexus Lesions

    PubMed Central

    van Gelein Vitringa, Valerie M.; van Noort, Arthur; Ritt, Marco J. P. F.; van Royen, Barend J.; van der Sluijs, Johannes A.

    2015-01-01

    Background and Objectives  Little is known about the relation between residual muscle strength and joint contracture formation in neuromuscular disorders. This study aimed to investigate the relation between residual muscle strength and shoulder joint contractures in children with sequelae of obstetric brachial plexus lesion (OBPL). In OBPL a shoulder joint contracture is a frequent finding. We hypothesize that residual internal and external rotator strength and their balance are related to the extent of shoulder joint contracture. Methods  Clinical assessment was performed in 34 children (mean 10.0 years) with unilateral OBPL and Narakas classes I–III. External and internal rotation strengths were measured with the shoulder in neutral position using a handheld dynamometer. Strength on the affected side was given as percentage of the normal side. Contracture was assessed by passive internal and external rotations in degrees (in 0° abduction). Mallet classification was used for active shoulder function. Results  External and internal rotation strengths on the affected side were approximately 50% of the normal side and on average both equally affected: 56% (SD 18%) respectively 51% (SD 27%); r = 0.600, p = 0.000. Residual strengths were not related to passive internal or external rotation (p > 0.200). Internal rotation strength (r =  − 0.425, p <0.05) was related to Narakas class. Mallet score was related to external and internal rotation strengths (r = 0.451 and r = 0.515, respectively; p < 0.01). Conclusion  The intuitive notion that imbalances in residual muscle strength influence contracture formation cannot be confirmed in this study. Our results are of interest for the understanding of contracture formation in OBPL. PMID:27917235