A Profile of Neuro-Ophthalmic Practice Around the World.

PubMed

Frohman, Larry P

2018-03-01

To compare contrast neuro-ophthalmic practice in various countries, an 18-question survey was sent to the international North American Neuro-Ophthalmology Society (NANOS) members in the spring of 2016. At least 1 NANOS member was contacted for each non-US nation in the NANOS membership roster. If there were multiple NANOS members from 1 country, multiple were contacted. If responses were received from more than 1 person from a single country, the first response received was used as the source data. The survey (in English) was emailed to 47 NANOS members from 31 countries. Twenty responses were received representing members from 15 nations. In all 15 nations, at least half of the neuro-ophthalmologists were trained as ophthalmologists. In 60% of nations, at least half of the neuro-ophthalmologists were trained internally, whereas in 33% of countries, at least half were trained in the United States. The number of physicians who practiced a significant amount of neuro-ophthalmology ranged from low (0.08/million, India) to high (3.10/million, Israel). Countries having the highest percentage of neuro-ophthalmologists exclusively practicing neuro-ophthalmology also were those with better patient access to neuro-ophthalmic care. Requirement of approval to see a neuro-ophthalmologist or for imaging studies requested by neuro-ophthalmologists was not typical. In most nations, academic neuro-ophthalmologists were paid a straight salary. In no nation were neuro-ophthalmologists paid more than another ophthalmic subspecialty. Individual national health care system designs and compensation models have had a profound influence on the rewards and challenges that face neuro-ophthalmologists. There seems to have been a connection between recognition of the discipline, financial rewards of neuro-ophthalmic practice, conditions that permit full-time neuro-ophthalmic practice, and patient access to care. A higher percentage of gross national product for health care did not seem to

Frequency and Pathological Phenotype of Bovine Astrovirus CH13/NeuroS1 Infection in Neurologically-Diseased Cattle: Towards Assessment of Causality.

PubMed

Selimovic-Hamza, Senija; Boujon, Céline L; Hilbe, Monika; Oevermann, Anna; Seuberlich, Torsten

2017-01-18

Next-generation sequencing (NGS) has opened up the possibility of detecting new viruses in unresolved diseases. Recently, astrovirus brain infections have been identified in neurologically diseased humans and animals by NGS, among them bovine astrovirus (BoAstV) CH13/NeuroS1, which has been found in brain tissues of cattle with non-suppurative encephalitis. Only a few studies are available on neurotropic astroviruses and a causal relationship between BoAstV CH13/NeuroS1 infections and neurological disease has been postulated, but remains unproven. Aiming at making a step forward towards assessing the causality, we collected brain samples of 97 cases of cattle diagnosed with unresolved non-suppurative encephalitis, and analyzed them by in situ hybridization and immunohistochemistry, to determine the frequency and neuropathological distribution of the BoAstV CH13/NeuroS1 and its topographical correlation to the pathology. We detected BoAstV CH13/NeuroS1 RNA or proteins in neurons throughout all parts of the central nervous system (CNS) in 34% of all cases, but none were detected in cattle of the control group. In general, brain lesions had a high correlation with the presence of the virus. These findings show that a substantial proportion of cattle with non-suppurative encephalitis are infected with BoAstV CH13/NeuroS1 and further substantiate the causal relationship between neurological disease and astrovirus infections.

Frequency and Pathological Phenotype of Bovine Astrovirus CH13/NeuroS1 Infection in Neurologically-Diseased Cattle: Towards Assessment of Causality

PubMed Central

Selimovic-Hamza, Senija; Boujon, Céline L.; Hilbe, Monika; Oevermann, Anna; Seuberlich, Torsten

2017-01-01

Next-generation sequencing (NGS) has opened up the possibility of detecting new viruses in unresolved diseases. Recently, astrovirus brain infections have been identified in neurologically diseased humans and animals by NGS, among them bovine astrovirus (BoAstV) CH13/NeuroS1, which has been found in brain tissues of cattle with non-suppurative encephalitis. Only a few studies are available on neurotropic astroviruses and a causal relationship between BoAstV CH13/NeuroS1 infections and neurological disease has been postulated, but remains unproven. Aiming at making a step forward towards assessing the causality, we collected brain samples of 97 cases of cattle diagnosed with unresolved non-suppurative encephalitis, and analyzed them by in situ hybridization and immunohistochemistry, to determine the frequency and neuropathological distribution of the BoAstV CH13/NeuroS1 and its topographical correlation to the pathology. We detected BoAstV CH13/NeuroS1 RNA or proteins in neurons throughout all parts of the central nervous system (CNS) in 34% of all cases, but none were detected in cattle of the control group. In general, brain lesions had a high correlation with the presence of the virus. These findings show that a substantial proportion of cattle with non-suppurative encephalitis are infected with BoAstV CH13/NeuroS1 and further substantiate the causal relationship between neurological disease and astrovirus infections. PMID:28106800

Co-incidence of Turner syndrome and Duchenne muscular dystrophy - an important problem for the clinician.

PubMed

Kaczorowska, Ewa; Zimowski, Janusz; Cichoń-Kotek, Monika; Mrozińska, Agnieszka; Purzycka, Joanna; Wierzba, Jolanta; Limon, Janusz; Lipska-Ziętkiewicz, Beata S

Turner syndrome is a relatively common chromosomal disorder which affects about one in 2000 live born females. Duchenne muscular dystrophy is an X-linked recessive disorder affecting 1:3600 live born males. Considering the above, the coexistence of these two diseases may occur only anecdotally. Here, we report a 4 ½ year-old female with classical 45,X Turner syndrome who also had Duchenne muscular dystrophy caused by a point mutation in the dystrophin gene (c.9055delG). The patient showed the typical phenotype of Turner syndrome including distinctive dysmorphic features (short neck, low posterior hairline, wide position of nipples), aortic coarctation and feet lymphedema. Besides, she presented with an unusually early beginning of muscular dystrophy symptoms with infantile-onset motor developmental delay, intellectual disability and early calf muscular hypertrophy. The coexistence of an X-linked recessive disorder should be considered in women affected by Turner syndrome presenting with additional atypical clinical features.

A Neuro-Musculo-Skeletal Model for Insects With Data-driven Optimization.

PubMed

Guo, Shihui; Lin, Juncong; Wöhrl, Toni; Liao, Minghong

2018-02-01

Simulating the locomotion of insects is beneficial to many areas such as experimental biology, computer animation and robotics. This work proposes a neuro-musculo-skeletal model, which integrates the biological inspirations from real insects and reproduces the gait pattern on virtual insects. The neural system is a network of spiking neurons, whose spiking patterns are controlled by the input currents. The spiking pattern provides a uniform representation of sensory information, high-level commands and control strategy. The muscle models are designed following the characteristic Hill-type muscle with customized force-length and force-velocity relationships. The model parameters, including both the neural and muscular components, are optimized via an approach of evolutionary optimization, with the data captured from real insects. The results show that the simulated gait pattern, including joint trajectories, matches the experimental data collected from real ants walking in the free mode. The simulated character is capable of moving at different directions and traversing uneven terrains.

Diffusion and imaging properties of three new lipophilic tracers, NeuroVue ™ Maroon, NeuroVue ™ Red and NeuroVue ™ Green and their use for double and triple labeling of neuronal profile.

PubMed Central

Fritzsch, B.; Muirhead, K.A.; Feng, Feng; D.Gray, B.; Ohlsson-Wilhelm, B. M.

2006-01-01

We describe here diffusion and imaging properties of three new lipophilic tracers, NeuroVue ™ Maroon (near infrared), NeuroVue ™ Red and NeuroVue ™ Green. Using pair wise comparisons between the new dyes and existing dyes (DiI, DiA, DiD, DiO, PKH2, PKH26) applied to the left and the right side of fixed spinal cord preparations, we show that NeuroVue Maroon (excitation max 647 nm) surpasses all other dyes in this study in signal to noise ratio. We also present data showing the utility of these new dyes for both double labeling and triple labeling in combination with each other or existing lipophilic tracers. Using mice bearing the PLP-eGFP transgene, we demonstrate that either NeuroVue Maroon or NeuroVue Red can readily be combined with eGFP labeling. Double labeling experiments using NeuroVue Red and eGFP allowed us to demonstrate that every fiber in the neonatal ear is surrounded by developing Schwann cells. PMID:16023922

Myoglobin in Primary Muscular Disease: I. Duchenne Muscular Dystrophy: and: II. Muscular Dystrophy of Distal Type

PubMed Central

Romero-Herrera, A. E.; Lehmann, H.; Tomlinson, B. E.; Walton, J. N.

1973-01-01

Skeletal myoglobin from two cases of muscular dystrophy, one of Duchenne muscular dystrophy, and one of muscular dystrophy of distal type, have been examined and no differences from normal human myoglobin were found. The opportunity has been taken to discuss the nature of minor fractions of myoglobin-like material which are found when human skeletal myoglobin is isolated. Those which have been observed in the present study have been artefacts and it was possible to demonstrate that they were due to deamidation of certain glutamine and asparagine residues. Images PMID:4590363

A neuro-immune, neuro-oxidative and neuro-nitrosative model of prenatal and postpartum depression.

PubMed

Roomruangwong, Chutima; Anderson, George; Berk, Michael; Stoyanov, Drozdstoy; Carvalho, André F; Maes, Michael

2018-02-02

A large body of evidence indicates that major affective disorders are accompanied by activated neuro-immune, neuro-oxidative and neuro-nitrosative stress (IO&NS) pathways. Postpartum depression is predicted by end of term prenatal depressive symptoms whilst a lifetime history of mood disorders appears to increase the risk for both prenatal and postpartum depression. This review provides a critical appraisal of available evidence linking IO&NS pathways to prenatal and postpartum depression. The electronic databases Google Scholar, PubMed and Scopus were sources for this narrative review focusing on keywords, including perinatal depression, (auto)immune, inflammation, oxidative, nitric oxide, nitrosative, tryptophan catabolites (TRYCATs), kynurenine, leaky gut and microbiome. Prenatal depressive symptoms are associated with exaggerated pregnancy-specific changes in IO&NS pathways, including increased C-reactive protein, advanced oxidation protein products and nitric oxide metabolites, lowered antioxidant levels, such as zinc, as well as lowered regulatory IgM-mediated autoimmune responses. The latter pathways coupled with lowered levels of endogenous anti-inflammatory compounds, including ω3 polyunsaturated fatty acids, may also underpin the pathophysiology of postpartum depression. Although increased bacterial translocation, lipid peroxidation and TRYCAT pathway activation play a role in mood disorders, similar changes do not appear to be relevant in perinatal depression. Some IO&NS biomarker characteristics of mood disorders are found in prenatal depression indicating that these pathways partly contribute to the association of a lifetime history of mood disorders and perinatal depression. However, available evidence suggests that some IO&NS pathways differ significantly between perinatal depression and mood disorders in general. This review provides a new IO&NS model of prenatal and postpartum depression. Copyright © 2017 Elsevier Inc. All rights reserved.

[To strengthen the education on basic knowledge and skills of neuro-ophthalmology].

PubMed

Zhang, Xiao-jun; Wang, Ning-li

2011-12-01

Basic knowledge and skills are cornerstone of the diagnosis and treatment of neuro-ophthalmology diseases in ophthalmology practice. Due to the interdisciplinary features of neuro-ophthalmology, neuro-anatomy, neuro-physiology related to eyes, neuro-image and neuro-electrodiagnosis, these should be included in the education for the ophthalmologist. Special attention should be paid to training on capability of logically thinking in neuro-ophthalmology. Multiple ways can be used for the education of ophthalmologists and neurologists for the enhancement of basic knowledge and skills of neuro-ophthalmology in China.

Evaluation of Limb-Girdle Muscular Dystrophy

ClinicalTrials.gov

2014-03-06

Becker Muscular Dystrophy; Limb-Girdle Muscular Dystrophy, Type 2A (Calpain-3 Deficiency); Limb-Girdle Muscular Dystrophy, Type 2B (Miyoshi Myopathy, Dysferlin Deficiency); Limb-Girdle Muscular Dystrophy, Type 2I (FKRP-deficiency)

Spinal Muscular Atrophy With Respiratory Distress Type 1-A Child With Atypical Presentation.

PubMed

Chiu, Annie Ting Gee; Chan, Sophelia Hoi Shan; Wu, Shun Ping; Ting, Shun Hin; Chung, Brian Hon Yin; Chan, Angel On Kei; Wong, Virginia Chun Nei

2018-01-01

The authors report a child with spinal muscular atrophy with respiratory distress type 1 (SMARD1). She presented atypically with hypothyroidism and heart failure due to septal defects that required early heart surgery and microcephaly in association with cerebral atrophy and thin corpus collosum. The subsequent asymmetrical onset of diaphragmatic paralysis, persistent hypotonia, and generalized muscle weakness led to the suspicion of spinal muscular atrophy with respiratory distress type 1. Sanger sequencing confirmed a compound heterozygous mutation in the Immunoglobulin Mu Binding Protein 2 (IGHMBP2) gene, with a known mutation c.2362C > T (p.Arg788*) and a novel frameshift mutation c.2048delG (p.Gly683A1afs*50). Serial nerve conduction study and electromyography confirmed progressive sensorimotor polyneuropathy and neuronopathy. In summary, this case report describes a child with spinal muscular atrophy with respiratory distress type 1 also with congenital cardiac disease and endocrine dysfunction, expanding the phenotypic spectrum of this condition. A high index of suspicion is needed in diagnosing this rare condition to guide the management and genetic counseling.

A novel FLNC frameshift and an OBSCN variant in a family with distal muscular dystrophy

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

Rossi, Daniela; Palmio, Johanna; Evilä, Anni

A novel FLNC c.5161delG (p.Gly1722ValfsTer61) mutation was identified in two members of a French family affected by distal myopathy and in one healthy relative. This FLNC c.5161delG mutation is one nucleotide away from a previously reported FLNC mutation (c.5160delC) that was identified in patients and in asymptomatic carriers of three Bulgarian families with distal muscular dystrophy, indicating a low penetrance of the FLNC frameshift mutations. Given these similarities, we believe that the two FLNC mutations alone can be causative of distal myopathy without full penetrance. Moreover, comparative analysis of the clinical manifestations indicates that patients of the French family showmore » an earlier onset and a complete segregation of the disease. As a possible explanation of this, the two French patients also carry a OBSCN c.13330C>T (p.Arg4444Trp) mutation. The p.Arg4444Trp variant is localized within the OBSCN Ig59 domain that, together with Ig58, binds to the ZIg9/ZIg10 domains of titin at Z-disks. Structural and functional studies indicate that this OBSCN p.Arg4444Trp mutation decreases titin binding by ~15-fold. On this line, we suggest that the combination of the OBSCN p.Arg4444Trp variant and of the FLNC c.5161delG mutation, can cooperatively affect myofibril stability and increase the penetrance of muscular dystrophy in the French family.« less

Randomized, multicenter, comparative study of NEURO versus CIMT in poststroke patients with upper limb hemiparesis: the NEURO-VERIFY Study.

PubMed

Abo, Masahiro; Kakuda, Wataru; Momosaki, Ryo; Harashima, Hiroaki; Kojima, Miki; Watanabe, Shigeto; Sato, Toshihiro; Yokoi, Aki; Umemori, Takuma; Sasanuma, Jinichi

2014-07-01

Many poststroke patients suffer functional motor limitation of the affected upper limb, which is associated with diminished health-related quality of life. The aim of this study is to conduct a randomized, multicenter, comparative study of low-frequency repetitive transcranial magnetic stimulation combined with intensive occupational therapy, NEURO (NovEl intervention Using Repetitive TMS and intensive Occupational therapy) versus constraint-induced movement therapy in poststroke patients with upper limb hemiparesis. In this randomized controlled study of NEURO and constraint-induced movement therapy, 66 poststroke patients with upper limb hemiparesis were randomly assigned at 2:1 ratio to low-frequency repetitive transcranial magnetic stimulation plus occupational therapy (NEURO group) or constraint-induced movement therapy (constraint-induced movement therapy group) for 15 days. Fugl-Meyer Assessment and Wolf Motor Function Test and Functional Ability Score of Wolf Motor Function Test were used for assessment. No differences in patients' characteristics were found between the two groups at baseline. The Fugl-Meyer Assessment score was significantly higher in both groups after the 15-day treatment compared with the baseline. Changes in Fugl-Meyer Assessment scores and Functional Ability Score of Wolf Motor Function Test were significantly higher in the NEURO group than in the constraint-induced movement therapy group, whereas the decrease in the Wolf Motor Function Test log performance time was comparable between the two groups (changes in Fugl-Meyer Assessment score, NEURO: 5·39 ± 4·28, constraint-induced movement therapy: 3·09 ± 4·50 points; mean ± standard error of the mean; P < 0·05) (changes in Functional Ability Score of Wolf Motor Function Test, NEURO: 3·98 ± 2·99, constraint-induced movement therapy: 2·09 ± 2·96 points; P < 0·05). The results of the 15-day rehabilitative protocol showed the superiority of NEURO

Transduction of NeuroD2 protein induced neural cell differentiation.

PubMed

Noda, Tomohide; Kawamura, Ryuzo; Funabashi, Hisakage; Mie, Masayasu; Kobatake, Eiry

2006-11-01

NeuroD2, one of the neurospecific basic helix-loop-helix transcription factors, has the ability to induce neural differentiation in undifferentiated cells. In this paper, we show that transduction of NeuroD2 protein induced mouse neuroblastoma cell line N1E-115 into neural differentiation. NeuroD2 has two basic-rich domains, one is nuclear localization signal (NLS) and the other is basic region of basic helix-loop-helix (basic). We constructed some mutants of NeuroD2, ND2(Delta100-115) (lack of NLS), ND2(Delta123-134) (lack of basic) and ND2(Delta100-134) (lack of both NLS and basic) for transduction experiments. Using these proteins, we have shown that NLS region of NeuroD2 plays a role of protein transduction. Continuous addition of NeuroD2 protein resulted in N1E-115 cells adopting neural morphology after 4 days and Tau mRNA expression was increased. These results suggest that neural differentiation can be induced by direct addition of NeuroD2 protein.

Muscular dystrophy in a dog resembling human becker muscular dystrophy.

PubMed

Baroncelli, A B; Abellonio, F; Pagano, T B; Esposito, I; Peirone, B; Papparella, S; Paciello, O

2014-05-01

A 3-year-old, male Labrador retriever dog was presented with clinical signs of progressive exercise intolerance, bilateral elbow extension, rigidity of the forelimbs, hindlimb flexion and kyphosis. Microscopical examination of muscle tissue showed marked variability in myofibre size, replacement of muscle with mature adipose tissue and degeneration/regeneration of muscle fibres, consistent with muscular dystrophy. Immunohistochemical examination for dystrophin showed markedly reduced labelling with monoclonal antibodies specific for the rod domain and the carboxy-terminal of dystrophin, while expression of β-sarcoglycan, γ-sarcoglycan and β-dystroglycan was normal. Immunoblotting revealed a truncated dystrophin protein of approximately 135 kDa. These findings supported a diagnosis of congenital canine muscular dystrophy resembling Becker muscular dystrophy in man. Copyright © 2014 Elsevier Ltd. All rights reserved.

Accelerating Translational Research through Open Science: The Neuro Experiment.

PubMed

Gold, E Richard

2016-12-01

Translational research is often afflicted by a fundamental problem: a limited understanding of disease mechanisms prevents effective targeting of new treatments. Seeking to accelerate research advances and reimagine its role in the community, the Montreal Neurological Institute (Neuro) announced in the spring of 2016 that it is launching a five-year experiment during which it will adopt Open Science-open data, open materials, and no patenting-across the institution. The experiment seeks to examine two hypotheses. The first is whether the Neuro's Open Science initiative will attract new private partners. The second hypothesis is that the Neuro's institution-based approach will draw companies to the Montreal region, where the Neuro is based, leading to the creation of a local knowledge hub. This article explores why these hypotheses are likely to be true and describes the Neuro's approach to exploring them.

Rhabdomyolysis featuring muscular dystrophies.

PubMed

Lahoria, Rajat; Milone, Margherita

2016-02-15

Rhabdomyolysis is a potentially life threatening condition of various etiology. The association between rhabdomyolysis and muscular dystrophies is under-recognized in clinical practice. To identify muscular dystrophies presenting with rhabdomyolysis at onset or as predominant feature. We retrospectively reviewed clinical and laboratory data of patients with a genetically confirmed muscular dystrophy in whom rhabdomyolysis was the presenting or main clinical manifestation. Thirteen unrelated patients (males=6; females=7) were identified. Median age at time of rhabdomyolysis was 18 years (range, 2-47) and median duration between the first episode of rhabdomyolysis and molecular diagnosis was 2 years. Fukutin-related protein (FKRP) muscular dystrophy (n=6) was the most common diagnosis, followed by anoctaminopathy-5 (n=3), calpainopathy-3 (n=2) and dystrophinopathy (n=2). Four patients experienced recurrent rhabdomyolysis. Eight patients were asymptomatic and 3 reported myalgia and exercise intolerance prior to the rhabdomyolysis. Exercise (n=6) and fever (n=4) were common triggers; rhabdomyolysis was unprovoked in 3 patients. Twelve patients required hospitalization. Baseline CK levels were elevated in all patients (median 1200 IU/L; range, 600-3600). Muscular dystrophies can present with rhabdomyolysis; FKRP mutations are particularly frequent in causing such complication. A persistently elevated CK level in patients with rhabdomyolysis warrants consideration for underlying muscular dystrophy. Copyright © 2015 Elsevier B.V. All rights reserved.

Spinal Muscular Atrophy (SMA)

MedlinePlus

... kids of the same age or have trouble lifting things. Kids with SMA can develop scoliosis (a ... Nervous System Your Muscles Wheelchairs Scoliosis Steven's Story: Power Player Kyphosis Muscular Dystrophy Spinal Muscular Atrophy: Steven's ...

Spinal Muscular Atrophy FAQ

MedlinePlus

... in SMA. What is Spinal Muscular Atrophy with Respiratory Distress (SMARD)? SMARD and SMA are separate diseases ... muscle weakness and atrophy. Spinal Muscular Atrophy with Respiratory Distress (SMARD) is a rare neuromuscular disease that ...

[Neuro-ophthalmology: the eye as a window to the brain].

PubMed

Kesler, Anat

2013-02-01

Neuro-ophthalmology focuses on the diagnosis and treatment of visual disorders related to the neurological system rather than the globe itself. Being a subspecialty of both neurology and ophthalmology, it requires specialized training and expertise in diseases of the eye, brain, nerves and muscles. Commonly encountered pathologies in neuro-ophthalmology include: optic neuropathies (such as optic neuritis and ischemic optic neuropathy), visual field loss (transient, constant, unexplained), transient visual loss, unspecified visual disturbances, diplopia, abnormal eye movements, thyroid eye disease, myasthenia gravis, anisocoria, and eyelid abnormalities. The current issue of "Harefuah" is dedicated to contemporary knowledge in neuro-opthalmology, and spans from studies of neuromyelitis optica (NMO), ischemic optic neuropathies, and optic neuropathies induced by phosphodiesterase inhibitors, to the management of sight-threatening carotid-cavernous fistulas, and more. These studies emphasize the importance of an interdisciplinary treatment team consisting of a neuro-ophthalmologist, a neuro-radiologist, and sometimes, even a neuro-surgeon. Such an approach may prove to be beneficial to the patient, by optimizing follow-up and treatment decisions. This issue emphasizes how a correct and timely diagnosis is of paramount significance in patients with neuro-ophthalmological disorders.

Improving English Instruction through Neuro-Linguistic Programming

ERIC Educational Resources Information Center

Helm, David Jay

2009-01-01

This study examines the background information and numerous applications of neuro-linguistic programming as it applies to improving English instruction. In addition, the N.L.P. modalities of eye movement, the use of predicates, and posturing are discussed. Neuro-linguistic programming presents all students of English an opportunity to reach their…

Approximation abilities of neuro-fuzzy networks

NASA Astrophysics Data System (ADS)

Mrówczyńska, Maria

2010-01-01

The paper presents the operation of two neuro-fuzzy systems of an adaptive type, intended for solving problems of the approximation of multi-variable functions in the domain of real numbers. Neuro-fuzzy systems being a combination of the methodology of artificial neural networks and fuzzy sets operate on the basis of a set of fuzzy rules "if-then", generated by means of the self-organization of data grouping and the estimation of relations between fuzzy experiment results. The article includes a description of neuro-fuzzy systems by Takaga-Sugeno-Kang (TSK) and Wang-Mendel (WM), and in order to complement the problem in question, a hierarchical structural self-organizing method of teaching a fuzzy network. A multi-layer structure of the systems is a structure analogous to the structure of "classic" neural networks. In its final part the article presents selected areas of application of neuro-fuzzy systems in the field of geodesy and surveying engineering. Numerical examples showing how the systems work concerned: the approximation of functions of several variables to be used as algorithms in the Geographic Information Systems (the approximation of a terrain model), the transformation of coordinates, and the prediction of a time series. The accuracy characteristics of the results obtained have been taken into consideration.

Structure of neuro-endocrine and neuro-epithelial interactions in human foetal pancreas.

PubMed

Krivova, Yuliya; Proshchina, Alexandra; Barabanov, Valeriy; Leonova, Olga; Saveliev, Sergey

2016-12-01

In the pancreas of many mammals including humans, endocrine islet cells can be integrated with the nervous system components into neuro-insular complexes. The mechanism of the formation of such complexes is not clearly understood. The present study evaluated the interactions between the nervous system components, epithelial cells and endocrine cells in the human pancreas. Foetal pancreas, gestational age 19-23 weeks (13 cases) and 30-34 weeks (7 cases), were studied using double immunohistochemical labeling with neural markers (S100 protein and beta III tubulin), epithelial marker (cytokeratin 19 (CK19)) and antibodies to insulin and glucagon. We first analyse the structure of neuro-insular complexes using confocal microscopy and provide immunohistochemical evidences of the presence of endocrine cells within the ganglia or inside the nerve bundles. We showed that the nervous system components contact with the epithelial cells located in ducts or in clusters outside the ductal epithelium and form complexes with separate epithelial cells. We observed CK19-positive cells inside the ganglia and nerve bundles which were located separately or were integrated with the islets. Therefore, we conclude that neuro-insular complexes may forms as a result of integration between epithelial cells and nervous system components at the initial stages of islets formation. Copyright © 2016 Elsevier Ltd. All rights reserved.

Palliative Care Consultations in the Neuro-ICU: A Qualitative Study.

PubMed

Tran, Len N; Back, Anthony L; Creutzfeldt, Claire J

2016-10-01

Integration of palliative care (PC) into the neurological intensive care unit (neuro-ICU) is increasingly recommended, but evidence regarding the best practice is lacking. We conducted a qualitative analysis exploring current practices and key themes of specialist PC consultations in patients admitted to a single neuro-ICU. We retrospectively identified all patients who were admitted to the neuro-ICU for ≥24 h and received a PC consultation between January and August 2014. We reviewed PC consultation notes and neuro-ICU progress notes from the electronic health records of these patients. We performed content analysis on the PC notes. Twenty-five neuro-ICU patients (4 %) received a PC consultation over 8 months with the most prevalent reason of clarifying goals of care. The main distinctions between patients with and those without (n = 580) a PC consultation were ICU length of stay (median 8.2 vs. 2.8 days) and death in the neuro-ICU (56 % vs. 11 %). The most prevalent themes addressed in the PC consultation notes were (1) discussing prognosis, (2) eliciting patient and family values, (3) understanding medical options, and (4) identifying conflict. PC consultations in the neuro-ICU emphasize family coping and decision-making by helping discuss prognosis and exploring patient and family values as well as their ability to understand the medical information. Several features suggest that earlier integration of PC into neuro-ICU care may enhance both coping and the decision-making process.

Ways of increasing muscular activity by means of isometric muscular exertion

NASA Technical Reports Server (NTRS)

Kovalik, A. V.

1980-01-01

The effect of isometric muscular exertion on the human body was investigated by having subjects perform basic movements in a sitting position in the conventional manner with additional muscle tension at 50% maximum force and at maximum force. The pulse, arterial pressure, skin temperature, respiratory rate, minute respiratory volume and electrical activity of the muscles involved were all measured. Performance of the exercises with maximum muscular exertion for 20 sec and without movement resulted in the greatest shifts in these indices; in the conventional manner substantial changes did not occur; and with isometric muscular exertion with 50% maximum force with and without movement, optimal functional shifts resulted. The latter is recommended for use in industrial exercises for the prevention of hypodynamia. Ten exercises are suggested.

Ophthalmologic Findings in Patients with Neuro-metabolic Disorders.

PubMed

Jafari, Narjes; Golnik, Karl; Shahriari, Mansoor; Karimzadeh, Parvaneh; Jabbehdari, Sayena

2018-01-01

We aimed to present the ophthalmic manifestations of neuro-metabolic disorders. Patients who were diagnosed with neuro-metabolic disorders in the Neurology Department of Mofid Pediatric Hospital in Tehran, Iran, between 2004 and 2014 were included in this study. Disorders were confirmed using clinical findings, neuroimaging, laboratory data, and genomic analyses. All enrolled patients were assessed for ophthalmological abnormalities. A total of 213 patients with 34 different neuro-metabolic disorders were included. Ophthalmological abnormalities were observed in 33.5% of patients. Abnormal findings in the anterior segment included Kayser-Fleischer rings, congenital or secondary cataracts, and lens dislocation into the anterior chamber. Posterior segment (i.e., retina, vitreous body, and optic nerve) evaluation revealed retinitis pigmentosa, cherry-red spots, and optic atrophy. In addition, strabismus, nystagmus, and lack of fixation were noted during external examination. Ophthalmological examination and assessment is essential in patients that may exhibit neuro-metabolic disorders.

Effect of Lumbar Progressive Resistance Exercise on Lumbar Muscular Strength and Core Muscular Endurance in Soldiers.

PubMed

Mayer, John M; Childs, John D; Neilson, Brett D; Chen, Henian; Koppenhaver, Shane L; Quillen, William S

2016-11-01

Low back pain is common, costly, and disabling for active duty military personnel and veterans. The evidence is unclear on which management approaches are most effective. The purpose of this study was to assess the effectiveness of lumbar extensor high-intensity progressive resistance exercise (HIPRE) training versus control on improving lumbar extension muscular strength and core muscular endurance in soldiers. A randomized controlled trial was conducted with active duty U.S. Army Soldiers (n = 582) in combat medic training at Fort Sam Houston, Texas. Soldiers were randomized by platoon to receive the experimental intervention (lumbar extensor HIPRE training, n = 298) or control intervention (core stabilization exercise training, n = 284) at one set, one time per week, for 11 weeks. Lumbar extension muscular strength and core muscular endurance were assessed before and after the intervention period. At 11-week follow-up, lumbar extension muscular strength was 9.7% greater (p = 0.001) for HIPRE compared with control. No improvements in core muscular endurance were observed for HIPRE or control. Lumbar extensor HIPRE training is effective to improve isometric lumbar extension muscular strength in U.S. Army Soldiers. Research is needed to explore the clinical relevance of these gains. Reprint & Copyright © 2016 Association of Military Surgeons of the U.S.

Intracranial Aneurysms of Neuro-Ophthalmologic Relevance.

PubMed

Micieli, Jonathan A; Newman, Nancy J; Barrow, Daniel L; Biousse, Valérie

2017-12-01

Intracranial saccular aneurysms are acquired lesions that often present with neuro-ophthalmologic symptoms and signs. Recent advances in neurosurgical techniques, endovascular treatments, and neurocritical care have improved the optimal management of symptomatic unruptured aneurysms, but whether the chosen treatment has an impact on neuro-ophthalmologic outcomes remains debated. A review of the literature focused on neuro-ophthalmic manifestations and treatment of intracranial aneurysms with specific relevance to neuro-ophthalmologic outcomes was conducted using Ovid MEDLINE and EMBASE databases. Cavernous sinus aneurysms were not included in this review. Surgical clipping vs endovascular coiling for aneurysms causing third nerve palsies was compared in 13 retrospective studies representing 447 patients. Complete recovery was achieved in 78% of surgical patients compared with 44% of patients treated with endovascular coiling. However, the complication rate, hospital costs, and days spent in intensive care were reported as higher in surgically treated patients. Retrospective reviews of surgical clipping and endovascular coiling for all ocular motor nerve palsies (third, fourth, or sixth cranial nerves) revealed similar results of complete resolution in 76% and 49%, respectively. Improvement in visual deficits related to aneurysmal compression of the anterior visual pathways was also better among patients treated with clipping than with coiling. The time to treatment from onset of visual symptoms was a predictive factor of visual recovery in several studies. Few reports have specifically assessed the improvement of visual deficits after treatment with flow diverters. Decisions regarding the choice of therapy for intracranial aneurysms causing neuro-ophthalmologic signs ideally should be made at high-volume centers with access to both surgical and endovascular treatments. The status of the patient, location of the aneurysm, and experience of the treating physicians

Myotonic Dystrophy and Facioscapulohumeral Muscular Dystrophy Registry

ClinicalTrials.gov

2017-08-11

Myotonic Dystrophy; Facioscapulohumeral Muscular Dystrophy; Muscular Dystrophy; Myotonic Dystrophy Type 1; Myotonic Dystrophy Type 2; Congenital Myotonic Dystrophy; PROMM (Proximal Myotonic Myopathy); Steinert's Disease; Myotonic Muscular Dystrophy

Treatment of neuro-ophthalmic sarcoidosis.

PubMed

Frohman, Larry P

2015-03-01

Because of the rarity of neuro-ophthalmic sarcoidosis, there are no therapeutic guidelines based on evidence-based medicine for this disorder. Review of literature combined with personal experience. Corticosteroids are the preferred initial therapy for neuro-ophthalmic sarcoidosis. If patients cannot tolerate the requisite dose of corticosteroid needed to control their disease, or if corticosteroids fail to adequately control the disease process, the choices of a second agent are based on the consideration of rapidity of clinical response and the safety profile. Although methotrexate and mycophenolate mofetil are the medications that are often selected after corticosteroid failure, more rapidly acting agents that have been used are infliximab and intravenous cyclophosphamide.

Parent perceptions of the quality of life of pet dogs living with neuro-typically developing and neuro-atypically developing children: An exploratory study.

PubMed

Hall, Sophie S; Wright, Hannah F; Mills, Daniel S

2017-01-01

There is growing scientific and societal recognition of the role that pet dogs can play in healthy development of children; both those who are neuro-typically developing and those who live with a neuro-developmental disorder, such as autism or attention deficit hyperactivity disorder. However, little attention has been paid to how living with children positively and negatively affects quality of life of a pet dog. In this exploratory study we conducted semi-structured interviews with parents of neuro-typically developing children (n = 18) and those with a neuro-developmental disorder (n = 18) who owned a pet dog, until no new factors were identified. Living with children brought potentially positive benefits to the dog's life including: imposition of a routine, participation in recreational activities and the development of a strong bond between the child and the dog. The importance of maintaining a routine was particularly prevalent in families with children with neuro-developmental disorders. Potential negative factors included having to cope with child meltdowns and tantrums, over stimulation from child visitors, harsh contact and rough and tumble play with the child. The regularity and intensity of meltdowns and tantrums was particularly evident in responses from parents with children with a neuro-developmental disorder. However, child visitors and rough play and contact were mentioned similarly across the groups. Protective factors included having a safe haven for the dog to escape to, parent's awareness of stress signs and child education in dog-interaction. Parents were also asked to complete a stress response scale to provide an initial quantitative comparison of stress responses between dogs living with the two family-types. Parents with neuro-typically developing children more frequently observed their dog rapidly running away from a situation and less frequently observed their dog widening their eyes, than parents with children with a neuro

Global muscular dystrophy research: A 25-year bibliometric perspective.

PubMed

Ram, Shri

2017-01-01

Muscular dystrophy is a genetic disorder leading to progressive weakness of muscles caused due to dysfunction in or lack of protein in muscle cells. The prevalence of muscular dystrophy has been observed globally and is becoming a critical area of study for better health services. The purpose of the study is to analyze the research strength of muscular dystrophy using bibliographic literature. A quantitative literature analysis was carried out on muscular dystrophy from 1991 to 2015 for assessing the global research trends. This literature-based study was conducted using the documents retrieved from the Science Citation Index using the keywords: Duchenne Muscular Dystrophy (DMD), Becker Muscular Dystrophy (BMD), Congenital Muscular Dystrophy (CMD), Myotonic Dystrophy, Emery-Dreifuss Muscular Dystrophy, Facioscapulohumeral Muscular Dystrophy, Oculopharyngeal Muscular Dystrophy, and Limb-Girdle Muscular Dystrophy. Analysis was done for annual productivity of publication, authorship, collaboration, country performance, citation frequency, characteristics of most cited document, journal productivity, etc.

From 'Hard' Neuro-Tools to 'Soft' Neuro-Toys? Refocussing the Neuro-Enhancement Debate.

PubMed

Brenninkmeijer, Jonna; Zwart, Hub

2017-01-01

Since the 1990's, the debate concerning the ethical, legal and societal aspects of 'neuro-enhancement' has evolved into a massive discourse, both in the public realm and in the academic arena. This ethical debate, however, tends to repeat the same sets of arguments over and over again. Normative disagreements between transhumanists and bioconservatives on invasive or radical brain stimulators, and uncertainties regarding the use and effectivity of nootropic pharmaceuticals dominate the field. Building on the results of an extensive European project on responsible research and innovation in neuro-enhancement (NERRI), we observe and encourage that the debate is now entering a new and, as we will argue, more realistic and societally relevant stage. This new stage concerns those technologies that enter the market as ostensibly harmless contrivances that consumers may use for self-care or entertainment. We use the examples and arguments of participants in NERRI debates to describe three case studies of such purportedly innocent 'toys'. Based upon this empirical material, we argue that these 'soft' enhancement gadgets are situated somewhere in the boundary zone between the internal and the external, between the intimate and the intrusive, between the familiar and the unfamiliar, between the friendly and the scary and, in Foucauldian terms, between technologies of the self and technologies of control. Therefore, we describe their physiognomy with the help of a term borrowed from Jacques Lacan, namely as "extimate" technologies.

Neuro-immune interactions at barrier surfaces

PubMed Central

Veiga-Fernandes, Henrique; Mucida, Daniel

2016-01-01

Multidirectional interactions between the nervous and immune systems have been documented in homeostasis and pathologies ranging from multiple sclerosis to autism, and from leukemia to acute and chronic inflammation. Recent studies have addressed this crosstalk using cell-specific targeting, novel sequencing, imaging and analytical tools, shedding light on unappreciated mechanisms of neuro-immune regulation. This review focuses on neuro-immune interactions at barrier surfaces, mostly the gut, but also including the skin and the airways, areas densely populated by neurons and immune cells that constantly sense and adapt to tissue-specific environmental challenges. PMID:27153494

Limb-Girdle Muscular Dystrophy (LGMD)

MedlinePlus

... Association (MDA) is a qualified 501(c)(3) tax-exempt organization. ©2018, Muscular Dystrophy Association Inc. All ... Association (MDA) is a qualified 501(c)(3) tax-exempt organization. ©2018, Muscular Dystrophy Association Inc. All ...

QL-10NEURO-ONCOLOGY TELEMEDICINE FOLLOW-UP VISITS

PubMed Central

Green, Richard; Woyshner, Emily

2014-01-01

We report our 18 month experience with the use of a videoconferencing system to perform neuro-oncology follow-up visits. The Neuro-oncology Program at the Kaiser Permanente-Los Angeles Medical center serves the majority of Kaiser HMO patients in the Southern California region. We installed a videoconferencing system (Cisco TelePresence EX90, Cisco Systems, San Jose, CA) in our office in Los Angeles and in a medical office building in Anaheim, CA at a distance of 35 miles. Established neuro-oncology patients from Orange County chose between in-person and remote visits. Patients were seated in an examination room and the neuro-oncology provider alerted by text page. A focused history and physical examination was performed, followed by desktop sharing of clinical and laboratory data using an electronic medical record (Epic Systems Corporation, Verona, WI) and of neuroimages (Phillips iSite PACS, Andover, MA). Patients were asked, but not required, to complete an anonymous online 16 question satisfaction survey after each visit. Visits were performed by either a neuro-oncologist (179) or a Physician's Assistant (12). Of the 191 visits, 174 included evaluation of neuroimaging and 77 included evaluation of response to ongoing chemotherapy. During 12 visits chemotherapy was initiated, and during 15 visits the chemotherapy regimen was changed based on imaging findings. One-hundred and eleven surveys (58% of visits) were completed. Patients reported a high level of satisfaction with the visits (average 9.6, on a 1-10 scale). The average estimated travel time saved was 118 minutes per visit. Four surveys reported technical problems and 1 indicated a preference for an in-person visit. No adverse events could be attributed to use of the telemedicine system. These data suggest that neuro-oncology follow-up visits can be practiced safely and effectively using a telemedicine system, with high levels of patient satisfaction.

Long-Term Outcomes of Ataluren in Duchenne Muscular Dystrophy

ClinicalTrials.gov

2018-05-16

Muscular Dystrophy, Duchenne; Muscular Dystrophies; Muscular Disorders, Atrophic; Muscular Diseases; Musculoskeletal Disease; Neuromuscular Diseases; Nervous System Diseases; Genetic Diseases, X-Linked; Genetic Diseases, Inborn

Dropped head congenital muscular dystrophy caused by de novo mutations in LMNA.

PubMed

Karaoglu, Pakize; Quizon, Nicolas; Pergande, Matthias; Wang, Haicui; Polat, Ayşe Ipek; Ersen, Ayca; Özer, Erdener; Willkomm, Lena; Hiz Kurul, Semra; Heredia, Raúl; Yis, Uluç; Selcen, Duygu; Çirak, Sebahattin

2017-04-01

Dropped head syndrome is an easily recognizable clinical presentation of Lamin A/C-related congenital muscular dystrophy. Patients usually present in the first year of life with profound neck muscle weakness, dropped head, and elevated serum creatine kinase. Two patients exhibited head drop during infancy although they were able to sit independently. Later they developed progressive axial and limb-girdle weakness. Creatine kinase levels were elevated and muscle biopsies of both patients showed severe dystrophic changes. The distinctive clinical hallmark of the dropped head led us to the diagnosis of Lamin A/C-related congenital muscular dystrophy, with a pathogenic de novo mutation p.Glu31del in the head domain of the Lamin A/C gene in both patients. Remarkably, one patient also had a central involvement with white matter changes on brain magnetic resonance imaging. Lamin A/C-related dropped-head syndrome is a rapidly progressive congenital muscular dystrophy and may lead to loss of ambulation, respiratory insufficiency, and cardiac complications. Thus, the genetic diagnosis of dropped-head syndrome as L-CMD and the implicated clinical care protocols are of vital importance for these patients. This disease may be underdiagnosed, as only a few genetically confirmed cases have been reported. Copyright © 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Parent perceptions of the quality of life of pet dogs living with neuro-typically developing and neuro-atypically developing children: An exploratory study

PubMed Central

Wright, Hannah F.; Mills, Daniel S.

2017-01-01

There is growing scientific and societal recognition of the role that pet dogs can play in healthy development of children; both those who are neuro-typically developing and those who live with a neuro-developmental disorder, such as autism or attention deficit hyperactivity disorder. However, little attention has been paid to how living with children positively and negatively affects quality of life of a pet dog. In this exploratory study we conducted semi-structured interviews with parents of neuro-typically developing children (n = 18) and those with a neuro-developmental disorder (n = 18) who owned a pet dog, until no new factors were identified. Living with children brought potentially positive benefits to the dog’s life including: imposition of a routine, participation in recreational activities and the development of a strong bond between the child and the dog. The importance of maintaining a routine was particularly prevalent in families with children with neuro-developmental disorders. Potential negative factors included having to cope with child meltdowns and tantrums, over stimulation from child visitors, harsh contact and rough and tumble play with the child. The regularity and intensity of meltdowns and tantrums was particularly evident in responses from parents with children with a neuro-developmental disorder. However, child visitors and rough play and contact were mentioned similarly across the groups. Protective factors included having a safe haven for the dog to escape to, parent’s awareness of stress signs and child education in dog-interaction. Parents were also asked to complete a stress response scale to provide an initial quantitative comparison of stress responses between dogs living with the two family-types. Parents with neuro-typically developing children more frequently observed their dog rapidly running away from a situation and less frequently observed their dog widening their eyes, than parents with children with a neuro

Patterns of neuroAIDS in Africa.

PubMed

Tadesse, Tizeta; Langford, Dianne; Manji, Karim; Mehari, Enawgaw

2005-01-01

According to UNAIDS, the African population accounts for greater than half of persons infected with HIV. Nevertheless, little information exists characterizing HIV in this population. Thus, the natural history and progression of HIV in the African population is virtually undocumented and therefore, poorly understood. Information regarding virtually every aspect of the disease including microbiology, pathogenicity, virulence, and clinical manifestation is based largely on data from select and limited populations. During the HAART-era, we have seen dramatic and significant changes in patterns of NeuroAIDS in patients in clinical cohorts from the United States and Western Europe. These observations have led to increased understanding of the progression of NeuroAIDS and have improved our ability to design treatment regimens to combat CNS complications resulting from HIV. Despite the existence of antiretroviral therapy for HIV, its absence in Africa along with poor treatments for opportunistic infections associated with HIV have become the main sources of neurological morbidity and mortality. In this context, we are presented with a unique opportunity to cultivate and enhance our understanding of the natural history and progression of NeuroAIDS in the African population thereby, better equipping healthcare providers, patients and their families in addressing this epidemic. This concept is particularly important as rapidly improving and more accessible anti-HIV medications and medications for the treatment of opportunistic infections become available to third world countries such as Africa. We believe that it is imperative to foster research, education and training between institutions in the industrialized world and Africa to close the gap in understanding patterns of NeuroAIDS in Africa.

Carrier testing for spinal muscular atrophy

PubMed Central

Gitlin, Jonathan M.; Fischbeck, Kenneth; Crawford, Thomas O.; Cwik, Valerie; Fleischman, Alan; Gonye, Karla; Heine, Deborah; Hobby, Kenneth; Kaufmann, Petra; Keiles, Steven; MacKenzie, Alex; Musci, Thomas; Prior, Thomas; Lloyd-Puryear, Michele; Sugarman, Elaine A.; Terry, Sharon F.; Urv, Tiina; Wang, Ching; Watson, Michael; Yaron, Yuval; Frosst, Phyllis; Howell, R. Rodney

2014-01-01

Spinal muscular atrophy is the most common fatal hereditary disease among newborns and infants. There is as yet no effective treatment. Although a carrier test is available, currently there is disagreement among professional medical societies who proffer standards of care as to whether or not carrier screening for spinal muscular atrophy should be offered as part of routine reproductive care. This leaves health care providers without clear guidance. In fall 2009, a meeting was held by National Institutes of Health to examine the scientific basis for spinal muscular atrophy carrier screening and to consider the issues that accompany such screening. In this article, the meeting participants summarize the discussions and conclude that pan-ethnic carrier screening for spinal muscular atrophy is technically feasible and that the specific study of implementing a spinal muscular atrophy carrier screening program raises broader issues about determining the scope and specifics of carrier screening in general. PMID:20808230

Neuro-Linguistic Programming: A Discussion of Why and How.

ERIC Educational Resources Information Center

Partridge, Susan

Intended for teachers, this article offers a definition of neuro-linguistic programming (NLP), discusses its relevance to instruction, and provides illustrations of the implementation of neuro-linguistic programming in instructional contexts. NLP is defined as an approach to instruction that recognizes the familiar visual, auditory, and…

Developmental neurogenetics and neuro-ophthalmology.

PubMed

Bennett, Jeffrey L

2002-12-01

The field of developmental neurogenetics has burgeoned over the past decade. Through the combined efforts of developmental biologists, geneticists, and clinicians, genetic defects resulting in neuro-ophthalmic disorders such as holoprosencephaly, microphthalmia, dominant optic atrophy, and optic nerve colobomas have been identified and characterized at the molecular level. Experimental studies in model organisms are continuing to identify novel genes critical for ocular and central nervous system development. Mutations in some of these genes have revealed a spectrum of pathology similar to that observed in septo-optic dysplasia, Möebius syndrome, and Duane retraction syndrome. This review examines our current knowledge of the molecular genetics of neuro-ophthalmic disease and focuses on several candidate genes for afferent and efferent visual system disorders.

Wasting Mechanisms in Muscular Dystrophy

PubMed Central

Shin, Jonghyun; Tajrishi, Marjan M.; Ogura, Yuji; Kumar, Ashok

2013-01-01

Muscular dystrophy is a group of more than 30 different clinical genetic disorders that are characterized by progressive skeletal muscle wasting and degeneration. Primary deficiency of specific extracellular matrix, sarcoplasmic, cytoskeletal, or nuclear membrane protein results in several secondary changes such as sarcolemmal instability, calcium influx, fiber necrosis, oxidative stress, inflammatory response, breakdown of extracellular matrix, and eventually fibrosis which leads to loss of ambulance and cardiac and respiratory failure. A number of molecular processes have now been identified which hasten disease progression in human patients and animal models of muscular dystrophy. Accumulating evidence further suggests that aberrant activation of several signaling pathways aggravate pathological cascades in dystrophic muscle. Although replacement of defective gene with wild-type is paramount to cure, management of secondary pathological changes has enormous potential to improving the quality of life and extending lifespan of muscular dystrophy patients. In this article, we have reviewed major cellular and molecular mechanisms leading to muscle wasting in muscular dystrophy. PMID:23669245

NeuroD modulates opioid agonist-selective regulation of adult neurogenesis and contextual memory extinction.

PubMed

Zheng, Hui; Zhang, Yue; Li, Wen; Loh, Horace H; Law, Ping-Yee

2013-04-01

Addictive drugs, including opioids, modulate adult neurogenesis. In order to delineate the probable implications of neurogenesis on contextual memory associated with addiction, we investigated opioid agonist-selective regulation of neurogenic differentiation 1 (NeuroD) activities under the conditioned place preference (CPP) paradigm. Training mice with equivalent doses of morphine and fentanyl produced different CPP extinction rates without measurable differences in the CPP acquisition rate or magnitude. Fentanyl-induced CPP required much longer time for extinction than morphine-induced CPP. We observed a parallel decrease in NeuroD activities and neurogenesis after morphine-induced CPP, but not after fentanyl-induced CPP. Increasing NeuroD activities with NeuroD-lentivirus (nd-vir) injection at the dentate gyrus before CPP training reversed morphine-induced decreases in NeuroD activities and neurogenesis, and prolonged the time required for extinction of morphine-induced CPP. On the other hand, decreasing NeuroD activities via injection of miRNA-190-virus (190-vir) reversed the fentanyl effect on NeuroD and neurogenesis and shortened the time required for extinction of fentanyl-induced CPP. Another contextual memory task, the Morris Water Maze (MWM), was affected similarly by alteration of NeuroD activities. The reduction in NeuroD activities either by morphine treatment or 190-vir injection decreased MWM task retention, while the increase in NeuroD activities by nd-vir prolonged MWM task retention. Thus, by controlling NeuroD activities, opioid agonists differentially regulate adult neurogenesis and subsequent contextual memory retention. Such drug-related memory regulation could have implications in eventual context-associated relapse.

Physiology of respiratory disturbances in muscular dystrophies

PubMed Central

Lo Mauro, Antonella

2016-01-01

Muscular dystrophy is a group of inherited myopathies characterised by progressive skeletal muscle wasting, including of the respiratory muscles. Respiratory failure, i.e. when the respiratory system fails in its gas exchange functions, is a common feature in muscular dystrophy, being the main cause of death, and it is a consequence of lung failure, pump failure or a combination of the two. The former is due to recurrent aspiration, the latter to progressive weakness of respiratory muscles and an increase in the load against which they must contract. In fact, both the resistive and elastic components of the work of breathing increase due to airway obstruction and chest wall and lung stiffening, respectively. The respiratory disturbances in muscular dystrophy are restrictive pulmonary function, hypoventilation, altered thoracoabdominal pattern, hypercapnia, dyspnoea, impaired regulation of breathing, inefficient cough and sleep disordered breathing. They can be present at different rates according to the type of muscular dystrophy and its progression, leading to different onset of each symptom, prognosis and degree of respiratory involvement. Key points A common feature of muscular dystrophy is respiratory failure, i.e. the inability of the respiratory system to provide proper oxygenation and carbon dioxide elimination. In the lung, respiratory failure is caused by recurrent aspiration, and leads to hypoxaemia and hypercarbia. Ventilatory failure in muscular dystrophy is caused by increased respiratory load and respiratory muscles weakness. Respiratory load increases in muscular dystrophy because scoliosis makes chest wall compliance decrease, atelectasis and fibrosis make lung compliance decrease, and airway obstruction makes airway resistance increase. The consequences of respiratory pump failure are restrictive pulmonary function, hypoventilation, altered thoracoabdominal pattern, hypercapnia, dyspnoea, impaired regulation of breathing, inefficient cough and

Physiology of respiratory disturbances in muscular dystrophies.

PubMed

Lo Mauro, Antonella; Aliverti, Andrea

2016-12-01

Muscular dystrophy is a group of inherited myopathies characterised by progressive skeletal muscle wasting, including of the respiratory muscles. Respiratory failure, i.e . when the respiratory system fails in its gas exchange functions, is a common feature in muscular dystrophy, being the main cause of death, and it is a consequence of lung failure, pump failure or a combination of the two. The former is due to recurrent aspiration, the latter to progressive weakness of respiratory muscles and an increase in the load against which they must contract. In fact, both the resistive and elastic components of the work of breathing increase due to airway obstruction and chest wall and lung stiffening, respectively. The respiratory disturbances in muscular dystrophy are restrictive pulmonary function, hypoventilation, altered thoracoabdominal pattern, hypercapnia, dyspnoea, impaired regulation of breathing, inefficient cough and sleep disordered breathing. They can be present at different rates according to the type of muscular dystrophy and its progression, leading to different onset of each symptom, prognosis and degree of respiratory involvement. A common feature of muscular dystrophy is respiratory failure, i.e. the inability of the respiratory system to provide proper oxygenation and carbon dioxide elimination.In the lung, respiratory failure is caused by recurrent aspiration, and leads to hypoxaemia and hypercarbia.Ventilatory failure in muscular dystrophy is caused by increased respiratory load and respiratory muscles weakness.Respiratory load increases in muscular dystrophy because scoliosis makes chest wall compliance decrease, atelectasis and fibrosis make lung compliance decrease, and airway obstruction makes airway resistance increase.The consequences of respiratory pump failure are restrictive pulmonary function, hypoventilation, altered thoracoabdominal pattern, hypercapnia, dyspnoea, impaired regulation of breathing, inefficient cough and sleep disordered

Muscular dystrophy

MedlinePlus

... are no known cures for the various muscular dystrophies. The goal of treatment is to control symptoms. Physical therapy may help maintain muscle strength and function. Leg braces and a wheelchair ...

Biochemistry and neurobiology of prosaposin: a potential therapeutic neuro-effector.

PubMed

Misasi, Roberta; Hozumi, Isao; Inuzuka, Takashi; Capozzi, Antonella; Mattei, Vincenzo; Kuramoto, Yukako; Shimeno, Hiroshi; Soeda, Shinji; Azuma, Norihiro; Yamauchi, Toyoaki; Hiraiwa, Masao

2009-06-01

Prosaposin, a 66 kDa glycoprotein, was identified initially as the precursor of the sphingolipid activator proteins, saposins A-D, which are required for the enzymatic hydrolysis of certain sphingolipids by lysosomal hydrolases. While mature saposins are distributed to lysosomes, prosaposin exists in secretory body fluids and plasma membranes. In addition to its role as the precursor, prosaposin shows a variety of neurotrophic and myelinotrophic activities through a receptor-mediated mechanism. In studies in vivo, prosaposin was demonstrated to exert a variety of neuro-efficacies capable of preventing neuro-degeneration following neuro-injury and promoting the amelioration of allodynia and hyperalgesia in pain models. Collective findings indicate that prosaposin is not a simple house-keeping precursor protein; instead, it is a protein essentially required for the development and maintenance of the central and peripheral nervous systems. Accumulating evidence over the last decade has attracted interests in exploring and developing new therapeutic approaches using prosaposin for human disorders associated with neuro-degeneration. In this review we detail the structure characteristics, cell biological feature, in vivo efficacy, and neuro-therapeutic potential of prosaposin, thereby providing future prospective in clinical application of this multifunctional protein.

Muscular Oxygen Uptake Kinetics in Aged Adults.

PubMed

Koschate, J; Drescher, U; Baum, K; Eichberg, S; Schiffer, T; Latsch, J; Brixius, K; Hoffmann, U

2016-06-01

Pulmonary oxygen uptake (V˙O2) kinetics and heart rate kinetics are influenced by age and fitness. Muscular V˙O2 kinetics can be estimated from heart rate and pulmonary V˙O2. In this study the applicability of a test using pseudo-random binary sequences in combination with a model to estimate muscular V˙O2 kinetics was tested. Muscular V˙O2 kinetics were expected to be faster than pulmonary V˙O2 kinetics, slowed in aged subjects and correlated with maximum V˙O2 and heart rate kinetics. 27 elderly subjects (73±3 years; 81.1±8.2 kg; 175±4.7 cm) participated. Cardiorespiratory kinetics were assessed using the maximum of cross-correlation functions, higher maxima implying faster kinetics. Muscular V˙O2 kinetics were faster than pulmonary V˙O2 kinetics (0.31±0.1 vs. 0.29±0.1 s; p=0.004). Heart rate kinetics were not correlated with muscular or pulmonary V˙O2 kinetics or maximum V˙O2. Muscular V˙O2 kinetics correlated with maximum V˙O2 (r=0.35; p=0.033). This suggests, that muscular V˙O2 kinetics are faster than estimates from pulmonary V˙O2 and related to maximum V˙O2 in aged subjects. In the future this experimental approach may help to characterize alterations in muscular V˙O2 under various conditions independent of motivation and maximal effort. © Georg Thieme Verlag KG Stuttgart · New York.

Muscular Dystrophy

MedlinePlus

... Gardner-Medwin D. Variability in clinical, genetic and protein abnormalities in manifesting carriers of Duchenne and Becker muscular dystrophy . Neuromuscul. Disord. Jan 1993;3(1):57-64. 3. Bushby KM, Appleton R, Anderson ...

Palliative Care Needs Assessment in the Neuro-ICU: Effect on Family.

PubMed

Creutzfeldt, Claire J; Hanna, Marina G; Cheever, C Sherry; Lele, Abhijit V; Spiekerman, Charles; Engelberg, Ruth A; Curtis, J Randall

2017-10-01

Examine the association of a daily palliative care needs checklist on outcomes for family members of patients discharged from the neurosciences intensive care unit (neuro-ICU). We conducted a prospective, longitudinal cohort study in a single, thirty-bed neuro-ICU in a regional comprehensive stroke and level 1 trauma center. One of two neuro-ICU services that admit patients to the same ICU on alternating days used a palliative care needs checklist during morning work rounds. Between March and October, 2015, surveys were mailed to family members of patients discharged from the neuro-ICU. Nearly half of surveys (n = 91, 48.1%) were returned at a median of 4.7 months. At the time of survey completion, mean Modified rankin scale score (mRS) of neuro-ICU patients was 3.1 (SD 2). Overall ratings of quality of care were relatively high (82.2 on a 0-100 scale) with 32% of family members meeting screening criteria for depressive syndrome. The primary outcome measuring family satisfaction, consisting of eight items from the Family Satisfaction in the ICU questionnaire, did not differ significantly between families of patients from either ICU service nor did family ratings of depression (PHQ-8) and post-traumatic stress (PCL-17). Among families of patients discharged from the neuro-ICU, the daily use of a palliative care needs checklist had no measurable effect on family satisfaction scores or long-term psychological outcomes. Further research is needed to identify optimal interventions to meet the palliative care needs specific to family members of patients treated in the neuro-ICU.

Statistically qualified neuro-analytic failure detection method and system

DOEpatents

Vilim, Richard B.; Garcia, Humberto E.; Chen, Frederick W.

2002-03-02

An apparatus and method for monitoring a process involve development and application of a statistically qualified neuro-analytic (SQNA) model to accurately and reliably identify process change. The development of the SQNA model is accomplished in two stages: deterministic model adaption and stochastic model modification of the deterministic model adaptation. Deterministic model adaption involves formulating an analytic model of the process representing known process characteristics, augmenting the analytic model with a neural network that captures unknown process characteristics, and training the resulting neuro-analytic model by adjusting the neural network weights according to a unique scaled equation error minimization technique. Stochastic model modification involves qualifying any remaining uncertainty in the trained neuro-analytic model by formulating a likelihood function, given an error propagation equation, for computing the probability that the neuro-analytic model generates measured process output. Preferably, the developed SQNA model is validated using known sequential probability ratio tests and applied to the process as an on-line monitoring system. Illustrative of the method and apparatus, the method is applied to a peristaltic pump system.

Neuro-Behçet’s disease in childhood: A focus on the neuro-ophthalmological features

PubMed Central

2013-01-01

Neuro-Behçet’s disease (NBD) involves the central nervous system; peripheral nervous system involvement is not often reported. NBD is quite common in adult patients and occurs rarely during childhood and adolescence. Young patients may share symptoms and signs of NBD with other neuro-ophthalmological disorders (e.g. idiopathic intracranial hypertension); thus, making the differential diagnosis difficult. Neuroimaging is mandatory and necessary for a correct NBD diagnosis but in children radiological examinations are often difficult to perform without sedation. From 1971 to 2011, 130 patients aged ≤16 years have been reported with NBD, according to retrospective surveys, case series, and case reports. The origin of the reported cases met the well-known geographical distribution of Behçet’s disease (BD); the mean age at presentation of neurological findings was 11.8 years, with male gender prevalence (ratio, 2.9:1). We considered in detail the neuro-ophthalmological features of the 53 cases whose neuroimaging alterations were described with an assigned radiological pattern of the disease (parenchymal: 14 cases, non-parechymal: 35 cases, and mixed: 4 cases). In 19/53 patients (36%), neuro-ophthalmological symptoms anticipated any pathognomonic sign for a BD diagnosis, or only occasional aphtae were recalled by the patients. Family history was positive in 17% of subjects. Headache was reported in 75% of the patients; in those presenting with cerebral vascular involvement, headache was combined to other symptoms of intracranial hypertension. Papilledema was the most frequently reported ophthalmological finding, followed by posterior uveitis. Treatment consisted of systemic steroids in 93% of patients, often combined with other immunosuppressive drugs (especially colchicine and azathioprine). Clinical recovery or improvement was documented in the large majority of patients. Nine subjects had definitive alterations, and one died. Based on our review and personal

Two modular neuro-fuzzy system for mobile robot navigation

NASA Astrophysics Data System (ADS)

Bobyr, M. V.; Titov, V. S.; Kulabukhov, S. A.; Syryamkin, V. I.

2018-05-01

The article considers the fuzzy model for navigation of a mobile robot operating in two modes. In the first mode the mobile robot moves along a line. In the second mode, the mobile robot looks for an target in unknown space. Structural and schematic circuit of four-wheels mobile robot are presented in the article. The article describes the movement of a mobile robot based on two modular neuro-fuzzy system. The algorithm of neuro-fuzzy inference used in two modular control system for movement of a mobile robot is given in the article. The experimental model of the mobile robot and the simulation of the neuro-fuzzy algorithm used for its control are presented in the article.

Intelligent multiagent coordination based on reinforcement hierarchical neuro-fuzzy models.

PubMed

Mendoza, Leonardo Forero; Vellasco, Marley; Figueiredo, Karla

2014-12-01

This paper presents the research and development of two hybrid neuro-fuzzy models for the hierarchical coordination of multiple intelligent agents. The main objective of the models is to have multiple agents interact intelligently with each other in complex systems. We developed two new models of coordination for intelligent multiagent systems, which integrates the Reinforcement Learning Hierarchical Neuro-Fuzzy model with two proposed coordination mechanisms: the MultiAgent Reinforcement Learning Hierarchical Neuro-Fuzzy with a market-driven coordination mechanism (MA-RL-HNFP-MD) and the MultiAgent Reinforcement Learning Hierarchical Neuro-Fuzzy with graph coordination (MA-RL-HNFP-CG). In order to evaluate the proposed models and verify the contribution of the proposed coordination mechanisms, two multiagent benchmark applications were developed: the pursuit game and the robot soccer simulation. The results obtained demonstrated that the proposed coordination mechanisms greatly improve the performance of the multiagent system when compared with other strategies.

Muscular Dystrophy

MedlinePlus

... forms of MD grow worse as the person's muscles get weaker. Most people with MD eventually lose the ability to walk. There is no cure for muscular dystrophy. Treatments can help with the symptoms and prevent complications. ...

Cascade control of superheated steam temperature with neuro-PID controller.

PubMed

Zhang, Jianhua; Zhang, Fenfang; Ren, Mifeng; Hou, Guolian; Fang, Fang

2012-11-01

In this paper, an improved cascade control methodology for superheated processes is developed, in which the primary PID controller is implemented by neural networks trained by minimizing error entropy criterion. The entropy of the tracking error can be estimated recursively by utilizing receding horizon window technique. The measurable disturbances in superheated processes are input to the neuro-PID controller besides the sequences of tracking error in outer loop control system, hence, feedback control is combined with feedforward control in the proposed neuro-PID controller. The convergent condition of the neural networks is analyzed. The implementation procedures of the proposed cascade control approach are summarized. Compared with the neuro-PID controller using minimizing squared error criterion, the proposed neuro-PID controller using minimizing error entropy criterion may decrease fluctuations of the superheated steam temperature. A simulation example shows the advantages of the proposed method. Copyright © 2012 ISA. Published by Elsevier Ltd. All rights reserved.

Statistically Qualified Neuro-Analytic system and Method for Process Monitoring

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

Vilim, Richard B.; Garcia, Humberto E.; Chen, Frederick W.

1998-11-04

An apparatus and method for monitoring a process involves development and application of a statistically qualified neuro-analytic (SQNA) model to accurately and reliably identify process change. The development of the SQNA model is accomplished in two steps: deterministic model adaption and stochastic model adaptation. Deterministic model adaption involves formulating an analytic model of the process representing known process characteristics,augmenting the analytic model with a neural network that captures unknown process characteristics, and training the resulting neuro-analytic model by adjusting the neural network weights according to a unique scaled equation emor minimization technique. Stochastic model adaptation involves qualifying any remaining uncertaintymore » in the trained neuro-analytic model by formulating a likelihood function, given an error propagation equation, for computing the probability that the neuro-analytic model generates measured process output. Preferably, the developed SQNA model is validated using known sequential probability ratio tests and applied to the process as an on-line monitoring system.« less

Muscular Dystrophy

MedlinePlus

... be affected. Limb-girdle muscular dystrophy (LGMD) affects boys and girls equally, weakening muscles in the shoulders and upper ... weakness and poor muscle tone. Occurring in both girls and boys, it can have different symptoms. It varies in ...

Neuro-transmitters in the central nervous system & their implication in learning and memory processes.

PubMed

Reis, Helton J; Guatimosim, Cristina; Paquet, Maryse; Santos, Magda; Ribeiro, Fabíola M; Kummer, Arthur; Schenatto, Grace; Salgado, João V; Vieira, Luciene B; Teixeira, Antônio L; Palotás, András

2009-01-01

This review article gives an overview of a number of central neuro-transmitters, which are essential for integrating many functions in the central nervous system (CNS), such as learning, memory, sleep cycle, body movement, hormone regulation and many others. Neurons use neuro-transmitters to communicate, and a great variety of molecules are known to fit the criteria to be classified as such. A process shared by all neuro-transmitters is their release by excocytosis, and we give an outline of the molecular events and protein complexes involved in this mechanism. Synthesis, transport, inactivation, and cellular signaling can be very diverse when different neuro-transmitters are compared, and these processes are described separately for each neuro-transmitter system. Here we focus on the most well known neuro-transmitters: acetyl-choline, catechol-amines (dopamine and nor-adrenalin), indole-amine (serotonin), glutamate, and gamma-amino-butyric acid (GABA). Glutamate is the major excitatory neuro-transmitter in the brain and its actions are counter-balanced by GABA, which is the major inhibitory substance in the CNS. A balance of neuronal transmission between these two neuro-transmitters is essential to normal brain function. Acetyl-choline, serotonin and catechol-amines have a more modulatory function in the brain, being involved in many neuronal circuits. Apart from summarizing the current knowledge about the synthesis, release and receptor signaling of these transmitters, some disease states due to alteration of their normal neuro-transmission are also described.

[Top ten progression of neuro-ophthalmology research in China in the latest five years].

PubMed

2014-12-01

Ten researches that represent the most advanced neuro-ophthalmology related studies in china were voted by specialists from Chinese Neuro-ophthalmology Society. These researches were concentrated in the following fields: clinical and basic researches of optic neuritis, studies of ischemic optic neuropathy, and clinical present of Leber's hereditary optic neuropathy. These researches represented the level of neuro-ophthalmology in China and also showed the focus of our Chinese neuro-ophthalmologists in recent years.

Gains in flexibility related to measures of muscular performance: impact of flexibility on muscular performance.

PubMed

Ferreira, Gustavo Nunes Tasca; Teixeira-Salmela, Luci Fuscaldi; Guimarães, Cristiano Queiroz

2007-07-01

Studies that investigated possible correlations between flexibility and muscular performance are scarce in the literature. Therefore, the purpose of this study was to investigate the impact of a program of static stretching on the flexibility of the hamstrings and on muscular performance of the knee flexors and extensors. Pre-post experimental design. University laboratory. Thirty subjects aged 22.8 +/- 4.9 years with bilaterally shortened hamstrings. Using a protocol that has been previously described, the intervention consisted of 30 sessions of static stretching, performed bilaterally five times a week for 6 weeks. Measures of knee range of motion and isokinetic muscular performance (peak torque, angle of peak torque, and work) of knee flexors and extensors at speeds of 60 and 300 degrees/s. After intervention, significant gains in measures of flexibility (P < 0.0001) were observed, with an average gain of the knee-extension angle of 12.6 degrees, ranging from -1.2 to 30.7 degrees. In addition, we found significant increases in the following parameters of muscular performance: angle of peak torque of hamstrings at 60 and 300 degrees/s (P < 0.0001 and 0.018) and for work at 60 and 300 degrees/s for knee flexors (P = 0.012 and 0.005) and for knee extensors (P < 0.0001). The intervention resulted in gains in measures of flexibility, and these gains had a positive impact on some parameters of muscular performance.

Translational Research for Muscular Dystrophy

DTIC Science & Technology

2012-05-01

common in-frame deletions for which clinical information from human Becker muscular dystrophy patients (deletion of exons 44-45, 49-51, 48-53) is lacking... Dystrophy PRINCIPAL INVESTIGATOR: Gregory A. Cox, Ph.D. CONTRACTING ORGANIZATION: The Jackson Laboratory...REPORT TYPE Annual 3. DATES COVERED 1 MAR 2011 - 30 APR 2012 4. TITLE AND SUBTITLE Translational Research for Muscular Dystrophy 5a. CONTRACT

Neuro Linguistic Programming for Counselors.

ERIC Educational Resources Information Center

Harman, Robert L.; O'Neill, Charles

1981-01-01

Describes contributions of Neuro Linguistic Programming (NLP) to counseling practice. The Meta-Model, representational systems, anchoring, and reframing are described. Counselors interested in learning NLP can integrate many valuable new ways of communicating with clients and changing client behaviors. (Author)

Neuro-Ophthalmology at a Tertiary Eye Care Centre in India.

PubMed

Dhiman, Rebika; Singh, Digvijay; Gantayala, Shiva P; Ganesan, Vaitheeswaran L; Sharma, Pradeep; Saxena, Rohit

2017-11-09

Neuro-ophthalmology as a specialty is underdeveloped in India. The aim of our study was to determine the spectrum and profile of patients presenting to a tertiary eye care center with neuro-ophthalmic disorders. A retrospective hospital-based study was conducted, and records of all patients seen at the neuro-ophthalmology clinic of Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India, over a 1-year period were retrieved and evaluated. Of a total of 30,111 patients referred to various specialty clinics in a span of 1 year, 1597 (5%) were referred for neuro-ophthalmology evaluation. The mean patient age was 30.8 ± 19.5 years, with a male dominance (M:F = 2.02:1). Among these patients, optic nerve disorders were noted in 63.8% (n = 1,020), cranial nerve palsy in 7% (n = 114), cortical visual impairment in 6.5% (n = 105), and others (eye/optic nerve hypophasia, blepharospasm, and optic disc drusen) in 6% (n = 95). Among the patients with optic nerve disorders, optic neuropathy without disc edema/(traumatic optic neuropathy, hereditary, tumor-related, retrobulbar neuritis, toxic, and idiopathic) was noted in 42.8% (n = 685) and optic neuropathy with disc edema (ischemic optic neuropathy, papilledema, post-papilledema optic atrophy, papillitis, neuroretinitis, and inflammatory optic neuropathy) in 20.9% (n = 335). Sixteen percent of patients (n = 263) were incorrect referrals. The neuro-ophthalmic clinic constitutes a significant referral unit in a tertiary eye care center in India. Traumatic and ischemic optic neuropathies are the most common diagnoses. Neuro-ophthalmology requires further development as a subspecialty in India to better serve the nation's population.

Industry progress report on neuro-oncology: a biotech update.

PubMed

Haber, Jessica S; Banu, Matei A; Ray, Ashley; Kesavabhotla, Kartik; Boockvar, John A

2013-04-01

With steadily rising revenue and large numbers of clinical trials utilizing novel treatment strategies, the field of neuro-oncology is at the core of the growing cancer therapy industry. In June 2012, the Weill Cornell Brain and Tumor Center hosted the first Brain Tumor Biotech Summit as a forum for fostering and encouraging collaboration between researches and investors to accelerate novel treatments for brain cancer. This event brought together neuro-oncologists, neurosurgeons, academicians, entrepreneurs, non-profits, CEOs and investors in an attempt to bring innovative treatments and concepts to the fore. Specific subjects presented at the meeting included new surgical devices and delivery techniques, targeted therapeutics, immunotherapy, and stem cell biology. The mission of the summit was to provide opportunities for researchers in neuro-oncology to directly interact with leaders from the investment community with insight into the commercial aspects of our work. Our shared goal is to shorten the time for basic science ideas to be translated into the clinical setting. The following serves as a progress report on the biotech industry in neuro-oncology, as presented at the Brain Tumor Biotech Summit.

Neuro-Sweet disease: report of the first autopsy case.

PubMed

Kokubo, Yasumasa; Kuzuhara, Shigeki; Isoda, Kenichi; Sato, Kenji; Kawada, Norikazu; Narita, Yugo

2007-09-01

Neuro-Sweet disease is a rare condition of central nervous involvement accompanied by cutaneous Sweet lesions. Neuropathological changes in neuro-Sweet disease are unknown. To describe post-mortem findings of the first case of neuro-Sweet disease. A 44-year-old Japanese man developed recurrent episodes of cerebral and brainstem encephalitis with cutaneous Sweet lesions from the age of 34 years. His HLA typing was B54 and Cw1, and the symptoms and MRI abnormalities markedly subsided following corticosteroid therapy. Histologically, there were multiple lesions of perivascular cuffing of small venules by macrophages without vasculitis in the thalamus, temporal lobe, basal ganglia, pons, leptomeninges or ventricular ependym. The core neuropathological findings were: perivascular cuffing around particularly small veins; absence of granulomatous or necrotic angitis; mainly macrophage infiltration; and the thalamus being most affected. In the present case, the diagnosis of neuro-Sweet disease was made by skin biopsy 5 years after the onset of the central neuron system symptoms. We should pay more attention to skin lesions in steroid responsive recurrent encephalitis in patients who are HLA-B54 or Cw1 positive.

Myasthenic syndrome: effect of choline, plasmapheresis and tests for circulating factor.

PubMed Central

Kranz, H; Caddy, D J; Williams, A M; Gay, W

1980-01-01

In a patient with myasthenic syndrome neuro-muscular transmission was characterised by depression and facilitation. The relative extent of these two processes varied between muscles, and in the one muscle with time. Guanidine HCl treatment corrected the electrophysiological defect. Oral choline increased muscle action potential amplitude in response to single shocks. Intravenous choline produced features indicating cholinergic autonomic stimulation. Pimozide and plasmapheresis had no effect. Animal in-vivo and in-vitro studies performed to detect a circulating factor which interferes with neuro-muscular transmission were negative. PMID:6110709

Neuro-QoL health-related quality of life measurement system: Validation in Parkinson's disease.

PubMed

Nowinski, Cindy J; Siderowf, Andrew; Simuni, Tanya; Wortman, Catherine; Moy, Claudia; Cella, David

2016-05-01

Neuro-QoL is a multidimensional patient-reported outcome measurement system assessing aspects of physical, mental, and social health identified by neurology patients and caregivers as important. One of the first neurology-specific patient-reported outcome measure systems created using modern test development methods, Neuro-Qol enables brief, yet precise, assessment and the ability to conduct both PD-specific and cross-disease comparisons. We present results of Neuro-QoL clinical validation using a sample of PD patients. A total of 120 PD patients recruited from academic medical centers were assessed at baseline, 1 week, and 6 months. Assessments included Neuro-QoL and general and PD-specific validity measures. Participants were 62% male and 95% white (average age = 66); H & Y stages were 1 (16%), 2 (61%), 3 (18%), and 4 (5%). Internal consistency and test-retest reliability of Neuro-QoL ranged from Cronbach's alphas = 0.81 to 0.94 with intraclass correlation coefficients = 0.66 to 0.80. Pearson's correlations between Neuro-QoL and legacy measures were generally moderate and in expected directions. UPDRS Part 2 was moderately correlated with Neuro-QoL Upper Extremity and Mobility, respectively (r's = -0.44; -0.59). Parkinson's Disease Questionnaire-39 and Neuro-QoL measures of similar constructs showed strong-to-moderate correlations (r's = 0.70-0.44). Neuro-QoL measures of fatigue, mobility, positive emotion, and emotional/behavioral control showed responsiveness to self-reported change. Neuro-QoL is valid for use in PD clinical research. Reliability for all but two measures is sufficient for group comparisons, with some evidence supporting responsiveness to change. Neuro-QoL possesses characteristics, such as brevity, flexibility in administration, and suitability, for cross-disease comparisons that may be advantageous to users in a variety of settings. © 2016 Movement Disorder Society. © 2016 International Parkinson and Movement Disorder

Global Perspective of Novel Therapeutic Strategies for the Management of NeuroAIDS.

PubMed

Kumar, Swatantra; Maurya, Vimal K; Dandu, Himanshu R; Bhatt, Madan Lb; Saxena, Shailendra K

2018-05-08

Among Human immunodeficiency virus (HIV) infected individuals, around two-thirds of patients present with neuroAIDS, where HIV-associated neurocognitive disorders (HAND), and HIV-associated dementia (HAD) are the most prevailing neurological complications. The neuropathology of neuroAIDS can be characterized by the presence of HIV infected macrophages and microglia in the brain, with the formation of multinucleated giant cells. Global predominant subtypes of HIV-1 clade B and C infections influence the differential effect of immune and neuronal dysfunctions, leading to clade-specific clinical variation in neuroAIDS patient cohorts. Highly active antiretroviral therapy (HAART) enhances the survival rate among AIDS patients, but due to the inability to cross the Blood-Brain-Barrier (BBB), incidence of neuroAIDS during disease progression may be envisaged. The complex structure of blood-brain-barrier, and poor pharmacokinetic profile coupled with weak bio-distribution of antiretroviral drugs, are the principle barriers for the treatment of neuroAIDS. In the combined antiretroviral therapy (cART) era, the frequency of HAD has decreased; however the incidence of asymptomatic neurocognitive impairment (ANI) and minor neurocognitive disorder (MND) remains consistent. Therefore, several effective novel nanotechnology based therapeutic approaches have been developed to improve the availability of antiretroviral drugs in the brain for the management of neuroAIDS.

Three novel serum biomarkers, miR-1, miR-133a, and miR-206 for Limb-girdle muscular dystrophy, Facioscapulohumeral muscular dystrophy, and Becker muscular dystrophy.

PubMed

Matsuzaka, Yasunari; Kishi, Soichiro; Aoki, Yoshitsugu; Komaki, Hirofumi; Oya, Yasushi; Takeda, Shin-Ichi; Hashido, Kazuo

2014-11-01

Muscular dystrophies are a clinically and genetically heterogeneous group of inherited myogenic disorders. In clinical tests for these diseases, creatine kinase (CK) is generally used as diagnostic blood-based biomarker. However, because CK levels can be altered by various other factors, such as vigorous exercise, etc., false positive is observed. Therefore, three microRNAs (miRNAs), miR-1, miR-133a, and miR-206, were previously reported as alternative biomarkers for duchenne muscular dystrophy (DMD). However, no alternative biomarkers have been established for the other muscular dystrophies. We, therefore, evaluated whether these miR-1, miR-133a, and miR-206 can be used as powerful biomarkers using the serum from muscular dystrophy patients including DMD, myotonic dystrophy 1 (DM1), limb-girdle muscular dystrophy (LGMD), facioscapulohumeral muscular dystrophy (FSHD), becker muscular dystrophy (BMD), and distal myopathy with rimmed vacuoles (DMRV) by qualitative polymerase chain reaction (PCR) amplification assay. Statistical analysis indicated that all these miRNA levels in serum represented no significant differences between all muscle disorders examined in this study and controls by Bonferroni correction. However, some of these indicated significant differences without correction for testing multiple diseases (P < 0.05). The median values of miR-1 levels in the serum of patients with LGMD, FSHD, and BMD were approximately 5.5, 3.3 and 1.7 compared to that in controls, 0.68, respectively. Similarly, those of miR-133a and miR-206 levels in the serum of BMD patients were about 2.5 and 2.1 compared to those in controls, 1.03 and 1.32, respectively. Taken together, our data demonstrate that levels of miR-1, miR-133a, and miR-206 in serum of BMD and miR-1 in sera of LGMD and FSHD patients showed no significant differences compared with those of controls by Bonferroni correction. However, the results might need increase in sample sizes to evaluate these three miRNAs as

A Portable Ultrasound System for Non-Invasive Ultrasonic Neuro-Stimulation.

PubMed

Qiu, Weibao; Zhou, Juan; Chen, Yan; Su, Min; Li, Guofeng; Zhao, Huixia; Gu, Xianyi; Meng, De; Wang, Congzhi; Xiao, Yang; Lam, Kwok Ho; Dai, Jiyan; Zheng, Hairong

2017-12-01

Fundamental insights into the function of the neural circuits often follows from the advances in methodologies and tools for neuroscience. Electrode- and optical- based stimulation methods have been used widely for neuro-modulation with high resolution. However, they are suffering from inherent invasive surgical procedure. Ultrasound has been proved as a promising technology for neuro-stimulation in a non-invasive manner. However, no portable ultrasound system has been developed particularly for neuro-stimulation. The utilities used currently are assembled by traditional functional generator, power amplifier, and general transducer, therefore, resulting in lack of flexibility. This paper presents a portable system to achieve ultrasonic neuro-stimulation to satisfy various studies. The system incorporated a high voltage waveform generator and a matching circuit that were optimized for neuro-stimulation. A new switching mode power amplifier was designed and fabricated. The noise generated by the power amplifier was reduced (about 30 dB), and the size and weight were smaller in contrast with commercial equipment. In addition, a miniaturized ultrasound transducer was fabricated using Pb(Mg 1/3 Nb 2/3 )O 3 -PbTiO 3 (PMN-PT) 1-3 composite single crystal for the improved ultrasonic performance. The spatial peak temporal average pressure was higher than 250 kPa in the range of 0.5-5 MHz. In vitro and in vivo studies were conducted to show the performance of the system.

OpenCL Implementation of NeuroIsing

NASA Astrophysics Data System (ADS)

Zapart, C. A.

Recent advances in graphics card hardware combined with anintroduction of the OpenCL standard promise to accelerate numerical simulations across diverse scientific disciplines. One such field benefiting from new hardware/software paradigms is econophysics. The paper describes an OpenCL implementation of a selected econophysics model: NeuroIsing, which has been designed to execute in parallel on a vendor-independent graphics card. Originally introduced in the paper [C.~A.~Zapart, ``Econophysics in Financial Time Series Prediction'', PhD thesis, Graduate University for Advanced Studies, Japan (2009)], at first it was implemented on a CELL processor running inside a SONY PS3 games console. The NeuroIsing framework can be applied to predicting and trading foreign exchange as well as stock market index futures.

The role of ethics in data governance of large neuro-ICT projects.

PubMed

Stahl, Bernd Carsten; Rainey, Stephen; Harris, Emma; Fothergill, B Tyr

2018-05-14

We describe current practices of ethics-related data governance in large neuro-ICT projects, identify gaps in current practice, and put forward recommendations on how to collaborate ethically in complex regulatory and normative contexts. We undertake a survey of published principles of data governance of large neuro-ICT projects. This grounds an approach to a normative analysis of current data governance approaches. Several ethical issues are well covered in the data governance policies of neuro-ICT projects, notably data protection and attribution of work. Projects use a set of similar policies to ensure users behave appropriately. However, many ethical issues are not covered at all. Implementation and enforcement of policies remain vague. The data governance policies we investigated indicate that the neuro-ICT research community is currently close-knit and that shared assumptions are reflected in infrastructural aspects. This explains why many ethical issues are not explicitly included in data governance policies at present. With neuro-ICT research growing in scale, scope, and international involvement, these shared assumptions should be made explicit and reflected in data governance.

Translational Research for Muscular Dystrophy

DTIC Science & Technology

2014-05-01

year of work was awarded to allow completion of our transgenic analysis of Becker -like muscular dystrophy rescue experiments, allow completion of the D2... Dystrophy PRINCIPAL INVESTIGATOR: Gregory A. Cox, Ph.D. CONTRACTING ORGANIZATION: The Jackson Laboratory Bar Harbor, ME 04609-1523...April 2014 4. TITLE AND SUBTITLE Translational Research for Muscular Dystrophy 5a. CONTRACT NUMBER 5b. GRANT NUMBER W81XWH-11-1-0330 5c

The Neurologic Assessment in Neuro-Oncology (NANO) scale: a tool to assess neurologic function for integration into the Response Assessment in Neuro-Oncology (RANO) criteria.

PubMed

Nayak, Lakshmi; DeAngelis, Lisa M; Brandes, Alba A; Peereboom, David M; Galanis, Evanthia; Lin, Nancy U; Soffietti, Riccardo; Macdonald, David R; Chamberlain, Marc; Perry, James; Jaeckle, Kurt; Mehta, Minesh; Stupp, Roger; Muzikansky, Alona; Pentsova, Elena; Cloughesy, Timothy; Iwamoto, Fabio M; Tonn, Joerg-Christian; Vogelbaum, Michael A; Wen, Patrick Y; van den Bent, Martin J; Reardon, David A

2017-05-01

The Macdonald criteria and the Response Assessment in Neuro-Oncology (RANO) criteria define radiologic parameters to classify therapeutic outcome among patients with malignant glioma and specify that clinical status must be incorporated and prioritized for overall assessment. But neither provides specific parameters to do so. We hypothesized that a standardized metric to measure neurologic function will permit more effective overall response assessment in neuro-oncology. An international group of physicians including neurologists, medical oncologists, radiation oncologists, and neurosurgeons with expertise in neuro-oncology drafted the Neurologic Assessment in Neuro-Oncology (NANO) scale as an objective and quantifiable metric of neurologic function evaluable during a routine office examination. The scale was subsequently tested in a multicenter study to determine its overall reliability, inter-observer variability, and feasibility. The NANO scale is a quantifiable evaluation of 9 relevant neurologic domains based on direct observation and testing conducted during routine office visits. The score defines overall response criteria. A prospective, multinational study noted a >90% inter-observer agreement rate with kappa statistic ranging from 0.35 to 0.83 (fair to almost perfect agreement), and a median assessment time of 4 minutes (interquartile range, 3-5). The NANO scale provides an objective clinician-reported outcome of neurologic function with high inter-observer agreement. It is designed to combine with radiographic assessment to provide an overall assessment of outcome for neuro-oncology patients in clinical trials and in daily practice. Furthermore, it complements existing patient-reported outcomes and cognition testing to combine for a global clinical outcome assessment of well-being among brain tumor patients. © The Author(s) 2017. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions

Parental attitudes toward newborn screening for Duchenne/Becker muscular dystrophy and spinal muscular atrophy.

PubMed

Wood, Molly F; Hughes, Sarah C; Hache, Lauren P; Naylor, Edwin W; Abdel-Hamid, Hoda Z; Barmada, M Michael; Dobrowolski, Steven F; Stickler, David E; Clemens, Paula R

2014-06-01

Disease inclusion in the newborn screening (NBS) panel should consider the opinions of those most affected by the outcome of screening. We assessed the level and factors that affect parent attitudes regarding NBS panel inclusion of Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), and spinal muscular atrophy (SMA). The attitudes toward NBS for DMD, BMD, and SMA were surveyed and compared for 2 categories of parents, those with children affected with DMD, BMD, or SMA and expectant parents unselected for known family medical history. The level of support for NBS for DMD, BMD, and SMA was 95.9% among parents of children with DMD, BMD, or SMA and 92.6% among expectant parents. There was strong support for NBS for DMD, BMD, and SMA in both groups of parents. Given advances in diagnostics and promising therapeutic approaches, discussion of inclusion in NBS should continue. Copyright © 2013 Wiley Periodicals, Inc.

NeuroLOG: a community-driven middleware design.

PubMed

Montagnat, Johan; Gaignard, Alban; Lingrand, Diane; Rojas Balderrama, Javier; Collet, Philippe; Lahire, Philippe

2008-01-01

The NeuroLOG project designs an ambitious neurosciences middleware, gaining from many existing components and learning from past project experiences. It is targeting a focused application area and adopting a user-centric perspective to meet the neuroscientists expectations. It aims at fostering the adoption of HealthGrids in a pre-clinical community. This paper details the project's design study and methodology which were proposed to achieve the integration of heterogeneous site data schemas and the definition of a site-centric policy. The NeuroLOG middleware will bridge HealthGrid and local resources to match user desires to control their resources and provide a transitional model towards HealthGrids.

NeuroManager: a workflow analysis based simulation management engine for computational neuroscience

PubMed Central

Stockton, David B.; Santamaria, Fidel

2015-01-01

We developed NeuroManager, an object-oriented simulation management software engine for computational neuroscience. NeuroManager automates the workflow of simulation job submissions when using heterogeneous computational resources, simulators, and simulation tasks. The object-oriented approach (1) provides flexibility to adapt to a variety of neuroscience simulators, (2) simplifies the use of heterogeneous computational resources, from desktops to super computer clusters, and (3) improves tracking of simulator/simulation evolution. We implemented NeuroManager in MATLAB, a widely used engineering and scientific language, for its signal and image processing tools, prevalence in electrophysiology analysis, and increasing use in college Biology education. To design and develop NeuroManager we analyzed the workflow of simulation submission for a variety of simulators, operating systems, and computational resources, including the handling of input parameters, data, models, results, and analyses. This resulted in 22 stages of simulation submission workflow. The software incorporates progress notification, automatic organization, labeling, and time-stamping of data and results, and integrated access to MATLAB's analysis and visualization tools. NeuroManager provides users with the tools to automate daily tasks, and assists principal investigators in tracking and recreating the evolution of research projects performed by multiple people. Overall, NeuroManager provides the infrastructure needed to improve workflow, manage multiple simultaneous simulations, and maintain provenance of the potentially large amounts of data produced during the course of a research project. PMID:26528175

NeuroManager: a workflow analysis based simulation management engine for computational neuroscience.

PubMed

Stockton, David B; Santamaria, Fidel

2015-01-01

We developed NeuroManager, an object-oriented simulation management software engine for computational neuroscience. NeuroManager automates the workflow of simulation job submissions when using heterogeneous computational resources, simulators, and simulation tasks. The object-oriented approach (1) provides flexibility to adapt to a variety of neuroscience simulators, (2) simplifies the use of heterogeneous computational resources, from desktops to super computer clusters, and (3) improves tracking of simulator/simulation evolution. We implemented NeuroManager in MATLAB, a widely used engineering and scientific language, for its signal and image processing tools, prevalence in electrophysiology analysis, and increasing use in college Biology education. To design and develop NeuroManager we analyzed the workflow of simulation submission for a variety of simulators, operating systems, and computational resources, including the handling of input parameters, data, models, results, and analyses. This resulted in 22 stages of simulation submission workflow. The software incorporates progress notification, automatic organization, labeling, and time-stamping of data and results, and integrated access to MATLAB's analysis and visualization tools. NeuroManager provides users with the tools to automate daily tasks, and assists principal investigators in tracking and recreating the evolution of research projects performed by multiple people. Overall, NeuroManager provides the infrastructure needed to improve workflow, manage multiple simultaneous simulations, and maintain provenance of the potentially large amounts of data produced during the course of a research project.

Chips of Hope: Neuro-Electronic Hybrids for Brain Repair

NASA Astrophysics Data System (ADS)

Ben-Jacob, Eshel

2010-03-01

The field of Neuro-Electronic Hybrids kicked off 30 years ago when researchers in the US first tweaked the technology of recording and stimulation of networks of live neurons grown in a Petri dish and interfaced with a computer via an array of electrodes. Since then, many researchers have searched for ways to imprint in neural networks new ``memories" without erasing old ones. I will describe our new generation of Neuro-Electronic Hybrids and how we succeeded to turn them into the first learning Neurochips - memory and information processing chips made of live neurons. To imprint multiple memories in our new chip we used chemical stimulation at specific locations that were selected by analyzing the networks activity in real time according to our new information encoding principle. Currently we develop new-generation of neuro chips using special carbon nano tubes (CNT). These electrodes enable to engineer the networks topology and efficient electrical interfacing with the neurons. This advance bears the promise to pave the way for building a new experimental platform for testing new drugs and developing new methods for neural networks repair and regeneration. Looking into the future, the development brings us a step closer towards the dream of Brain Repair by implementable Neuro-Electronic hybrid chips.

Physical Therapy and Facioscapulohumeral Muscular Dystrophy (FSHD)

MedlinePlus

... 43. 2. The FSH‐DY Group. A prospective, quantitative study of the natural history of facioscapulohumeral muscular ... regarding facioscapulohumeral muscular dystrophy (FSHD). It actively promotes research toward the prevention, cause and treatment of FSHD. ...

A Case of Neuro-Behcet's Disease Presenting with Central Neurogenic Hyperventilation.

PubMed

Alkhachroum, Ayham M; Saeed, Saba; Kaur, Jaspreet; Shams, Tanzila; DeGeorgia, Michael A

2016-03-11

Behcet's disease is a chronic inflammatory disorder usually characterized by the triad of oral ulcers, genital ulcers, and uveitis. Central to the pathogenesis of Behcet's disease is an autoimmune vasculitis. Neurological involvement, so called "Neuro-Behcet's disease", occurs in 10-20% of patients, usually from a meningoencephalitis or venous thrombosis. We report the case of a 46-year-old patient with Neuro-Behcet's disease who presented with central neurogenic hyperventilation as a result of brainstem involvement from venulitis. To the best of our knowledge, central neurogenic hyperventilation has not previously been described in a patient with Neuro-Behcet's disease.

[Myostatin blockade therapy for muscular atrophy].

PubMed

Sunada, Yoshihide

2011-11-01

Myostatin, a member of the muscle-specific transforming growth factor (TGF)-β family, negatively regulates skeletal muscle growth. It inhibits muscle stem cell proliferation and differentiation and attenuates adult muscle fiber protein accretion, resulting in decreased skeletal muscle mass. As such, it has been considered a therapeutic target of muscular dystrophy. Notably, administration of a blocking antibody against myostatin ameliorated the pathophysiology of dystrophin-deficient mdx mice. Although a clinical trial of anti-myostatin antibody MYO-029 failed to achieve a significant outcome in patients with muscular dystrophies, various distinct approaches have been taken to establish anti-myostatin therapy, including myostatin decoy receptor ACE-031, small-molecule inhibitors against the myostatin receptor, and myostatin short intertering RNA with collagen-derived carrier particles. The clinical application of anti-myostatin therapeutics in treatment of patients with muscular dystrophy needs further evaluation for safety and specification of the target disease types among the various muscular dystrophies. In addition, myostatin inhibition could be effective for muscle-wasting conditions other than muscular dystrophy- for instance, steroid-induced myopathy, mitochondrial myopathy, or sarcopenia in elderly patients. Moreover, considerable evidence shows that myostatin regulates energy metabolism and that its inhibition can significantly attenuate the progression of obesity and diabetes. It may also be applicable for the prevention of metabolic syndrome. Thus, safe and potent anti-myostatin therapy will have a wide variety of applications in modern medicine.

Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet): case definition in surveillance for childhood-onset Duchenne/Becker muscular dystrophy.

PubMed

Mathews, Katherine D; Cunniff, Chris; Kantamneni, Jiji R; Ciafaloni, Emma; Miller, Timothy; Matthews, Dennis; Cwik, Valerie; Druschel, Charlotte; Miller, Lisa; Meaney, F John; Sladky, John; Romitti, Paul A

2010-09-01

The Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet) is a multisite collaboration to determine the prevalence of childhood-onset Duchenne/Becker muscular dystrophy and to characterize health care and health outcomes in this population. MD STARnet uses medical record abstraction to identify patients with Duchenne/Becker muscular dystrophy born January 1, 1982 or later who resided in 1 of the participating sites. Critical diagnostic elements of each abstracted record are reviewed independently by >4 clinicians and assigned to 1 of 6 case definition categories (definite, probable, possible, asymptomatic, female, not Duchenne/Becker muscular dystrophy) by consensus. As of November 2009, 815 potential cases were reviewed. Of the cases included in analysis, 674 (82%) were either ''definite'' or ''probable'' Duchenne/Becker muscular dystrophy. These data reflect a change in diagnostic testing, as case assignment based on genetic testing increased from 67% in the oldest cohort (born 1982-1987) to 94% in the cohort born 2004 to 2009.

Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet): Case Definition in Surveillance for Childhood-Onset Duchenne/Becker Muscular Dystrophy

PubMed Central

Mathews, Katherine D.; Cunniff, Chris; Kantamneni, Jiji R.; Ciafaloni, Emma; Miller, Timothy; Matthews, Dennis; Cwik, Valerie; Druschel, Charlotte; Miller, Lisa; Meaney, F. John; Sladky, John; Romitti, Paul A.

2013-01-01

The Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet) is a multisite collaboration to determine the prevalence of childhood-onset Duchenne/Becker muscular dystrophy and to characterize health care and health outcomes in this population. MD STARnet uses medical record abstraction to identify patients with Duchenne/Becker muscular dystrophy born January 1, 1982 or later who resided in one of the participating sites. Critical diagnostic elements of each abstracted record are reviewed independently by ≥4 clinicians and assigned to 1 of 6 case definition categories (definite, probable, possible, asymptomatic, female, not Duchenne/Becker muscular dystrophy) by consensus. As of November 2009, 815 potential cases were reviewed. Of the cases included in analysis, 674 (82%) were either “definite” or “probable” Duchenne/Becker muscular dystrophy. These data reflect a change in diagnostic testing, as case assignment based on genetic testing increased from 67% in the oldest cohort (born 1982–1987) to 94% in the cohort born 2004–2009. PMID:20817884

A heterozygous 21-bp deletion in CAPN3 causes dominantly inherited limb girdle muscular dystrophy.

PubMed

Vissing, John; Barresi, Rita; Witting, Nanna; Van Ghelue, Marijke; Gammelgaard, Lise; Bindoff, Laurence A; Straub, Volker; Lochmüller, Hanns; Hudson, Judith; Wahl, Christoph M; Arnardottir, Snjolaug; Dahlbom, Kathe; Jonsrud, Christoffer; Duno, Morten

2016-08-01

Limb girdle muscular dystrophy type 2A is the most common limb girdle muscular dystrophy form worldwide. Although strict recessive inheritance is assumed, patients carrying a single mutation in the calpain 3 gene (CAPN3) are reported. Such findings are commonly attributed to incomplete mutation screening. In this investigation, we report 37 individuals (age range: 21-85 years, 21 females and 16 males) from 10 families in whom only one mutation in CAPN3 could be identified; a 21-bp, in-frame deletion (c.643_663del21). This mutation co-segregated with evidence of muscle disease and autosomal dominant transmission in several generations. Evidence of muscle disease was indicated by muscle pain, muscle weakness and wasting, significant fat replacement of muscles on imaging, myopathic changes on muscle biopsy and loss of calpain 3 protein on western blotting. Thirty-one of 34 patients had elevated creatine kinase or myoglobin. Muscle weakness was generally milder than observed in limb girdle muscular dystrophy type 2A, but affected the same muscle groups (proximal leg, lumbar paraspinal and medial gastrocnemius muscles). In some cases, the weakness was severely disabling. The 21-bp deletion did not affect mRNA maturation. Calpain 3 expression in muscle, assessed by western blot, was below 15% of normal levels in the nine mutation carriers in whom this could be tested. Haplotype analysis in four families from three different countries suggests that the 21-bp deletion is a founder mutation. This study provides strong evidence that heterozygosity for the c.643_663del21 deletion in CAPN3 results in a dominantly inherited muscle disease. The normal expression of mutated mRNA and the severe loss of calpain 3 on western blotting, suggest a dominant negative effect with a loss-of-function mechanism affecting the calpain 3 homodimer. This renders patients deficient in calpain 3 as in limb girdle muscular dystrophy type 2A, albeit in a milder form in most cases. Based on findings

Oxidative muscular injury and its relevance to hyperthyroidism.

PubMed

Asayama, K; Kato, K

1990-01-01

In experimental hyperthyroidism, acceleration of lipid peroxidation occurs in heart and slow-oxidative muscles, suggesting the contribution of reactive oxygen species to the muscular injury caused by thyroid hormones. This article reviews various models of oxidative muscular injury and considers the relevance of the accompanying metabolic derangements to thyrotoxic myopathy and cardiomyopathy, which are the major complications of hyperthyroidism. The muscular injury models in which reactive oxygen species are supposed to play a role are ischemia/reperfusion syndrome, exercise-induced myopathy, heart and skeletal muscle diseases related to the nutritional deficiency of selenium and vitamin E and related disorders, and genetic muscular dystrophies. These models provide evidence that mitochondrial function and the glutathione-dependent antioxidant system are important for the maintenance of the structural and functional integrity of muscular tissues. Thyroid hormones have a profound effect on mitochondrial oxidative activity, synthesis and degradation of proteins and vitamin E, the sensitivity of the tissues to catecholamine, the differentiation of muscle fibers, and the levels of antioxidant enzymes. The large volume of circumstantial evidence presented here indicates that hyperthyroid muscular tissues undergo several biochemical changes that predispose them to free radical-mediated injury.

Genetic Modifiers of Duchenne and Facioscapulohumeral Muscular Dystrophies

PubMed Central

Hightower, Rylie M.; Alexander, Matthew S.

2017-01-01

Muscular dystrophy is defined as the progressive wasting of skeletal muscles that is caused by inherited or spontaneous genetic mutations. Next-generation sequencing (NGS) has greatly improved the accuracy and speed of diagnosis for different types of muscular dystrophy. Advancements in depth of coverage, convenience, and overall reduced cost, have led to the identification of genetic modifiers that are responsible for phenotypic variability in affected patients. These genetic modifiers have been postulated to explain key differences in disease phenotypes including age of loss of ambulation, steroid-responsiveness, and the presence or absence of cardiac defects in patients with the same form of muscular dystrophy. Here we review and highlight recent findings on genetic modifiers of Duchenne and Facioscapulohumeral muscular dystrophies based on animal and clinical studies. These genetic modifiers hold great promise to be developed into novel therapeutic targets for the treatment of muscular dystrophies. PMID:28877560

The NeuroAiD Safe Treatment (NeST) Registry: a protocol

PubMed Central

Venketasubramanian, Narayanaswamy; Kumar, Ramesh; Soertidewi, Lyna; Abu Bakar, Azizi; Laik, Carine; Gan, Robert

2015-01-01

Introduction NeuroAiD (MLC601, MLC901), a combination of natural products, has been shown to be safe and to aid neurological recovery after brain injuries. The NeuroAiD Safe Treatment (NeST) Registry aims to assess its use and safety in the real-world setting. Methods and analysis The NeST Registry is designed as a product registry that would provide information on the use and safety of NeuroAiD in clinical practice. An online NeST Registry was set up to allow easy entry and retrieval of essential information including demographics, medical conditions, clinical assessments of neurological, functional and cognitive state, compliance, concomitant medications, and side effects, if any, among patients on NeuroAiD. Patients who are taking or have been prescribed NeuroAiD may be included. Participation is voluntary. Data collected are similar to information obtained during standard care and are prospectively entered by the participating physicians at baseline (before initialisation of NeuroAiD) and during subsequent visits. The primary outcome assessed is safety (ie, non-serious and serious adverse event), while compliance and neurological status over time are secondary outcomes. The in-person follow-up assessments are timed with clinical appointments. Anonymised data will be extracted and collectively analysed. Initial target sample size for the registry is 2000. Analysis will be performed after every 500 participants entered with completed follow-up information. Ethics and dissemination Doctors who prescribe NeuroAiD will be introduced to the registry by local partners. The central coordinator of the registry will discuss the protocol and requirements for implementation with doctors who show interest. Currently, the registry has been approved by the Ethics Committees of Universiti Kebangsaan Malaysia (Malaysia) and National Brain Center (Indonesia). In addition, for other countries, Ethics Committee approval will be obtained in accordance with local requirements. Trial

Reversible neuronal and muscular toxicity of caffeine in developing vertebrates.

PubMed

Rodriguez, Rufino S; Haugen, Rebecca; Rueber, Alexandra; Huang, Cheng-Chen

2014-06-01

This study utilizes zebrafish embryos to understand the cellular and molecular mechanisms of caffeine toxicity in developing vertebrate embryos. By using a high concentration of caffeine, we observed almost all the phenotypes that have been described in humans and/or in other animal models, including neural tube closure defect, jittery, touch insensitivity, and growth retardation as well as a drastic coiled body phenotype. Zebrafish embryos exposed to 5mM caffeine exhibited high frequent movement, 10 moves/min comparing with around 3 moves/min in control embryos, within half an hour post exposure (HPE). They later showed twitching, uncoordinated movement, and eventually severe body curvature by 6HPE. Exposure at later stages resulted in the same phenotypes but more posteriorly. Surprisingly, when caffeine was removed before 6HPE, the embryos were capable of recovering but still exhibited mild curvature and shorter bodies. Longer exposure caused irreversible body curvature and lethality. These results suggest that caffeine likely targets the neuro-muscular physiology in developing embryos. Immunohistochemistry revealed that the motorneurons in treated embryos developed shorter axons, abnormal branching, and excessive synaptic vesicles. Developing skeletal muscles also appeared smaller and lacked the well-defined boundaries seen in control embryos. Finally, caffeine increases the expression of genes involved in synaptic vesicle migration. In summary, our results provide molecular understanding of caffeine toxicity on developing vertebrate embryos. Published by Elsevier Inc.

Functional coordination of muscles underlying changes in behavioural dynamics.

PubMed

Vernooij, Carlijn A; Rao, Guillaume; Perdikis, Dionysios; Huys, Raoul; Jirsa, Viktor K; Temprado, Jean-Jacques

2016-06-10

The dynamical systems approach addresses Bernstein's degrees of freedom problem by assuming that the neuro-musculo-skeletal system transiently assembles and dismantles its components into functional units (or synergies) to meet task demands. Strikingly, little is known from a dynamical point of view about the functioning of the muscular sub-system in this process. To investigate the interaction between the dynamical organisation at muscular and behavioural levels, we searched for specific signatures of a phase transition in muscular coordination when a transition is displayed at the behavioural level. Our results provide evidence that, during Fitts' task when behaviour switches to a different dynamical regime, muscular activation displays typical signatures of a phase transition; a reorganisation in muscular coordination patterns accompanied by a peak in the variability of muscle activation. This suggests that consistent changes occur in coordination processes across the different levels of description (i.e., behaviour and muscles). Specifically, in Fitts' task, target size acts as a control parameter that induces a destabilisation and a reorganisation of coordination patterns at different levels of the neuro-musculo-skeletal system.

Threatened masculinity and muscularity: an experimental examination of multiple aspects of muscularity in men.

PubMed

Hunt, Christopher John; Gonsalkorale, Karen; Murray, Stuart B

2013-06-01

Two studies examined the threatened masculinity theory of male body dissatisfaction, which posits that threats to masculinity result in increased muscle dissatisfaction. In Study 1, a masculinity threat was followed by tasks examining confidence in physical ability and perceptions of current and ideal body shapes. Results showed that men who experienced a masculinity threat reported lower confidence in their physical ability and perceived themselves as less muscular than men who experienced an affirmation of their masculinity. In Study 2, men were asked to report their intention to increase muscularity and their appearance anxiety following a threat to masculinity. Results showed that men reported lower appearance anxiety and drive for muscularity when their masculinity was threatened than when their masculinity was affirmed. This apparent contradiction can be explained by noting that men may be motivated to deny appearance concerns following a threat to masculinity, as such concerns are equated with femininity. Copyright © 2013. Published by Elsevier Ltd.

9 CFR 311.35 - Muscular inflammation, degeneration, or infiltration.

Code of Federal Regulations, 2011 CFR

2011-01-01

... 9 Animals and Animal Products 2 2011-01-01 2011-01-01 false Muscular inflammation, degeneration, or infiltration. 311.35 Section 311.35 Animals and Animal Products FOOD SAFETY AND INSPECTION SERVICE... PARTS § 311.35 Muscular inflammation, degeneration, or infiltration. (a) If muscular lesions are found...

9 CFR 311.35 - Muscular inflammation, degeneration, or infiltration.

Code of Federal Regulations, 2014 CFR

2014-01-01

... 9 Animals and Animal Products 2 2014-01-01 2014-01-01 false Muscular inflammation, degeneration, or infiltration. 311.35 Section 311.35 Animals and Animal Products FOOD SAFETY AND INSPECTION SERVICE... PARTS § 311.35 Muscular inflammation, degeneration, or infiltration. (a) If muscular lesions are found...

9 CFR 311.35 - Muscular inflammation, degeneration, or infiltration.

Code of Federal Regulations, 2012 CFR

2012-01-01

... 9 Animals and Animal Products 2 2012-01-01 2012-01-01 false Muscular inflammation, degeneration, or infiltration. 311.35 Section 311.35 Animals and Animal Products FOOD SAFETY AND INSPECTION SERVICE... PARTS § 311.35 Muscular inflammation, degeneration, or infiltration. (a) If muscular lesions are found...

9 CFR 311.35 - Muscular inflammation, degeneration, or infiltration.

Code of Federal Regulations, 2010 CFR

2010-01-01

... 9 Animals and Animal Products 2 2010-01-01 2010-01-01 false Muscular inflammation, degeneration, or infiltration. 311.35 Section 311.35 Animals and Animal Products FOOD SAFETY AND INSPECTION SERVICE... PARTS § 311.35 Muscular inflammation, degeneration, or infiltration. (a) If muscular lesions are found...

9 CFR 311.35 - Muscular inflammation, degeneration, or infiltration.

Code of Federal Regulations, 2013 CFR

2013-01-01

... 9 Animals and Animal Products 2 2013-01-01 2013-01-01 false Muscular inflammation, degeneration, or infiltration. 311.35 Section 311.35 Animals and Animal Products FOOD SAFETY AND INSPECTION SERVICE... PARTS § 311.35 Muscular inflammation, degeneration, or infiltration. (a) If muscular lesions are found...

Muscular Subaortic Stenosis

PubMed Central

Wigle, E. Douglas; Auger, Pierre; Marquis, Yves

1966-01-01

Two types of intraventricular pressure differences within the left ventricle of man are described. The first is encountered in cases of muscular (or fibrous) subaortic stenosis, in which the outflow tract pressure distal to the stenosis (and proximal to the aortic valve) is low, whereas all pressures recorded in the left ventricle proximal to the stenosis, including that just inside the mitral valve (the initial inflow tract pressure) are high. The second type of intraventricular pressure difference may be recorded in patients without muscular subaortic stenosis when a heart catheter is advanced to the left ventricular wall in such a manner that it becomes imbedded or entrapped by cardiac muscle in systole. Such an entrapped catheter records a high intraventricular pressure that is believed to reflect intramyocardial tissue pressure, which normally exceeds intracavitary pressure. In such cases the initial inflow tract pressure is not high and is precisely equal to the outflow tract systolic pressure, i.e. both are recording intracavity pressure. This type of intramyocardial to intracavitary pressure difference may also be encountered in the left ventricle of dogs. The recent suggestion that intraventricular pressure differences in the left ventricle of cases of muscular subaortic stenosis are due to catheter entrapment by cardiac muscle is refuted by using the initial inflow tract pressure as the means of differentiation between the two types of intraventricular pressure differences outlined. PMID:5951625

Genetic modifiers of Duchenne and facioscapulohumeral muscular dystrophies.

PubMed

Hightower, Rylie M; Alexander, Matthew S

2018-01-01

Muscular dystrophy is defined as the progressive wasting of skeletal muscles that is caused by inherited or spontaneous genetic mutations. Next-generation sequencing has greatly improved the accuracy and speed of diagnosis for different types of muscular dystrophy. Advancements in depth of coverage, convenience, and overall reduced cost have led to the identification of genetic modifiers that are responsible for phenotypic variability in affected patients. These genetic modifiers have been postulated to explain key differences in disease phenotypes, including age of loss of ambulation, steroid responsiveness, and the presence or absence of cardiac defects in patients with the same form of muscular dystrophy. This review highlights recent findings on genetic modifiers of Duchenne and facioscapulohumeral muscular dystrophies based on animal and clinical studies. These genetic modifiers hold great promise to be developed into novel therapeutic targets for the treatment of muscular dystrophies. Muscle Nerve 57: 6-15, 2018. © 2017 Wiley Periodicals, Inc.

Severe panuveitis in neuro-Behçet's disease in Malaysia: a case series.

PubMed

Othman, Khairuddin; Liza-Sharmini, Ahmad Tajudin; Ibrahim, Mohtar; Tharakan, John; Yanai, Ryoji; Zunaina, Embong

2017-01-01

Behçet's disease (BD) is a multisystemic disease that is very rare in Malaysia. About 5% of patients develop central nervous system involvement, termed neuro-Behçet's. Neuro-Behçet's is one of the most serious causes of long-term morbidity and mortality. We report two cases of neuro-Behçet's associated with uveitis (ocular BD) highlighting the clinical presentation, diagnostic measurement, and therapeutic management of these cases.

Lin4Neuro: a customized Linux distribution ready for neuroimaging analysis

PubMed Central

2011-01-01

Background A variety of neuroimaging software packages have been released from various laboratories worldwide, and many researchers use these packages in combination. Though most of these software packages are freely available, some people find them difficult to install and configure because they are mostly based on UNIX-like operating systems. We developed a live USB-bootable Linux package named "Lin4Neuro." This system includes popular neuroimaging analysis tools. The user interface is customized so that even Windows users can use it intuitively. Results The boot time of this system was only around 40 seconds. We performed a benchmark test of inhomogeneity correction on 10 subjects of three-dimensional T1-weighted MRI scans. The processing speed of USB-booted Lin4Neuro was as fast as that of the package installed on the hard disk drive. We also installed Lin4Neuro on a virtualization software package that emulates the Linux environment on a Windows-based operation system. Although the processing speed was slower than that under other conditions, it remained comparable. Conclusions With Lin4Neuro in one's hand, one can access neuroimaging software packages easily, and immediately focus on analyzing data. Lin4Neuro can be a good primer for beginners of neuroimaging analysis or students who are interested in neuroimaging analysis. It also provides a practical means of sharing analysis environments across sites. PMID:21266047

Lin4Neuro: a customized Linux distribution ready for neuroimaging analysis.

PubMed

Nemoto, Kiyotaka; Dan, Ippeita; Rorden, Christopher; Ohnishi, Takashi; Tsuzuki, Daisuke; Okamoto, Masako; Yamashita, Fumio; Asada, Takashi

2011-01-25

A variety of neuroimaging software packages have been released from various laboratories worldwide, and many researchers use these packages in combination. Though most of these software packages are freely available, some people find them difficult to install and configure because they are mostly based on UNIX-like operating systems. We developed a live USB-bootable Linux package named "Lin4Neuro." This system includes popular neuroimaging analysis tools. The user interface is customized so that even Windows users can use it intuitively. The boot time of this system was only around 40 seconds. We performed a benchmark test of inhomogeneity correction on 10 subjects of three-dimensional T1-weighted MRI scans. The processing speed of USB-booted Lin4Neuro was as fast as that of the package installed on the hard disk drive. We also installed Lin4Neuro on a virtualization software package that emulates the Linux environment on a Windows-based operation system. Although the processing speed was slower than that under other conditions, it remained comparable. With Lin4Neuro in one's hand, one can access neuroimaging software packages easily, and immediately focus on analyzing data. Lin4Neuro can be a good primer for beginners of neuroimaging analysis or students who are interested in neuroimaging analysis. It also provides a practical means of sharing analysis environments across sites.

Cardiac treatment in neuro-muscular diseases.

PubMed

Nigro, G; Politano, L; Passamano, L; Palladino, A; De Luca, F; Nigro, Ge; Comi, L I

2006-12-01

The authors have been treating heart involvement in muscle dystrophy since 1978. However, this study aimed to define recent therapeutic protocols, evaluating the results of cardiac treatment, performed between 1st February 2004 and 31st July, 2006. In this period, 100 Becker, 136 Duchenne, 44 Limb-girdle and 116 Steinert patients were treated. In that same period, a large group of MD patients refusing cardiac therapy have also been followed. All patients had previously been classified in the appropriate stage of cardiomyopathy and examined at least twice every year and even every week if presenting heart failure. The results show the usefulness of the recent protocols of treatment of cardiac involvement in muscle dystrophy patients.

Adaptive Neuro-Fuzzy Modeling of UH-60A Pilot Vibration

NASA Technical Reports Server (NTRS)

Kottapalli, Sesi; Malki, Heidar A.; Langari, Reza

2003-01-01

Adaptive neuro-fuzzy relationships have been developed to model the UH-60A Black Hawk pilot floor vertical vibration. A 200 point database that approximates the entire UH-60A helicopter flight envelope is used for training and testing purposes. The NASA/Army Airloads Program flight test database was the source of the 200 point database. The present study is conducted in two parts. The first part involves level flight conditions and the second part involves the entire (200 point) database including maneuver conditions. The results show that a neuro-fuzzy model can successfully predict the pilot vibration. Also, it is found that the training phase of this neuro-fuzzy model takes only two or three iterations to converge for most cases. Thus, the proposed approach produces a potentially viable model for real-time implementation.

Induction of neural differentiation by electrically stimulated gene expression of NeuroD2.

PubMed

Mie, Masayasu; Endoh, Tamaki; Yanagida, Yasuko; Kobatake, Eiry; Aizawa, Masuo

2003-02-13

Regulation of cell differentiation is an important assignment for cellular engineering. One of the techniques for regulation is gene transfection into undifferentiated cells. Transient expression of NeuroD2, one of neural bHLH transcription factors, converted mouse N1E-115 neuroblastoma cells into differentiated neurons. The regulation of neural bHLH expression should be a novel strategy for cell differentiation. In this study, we tried to regulate neural differentiation by NeuroD2 gene inserted under the control of heat shock protein-70 (HSP) promoter, which can be activated by electrical stimulation. Mouse neuroblastoma cell line, N1E-115, was stably transfected with expression vector containing mouse NeuroD2 cDNA under HSP promoter. Transfected cells were cultured on the electrode surface and applied electrical stimulation. After stimulation, NeuroD2 expression was induced, and transfected cells adopt a neuronal morphology at 3 days after stimulation. These results suggest that neural differentiation can be induced by electrically stimulated gene expression of NeuroD2.

Symptomatic muscular sarcoidosis: Lessons from a nationwide multicenter study.

PubMed

Cohen Aubart, Fleur; Abbara, Salam; Maisonobe, Thierry; Cottin, Vincent; Papo, Thomas; Haroche, Julien; Mathian, Alexis; Pha, Micheline; Gilardin, Laurent; Hervier, Baptiste; Soussan, Michael; Morlat, Philippe; Nunes, Hilario; Benveniste, Olivier; Amoura, Zahir; Valeyre, Dominique

2018-05-01

To describe clinicopathologic features of muscular sarcoidosis and the associated sarcoidosis phenotype through a nationwide multicenter study. Patients were included if they had histologically proven sarcoidosis and symptomatic muscular involvement confirmed by biological, imaging, or histologic examinations. Forty-eight patients (20 males) were studied, with a median age at muscular symptoms onset of 45 years (range 18-71). Four patterns were identified: a nodular pattern (27%); smoldering phenotype (29%); acute, subacute, or progressive myopathic type (35%); and combined myopathic and neurogenic pattern (10%). In all patterns, sarcoidosis was multivisceral with a median of 3 extramuscular organs involved (mostly lungs, lymph nodes, eyes, and skin) and a prolonged course with long-term use of corticosteroids and immunosuppressive drugs. Muscular patterns differed according to clinical presentation (myalgia, nodules, or weakness), electromyographic findings, muscular MRI, and response to sarcoidosis treatment. The myopathic and neuromuscular patterns were more severe. This nationwide study of muscular sarcoidosis allowed the identification of 4 patterns of granulomatous myositis, which differed by phenotypes and the clinical course.

Pain in adolescents with spinal muscular atrophy and Duchenne and Becker muscular dystrophy.

PubMed

Lager, Christina; Kroksmark, Anna-Karin

2015-09-01

The purpose of this study was to explore the prevalence, nature and scope of pain in adolescents with spinal muscular atrophy and Duchenne and Becker muscular dystrophy and whether the pain differs between diagnostic groups or between adolescents with different ambulation status. Furthermore to study the consequences of pain and to identify pain-exacerbating and pain-relieving factors. In a national survey, fifty-five adolescents with spinal muscular atrophy and dystrophinopathy completed a questionnaire assessing pain frequency, duration, location using a body map, intensity and discomfort using visual analogue scales, pain interference using a modified version of Brief Pain Inventory and factors exacerbating and relieving pain. Sixty-nine per cent of the adolescents reported pain during the past three months and 50% reported chronic pain. The pain prevalence did not differ significantly between diagnostic groups or between ambulators and non-ambulators. The average pain intensity was graded as mild and the worst pain as moderate. The pain typically occurred weekly, most frequently in the neck/back or legs. General activity and mood were the areas that were most affected by pain. Common pain-exacerbating factors were sitting, too much movement/activity and being lifted or transferred. Pain is a frequent problem in adolescents with spinal muscular atrophy and dystrophinopathy. The assessments used enable an understanding both of the nature and scope of pain and of the impact of pain in everyday life. The study highlights the importance of assessing pain in a systematic manner and offering an individual approach to interventions designed to reduce pain in this population. Copyright © 2015 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

A comparison of swallowing dysfunction in Becker muscular dystrophy and Duchenne muscular dystrophy.

PubMed

Yamada, Yuka; Kawakami, Michiyuki; Wada, Ayako; Otsuka, Tomoyoshi; Muraoka, Kaori; Liu, Meigen

2018-06-01

Swallowing dysfunction has been reported in Duchenne muscular dystrophy (DMD), but has not been studied in Becker muscular dystrophy (BMD). The aims of this study were to report the characteristics of swallowing dysfunction in BMD compared with DMD. The study participants were 18 patients with BMD and 18 patients with DMD. All the patients were examined using videofluorography during swallowing of 5 mL of fluid. The penetration-aspiration scale (P-A scale) and the videofluorographic dysphagia scale (VDS) were used to evaluate dysphagia. Swinyard functional ability stage was not significantly different between the BMD and DMD groups. Rate of aspiration, P-A scale score, and total VDS score did not differ across groups, but the VDS item score for laryngeal elevation was lower in the BMD group than in the DMD group (median scores 4.5 and 9, respectively; p < 0.001). In the BMD group, total VDS score significantly correlated with Swinyard stage (r = 0.78, p < 0.001), but not with age or lung function. Patients with BMD have swallowing problems similar to those observed in patients with DMD when matched according to physical functional status. These patients should be evaluated and followed-up for the duration of their disease. Implications for rehabiliation Dysphagia is one of the most critical problems in patients with progressive neuromuscular disease but dysphagia in patients with Becker muscular dystrophy (BMD) was not well known. Eighteen patients with BMD and 18 patients with Duchenne muscular dystrophy were examined with videofluorography. Patients with BMD have swallowing problems similar to those observed in patients with DMD.

Comparison of Automated Brain Volume Measures obtained with NeuroQuant and FreeSurfer.

PubMed

Ochs, Alfred L; Ross, David E; Zannoni, Megan D; Abildskov, Tracy J; Bigler, Erin D

2015-01-01

To examine intermethod reliabilities and differences between FreeSurfer and the FDA-cleared congener, NeuroQuant, both fully automated methods for structural brain MRI measurements. MRI scans from 20 normal control subjects, 20 Alzheimer's disease patients, and 20 mild traumatically brain-injured patients were analyzed with NeuroQuant and with FreeSurfer. Intermethod reliability was evaluated. Pairwise correlation coefficients, intraclass correlation coefficients, and effect size differences were computed. NeuroQuant versus FreeSurfer measures showed excellent to good intermethod reliability for the 21 regions evaluated (r: .63 to .99/ICC: .62 to .99/ES: -.33 to 2.08) except for the pallidum (r/ICC/ES = .31/.29/-2.2) and cerebellar white matter (r/ICC/ES = .31/.31/.08). Volumes reported by NeuroQuant were generally larger than those reported by FreeSurfer with the whole brain parenchyma volume reported by NeuroQuant 6.50% larger than the volume reported by FreeSurfer. There was no systematic difference in results between the 3 subgroups. NeuroQuant and FreeSurfer showed good to excellent intermethod reliability in volumetric measurements for all brain regions examined with the only exceptions being the pallidum and cerebellar white matter. This finding was robust for normal individuals, patients with Alzheimer's disease, and patients with mild traumatic brain injury. Copyright © 2015 by the American Society of Neuroimaging.

Muscular contraction stimulates posterior hypothalamic neurons.

PubMed

Waldrop, T G; Stremel, R W

1989-02-01

Recent studies have suggested that the subthalamic locomotor region (STLR) of the posterior hypothalamus is involved in modulating cardiorespiratory responses to feedback from contracting muscles. The purpose of this study was to determine whether neurons in this hypothalamic region alter their discharge frequency during contraction of hindlimb muscles. Stainless steel electrodes were used to record single-unit activity of STLR neurons during static and rhythmic contractions of hindlimb muscles in anesthetized cats. Recordings were also made from neurons in areas outside but surrounding the subthalamic locomotor region. Contraction of the triceps surae muscles was induced by stimulation of the peripheral cut ends of the L7 and S1 ventral roots. Both static and rhythmic contractions of the triceps surae evoked an increase in the discharge rate of the majority of the STLR cells studied. Two types of excitatory responses were observed: 1) abrupt increases in discharge frequency at the onset of muscular contraction and 2) a delayed more gradual increase in firing. Most of the cells that responded to muscular contraction could be activated by mechanical probing of the triceps surae muscles. However, the changes in discharge frequency were unrelated to changes in arterial pressure occurring during muscular contraction. Most of the neurons located outside the STLR were slightly inhibited by or did not respond to muscular contraction. Thus input from contracting muscles exerts predominantly an excitatory effect on neurons in the posterior hypothalamus. These results are consistent with other studies which have concluded that this hypothalamic site is involved in influencing the cardiorespiratory responses to muscular contraction.

Clinical neuro-oncology formal education opportunities for medical students in the United States and Canada.

PubMed

Dixit, Karan S; Nicholas, Martin Kelly; Lukas, Rimas V

2014-12-01

To develop an understanding of the availability of the formal clinical neuro-oncology educational opportunities for medical students. The curriculum websites of all medical schools accredited by the Liaison Committee on Medical Education were reviewed for the presence of clinical neuro-oncology electives as well as other relevant data. Ten (6.8%) of medical schools accredited by the Liaison Committee on Medical Education offer formal neuro-oncology electives. Half are clustered in the Midwest. Forty percent are at institutions with neuro-oncology fellowships. All are at institutions with neurosurgery and neurology residency programs. Formal clinical neuro-oncology elective opportunities for medical students in the United States and Canada are limited. Additional such opportunities may be of value in the education of medical students. Copyright © 2014 Elsevier Inc. All rights reserved.

Neuro-Linguistic Programming and Family Therapy.

ERIC Educational Resources Information Center

Davis, Susan L. R.; Davis, Donald I.

1983-01-01

Presents a brief introduction to Neuro-Linguistic Programming (NLP), followed by case examples which illustrate some of the substantive gains which NLP techniques have provided in work with couples and families. NLP's major contributions involve understanding new models of human experience. (WAS)

Translational Research in NeuroAIDS: A Neuroimmune Pharmacology-Related Course

PubMed Central

Brown, Amanda; Shiramizu, Bruce; Nath, Avindra; Wojna, Valerie

2010-01-01

Neuroimmune pharmacology (NIP) can be considered a multidisciplinary science where areas of neuroscience, immunology, and pharmacology intersect in neurological disorders. The R25 training program titled “Translational Research in NeuroAIDS and Mental Health (TR-NAMH): An innovative mentoring program to promote diversity in NeuroAIDS Research (R25 MH080661)” at the Johns Hopkins University is a web-based interactive course with the goal to improve the capacity of high quality research by developing mentoring programs for (1) doctoral and postdoctoral candidates and junior faculty from racial and ethnic minorities and (2) non-minority individuals at the same levels, whose research focuses on NeuroAIDS disparity issues such as HIV-associated neurocognitive disorders (HAND). This web-based interactive course overcomes the limitations of traditional education such as access to expert faculty and financial burden of scientists from racial and ethnic minority groups in the field of NeuroAIDS research and NIP and identifies rich nurturing environments for investigators to support their careers. The TR-NAMH program identifies a cadre of talented students and investigators eager to commit to innovative educational and training sessions in NeuroAIDS and NIP. The interplay between NIP changes precipitated by HIV infection in the brain makes the study of HAND an outstanding way to integrate important concepts from these two fields. The course includes activities besides those related to didactic learning such as research training and long-term mentoring; hence, the newly learned topics in NIP are continually reinforced and implemented in real-time experiences. We describe how NIP is integrated in the TR-NAMH program in the context of HAND. PMID:20496178

Translational research in NeuroAIDS: a neuroimmune pharmacology-related course.

PubMed

Brown, Amanda; Shiramizu, Bruce; Nath, Avindra; Wojna, Valerie

2011-03-01

Neuroimmune pharmacology (NIP) can be considered a multidisciplinary science where areas of neuroscience, immunology, and pharmacology intersect in neurological disorders. The R25 training program titled "Translational Research in NeuroAIDS and Mental Health (TR-NAMH): An innovative mentoring program to promote diversity in NeuroAIDS Research (R25 MH080661)" at the Johns Hopkins University is a web-based interactive course with the goal to improve the capacity of high quality research by developing mentoring programs for (1) doctoral and postdoctoral candidates and junior faculty from racial and ethnic minorities and (2) non-minority individuals at the same levels, whose research focuses on NeuroAIDS disparity issues such as HIV-associated neurocognitive disorders (HAND). This web-based interactive course overcomes the limitations of traditional education such as access to expert faculty and financial burden of scientists from racial and ethnic minority groups in the field of NeuroAIDS research and NIP and identifies rich nurturing environments for investigators to support their careers. The TR-NAMH program identifies a cadre of talented students and investigators eager to commit to innovative educational and training sessions in NeuroAIDS and NIP. The interplay between NIP changes precipitated by HIV infection in the brain makes the study of HAND an outstanding way to integrate important concepts from these two fields. The course includes activities besides those related to didactic learning such as research training and long-term mentoring; hence, the newly learned topics in NIP are continually reinforced and implemented in real-time experiences. We describe how NIP is integrated in the TR-NAMH program in the context of HAND.

Evidence-based Neuro Linguistic Psychotherapy: a meta-analysis.

PubMed

Zaharia, Cătălin; Reiner, Melita; Schütz, Peter

2015-12-01

Neuro Linguistic Programming (NLP) Framework has enjoyed enormous popularity in the field of applied psychology. NLP has been used in business, education, law, medicine and psychotherapy to identify people's patterns and alter their responses to stimuli, so they are better able to regulate their environment and themselves. NLP looks at achieving goals, creating stable relationships, eliminating barriers such as fears and phobias, building self-confidence, and self-esteem, and achieving peak performance. Neuro Linguistic Psychotherapy (NLPt) encompasses NLP as framework and set of interventions in the treatment of individuals with different psychological and/or social problems. We aimed systematically to analyse the available data regarding the effectiveness of Neuro Linguistic Psychotherapy (NLPt). The present work is a meta-analysis of studies, observational or randomized controlled trials, for evaluating the efficacy of Neuro Linguistic Programming in individuals with different psychological and/or social problems. The databases searched to identify studies in English and German language: CENTRAL in the Cochrane Library; PubMed; ISI Web of Knowledge (include results also from Medline and the Web of Science); PsycINFO (including PsycARTICLES); Psyndex; Deutschsprachige Diplomarbeiten der Psychologie (database of theses in Psychology in German language), Social SciSearch; National library of health and two NLP-specific research databases: one from the NLP Community (http://www.nlp.de/cgi-bin/research/nlprdb.cgi?action=res_entries) and one from the NLP Group (http://www.nlpgrup.com/bilimselarastirmalar/bilimsel-arastirmalar-4.html#Zweig154). From a total number of 425 studies, 350 were removed and considered not relevant based on the title and abstract. Included, in the final analysis, are 12 studies with numbers of participants ranging between 12 and 115 subjects. The vast majority of studies were prospective observational. The actual paper represents the first

Neuro-fuzzy controller to navigate an unmanned vehicle.

PubMed

Selma, Boumediene; Chouraqui, Samira

2013-12-01

A Neuro-fuzzy control method for an Unmanned Vehicle (UV) simulation is described. The objective is guiding an autonomous vehicle to a desired destination along a desired path in an environment characterized by a terrain and a set of distinct objects, such as obstacles like donkey traffic lights and cars circulating in the trajectory. The autonomous navigate ability and road following precision are mainly influenced by its control strategy and real-time control performance. Fuzzy Logic Controller can very well describe the desired system behavior with simple "if-then" relations owing the designer to derive "if-then" rules manually by trial and error. On the other hand, Neural Networks perform function approximation of a system but cannot interpret the solution obtained neither check if its solution is plausible. The two approaches are complementary. Combining them, Neural Networks will allow learning capability while Fuzzy-Logic will bring knowledge representation (Neuro-Fuzzy). In this paper, an artificial neural network fuzzy inference system (ANFIS) controller is described and implemented to navigate the autonomous vehicle. Results show several improvements in the control system adjusted by neuro-fuzzy techniques in comparison to the previous methods like Artificial Neural Network (ANN).

The Neurologic Assessment in Neuro-Oncology (NANO) scale: a tool to assess neurologic function for integration into the Response Assessment in Neuro-Oncology (RANO) criteria

PubMed Central

DeAngelis, Lisa M.; Brandes, Alba A.; Peereboom, David M.; Galanis, Evanthia; Lin, Nancy U.; Soffietti, Riccardo; Macdonald, David R.; Chamberlain, Marc; Perry, James; Jaeckle, Kurt; Mehta, Minesh; Stupp, Roger; Muzikansky, Alona; Pentsova, Elena; Cloughesy, Timothy; Iwamoto, Fabio M.; Tonn, Joerg-Christian; Vogelbaum, Michael A.; Wen, Patrick Y.; van den Bent, Martin J.; Reardon, David A.

2017-01-01

Abstract Background. The Macdonald criteria and the Response Assessment in Neuro-Oncology (RANO) criteria define radiologic parameters to classify therapeutic outcome among patients with malignant glioma and specify that clinical status must be incorporated and prioritized for overall assessment. But neither provides specific parameters to do so. We hypothesized that a standardized metric to measure neurologic function will permit more effective overall response assessment in neuro-oncology. Methods. An international group of physicians including neurologists, medical oncologists, radiation oncologists, and neurosurgeons with expertise in neuro-oncology drafted the Neurologic Assessment in Neuro-Oncology (NANO) scale as an objective and quantifiable metric of neurologic function evaluable during a routine office examination. The scale was subsequently tested in a multicenter study to determine its overall reliability, inter-observer variability, and feasibility. Results. The NANO scale is a quantifiable evaluation of 9 relevant neurologic domains based on direct observation and testing conducted during routine office visits. The score defines overall response criteria. A prospective, multinational study noted a >90% inter-observer agreement rate with kappa statistic ranging from 0.35 to 0.83 (fair to almost perfect agreement), and a median assessment time of 4 minutes (interquartile range, 3–5). Conclusion. The NANO scale provides an objective clinician-reported outcome of neurologic function with high inter-observer agreement. It is designed to combine with radiographic assessment to provide an overall assessment of outcome for neuro-oncology patients in clinical trials and in daily practice. Furthermore, it complements existing patient-reported outcomes and cognition testing to combine for a global clinical outcome assessment of well-being among brain tumor patients. PMID:28453751

Neuro-Immune Mechanisms in Response to Venezuelan Equine Encephalitis Virus Infection

DTIC Science & Technology

2000-01-01

iii ABSTRACT NEURO-IMMUNE MECHANISMS IN RESPONSE TO VENEZUELAN EQUINE ENCEPHALITIS VIRUS INFECTION Major Bruce A. Schoneboom directed by Franziska B...Grieder, DVM, Ph.D., Assistant Professor of Microbiology and Immunology, Molecular and Cellular Biology, and Neuroscience Venezuelan equine ...3. DATES COVERED - 4. TITLE AND SUBTITLE NEURO-IMMUNE MECHANISMS IN RESPONSE TO VENEZUELAN EQUINE ENCEPHALITIS VIRUS INFECTION 5a. CONTRACT

Dioscin relieves endotoxemia induced acute neuro-inflammation and protect neurogenesis via improving 5-HT metabolism

PubMed Central

Yang, Rui; Chen, Wei; Lu, Ye; Li, Yingke; Du, Hongli; Gao, Songyan; Dong, Xin; Yuan, Hongbin

2017-01-01

Sepsis, in addition to causing fatality, is an independent risk factor for cognitive impairment among sepsis survivors. The pathologic mechanism of endotoxemia induced acute neuro-inflammation still has not been fully understood. For the first time, we found the disruption of neurotransmitters 5-HT, impaired neurogenesis and activation of astrocytes coupled with concomitant neuro-inflammation were the potential pathogenesis of endotoxemia induced acute neuro-inflammation in sepsis survivors. In addition, dioscin a natural steroidal saponin isolated from Chinese medicinal herbs, enhanced the serotonergic system and produced anti-depressant effect by enhancing 5-HT levels in hippocampus. What is more, this finding was verified by metabolic analyses of hippocampus, indicating 5-HT related metabolic pathway was involved in the pathogenesis of endotoxemia induced acute neuro-inflammation. Moreover, neuro-inflammation and neurogenesis within hippocampus were indexed using quantitative immunofluorescence analysis of GFAP DCX and Ki67, as well as real-time RT-PCR analysis of some gene expression levels in hippocampus. Our in vivo and in vitro studies show dioscin protects hippocampus from endotoxemia induced cascade neuro-inflammation through neurotransmitter 5-HT and HMGB-1/TLR4 signaling pathway, which accounts for the dioscin therapeutic effect in behavioral tests. Therefore, the current findings suggest that dioscin could be a potential approach for the therapy of endotoxemia induced acute neuro-inflammation. PMID:28059131

NeuroFlow: A General Purpose Spiking Neural Network Simulation Platform using Customizable Processors.

PubMed

Cheung, Kit; Schultz, Simon R; Luk, Wayne

2015-01-01

NeuroFlow is a scalable spiking neural network simulation platform for off-the-shelf high performance computing systems using customizable hardware processors such as Field-Programmable Gate Arrays (FPGAs). Unlike multi-core processors and application-specific integrated circuits, the processor architecture of NeuroFlow can be redesigned and reconfigured to suit a particular simulation to deliver optimized performance, such as the degree of parallelism to employ. The compilation process supports using PyNN, a simulator-independent neural network description language, to configure the processor. NeuroFlow supports a number of commonly used current or conductance based neuronal models such as integrate-and-fire and Izhikevich models, and the spike-timing-dependent plasticity (STDP) rule for learning. A 6-FPGA system can simulate a network of up to ~600,000 neurons and can achieve a real-time performance of 400,000 neurons. Using one FPGA, NeuroFlow delivers a speedup of up to 33.6 times the speed of an 8-core processor, or 2.83 times the speed of GPU-based platforms. With high flexibility and throughput, NeuroFlow provides a viable environment for large-scale neural network simulation.

NeuroFlow: A General Purpose Spiking Neural Network Simulation Platform using Customizable Processors

PubMed Central

Cheung, Kit; Schultz, Simon R.; Luk, Wayne

2016-01-01

NeuroFlow is a scalable spiking neural network simulation platform for off-the-shelf high performance computing systems using customizable hardware processors such as Field-Programmable Gate Arrays (FPGAs). Unlike multi-core processors and application-specific integrated circuits, the processor architecture of NeuroFlow can be redesigned and reconfigured to suit a particular simulation to deliver optimized performance, such as the degree of parallelism to employ. The compilation process supports using PyNN, a simulator-independent neural network description language, to configure the processor. NeuroFlow supports a number of commonly used current or conductance based neuronal models such as integrate-and-fire and Izhikevich models, and the spike-timing-dependent plasticity (STDP) rule for learning. A 6-FPGA system can simulate a network of up to ~600,000 neurons and can achieve a real-time performance of 400,000 neurons. Using one FPGA, NeuroFlow delivers a speedup of up to 33.6 times the speed of an 8-core processor, or 2.83 times the speed of GPU-based platforms. With high flexibility and throughput, NeuroFlow provides a viable environment for large-scale neural network simulation. PMID:26834542

Validation of the Neuro-QoL Measurement System in Children with Epilepsy

PubMed Central

Lai, Jin-Shei; Nowinski, Cindy J.; Zelko, Frank; Wortman, Katy; Burns, James; Nordli, Douglas R.; Cella, David

2015-01-01

OBJECTIVE Children with epilepsy often face complex psychosocial consequences that are not fully captured by existing patient-reported outcome (PRO) measures. The Neurology Quality of Life Measurement System “Neuro-QoL” was developed to provide a set of common PRO measures that address issues important to people with neurologic disorders. This paper reports Neuro-QoL (Anxiety, Depression, Interaction with peers, Fatigue, Pain, Cognition, Stigma, and Upper and Lower Extremity Function) validation in children with epilepsy. METHOD Patients (aged 10–18 years) diagnosed with epilepsy completed Neuro-QoL and legacy measures at time-1 and 6-month follow-up. Internal consistency reliability was also evaluated. Concurrent validity was assessed by comparing Neuro-QoL measures with more established “legacy” measures of the same concepts. Clinical validity was evaluated by comparing mean Neuro-QoL scores of patients grouped by clinical anchors such as disease severity. Responsiveness of the Neuro-QoL from time-1 to 6-month was evaluated using self-reported change as the primary anchor. RESULTS 61 patients (mean age=13.4 years; 62.3% male, 75.9% white) participated. Most patients (64.2%) had been seizure free in the 3 months prior to participation, and seizure frequency was otherwise described as follows: 17.8% daily, 13.3% weekly, 35.6% monthly and 33.3% yearly. All patients were taking anti-epileptic drugs. Patients reported better function/less symptoms compared to the reference groups. Internal consistency (alpha) coefficients ranged from 0.76 – 0.87. Patients with different seizure frequency differed on Anxiety (p<.01) and Cognition (p<.05). Compared to patients on polytherapy, those on monotherapy had better Upper Extremity scores (p<.05). Compared to those with localized seizures, those experiencing generalized seizures reported worse stigma (p<.05). Depression, Anxiety, Lower Extremity, Fatigue, Pain, Interaction with peers, and Stigma also significantly

Phase 3 Study of Ataluren in Patients With Nonsense Mutation Duchenne Muscular Dystrophy

ClinicalTrials.gov

2017-12-11

Muscular Dystrophy, Duchenne; Muscular Dystrophies; Muscular Disorders, Atrophic; Muscular Diseases; Musculoskeletal Diseases; Neuromuscular Diseases; Nervous System Diseases; Genetic Diseases, X-Linked; Genetic Diseases, Inborn

Space flight-associated neuro-ocular syndrome (SANS).

PubMed

Lee, Andrew G; Mader, Thomas H; Gibson, C Robert; Brunstetter, Tyson J; Tarver, William J

2018-03-12

Interesting novel and somewhat perplexing physiologic and pathologic neuro-ocular findings have been documented in astronauts during and after long duration space flight (LDSF). These findings collectively have been termed the "space flight-associated neuro-ocular syndrome" (SANS). The National Aeronautics and Space Administration (NASA) in the United States has meticulously and prospectively documented the clinical, ultrasound, optical coherence tomography imaging, and radiographic findings of SANS including unilateral and bilateral optic disc edema, globe flattening, choroidal and retinal folds, hyperopic refractive error shifts, and nerve fiber layer infarcts (i.e., cotton wool spots). NASA and collaborating researchers continue to study SANS in preparation for future manned missions to space, including continued trips to the ISS, a return to the moon, or perhaps new voyages to the asteroid belt, or the planet, Mars.

A Survey of the Neuro-Oncology Landscape

PubMed Central

Wu, Jing; Dey, Mahua; Buerki, Robin A.; Byrne, Richard W.; Dohrmann, George J.

2018-01-01

The field of neuro-oncology is evolving rapidly. Many important advances have recently been reported, and other promising investigations have the potential to soon make substantial impacts in the field, especially in the areas of high-grade gliomas and brain metastases. We present an overview of the current status of this field, highlighting the key recent advances as well as representative work of key clinical investigations, since these concepts have the potential to influence clinical management if they are demonstrated to be safe and efficacious. This overview includes some work that has only appeared in abstract form in order to provide a timely understanding of how the field is actively changing and what may lie on the horizon. We focus on both medical and surgical neuro-oncology advances in this highly multidisciplinary subspecialty. PMID:29141278

Neurology diagnostics security and terminal adaptation for PocketNeuro project.

PubMed

Chemak, C; Bouhlel, M-S; Lapayre, J-C

2008-09-01

This paper presents new approaches of medical information security and terminal mobile phone adaptation for the PocketNeuro project. The latter term refers to a project created for the management of neurological diseases. It consists of transmitting information about patients ("desk of patients") to a doctor's mobile phone during a visit and examination of a patient. These new approaches for the PocketNeuro project were analyzed in terms of medical information security and adaptation of the diagnostic images to the doctor's mobile phone. Images were extracted from a DICOM library. Matlab and its library were used as software to test our approaches and to validate our results. Experiments performed on a database of 30 256 x 256 pixel-sized neuronal medical images indicated that our new approaches for PocketNeuro project are valid and support plans for large-scale studies between French and Swiss hospitals using secured connections.

Research Registries: A Tool to Advance Understanding of Rare Neuro-Ophthalmic Diseases

PubMed Central

Blankshain, Kimberly D; Moss, Heather E

2016-01-01

Background Medical research registries (MRR) are organized systems used to collect, store and analyze patient information. They are important tools for medical research with particular application to the study of rare diseases, including those seen in neuro-ophthalmic practice. Evidence Acquisition Evidence for this review was gathered from the writers’ experiences creating a comprehensive neuro-ophthalmology registry and review of the literature. Results MRR are typically observational and prospective databases of de-identified patient information. The structure is flexible and can accommodate a focus on specific diseases or treatments, surveillance of patient populations, physician quality improvement, or recruitment for future studies. They are particularly useful for the study of rare diseases. They can be integrated into the hierarchy of medical research at many levels provided their construction is well organized and they have several key characteristics including an easily manipulated database, comprehensive information on carefully selected patients and comply with human subjects regulations. MRR pertinent to neuro-ophthalmology include the UIC neuro-ophthalmology registry, Susac Syndrome Registry, Intracranial Hypertension Registry as well as larger scale patient outcome registries being developed by professional societies. Conclusion Medical research registries have a variety of forms and applications. With careful planning and clear goals, they are flexible and powerful research tools that can support multiple different study designs, and through this have the potential to advance understanding and care of neuro-ophthalmic diseases. PMID:27389624

Closed-Loop, Multichannel Experimentation Using the Open-Source NeuroRighter Electrophysiology Platform

PubMed Central

Newman, Jonathan P.; Zeller-Townson, Riley; Fong, Ming-Fai; Arcot Desai, Sharanya; Gross, Robert E.; Potter, Steve M.

2013-01-01

Single neuron feedback control techniques, such as voltage clamp and dynamic clamp, have enabled numerous advances in our understanding of ion channels, electrochemical signaling, and neural dynamics. Although commercially available multichannel recording and stimulation systems are commonly used for studying neural processing at the network level, they provide little native support for real-time feedback. We developed the open-source NeuroRighter multichannel electrophysiology hardware and software platform for closed-loop multichannel control with a focus on accessibility and low cost. NeuroRighter allows 64 channels of stimulation and recording for around US $10,000, along with the ability to integrate with other software and hardware. Here, we present substantial enhancements to the NeuroRighter platform, including a redesigned desktop application, a new stimulation subsystem allowing arbitrary stimulation patterns, low-latency data servers for accessing data streams, and a new application programming interface (API) for creating closed-loop protocols that can be inserted into NeuroRighter as plugin programs. This greatly simplifies the design of sophisticated real-time experiments without sacrificing the power and speed of a compiled programming language. Here we present a detailed description of NeuroRighter as a stand-alone application, its plugin API, and an extensive set of case studies that highlight the system’s abilities for conducting closed-loop, multichannel interfacing experiments. PMID:23346047

Research Registries: A Tool to Advance Understanding of Rare Neuro-Ophthalmic Diseases.

PubMed

Blankshain, Kimberly D; Moss, Heather E

2016-09-01

Medical research registries (MRR) are organized systems used to collect, store, and analyze patient information. They are important tools for medical research with particular application to the study of rare diseases, including those seen in neuro-ophthalmic practice. Evidence for this review was gathered from the writers' experiences creating a comprehensive neuro-ophthalmology registry and review of the literature. MRR are typically observational and prospective databases of de-identified patient information. The structure is flexible and can accommodate a focus on specific diseases or treatments, surveillance of patient populations, physician quality improvement, or recruitment for future studies. They are particularly useful for the study of rare diseases. They can be integrated into the hierarchy of medical research at many levels provided their construction is well organized and they have several key characteristics including an easily manipulated database, comprehensive information on carefully selected patients, and comply with human subjects regulations. MRR pertinent to neuro-ophthalmology include the University of Illinois at Chicago neuro-ophthalmology registry, Susac Syndrome Registry, Intracranial Hypertension Registry, and larger-scale patient outcome registries being developed by professional societies. MRR have a variety of forms and applications. With careful planning and clear goals, they are flexible and powerful research tools that can support multiple different study designs, and this can provide the potential to advance understanding and care of neuro-ophthalmic diseases.

Effect of NeuroD gene silencing on the migration and invasion of human pancreatic cancer cells PANC-1.

PubMed

Wang, Yang; Su, Dong Wei; Gao, Li; Ding, Gui Ling; Ni, Can Rong; Zhu, Ming Hua

2014-07-01

The aim of this study is to investigate the influence of Lenti-EGFP-NeuroD-miR, RNAi lentiviral expression vector, on the expression level of NeuroD and migration, and invasion of PANC-1 cell line. PANC-1 cells were cultured and cotransfected with Lenti-EGFP-NeuroD-miR and Lenti-GFP. The infection rate of lentivirus was determined by fluorescence. The interfering effection by the expression of NeuroD mRNA in PANC-1 cells was analyzed by real-time PCR after transfected. Biological behavior of PANC-1 cells transinfected was observed, and the migration and invasion were studied by transwell assay. Intrapancreatic allografts model in nude mice was established to observe the effects of NeuroD on tumorigenesis, tumor growth, and invasion in vivo. The expression of NeuroD mRNA decreased significantly after RNAi lentivirus transinfecting PANC-1 cell. The cell's migration and invasion ability decreased obviously as soon as down regulate of NeuroD in PANC-1 cells. Comparing with control group, the tumors were smaller in size and the invasiveness was inhibited after 8 weeks intrapancreatic allografts in nude mice. Lenti-EGFP-NeuroD-miR transfected into PANC-1 cells shows a stable, effective, and especial blocking expression of NeuroD in mRNA level. The RNAi of lentiviral vector target NeuroD can reduce the migration and invasion abilities of PANC-1 cells.

Space Flight-Associated Neuro-ocular Syndrome.

PubMed

Lee, Andrew G; Mader, Thomas H; Gibson, C Robert; Tarver, William

2017-09-01

New and unique physiologic and pathologic systemic and neuro-ocular responses have been documented in astronauts during and after long-duration space flight. Although the precise cause remains unknown, space flight-associated neuro-ocular syndrome (SANS) has been adopted as an appropriate descriptive term. The Space Medicine Operations Division of the US National Aeronautics and Space Administration (NASA) has documented the variable occurrence of SANS in astronauts returning from long-duration space flight on the International Space Station. These clinical findings have included unilateral and bilateral optic disc edema, globe flattening, choroidal and retinal folds, hyperopic refractive error shifts, and nerve fiber layer infarcts. The clinical findings of SANS have been correlated with structural changes on intraorbital and intracranial magnetic resonance imaging and in-flight and terrestrial ultrasonographic studies and ocular optical coherence tomography. Further study of SANS is ongoing for consideration of future manned missions to space, including a return trip to the moon or Mars.

The lack of CD131 and the inhibition of Neuro-2a growth by carbamylated erythropoietin.

PubMed

Ding, Jing; Li, Qin-Ying; Yu, Jie-Zhong; Wang, Xin; Lu, Chuan-Zhen; Ma, Cun-Gen; Xiao, Bao-Guo

2015-02-01

Recombinant human erythropoietin (EPO), a glycohormone, is one of the leading biopharmaceutical products, while carbamylated erythropoietin (CEPO), an EPO derivative, is attracting widespread interest due to its neuroprotective effects without erythropoiesis in several cells and animal models. However, exogenous EPO promotes an angiogenic response from tumor cells and is associated with tumor growth, but knowledge of CEPO on tumor growth is lacking. Here we show that CEPO, but not EPO, inhibited Neuro-2a growth and viability. As expected, CEPO--unlike EPO--did not activate JAK-2 either in primary neurons or in Neuro-2a cells. Interestingly, CEPO did not induce GDNF expression and subsequent AKT activation in Neuro-2a cells. Before CEPO/EPO treatment, glial cell line-derived neurotrophic factor (GDNF) neutralization and GFR receptor blocking decreased the viability of EPO-treated Neuro-2a cells but did not influence CEPO-treated Neuro-2a cells. As compared to primary neurons, the expression of CD131, as a receptor complex binding to CEPO, is almost lacking in Neuro-2a cells. In BABL/C-nu mice, CEPO did not promote the growth of Neuro-2a cells nor extended the survival time compared to mice treated with EPO. The results indicate that CEPO did not promote tumor growth because of lower expression of CD131 and subsequent dysfunction of CD131/GDNF/AKT pathway in Neuro-2a cells, revealing its therapeutic potential in future clinical application.

A Hybrid Neuro-Fuzzy Model For Integrating Large Earth-Science Datasets

NASA Astrophysics Data System (ADS)

Porwal, A.; Carranza, J.; Hale, M.

2004-12-01

A GIS-based hybrid neuro-fuzzy approach to integration of large earth-science datasets for mineral prospectivity mapping is described. It implements a Takagi-Sugeno type fuzzy inference system in the framework of a four-layered feed-forward adaptive neural network. Each unique combination of the datasets is considered a feature vector whose components are derived by knowledge-based ordinal encoding of the constituent datasets. A subset of feature vectors with a known output target vector (i.e., unique conditions known to be associated with either a mineralized or a barren location) is used for the training of an adaptive neuro-fuzzy inference system. Training involves iterative adjustment of parameters of the adaptive neuro-fuzzy inference system using a hybrid learning procedure for mapping each training vector to its output target vector with minimum sum of squared error. The trained adaptive neuro-fuzzy inference system is used to process all feature vectors. The output for each feature vector is a value that indicates the extent to which a feature vector belongs to the mineralized class or the barren class. These values are used to generate a prospectivity map. The procedure is demonstrated by an application to regional-scale base metal prospectivity mapping in a study area located in the Aravalli metallogenic province (western India). A comparison of the hybrid neuro-fuzzy approach with pure knowledge-driven fuzzy and pure data-driven neural network approaches indicates that the former offers a superior method for integrating large earth-science datasets for predictive spatial mathematical modelling.

"Too Withdrawn" or "Too Friendly": Considering Social Vulnerability in Two Neuro-Developmental Disorders

ERIC Educational Resources Information Center

Jawaid, A.; Riby, D. M.; Owens, J.; White, S. W.; Tarar, T.; Schulz, P. E.

2012-01-01

In some neuro-developmental disorders, the combined effect of intellectual disability and atypicalities of social cognition may put individuals at increased vulnerability in their social environment. The neuro-developmental disorders Williams syndrome, characterised by "hypersociability", and autism spectrum disorders, characterised by "social…

A Neuro-Fuzzy Approach in the Classification of Students' Academic Performance

PubMed Central

2013-01-01

Classifying the student academic performance with high accuracy facilitates admission decisions and enhances educational services at educational institutions. The purpose of this paper is to present a neuro-fuzzy approach for classifying students into different groups. The neuro-fuzzy classifier used previous exam results and other related factors as input variables and labeled students based on their expected academic performance. The results showed that the proposed approach achieved a high accuracy. The results were also compared with those obtained from other well-known classification approaches, including support vector machine, Naive Bayes, neural network, and decision tree approaches. The comparative analysis indicated that the neuro-fuzzy approach performed better than the others. It is expected that this work may be used to support student admission procedures and to strengthen the services of educational institutions. PMID:24302928

Spacecraft attitude control using neuro-fuzzy approximation of the optimal controllers

NASA Astrophysics Data System (ADS)

Kim, Sung-Woo; Park, Sang-Young; Park, Chandeok

2016-01-01

In this study, a neuro-fuzzy controller (NFC) was developed for spacecraft attitude control to mitigate large computational load of the state-dependent Riccati equation (SDRE) controller. The NFC was developed by training a neuro-fuzzy network to approximate the SDRE controller. The stability of the NFC was numerically verified using a Lyapunov-based method, and the performance of the controller was analyzed in terms of approximation ability, steady-state error, cost, and execution time. The simulations and test results indicate that the developed NFC efficiently approximates the SDRE controller, with asymptotic stability in a bounded region of angular velocity encompassing the operational range of rapid-attitude maneuvers. In addition, it was shown that an approximated optimal feedback controller can be designed successfully through neuro-fuzzy approximation of the optimal open-loop controller.

A neuro-fuzzy approach in the classification of students' academic performance.

PubMed

Do, Quang Hung; Chen, Jeng-Fung

2013-01-01

Classifying the student academic performance with high accuracy facilitates admission decisions and enhances educational services at educational institutions. The purpose of this paper is to present a neuro-fuzzy approach for classifying students into different groups. The neuro-fuzzy classifier used previous exam results and other related factors as input variables and labeled students based on their expected academic performance. The results showed that the proposed approach achieved a high accuracy. The results were also compared with those obtained from other well-known classification approaches, including support vector machine, Naive Bayes, neural network, and decision tree approaches. The comparative analysis indicated that the neuro-fuzzy approach performed better than the others. It is expected that this work may be used to support student admission procedures and to strengthen the services of educational institutions.

Validity of the Neurology Quality of Life (Neuro-QoL) Measurement System in Adult Epilepsy

PubMed Central

Victorson, David; Cavazos, Jose E.; Holmes, Gregory L.; Reder, Anthony T.; Wojna, Valerie; Nowinski, Cindy; Miller, Deborah; Buono, Sarah; Mueller, Allison; Moy, Claudia; Cella, David

2014-01-01

Epilepsy is a chronic neurological disorder that results in recurring seizures and can have a significant adverse effect on health related quality of life (HRQL). Neuro-QoL is an NINDS-funded system of patient reported outcome measures for neurology clinical research, which was designed to provide a precise and standardized way to measure HRQL in epilepsy and other neurological disorders. Using mixed-methods and item response theory-based approaches, we developed generic item banks and targeted scales for adults and children with major neurological disorders. This paper provides empirical results from a clinical validation study with a sample of adults diagnosed with epilepsy. One hundred twenty one people diagnosed with epilepsy participated, of which the majority were male (62%), Caucasian (95%), with a mean age of 47.3 (SD=16.9). Baseline assessments included Neuro-QoL short forms and general and external validity measures. Neuro-QoL short forms that are not typically found in other epilepsy-specific HRQL instruments include Stigma, Sleep Disturbance, Emotional and Behavioral Dyscontrol and Positive Affect & Well-being. Neuro-QoL short forms demonstrated adequate reliability (internal consistency range = .86–.96; test-retest range = .57–.89). Pearson correlations (p<.01) between Neuro-QoL forms of emotional distress (Anxiety, Depression, Stigma) and the QOLIE-31 Emotional Well-being Subscale were in the moderate to strong range (r’s = .66, .71 & .53, respectively), as were relations with the PROMIS Global Mental Health subscale (r’s = .59, .74 & .52, respectively). Moderate correlations were observed between Neuro-QoL Social Role Performance and Satisfaction and the QOLIE-31 Social Function (r’s = .58 & .52, respectively). In measuring aspects of physical function, the Neuro-QoL Mobility and Upper Extremity forms demonstrated moderate associations with the PROMIS Global Physical Function Subscale (r’s = .60 & .61, respectively). Neuro-QoL measures of

Becker muscular dystrophy-like myopathy regarded as so-called "fatty muscular dystrophy" in a pig: a case report and its diagnostic method.

PubMed

Horiuchi, Noriyuki; Aihara, Naoyuki; Mizutani, Hiroshi; Kousaka, Shinichi; Nagafuchi, Tsuneyuki; Ochiai, Mariko; Ochiai, Kazuhiko; Kobayashi, Yoshiyasu; Furuoka, Hidefumi; Asai, Tetsuo; Oishi, Koji

2014-03-01

We describe a case of human Becker muscular dystrophy (BMD)-like myopathy that was characterized by the declined stainability of dystrophin at sarcolemma in a pig and the immunostaining for dystrophin on the formalin-fixed, paraffin-embedded (FFPE) tissue. The present case was found in a meat inspection center. The pig looked appeared healthy at the ante-mortem inspection. Muscular abnormalities were detected after carcass dressing as pale, discolored skeletal muscles with prominent fat infiltrations and considered so-called "fatty muscular dystrophy". Microscopic examination revealed following characteristics: diffused fat infiltration into the skeletal muscle and degeneration and regeneration of the remaining skeletal muscle fibers. Any lesions that were suspected of neurogenic atrophy, traumatic muscular degeneration, glycogen storage disease or other porcine muscular disorders were not observed. The immunostaining for dystrophin was conducted and confirmed to be applicable on FFPE porcine muscular tissues and revealed diminished stainability of dystrophin at the sarcolemma in the present case. Based on the histological observations and immunostaining results, the present case was diagnosed with BMD-like myopathy associated with dystrophin abnormality in a pig. Although the genetic properties were not clear, the present BMD-like myopathy implied the occurrence of dystrophinopathy in pigs. To the best of our knowledge, this is the first report of a natural case of myopathy associated with dystrophin abnormalities in a pig.

The role of tumor board conferences in neuro-oncology: a nationwide provider survey.

PubMed

Snyder, James; Schultz, Lonni; Walbert, Tobias

2017-05-01

The tumor board or multidisciplinary cancer meeting (MCM) is the foundation of high value multidisciplinary oncology care, coordinating teams of specialists. Little is known on how these meetings are implemented in Neuro-oncology. Benefits of MCMs include coordination, direction for complicated cases, education, and a forum for communication, emerging technology, and clinical trials. This study identifies participation and utilization of neuro-oncology MCMs. A cross-sectional descriptive survey was dispersed through an internet questionnaire. The Society of Neuro-Oncology and the American Brain Tumor Association provided a list of dedicated neuro-oncology centers. All National Cancer Institute designated centers, and participants in the Adult Brain Tumor Consortium or the Brain Tumor Trials Collaborative were included, identifying 85 centers. Discussion included primary brain tumors (100%), challenging cases (98%), recurrent disease (96%), neoplastic spine disease (93%), metastatic brain lesions (89%), pre-surgical cases (82%), pathology (76%), and paraneoplastic disease (40%). MCMs were composed of neuro-oncologists, neurosurgeons, and radiation oncologists (100%), radiologists (98%), pathologists (96%), and clinical trial participants (64%). Individual preparation ranged from 15 to 300 min. MCMs were valued for clinical decision making (94%), education (89%), and access to clinical trials (69%). 13% documented MCMs in the medical record. 38% of centers used a molecular tumor board; however, many commented with uncertainty as to how this is defined. Neuro-oncology MCMs at leading U.S. institutions demonstrate congruity of core disciplines, cases discussed, and perceived value. We identified variability in preparation time and implementation of MCM recommendations. There is high uncertainty as to the definition and application of a molecular tumor board.

A new learning algorithm for a fully connected neuro-fuzzy inference system.

PubMed

Chen, C L Philip; Wang, Jing; Wang, Chi-Hsu; Chen, Long

2014-10-01

A traditional neuro-fuzzy system is transformed into an equivalent fully connected three layer neural network (NN), namely, the fully connected neuro-fuzzy inference systems (F-CONFIS). The F-CONFIS differs from traditional NNs by its dependent and repeated weights between input and hidden layers and can be considered as the variation of a kind of multilayer NN. Therefore, an efficient learning algorithm for the F-CONFIS to cope these repeated weights is derived. Furthermore, a dynamic learning rate is proposed for neuro-fuzzy systems via F-CONFIS where both premise (hidden) and consequent portions are considered. Several simulation results indicate that the proposed approach achieves much better accuracy and fast convergence.

libNeuroML and PyLEMS: using Python to combine procedural and declarative modeling approaches in computational neuroscience.

PubMed

Vella, Michael; Cannon, Robert C; Crook, Sharon; Davison, Andrew P; Ganapathy, Gautham; Robinson, Hugh P C; Silver, R Angus; Gleeson, Padraig

2014-01-01

NeuroML is an XML-based model description language, which provides a powerful common data format for defining and exchanging models of neurons and neuronal networks. In the latest version of NeuroML, the structure and behavior of ion channel, synapse, cell, and network model descriptions are based on underlying definitions provided in LEMS, a domain-independent language for expressing hierarchical mathematical models of physical entities. While declarative approaches for describing models have led to greater exchange of model elements among software tools in computational neuroscience, a frequent criticism of XML-based languages is that they are difficult to work with directly. Here we describe two Application Programming Interfaces (APIs) written in Python (http://www.python.org), which simplify the process of developing and modifying models expressed in NeuroML and LEMS. The libNeuroML API provides a Python object model with a direct mapping to all NeuroML concepts defined by the NeuroML Schema, which facilitates reading and writing the XML equivalents. In addition, it offers a memory-efficient, array-based internal representation, which is useful for handling large-scale connectomics data. The libNeuroML API also includes support for performing common operations that are required when working with NeuroML documents. Access to the LEMS data model is provided by the PyLEMS API, which provides a Python implementation of the LEMS language, including the ability to simulate most models expressed in LEMS. Together, libNeuroML and PyLEMS provide a comprehensive solution for interacting with NeuroML models in a Python environment.

libNeuroML and PyLEMS: using Python to combine procedural and declarative modeling approaches in computational neuroscience

PubMed Central

Vella, Michael; Cannon, Robert C.; Crook, Sharon; Davison, Andrew P.; Ganapathy, Gautham; Robinson, Hugh P. C.; Silver, R. Angus; Gleeson, Padraig

2014-01-01

NeuroML is an XML-based model description language, which provides a powerful common data format for defining and exchanging models of neurons and neuronal networks. In the latest version of NeuroML, the structure and behavior of ion channel, synapse, cell, and network model descriptions are based on underlying definitions provided in LEMS, a domain-independent language for expressing hierarchical mathematical models of physical entities. While declarative approaches for describing models have led to greater exchange of model elements among software tools in computational neuroscience, a frequent criticism of XML-based languages is that they are difficult to work with directly. Here we describe two Application Programming Interfaces (APIs) written in Python (http://www.python.org), which simplify the process of developing and modifying models expressed in NeuroML and LEMS. The libNeuroML API provides a Python object model with a direct mapping to all NeuroML concepts defined by the NeuroML Schema, which facilitates reading and writing the XML equivalents. In addition, it offers a memory-efficient, array-based internal representation, which is useful for handling large-scale connectomics data. The libNeuroML API also includes support for performing common operations that are required when working with NeuroML documents. Access to the LEMS data model is provided by the PyLEMS API, which provides a Python implementation of the LEMS language, including the ability to simulate most models expressed in LEMS. Together, libNeuroML and PyLEMS provide a comprehensive solution for interacting with NeuroML models in a Python environment. PMID:24795618

Early-Onset LMNA-Associated Muscular Dystrophy with Later Involvement of Contracture.

PubMed

Lee, Younggun; Lee, Jung Hwan; Park, Hyung Jun; Choi, Young Chul

2017-10-01

The early diagnosis of LMNA-associated muscular dystrophy is important for preventing sudden arrest related to cardiac conduction block. However, diagnosing early-onset Emery-Dreifuss muscular dystrophy (EDMD) with later involvement of contracture and limb-girdle muscular dystrophy type 1B is often delayed due to heterogeneous clinical presentations. We aimed to determine the clinical features that contribute to a delayed diagnosis. We reviewed four patients who were recently diagnosed with LMNA-associated muscular dystrophy by targeted exome sequencing and who were initially diagnosed with nonspecific or other types of muscular dystrophy. Certain clinical features such as delayed contracture involvement and calf hypertrophy were found to contribute to a delayed diagnosis. Muscle biopsies were not informative for the diagnosis in these patients. Genetic testing of single or multiple genes is useful for confirming a diagnosis of LMNA-associated muscular dystrophy. Even EDMD patients could experience the later involvement of contracture, so clinicians should consider early genetic testing for patients with undiagnosed muscular dystrophy or laminopathy. Copyright © 2017 Korean Neurological Association

[Supplementary device for a dynamometer to evaluate and register muscular endurance indices].

PubMed

Timoshenko, D A; Bokser, O Ia

1986-01-01

In practice of psychophysiologic research muscular endurance index is used for estimation of CNS function. Muscular endurance index is defined as relative time needed for maintaining the preset muscular effort. The described device widens the possibilities of a digital dynamometer for automatic estimation and recording of muscular endurance index in real time.

A wearable neuro-feedback system with EEG-based mental status monitoring and transcranial electrical stimulation.

PubMed

Roh, Taehwan; Song, Kiseok; Cho, Hyunwoo; Shin, Dongjoo; Yoo, Hoi-Jun

2014-12-01

A wearable neuro-feedback system is proposed with a low-power neuro-feedback SoC (NFS), which supports mental status monitoring with encephalography (EEG) and transcranial electrical stimulation (tES) for neuro-modulation. Self-configured independent component analysis (ICA) is implemented to accelerate source separation at low power. Moreover, an embedded support vector machine (SVM) enables online source classification, configuring the ICA accelerator adaptively depending on the types of the decomposed components. Owing to the hardwired accelerating functions, the NFS dissipates only 4.45 mW to yield 16 independent components. For non-invasive neuro-modulation, tES stimulation up to 2 mA is implemented on the SoC. The NFS is fabricated in 130-nm CMOS technology.

Rare Muscular Dystrophies: Congenital, Distal, Emery-Dreifuss and Oculopharyngeal Muscular Dystrophies

MedlinePlus

... although EDMD and OPMD can have more than one genetic cause). In most cases of muscular dystrophy, muscle mass in the affected ... categories. Many of these diseases can vary from one person to the next, and in some cases, research- ers are still in the process of ...

Immunotherapy Response Assessment in Neuro-Oncology (iRANO): A Report of the RANO Working Group

PubMed Central

Okada, Hideho; Weller, Michael; Huang, Raymond; Finocchiaro, Gaetano; Gilbert, Mark R.; Wick, Wolfgang; Ellingson, Benjamin M.; Hashimoto, Naoya; Pollack, Ian F.; Brandes, Alba A.; Franceschi, Enrico; Herold-Mende, Christel; Nayak, Lakshmi; Panigrahy, Ashok; Pope, Whitney B.; Prins, Robert; Sampson, John H.; Wen, Patrick Y.; Reardon, David A.

2015-01-01

Immunotherapy represents a promising area of therapy among neuro-oncology patients. However, early phase studies reveal unique challenges associated with assessment of radiological changes reflecting delayed responses or therapy-induced inflammation. Clinical benefit, including long-term survival and tumor regression, can still occur following initial apparent progression or appearance of new lesions. Refinement of response assessment criteria for neuro-oncology patients undergoing immunotherapy is therefore warranted. A multinational and multidisciplinary panel of neuro-oncology immunotherapy experts describes immunotherapy response assessment for neuro-oncology (iRANO) criteria that are based on guidance for determination of tumor progression outlined by the immune-related response criteria (irRC) and the response assessment in neuro-oncology (RANO) working group. Among patients who demonstrate imaging findings meeting RANO criteria for progressive disease (PD) within six months of initiating immunotherapy including the development of new lesions, confirmation of radiographic progression on follow-up imaging is recommended provided that the patient is not significantly worse clinically. The proposed criteria also include guidelines for use of corticosteroids. The role of advanced imaging techniques and measurement of clinical benefit endpoints including neurologic and immunologic functions are reviewed. The iRANO guidelines put forth herein will evolve successively to improve their utility as further experience from immunotherapy trials in neuro-oncology accumulate. PMID:26545842

Limb-girdle muscular dystrophies

MedlinePlus

... diseases in which there is muscle weakness and wasting (muscular dystrophy). In most cases, both parents must ... which are not actually strong Loss of muscle mass, thinning of certain body parts Low back pain ...

Limb-girdle muscular dystrophy subtypes: First-reported cohort from northeastern China

PubMed Central

Mahmood, Omar Abdulmonem; Jiang, Xinmei; Zhang, Qi

2013-01-01

The relative frequencies of different subtypes of limb-girdle muscular dystrophies vary widely among different populations. We estimated the percentage of limb-girdle muscular dystrophy subtypes in Chinese people based on 68 patients with limb-girdle muscular dystrophy from the Myology Clinic, Neurology Department, First Hospital of Jilin University, China. A diagnosis of calpainopathy was made in 12 cases (17%), and dysferlin deficiency in 10 cases (15%). Two biopsies revealed α-sarcoglycan deficiency (3%), and two others revealed a lack of caveolin-3 (3%). A diagnosis of unclassified limb-girdle muscular dystrophy was made in the remaining patients (62%). The appearances of calpain 3- and dysferlin-deficient biopsies were similar, though rimmed vacuoles were unique to dysferlinopathy, while inflammatory infiltrates were present in both these limb-girdle muscular dystrophy type 2D biopsies. Macrophages were detected in seven dysferlinopathy biopsies. The results of this study suggest that the distribution of limb-girdle muscular dystrophy subtypes in the Han Chinese population is similar to that reported in the West. The less necrotic, regenerating and inflammatory appearance of limb-girdle muscular dystrophy type 2A, but with more lobulated fibers, supports the idea that calpainopathy is a less active, but more chronic disease than dysferlinopathy. Unusual features indicated an extended limb-girdle muscular dystrophy disease spectrum. The use of acid phosphatase stain should be considered in suspected dysferlinopathies. To the best of our knowledge, this is the first report to define the relative proportions of the various forms of limb-girdle muscular dystrophy in China, based on protein testing. PMID:25206500

Lucien J. Rubinstein: enduring contributions to neuro-oncology.

PubMed

Mut, Melike; Lopes, M Beatriz S; Shaffrey, Mark

2005-04-15

Dr. Lucien Rubinstein is best remembered for his significant contributions to the field of neuropathology, particularly in the classification of nervous system tumors. His accomplishments in basic neuro-oncology and in the formulation of diagnostic principles reflected a unique talent for synthesizing fundamental clinicopathological concepts based on skillful diagnostic investigation and a thorough understanding of neurobiology. Dr. Rubinstein was the leader in the establishment of cell cultures from central nervous system (CNS) tumors. He meticulously analyzed both light and electron microscopic features of CNS tumors, recorded his findings, and patiently drew sketches to be shared generously with his colleagues and students. As a pioneer in neuropathology, in his work Dr. Rubinstein set the foundation for many enduring concepts in neurosurgery, neuro-oncology, neurology, and basic tumor biology.

Functional Neuro-Imaging and Post-Traumatic Olfactory Impairment

PubMed Central

Roberts, Richard J.; Sheehan, William; Thurber, Steven; Roberts, Mary Ann

2010-01-01

Objective: To evaluate via a research literature survey the anterior neurological significance of decreased olfactory functioning following traumatic brain injuries. Materials and Methods: A computer literature review was performed to locate all functional neuro-imaging studies on patients with post-traumatic anosmia and other olfactory deficits. Results: A convergence of findings from nine functional neuro-imaging studies indicating evidence for reduced metabolic activity at rest or relative hypo-perfusion during olfactory activations. Hypo-activation of the prefrontal regions was apparent in all nine post-traumatic samples, with three samples yielding evidence of reduced activity in the temporal regions as well. Conclusions: The practical ramifications include the reasonable hypothesis that a total anosmic head trauma patient likely has frontal lobe involvement. PMID:21716782

In-stream attenuation of neuro-active pharmaceuticals and their metabolites

USGS Publications Warehouse

Writer, Jeffrey; Antweiler, Ronald C.; Ferrar, Imma; Ryan, Joseph N.; Thurman, Michael

2013-01-01

In-stream attenuation was determined for 14 neuro-active pharmaceuticals and associated metabolites. Lagrangian sampling, which follows a parcel of water as it moves downstream, was used to link hydrological and chemical transformation processes. Wastewater loading of neuro-active compounds varied considerably over a span of several hours, and thus a sampling regime was used to verify that the Lagrangian parcel was being sampled and a mechanism was developed to correct measured concentrations if it was not. In-stream attenuation over the 5.4-km evaluated reach could be modeled as pseudo-first-order decay for 11 of the 14 evaluated neuro-active pharmaceutical compounds, illustrating the capacity of streams to reduce conveyance of neuro-active compounds downstream. Fluoxetine and N-desmethyl citalopram were the most rapidly attenuated compounds (t1/2 = 3.6 ± 0.3 h, 4.0 ± 0.2 h, respectively). Lamotrigine, 10,11,-dihydro-10,11,-dihydroxy-carbamazepine, and carbamazepine were the most persistent (t1/2 = 12 ± 2.0 h, 12 ± 2.6 h, 21 ± 4.5 h, respectively). Parent compounds (e.g., buproprion, carbamazepine, lamotrigine) generally were more persistent relative to their metabolites. Several compounds (citalopram, venlafaxine, O-desmethyl-venlafaxine) were not attenuated. It was postulated that the primary mechanism of removal for these compounds was interaction with bed sediments and stream biofilms, based on measured concentrations in stream biofilms and a column experiment using stream sediments.

Muscular anatomy of an entoproct creeping-type larva reveals extraordinary high complexity and potential shared characters with mollusks.

PubMed

Merkel, Julia; Lieb, Bernhard; Wanninger, Andreas

2015-07-03

Entoprocta (Kamptozoa) is an enigmatic, acoelomate, tentacle-bearing phylum with indirect development, either via a swimming- or a creeping-type larva and still debated phylogenetic position within Lophotrochozoa. Recent morphological and neuro-anatomical studies on the creeping-type larva support a close relationship of Entoprocta and Mollusca, with a number of shared apomorphies including a tetraneurous nervous system and a complex serotonin-expressing apical organ. However, many morphological traits of entoproct larvae, in particular of the putative basal creeping-type larva, remain elusive. Applying fluorescent markers and 3D modeling, we found that this larval type has the most complex musculature hitherto described for any lophotrochozoan larva. The muscle systems identified include numerous novel and most likely creeping-type larva-specific structures such as frontal organ retractors, several other muscle fibers originating from the frontal organ, and longitudinal prototroch muscles. Interestingly, we found distinct muscle sets that are also present in several mollusks. These include paired sets of dorso-ventral muscles that intercross ventrally above the foot sole and a paired enrolling muscle that is distinct from the musculature of the body wall. Our data add further morphological support for an entoproct-mollusk relationship (Tetraneuralia) and strongly argue for the presence of an enrolling musculature as well as seriality (but not segmentation) in the last common tetraneuralian ancestor. The evolutionary driving forces that have led to the emergence of the extraordinarily complex muscular architecture in this short-lived, non-feeding entoproct larval type remain unknown, as are the processes that give rise to the highly different and much simpler muscular bodyplan of the adult entoproct during metamorphosis.

Delayed Diagnosis in Duchenne Muscular Dystrophy: Data from the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet)

PubMed Central

Ciafaloni, Emma; Fox, Deborah J.; Pandya, Shree; Westfield, Christina P.; Puzhankara, Soman; Romitti, Paul A.; Mathews, Katherine D.; Miller, Timothy M.; Matthews, Dennis J.; Miller, Lisa A.; Cunniff, Christopher; Druschel, Charlotte M.; Moxley, Richard T.

2018-01-01

Objective To identify key factors for the delay in diagnosis of Duchenne muscular dystrophy (DMD) without known family history. Study design The cohort comes from the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet), a multistate, multiple-source, population-based surveillance system that identifies and gathers information on all cases of Duchenne and Becker muscular dystrophy born since 1982. We analyzed medical records of 453 Duchenne and Becker muscular dystrophy boys to document the time course and steps taken to reach a definitive diagnosis. Results Among 156 boys without known family history of DMD prior to birth, first signs or symptoms were noted at a mean age of 2.5 years. Concerns resulted in primary care provider evaluation of the child at a mean age of 3.6 years. Mean age at time of initial creatine kinase was 4.7 years. Mean age at definitive diagnosis of DMD was 4.9 years. Conclusions There is a delay of about 2.5 years between onset of DMD symptoms and the time of definitive diagnosis, unchanged over the previous 2 decades. This delay results in lost opportunities for timely genetic counseling and initiation of corticosteroid treatment. We recommend checking creatine kinase early in the evaluation of boys with unexplained developmental delay. PMID:19394035

Muscle Dysmorphia and the Perception of Men's Peer Muscularity Preferences.

PubMed

Lin, Linda; DeCusati, Frank

2016-11-01

Research suggests that peer muscularity norms preferences are related to men's body image, but little information is known about how perceptions of specific peer group norms preferences are related to men's body image disturbances and specific health behaviors. This study investigated how men perceived the muscularity preferences of male, female, close, and distant peers and whether the perceptions of specific peer preferences were related to muscle dysmorphia and steroid use. Data on muscle dysmorphia and the perceptions of peer muscularity norms were collected from 117 male college students. Results indicated that men perceived distant and male peers as having the most exaggerated preferences for muscularity and that those perceptions were not an accurate reflection of their distant male peers' reported preferences. Results also indicated that perceptions of close female peer muscularity preferences were predictive of symptoms of muscle dysmorphia, but this relationship did not exist for other peer groups, suggesting that the perceptions of close female peer preferences may play a role in the development of muscle dysmorphia. No relationship was found between perceptions of peer muscularity preferences and steroid use. © The Author(s) 2015.

Neuro-Linguistic Programming in Couple Therapy.

ERIC Educational Resources Information Center

Forman, Bruce D.

Neuro-Linguistic Programming (NLP) is a method of understanding the organization of subjective human experience. The NLP model provides a theoretical framework for directing or guiding therapeutic change. According to NLP, people experience the so-called real world indirectly and operate on the real world as if it were like the model of it they…

Validity of the Neurology Quality-of-Life (Neuro-QoL) measurement system in adult epilepsy.

PubMed

Victorson, David; Cavazos, Jose E; Holmes, Gregory L; Reder, Anthony T; Wojna, Valerie; Nowinski, Cindy; Miller, Deborah; Buono, Sarah; Mueller, Allison; Moy, Claudia; Cella, David

2014-02-01

Epilepsy is a chronic neurological disorder that results in recurring seizures and can have a significant adverse effect on health-related quality of life (HRQL). The Neuro-QoL measurement initiative is an NINDS-funded system of patient-reported outcome measures for neurology clinical research, which was designed to provide a precise and standardized way to measure HRQL in epilepsy and other neurological disorders. Using mixed-method and item response theory-based approaches, we developed generic item banks and targeted scales for adults and children with major neurological disorders. This paper provides empirical results from a clinical validation study with a sample of adults diagnosed with epilepsy. One hundred twenty-one people diagnosed with epilepsy participated, the majority of which were male (62%) and Caucasian (95%), with a mean age of 47.3 (SD=16.9). Baseline assessments included Neuro-QoL short forms and general and external validity measures. The Neuro-QoL short forms that are not typically found in other epilepsy-specific HRQL instruments include Stigma, Sleep Disturbance, Emotional and Behavioral Dyscontrol, and Positive Affect and Well-Being. Neurology Quality-of-Life short forms demonstrated adequate reliability (internal consistency range=.86-.96; test-retest range=.57-.89). Pearson correlations (p<.01) between Neuro-QoL forms of emotional distress (anxiety, depression, stigma) and the QOLIE-31 Emotional Well-Being subscale were in the moderate-to-strong range (r's=.66, .71 and .53, respectively), as were relations with the PROMIS Global Mental Health subscale (r's=.59, .74 and .52, respectively). Moderate correlations were observed between Neuro-QoL Social Role Performance and Satisfaction and the QOLIE-31 Social Function (r's=.58 and .52, respectively). In measuring aspects of physical function, the Neuro-QoL Mobility and Upper Extremity forms demonstrated moderate associations with the PROMIS Global Physical Function subscale (r's=.60 and .61

Angiographic assessment of atherosclerotic load at the lower extremity in patients with diabetic foot and charcot neuro-arthropathy.

PubMed

Çildağ, Mehmet B; Ertuğrul, Bülent M; Köseoğlu, Ömer Fk; Çildağ, Songül; Armstrong, David G

2018-06-01

The aim of this study was to investigate atherosclerotic load at the lower extremity in patients with diabetic foot and charcot neuro-arthropathy and compare them with patients with diabetic foot without charcot neuro-arthropathy. This retrospective study consists of 78 patients with diabetic foot who had lower extremity angiography with antegrade approach. All patients were classified into two groups; neuro ischemic wounds with charcot neuro-arthropathy (30/78) and without charcot neuro-arthropathy (48/78).Atherosclerotic load at the side of diabetic foot was determined by using the Bollinger angiogram scoring method. Comparison of atherosclerotic load between the two groups was performed. The mean of total and infrapopliteal level angiogram scoring of all patients was 33.3 (standard deviation, sd:±17.2) and 29.3 (sd:±15.6), respectively. The mean of total and infrapopliteal level angiogram scoring of neuroischemic wounds with charcot neuro-arthropathy group was 18.1 (sd:±11.6) and 15.7 (sd:±10.4), respectively. The mean of total and infrapopliteal level angiogram scoring of neuroischemic wounds without charcot neuro-arthropathy group was 42.8 (sd:±12.7) and 37.7 (sd:±12.0), respectively. There was a statistically significant difference between the two groups of mean total and infrapopliteal angiogram scoring (p < 0.01). This angiographic study confirms that the atherosclerotic load in patients with diabetic foot and chronic charcot neuro-arthropathy is significantly less than in patients with neuroischemic diabetic foot wounds without chronic charcot neuro-arthropathy. Copyright © 2017. Published by Elsevier Taiwan LLC.

What is muscular dystrophy? Forty years of progressive ignorance.

PubMed

Dubowitz, V

2000-01-01

This lecture traces recent advances in knowledge of the muscular dystrophies, as well as their increasing complexity. They are described through the eyes of the author from his first exposure to and complete ignorance of the disease in the late 1950s, through the advent of modern techniques, to the molecular genetic revolution, with the recognition of individual genes and proteins for disorders within the muscular dystrophy umbrella. There initially seemed to be a logical sequence of linked membrane proteins from dystrophin in Duchenne and Becker dystrophy, through the dystrophin-associated glycoproteins (sarcoglycans) in some of the limb girdle muscular dystrophies (LGMD), to the extracellular matrix protein merosin (alpha-2 laminin) in congenital muscular dystrophy (CMD). The first spoke in the wheel came with the discovery of a calcium activated protease enzyme, calpain 3, in one form of LGMD, and subsequently another novel non-membrane protein, dysferlin, in another. There are currently at least eight distinct genetic forms of LGMD alone, and another eight separate genetic entities in the CMD group. This has highlighted our ignorance of the pathogenesis of the muscular dystrophies in relation to a diverse array of protein deficiencies. To compound things further, the X-linked and dominant forms of Emery-Dreifuss muscular dystrophy have recently been linked to emerin and lamin A/C, respectively, two proteins of the nuclear membrane, opening up yet another new ballpark of discovery.

Neuro-Linguistic Programming: The New Eclectic Therapy.

ERIC Educational Resources Information Center

Betts, Nicoletta C.

Richard Bandler and John Grinder developed neuro-linguisitc programming (NLP) after observing "the magical skills of potent psychotherapists" Frederick Perls, Virginia Satir, and Milton Erikson. They compiled the most effective techniques for building rapport, gathering data, and influencing change in psychotherapy, offering them only as…

Why is muscularity sexy? Tests of the fitness indicator hypothesis.

PubMed

Frederick, David A; Haselton, Martie G

2007-08-01

Evolutionary scientists propose that exaggerated secondary sexual characteristics are cues of genes that increase offspring viability or reproductive success. In six studies the hypothesis that muscularity is one such cue is tested. As predicted, women rate muscular men as sexier, more physically dominant and volatile, and less committed to their mates than nonmuscular men. Consistent with the inverted-U hypothesis of masculine traits, men with moderate muscularity are rated most attractive. Consistent with past research on fitness cues, across two measures, women indicate that their most recent short-term sex partners were more muscular than their other sex partners (ds = .36, .47). Across three studies, when controlling for other characteristics (e.g., body fat), muscular men rate their bodies as sexier to women (partial rs = .49-.62) and report more lifetime sex partners (partial rs = .20-.27), short-term partners (partial rs = .25-.28), and more affairs with mated women (partial r = .28).

Facioscapulohumeral Muscular Dystrophy.

PubMed

DeSimone, Alec M; Pakula, Anna; Lek, Angela; Emerson, Charles P

2017-09-12

Facioscapulohumeral Muscular Dystrophy is a common form of muscular dystrophy that presents clinically with progressive weakness of the facial, scapular, and humeral muscles, with later involvement of the trunk and lower extremities. While typically inherited as autosomal dominant, facioscapulohumeral muscular dystrophy (FSHD) has a complex genetic and epigenetic etiology that has only recently been well described. The most prevalent form of the disease, FSHD1, is associated with the contraction of the D4Z4 microsatellite repeat array located on a permissive 4qA chromosome. D4Z4 contraction allows epigenetic derepression of the array, and possibly the surrounding 4q35 region, allowing misexpression of the toxic DUX4 transcription factor encoded within the terminal D4Z4 repeat in skeletal muscles. The less common form of the disease, FSHD2, results from haploinsufficiency of the SMCHD1 gene in individuals carrying a permissive 4qA allele, also leading to the derepression of DUX4, further supporting a central role for DUX4. How DUX4 misexpression contributes to FSHD muscle pathology is a major focus of current investigation. Misexpression of other genes at the 4q35 locus, including FRG1 and FAT1, and unlinked genes, such as SMCHD1, has also been implicated as disease modifiers, leading to several competing disease models. In this review, we describe recent advances in understanding the pathophysiology of FSHD, including the application of MRI as a research and diagnostic tool, the genetic and epigenetic disruptions associated with the disease, and the molecular basis of FSHD. We discuss how these advances are leading to the emergence of new approaches to enable development of FSHD therapeutics. © 2017 American Physiological Society. Compr Physiol 7:1229-1279, 2017. Copyright © 2017 John Wiley & Sons, Inc.

[Diagnosis and treatment with steroids for patients with Duchenne muscular dystrophy: experience and recommendations for Mexico. Administración del Patrimonio de la Beneficencia Pública. Asociación de Distrofia Muscular de Occidente].

PubMed

Vázquez-Cárdenas, Norma A; Ibarra-Hernández, Francisco; López-Hernández, Luz B; Escobar-Cedillo, Rosa E; Ruano-Calderón, Luis A; Gómez-Díaz, Benjamín; García-Calderón, Noemí; Carriedo-Dávila, M Fernanda; Rojas-Hurtado, Liliana G; Luna-Padrón, Emilia; Coral-Vázquez, Ramón M

2013-11-16

Duchenne muscular dystrophy is a severe, debilitating and progressive disease that affects 1 in 3,500 live male births in the world. The diagnosis should be confirmed by genetic testing to identify the mutation in the DMD gene or muscle biopsy and immunostaining to demonstrate the absence of dystrophin. Although up to now continues to be an incurable disease, this does not mean it has no treatment. Treatment should be multidisciplinary, looking for the functionality of the patient and avoiding or correcting complications, mainly cardio-respiratory and skeletal. Many proposals have been evaluated and implemented with the aim of improving the quality of life for these patients. The long-term steroids have shown significant benefits, such as prolonging ambulation, reduce the need for spinal surgery, improve cardiorespiratory function and increase survival and the quality of life. This document presents the recommendations based on the experience of the working group and experts worldwide on the diagnosis and treatment with steroids for patients with Duchenne muscular dystrophy.

Validation of the Neuro-QoL measurement system in children with epilepsy.

PubMed

Lai, Jin-Shei; Nowinski, Cindy J; Zelko, Frank; Wortman, Katy; Burns, James; Nordli, Douglas R; Cella, David

2015-05-01

Children with epilepsy often face complex psychosocial consequences that are not fully captured by existing patient-reported outcome (PRO) measures. The Neurology Quality of Life Measurement System "Neuro-QoL" was developed to provide a set of common PRO measures that address issues important to people with neurologic disorders. This paper reports Neuro-QoL (anxiety, depression, interaction with peers, fatigue, pain, cognitive function, stigma, and upper and lower extremity functions) validation in children with epilepsy. Patients (aged 10-18years) diagnosed with epilepsy completed Neuro-QoL and legacy measures at time 1 (initial study visit) and 6-month follow-up. Internal consistency reliability was also evaluated. Concurrent validity was assessed by comparing Neuro-QoL measures with more established "legacy" measures of the same concepts. Clinical validity was evaluated by comparing mean Neuro-QoL scores of patients grouped by clinical anchors such as disease severity. Responsiveness of the Neuro-QoL from time 1 (initial study visit) to 6months was evaluated using self-reported change as the primary anchor. Sixty-one patients (mean age=13.4years; 62.3% male, 75.9% white) participated. Most patients (64.2%) had been seizure-free in the 3months prior to participation, and seizure frequency was otherwise described as follows: 17.8% daily, 13.3% weekly, 35.6% monthly, and 33.3% yearly. All patients were taking antiepileptic drugs. Patients reported better function/less symptoms compared to the reference groups. Internal consistency (alpha) coefficients ranged from 0.76 to 0.87. Patients with different seizure frequencies differed on anxiety (p<.01) and cognitive function (p<.05). Compared to patients on polytherapy, those on monotherapy had better upper extremity scores (p<.05). Compared to those with localized seizures, those experiencing generalized seizures reported worse stigma (p<.05). Depression, anxiety, lower extremity, fatigue, pain, interaction with peers

Guidelines for the Perianesthesia Care of the Duchenne Muscular Dystrophy/Becker Muscular Dystrophy Patient.

PubMed

Alliod, Barbara A; Ash, Rebecca A

2016-12-01

More patients suffering with Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are presenting to perianesthesia settings for emergent and nonemergent treatment and care. A group of collaborative health care providers at Rush University Medical Center in Chicago developed a multidisciplinary DMD/BMD Task Force to study this disorder and create a set of guidelines to aid those engaging in the planning, execution of care, and recovery of this unique population in the perianesthesia setting. Attention to detail, well-executed preplanning, meticulous awareness of the patient, and prearranged implementation and intervention has proven to offset potential problems and complications and is the key to a successful perianesthesia period. Copyright © 2016 American Society of PeriAnesthesia Nurses. Published by Elsevier Inc. All rights reserved.

A Case of Neuro-Behcet’s Disease Presenting with Central Neurogenic Hyperventilation

PubMed Central

Alkhachroum, Ayham M.; Saeed, Saba; Kaur, Jaspreet; Shams, Tanzila; De Georgia, Michael A.

2016-01-01

Patient: Female, 46 Final Diagnosis: Central hyperventilation Symptoms: Hyperventilation Medication: — Clinical Procedure: None Specialty: Neurology Objective: Unusual clinical course Background: Behcet’s disease is a chronic inflammatory disorder usually characterized by the triad of oral ulcers, genital ulcers, and uveitis. Central to the pathogenesis of Behcet’s disease is an autoimmune vasculitis. Neurological involvement, so called “Neuro-Behcet’s disease”, occurs in 10–20% of patients, usually from a meningoencephalitis or venous thrombosis. Case Report: We report the case of a 46-year-old patient with Neuro-Behcet’s disease who presented with central neurogenic hyperventilation as a result of brainstem involvement from venulitis. Conclusions: To the best of our knowledge, central neurogenic hyperventilation has not previously been described in a patient with Neuro-Behcet’s disease. PMID:26965646

Tocotrienol prevents AAPH-induced neurite degeneration in neuro2a cells.

PubMed

Fukui, Koji; Sekiguchi, Hidekazu; Takatsu, Hirokatsu; Koike, Taisuke; Koike, Tatsuro; Urano, Shiro

2013-01-01

Reactive oxygen species induce neurite degeneration before inducing cell death. However, the degenerative mechanisms have not yet been elucidated. While tocotrienols have a known neuroprotective function, the underlying mechanism remains unclear and may or may not involve antioxidant action. In this study, we hypothesize that free radical-derived membrane injury is one possible mechanism for inducing neurite degeneration. Therefore, we examined the potential neuroprotective effect of tocotrienols mediated through its antioxidant activity. Mouse neuroblastoma neuro2a cells were used to examine the effect of the water-soluble free radical generator 2,2'-azobis(2-methylpropionamide) dihydrochloride (AAPH) on neurite dynamics. After 24 hours of AAPH treatment, cell viability, neurite number, and the number of altered neurites were measured in the presence or absence of α-tocotrienol. Treatment of neuro2a cells with a low concentration of AAPH induces neurite degeneration, but not cell death. Treatment with 5 µM α-tocotrienol significantly inhibited neurite degeneration in AAPH-treated neuro2a cells. Furthermore, morphological changes in AAPH-treated neuro2a cells were similar to those observed with colchicine treatment. α-Tocotrienol may scavenge AAPH-derived free radicals and alkoxyl radicals that are generated from AAPH-derived peroxyl radicals on cell membranes. Therefore, α-tocotrienol may have a neuroprotective effect mediated by its antioxidant activity.

Respiratory function in facioscapulohumeral muscular dystrophy 1.

PubMed

Wohlgemuth, M; Horlings, C G C; van der Kooi, E L; Gilhuis, H J; Hendriks, J C M; van der Maarel, S M; van Engelen, B G M; Heijdra, Y F; Padberg, G W

2017-06-01

To test the hypothesis that wheelchair dependency and (kypho-)scoliosis are risk factors for developing respiratory insufficiency in facioscapulohumeral muscular dystrophy, we examined 81 patients with facioscapulohumeral muscular dystrophy 1 of varying degrees of severity ranging from ambulatory patients to wheelchair-bound patients. We examined the patients neurologically and by conducting pulmonary function tests: Forced Vital Capacity, Forced Expiratory Volume in 1 second, and static maximal inspiratory and expiratory mouth pressures. We did not find pulmonary function test abnormalities in ambulant facioscapulohumeral muscular dystrophy patients. Even though none of the patients complained of respiratory dysfunction, mild to severe respiratory insufficiency was found in more than one third of the wheelchair-dependent patients. Maximal inspiratory pressures and maximal expiratory pressures were decreased in most patients, with a trend that maximal expiratory pressures were more affected than maximal inspiratory pressures. Wheelchair-dependent patients with (kypho-)scoliosis showed the most restricted lung function. Wheelchair-dependent patients with (kypho-)scoliosis are at risk for developing respiratory function impairment. We advise examining this group of facioscapulohumeral muscular dystrophy patients periodically, even in the absence of symptoms of respiratory insufficiency, given its frequency and impact on daily life and the therapeutic consequences. Copyright © 2017 Elsevier B.V. All rights reserved.

Early-progressive dilated cardiomyopathy in a family with Becker muscular dystrophy related to a novel frameshift mutation in the dystrophin gene exon 27.

PubMed

Tsuda, Takeshi; Fitzgerald, Kristi; Scavena, Mena; Gidding, Samuel; Cox, Mary O; Marks, Harold; Flanigan, Kevin M; Moore, Steven A

2015-03-01

We report a family in which two male siblings with Becker muscular dystrophy (BMD) developed severe dilated cardiomyopathy (DCM) and progressive heart failure (HF) at age 11 years; one died at age 14 years while awaiting heart transplant and the other underwent left ventricular assist device implantation at the same age. Genetic analysis of one sibling showed a novel frameshift mutation in exon 27 of Duchenne muscular dystrophy (DMD) gene (c.3779_3785delCTTTGGAinsGG), in which seven base pairs are deleted and two are inserted. Although this predicts an amino-acid substitution and premature termination (p.Thr1260Argfs*8), muscle biopsy dystrophin immunostaining instead indicates that the mutation is more likely to alter splicing. Despite relatively preserved skeletal muscular performance, both the siblings developed progressive HF secondary to early-onset DCM. In addition, their 7-year-old nephew with delayed gross motor development, mild proximal muscle weakness and markedly elevated serum creatine kinase level (>13 000 IU l(-1)) at 16 months was recently demonstrated to have the familial DMD mutation. Here, we report a novel genotype of BMD with early-onset DCM and progressive lethal HF during early adolescence.

Cardiac function in muscular dystrophy associates with abdominal muscle pathology.

PubMed

Gardner, Brandon B; Swaggart, Kayleigh A; Kim, Gene; Watson, Sydeaka; McNally, Elizabeth M

The muscular dystrophies target muscle groups differentially. In mouse models of muscular dystrophy, notably the mdx model of Duchenne Muscular Dystrophy, the diaphragm muscle shows marked fibrosis and at an earlier age than other muscle groups, more reflective of the histopathology seen in human muscular dystrophy. Using a mouse model of limb girdle muscular dystrophy, the Sgcg mouse, we compared muscle pathology across different muscle groups and heart. A cohort of nearly 200 Sgcg mice were studied using multiple measures of pathology including echocardiography, Evans blue dye uptake and hydroxyproline content in multiple muscle groups. Spearman rank correlations were determined among echocardiographic and pathological parameters. The abdominal muscles were found to have more fibrosis than other muscle groups, including the diaphragm muscle. The abdominal muscles also had more Evans blue dye uptake than other muscle groups. The amount of diaphragm fibrosis was found to correlate positively with fibrosis in the left ventricle, and abdominal muscle fibrosis correlated with impaired left ventricular function. Fibrosis in the abdominal muscles negatively correlated with fibrosis in the diaphragm and right ventricles. Together these data reflect the recruitment of abdominal muscles as respiratory muscles in muscular dystrophy, a finding consistent with data from human patients.

Consensus statement for standard of care in spinal muscular atrophy.

PubMed

Wang, Ching H; Finkel, Richard S; Bertini, Enrico S; Schroth, Mary; Simonds, Anita; Wong, Brenda; Aloysius, Annie; Morrison, Leslie; Main, Marion; Crawford, Thomas O; Trela, Anthony

2007-08-01

Spinal muscular atrophy is a neurodegenerative disease that requires multidisciplinary medical care. Recent progress in the understanding of molecular pathogenesis of spinal muscular atrophy and advances in medical technology have not been matched by similar developments in the care for spinal muscular atrophy patients. Variations in medical practice coupled with differences in family resources and values have resulted in variable clinical outcomes that are likely to compromise valid measure of treatment effects during clinical trials. The International Standard of Care Committee for Spinal Muscular Atrophy was formed in 2005, with a goal of establishing practice guidelines for clinical care of these patients. The 12 core committee members worked with more than 60 spinal muscular atrophy experts in the field through conference calls, e-mail communications, a Delphi survey, and 2 in-person meetings to achieve consensus on 5 care areas: diagnostic/new interventions, pulmonary, gastrointestinal/nutrition, orthopedics/rehabilitation, and palliative care. Consensus was achieved on several topics related to common medical problems in spinal muscular atrophy, diagnostic strategies, recommendations for assessment and monitoring, and therapeutic interventions in each care area. A consensus statement was drafted to address the 5 care areas according to 3 functional levels of the patients: nonsitter, sitter, and walker. The committee also identified several medical practices lacking consensus and warranting further investigation. It is the authors' intention that this document be used as a guideline, not as a practice standard for their care. A practice standard for spinal muscular atrophy is urgently needed to help with the multidisciplinary care of these patients.

[Neuro-skeletal biology and its importance for clinical osteology].

PubMed

Zofková, I

2012-01-01

Bone remodeling is determined by function of two basic cell forms--bone resorbing osteoclasts and bone formation activating osteoblasts. Both cells are under control of a variety of endogenic and environmental factors, which ensure balance between bone resorption and bone formation. This article reviews the neuro-hormonal factors with osteoanabolic (central isoform of serotonin, melatonin, cannabinoids, beta 1 adrenergic system, oxytocin, ACTH and TSH) or osteocatabolic effects (neuropeptide Y, neuromedin U, beta2 adrenergic system). The dual effects of the beta-adrenergic system, serotonin and leptin are also discussed. The goal of studies focused on neuro-skeletal interaction is to synthesize new molecules, which can modify osteo-anabolic or osteo-catabolic pathways.

Neuro-Fuzzy Support of Knowledge Management in Social Regulation

NASA Astrophysics Data System (ADS)

Petrovic-Lazarevic, Sonja; Coghill, Ken; Abraham, Ajith

2002-09-01

The aim of the paper is to demonstrate the neuro-fuzzy support of knowledge management in social regulation. Knowledge could be understood for social regulation purposes as explicit and tacit. Explicit knowledge relates to the community culture indicating how things work in the community based on social policies and procedures. Tacit knowledge is ethics and norms of the community. The former could be codified, stored and transferable in order to support decision making, while the latter being based on personal knowledge, experience and judgments is difficult to codify and store. Tacit knowledge expressed through linguistic information can be stored and used to support knowledge management in social regulation through the application of fuzzy and neuro-fuzzy logic.

A Study of CAP-1002 in Ambulatory and Non-Ambulatory Patients With Duchenne Muscular Dystrophy

ClinicalTrials.gov

2018-06-16

Muscular Dystrophies; Muscular Dystrophy, Duchenne; Muscular Disorders, Atrophic; Muscular Diseases; Neuromuscular Diseases; Nervous System Diseases; Genetic Diseases, X-Linked; Genetic Diseases, Inborn

NeuroGrid: recording action potentials from the surface of the brain.

PubMed

Khodagholy, Dion; Gelinas, Jennifer N; Thesen, Thomas; Doyle, Werner; Devinsky, Orrin; Malliaras, George G; Buzsáki, György

2015-02-01

Recording from neural networks at the resolution of action potentials is critical for understanding how information is processed in the brain. Here, we address this challenge by developing an organic material-based, ultraconformable, biocompatible and scalable neural interface array (the 'NeuroGrid') that can record both local field potentials(LFPs) and action potentials from superficial cortical neurons without penetrating the brain surface. Spikes with features of interneurons and pyramidal cells were simultaneously acquired by multiple neighboring electrodes of the NeuroGrid, allowing for the isolation of putative single neurons in rats. Spiking activity demonstrated consistent phase modulation by ongoing brain oscillations and was stable in recordings exceeding 1 week's duration. We also recorded LFP-modulated spiking activity intraoperatively in patients undergoing epilepsy surgery. The NeuroGrid constitutes an effective method for large-scale, stable recording of neuronal spikes in concert with local population synaptic activity, enhancing comprehension of neural processes across spatiotemporal scales and potentially facilitating diagnosis and therapy for brain disorders.

Inhibition of Prostaglandin D Synthase Suppresses Muscular Necrosis

PubMed Central

Mohri, Ikuko; Aritake, Kosuke; Taniguchi, Hidetoshi; Sato, Yo; Kamauchi, Shinya; Nagata, Nanae; Maruyama, Toshihiko; Taniike, Masako; Urade, Yoshihiro

2009-01-01

Duchenne muscular dystrophy is a fatal muscle wasting disease that is characterized by a deficiency in the protein dystrophin. Previously, we reported that the expression of hematopoietic prostaglandin D synthase (HPGDS) appeared in necrotic muscle fibers from patients with either Duchenne muscular dystrophy or polymyositis. HPGDS is responsible for the production of the inflammatory mediator, prostaglandin D2. In this paper, we validated the hypothesis that HPGDS has a role in the etiology of muscular necrosis. We investigated the expression of HPGDS/ prostaglandin D2 signaling using two different mouse models of muscle necrosis, that is, bupivacaine-induced muscle necrosis and the mdx mouse, which has a genetic muscular dystrophy. We treated each mouse model with the HPGDS-specific inhibitor, HQL-79, and measured both necrotic muscle volume and selected cytokine mRNA levels. We confirmed that HPGDS expression was induced in necrotic muscle fibers in both bupivacaine-injected muscle and mdx mice. After administration of HQL-79, necrotic muscle volume was significantly decreased in both mouse models. Additionally, mRNA levels of both CD11b and transforming growth factor β1 were significantly lower in HQL-79-treated mdx mice than in vehicle-treated animals. We also demonstrated that HQL-79 suppressed prostaglandin D2 production and improved muscle strength in the mdx mouse. Our results show that HPGDS augments inflammation, which is followed by muscle injury. Furthermore, the inhibition of HPGDS ameliorates muscle necrosis even in cases of genetic muscular dystrophy. PMID:19359520

Bupivacaine-induced apoptosis independently of WDR35 expression in mouse neuroblastoma Neuro2a cells

PubMed Central

2012-01-01

Background Bupivacaine-induced neurotoxicity has been shown to occur through apoptosis. Recently, bupivacaine was shown to elicit reactive oxygen species (ROS) production and induce apoptosis accompanied by activation of p38 mitogen-activated protein kinase (MAPK) in a human neuroblastoma cell line. We have reported that WDR35, a WD40-repeat protein, may mediate apoptosis through caspase-3 activation. The present study was undertaken to test whether bupivacaine induces apoptosis in mouse neuroblastoma Neuro2a cells and to determine whether ROS, p38 MAPK, and WDR35 are involved. Results Our results showed that bupivacaine induced ROS generation and p38 MAPK activation in Neuro2a cells, resulting in apoptosis. Bupivacaine also increased WDR35 expression in a dose- and time-dependent manner. Hydrogen peroxide (H2O2) also increased WDR35 expression in Neuro2a cells. Antioxidant (EUK-8) and p38 MAPK inhibitor (SB202190) treatment attenuated the increase in caspase-3 activity, cell death and WDR35 expression induced by bupivacaine or H2O2. Although transfection of Neuro2a cells with WDR35 siRNA attenuated the bupivacaine- or H2O2-induced increase in expression of WDR35 mRNA and protein, in contrast to our previous studies, it did not inhibit the increase in caspase-3 activity in bupivacaine- or H2O2-treated cells. Conclusions In summary, our results indicated that bupivacaine induced apoptosis in Neuro2a cells. Bupivacaine induced ROS generation and p38 MAPK activation, resulting in an increase in WDR35 expression, in these cells. However, the increase in WDR35 expression may not be essential for the bupivacaine-induced apoptosis in Neuro2a cells. These results may suggest the existence of another mechanism of bupivacaine-induced apoptosis independent from WDR35 expression in Neuro2a cells. PMID:23227925

Citation classics in neuro-oncology: assessment of historical trends and scientific progress.

PubMed

Hachem, Laureen D; Mansouri, Alireza; Juraschka, Kyle; Taslimi, Shervin; Pirouzmand, Farhad; Zadeh, Gelareh

2017-09-01

Citation classics represent the highest cited works in a field and are often regarded as the most influential literature. Analyzing thematic trends in citation classics across eras enables recognition of important historical advances within a field. We present the first analysis of the citation classics in neuro-oncology. The Web of Science database was searched using terms relevant to "neuro-oncology." Articles with >400 citations were identified and the top 100 cited articles were evaluated. The top 100 neuro-oncology citation classics consisted of 43 clinical studies (17 retrospective, 10 prospective, 16 randomized trials), 43 laboratory investigations, 8 reviews/meta-analyses, and 6 guidelines/consensus statements. Articles were classified into 4 themes: 13 pertained to tumor classification, 37 to tumor pathogenesis/clinical presentation, 6 to imaging, 44 to therapy (15 chemotherapy, 10 radiotherapy, 5 surgery, 14 new agents). Gliomas were the most common tumor type examined, with 70 articles. There was a significant increase in the number of citation classics in the late 1990s, which was paralleled by an increase in studies examining tumor pathogenesis, chemotherapy, and new agents along with laboratory and randomized studies. The majority of citation classics in neuro-oncology are related to gliomas and pertain to tumor pathogenesis and treatment. The rise in citation classics in recent years investigating tumor biology, new treatment agents, and chemotherapeutics may reflect increasing scientific interest in nonsurgical treatments for CNS tumors and the need for fundamental investigations into disease processes. © The Author(s) 2017. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com

A mixed analog/digital chaotic neuro-computer system for quadratic assignment problems.

PubMed

Horio, Yoshihiko; Ikeguchi, Tohru; Aihara, Kazuyuki

2005-01-01

We construct a mixed analog/digital chaotic neuro-computer prototype system for quadratic assignment problems (QAPs). The QAP is one of the difficult NP-hard problems, and includes several real-world applications. Chaotic neural networks have been used to solve combinatorial optimization problems through chaotic search dynamics, which efficiently searches optimal or near optimal solutions. However, preliminary experiments have shown that, although it obtained good feasible solutions, the Hopfield-type chaotic neuro-computer hardware system could not obtain the optimal solution of the QAP. Therefore, in the present study, we improve the system performance by adopting a solution construction method, which constructs a feasible solution using the analog internal state values of the chaotic neurons at each iteration. In order to include the construction method into our hardware, we install a multi-channel analog-to-digital conversion system to observe the internal states of the chaotic neurons. We show experimentally that a great improvement in the system performance over the original Hopfield-type chaotic neuro-computer is obtained. That is, we obtain the optimal solution for the size-10 QAP in less than 1000 iterations. In addition, we propose a guideline for parameter tuning of the chaotic neuro-computer system according to the observation of the internal states of several chaotic neurons in the network.

The EPTN consensus-based atlas for CT- and MR-based contouring in neuro-oncology.

PubMed

Eekers, Daniëlle Bp; In 't Ven, Lieke; Roelofs, Erik; Postma, Alida; Alapetite, Claire; Burnet, Neil G; Calugaru, Valentin; Compter, Inge; Coremans, Ida E M; Høyer, Morton; Lambrecht, Maarten; Nyström, Petra Witt; Romero, Alejandra Méndez; Paulsen, Frank; Perpar, Ana; de Ruysscher, Dirk; Renard, Laurette; Timmermann, Beate; Vitek, Pavel; Weber, Damien C; van der Weide, Hiske L; Whitfield, Gillian A; Wiggenraad, Ruud; Troost, Esther G C

2018-03-13

To create a digital, online atlas for organs at risk (OAR) delineation in neuro-oncology based on high-quality computed tomography (CT) and magnetic resonance (MR) imaging. CT and 3 Tesla (3T) MR images (slice thickness 1 mm with intravenous contrast agent) were obtained from the same patient and subsequently fused. In addition, a 7T MR without intravenous contrast agent was obtained from a healthy volunteer. Based on discussion between experienced radiation oncologists, the clinically relevant organs at risk (OARs) to be included in the atlas for neuro-oncology were determined, excluding typical head and neck OARs previously published. The draft atlas was delineated by a senior radiation oncologist, 2 residents in radiation oncology, and a senior neuro-radiologist incorporating relevant available literature. The proposed atlas was then critically reviewed and discussed by European radiation oncologists until consensus was reached. The online atlas includes one CT-scan at two different window settings and one MR scan (3T) showing the OARs in axial, coronal and sagittal view. This manuscript presents the three-dimensional descriptions of the fifteen consensus OARs for neuro-oncology. Among these is a new OAR relevant for neuro-cognition, the posterior cerebellum (illustrated on 7T MR images). In order to decrease inter- and intra-observer variability in delineating OARs relevant for neuro-oncology and thus derive consistent dosimetric data, we propose this atlas to be used in photon and particle therapy. The atlas is available online at www.cancerdata.org and will be updated whenever required. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

NeuroNames: an ontology for the BrainInfo portal to neuroscience on the web.

PubMed

Bowden, Douglas M; Song, Evan; Kosheleva, Julia; Dubach, Mark F

2012-01-01

BrainInfo ( http://braininfo.org ) is a growing portal to neuroscientific information on the Web. It is indexed by NeuroNames, an ontology designed to compensate for ambiguities in neuroanatomical nomenclature. The 20-year old ontology continues to evolve toward the ideal of recognizing all names of neuroanatomical entities and accommodating all structural concepts about which neuroscientists communicate, including multiple concepts of entities for which neuroanatomists have yet to determine the best or 'true' conceptualization. To make the definitions of structural concepts unambiguous and terminologically consistent we created a 'default vocabulary' of unique structure names selected from existing terminology. We selected standard names by criteria designed to maximize practicality for use in verbal communication as well as computerized knowledge management. The ontology of NeuroNames accommodates synonyms and homonyms of the standard terms in many languages. It defines complex structures as models composed of primary structures, which are defined in unambiguous operational terms. NeuroNames currently relates more than 16,000 names in eight languages to some 2,500 neuroanatomical concepts. The ontology is maintained in a relational database with three core tables: Names, Concepts and Models. BrainInfo uses NeuroNames to index information by structure, to interpret users' queries and to clarify terminology on remote web pages. NeuroNames is a resource vocabulary of the NLM's Unified Medical Language System (UMLS, 2011) and the basis for the brain regions component of NIFSTD (NeuroLex, 2011). The current version has been downloaded to hundreds of laboratories for indexing data and linking to BrainInfo, which attracts some 400 visitors/day, downloading 2,000 pages/day.

The incidence of neuro-ophthalmic diseases in Singapore: a prospective study in public hospitals.

PubMed

Lim, Su Ann; Wong, Wan Ling; Fu, Esther; Goh, Kong Yong; Seah, Alvin; Tan, Clement; Tow, Sharon; Cullen, James F; Wong, Tien Y

2009-01-01

To describe the incidence of neuro-ophthalmic diseases in a multi-ethnic Asian population in Singapore. Prospective study in public hospitals in Singapore. All neuro-ophthalmic cases seen in four public sector hospitals over a 22-month period (September 2002 to June 2004) were identified using a standardized protocol. The 2004 Singapore population was used as a denominator to estimate annual incidence. The prevalence of ischemic risk factors (hypertension, diabetes, and hypercholesterolemia) among cases was compared to population data. A total of 1,356 patients with neuro-ophthalmic diseases were seen during the study period, of which 627 were new incident cases. The overall annual incidence of neuro-ophthalmic diseases was 9.81 per 100,000 (95% confidence interval, 8.80-10.90). The incidence increased with age. After controlling for age, the annual incidence was similar between men (10.75 per 100,000) and women (9.00 per 100,000), but was higher in Chinese (10.33 per 100,000) and Indians (9.34 per 100,000) than in Malays (6.62 per 100,000). The three commonest specific neuro-ophthalmic conditions were abducens nerve palsy (1.27 per 100,000), anterior ischemic optic neuropathy (1.08 per 100,000) and oculomotor nerve palsy (0.91 per 100,000). The incidence of optic neuritis was 0.83 per 100,000. Compared with the Singapore general population, the prevalence of diabetes was significantly higher in people aged 40-59, while the prevalence of hypercholesterolemia was significantly higher in 60-69 year age group. In this study of public hospitals in Singapore, the incidence of neuro-ophthalmic diseases was higher in Chinese and Indians compared to Malays.

A selected review of abstracts from the 20th Annual Meeting of the Society for Neuro-Oncology (SNO).

PubMed

Chamberlain, Marc C

2016-07-01

20th Annual Meeting of the Society for Neuro-Oncology, San Antonio, TX, USA, 18-22 November 2015 The Society for Neuro-Oncology is the largest neuro-oncology meeting in the USA that meets annually and provides a multiday venue that showcases new brain cancer clinical trial results and basic research primarily pertaining to gliomas. The Society for Neuro-Oncology 2015 meeting comprising one education day, 2 days of premeetings and 3 days of presentation, over 200 oral presentations and 900 abstracts provides an overview of contemporary neuro-oncology that includes metastatic disease of the central nervous system as well as primary brain tumors. This review attempts to highlight select abstracts presented at this year's meeting in a short summary that provides a synopsis of a large and multifaceted meeting.

The relationship between muscularity, muscle:bone ratio and cut dimensions in male and female lamb carcasses and the measurement of muscularity using image analysis.

PubMed

Hopkins, D L

1996-12-01

Dorsal images of 57 whole lamb carcasses (mean 22.5 kg, SD 2.3 kg) were obtained on a slaughter chain using a video camera. The lambs represented two sexes (29 cryptorchids, 28 ewes) and one genotype (Poll Dorset × Border Leicester × Merino). Cryptorchid carcasses were significantly (P < 0.05) leaner than ewe carcasses at a common weight but there was little difference in dimensional measurements of M. longissimus thoracis et lumborum (LL). The cryptorchid carcasses had a significantly better conformation (based on the EUROP system) even when adjusted to the same carcass weight and subcutaneous fat level. From the hindleg and chump the following muscles were dissected and weighed: M. semimembranosus, M. adductor femoris, M. semitendinosus, M. biceps femoris, and M. quadriceps femoris. The femur was weighed, the length measured and a muscularity value calculated as described by Purchas et al. (1991 Meat Sci., 30, 181). There was no significant effect of sex on muscularity or muscle to bone ratio (M:B). Cryptorchid carcasses produced heavier (P < 0.05) round and midloin cuts but lighter (P < 0.05) chump and ribloin cuts. Overall there was no significant sex effect on the yield of hindquarter cuts. Correlation showed a significant (P < 0.001) association between LL area and muscularity, with a lower correlation between round and topside cross-sectional area and muscularity. Neither muscle cross-sectional area nor muscularity was significantly related to M:B ratios. Muscularity increased with increasing carcass weight (P < 0.001) but M:B did not. Prediction of muscularity was significantly (P < 0.05) improved by adding to hot carcass weight a measure of the combined width across the hind legs at interval three, as taken from video images, there being five equally-spaced intervals from the groin to the gambrel. A similar result was achieved by using carcass width at the third interval of five-eventy spaced intervals between the minimum shoulder width and the point of

Dysphagia in Duchenne Muscular Dystrophy Assessed by Validated Questionnaire

ERIC Educational Resources Information Center

Archer, Sally K.; Garrod, Rachel; Hart, Nicholas; Miller, Simon

2013-01-01

Background: Duchenne muscular dystrophy (DMD) leads to progressive muscular weakness and death, most typically from respiratory complications. Dysphagia is common in DMD; however, the most appropriate swallowing assessments have not been universally agreed and the symptoms of dysphagia remain under-reported. Aims: To investigate symptoms of…

From Emotions to Consciousness – A Neuro-Phenomenal and Neuro-Relational Approach

PubMed Central

Northoff, Georg

2012-01-01

The James–Lange theory considers emotional feelings as perceptions of physiological body changes. This approach has recently resurfaced and modified in both neuroscientific and philosophical concepts of embodiment of emotional feelings. In addition to the body, the role of the environment in emotional feeling needs to be considered. I here claim that the environment has not merely an indirect and instrumental, i.e., modulatory role on emotional feelings via the body and its sensorimotor and vegetative functions. Instead, the environment may have a direct and non-instrumental, i.e., constitutional role in emotional feelings. This implies that the environment itself is constitutive of emotional feeling rather than the bodily representation of the environment. I call this the relational concept of emotional feeling. The present paper discusses recent data from neuroimaging that investigate emotions in relation to interoceptive processing and the brain’s intrinsic activity. These data show the intrinsic linkage of interoceptive stimulus processing to both exteroceptive stimuli and the brain’s intrinsic activity. This is possible only if the differences between intrinsic activity and intero- and exteroceptive stimuli is encoded into neural activity. Such relational coding makes possible the assignment of subjective and affective features to the otherwise objective and non-affective stimulus. I therefore consider emotions to be intrinsically affective and subjective as it is manifest in emotional feelings. The relational approach thus goes together with what may be described as neuro-phenomenal approach. Such neuro-phenomenal approach does not only inform emotions and emotional feeling but is also highly relevant to better understand the neuronal mechanisms underlying consciousness in general. PMID:22969736

Muscular and Aerobic Fitness, Working Memory, and Academic Achievement in Children.

PubMed

Kao, Shih-Chun; Westfall, Daniel R; Parks, Andrew C; Pontifex, Matthew B; Hillman, Charles H

2017-03-01

This study investigated the relationship between aerobic and muscular fitness with working memory and academic achievement in preadolescent children. Seventy-nine 9- to 11-yr-old children completed an aerobic fitness assessment using a graded exercise test; a muscular fitness assessment consisting of upper body, lower body, and core exercises; a serial n-back task to assess working memory; and an academic achievement test of mathematics and reading. Hierarchical regression analyses indicated that after controlling for demographic variables (age, sex, grade, IQ, socioeconomic status), aerobic fitness was associated with greater response accuracy and d' in the 2-back condition and increased mathematic performance in algebraic functions. Muscular fitness was associated with increased response accuracy and d', and longer reaction time in the 2-back condition. Further, the associations of muscular fitness with response accuracy and d' in the 2-back condition were independent of aerobic fitness. The current findings suggest the differential relationships between the aerobic and the muscular aspects of physical fitness with working memory and academic achievement. With the majority of research focusing on childhood health benefits of aerobic fitness, this study suggests the importance of muscular fitness to cognitive health during preadolescence.

Genetics Home Reference: spinal muscular atrophy with progressive myoclonic epilepsy

MedlinePlus

... myoclonic epilepsy Spinal muscular atrophy with progressive myoclonic epilepsy Printable PDF Open All Close All Enable Javascript ... boxes. Description Spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME) is a neurological condition that causes ...

Heidelberg Neuro-Music Therapy for chronic-tonal tinnitus - treatment outline and psychometric evaluation.

PubMed

Argstatter, Heike; Grapp, Miriam; Plinkert, Peter K; Bolay, Hans Volker

2012-01-01

Musical training positively influences the cortical plasticity of the brain and has proven to be effective in treating chronic tinnitus. A neuro-music therapy concept, the "Heidelberg Neuro-Music Therapy" treatment was developed and evaluated. A prospective, cross-sectional design was used. N = 135 patients (mean age 47 years) with chronic, tonal tinnitus attended a standardized protocol for Neuro-Music Therapy (either "standard therapy" ST or "compact therapy" CT). The results were compared to a cognitive behavioral placebo music therapy procedure (PT). Tinnitus distress was assessed using the German version of the Tinnitus-Questionnaire (TQ) at admission, at discharge and six months after therapy. Changes were assessed statistically and by means of clinical significance. TQ scores significantly improved - independent of group allocation. But more than 80% of the music therapy patients (both ST and CT) revealed a reliable improvement ("responder") compared to 44% in the PT group. Therapy impact seems to be lasting since TQ scores remained stable until follow-up at six months. The "Heidelberg Neuro-Music Therapy" is a method with fast onset and long lasting effect for patients with "tonal" tinnitus. A number of potential working factors accounting for the treatment success are highlighted.

GNOSIS: guidelines for neuro-oncology: standards for investigational studies--reporting of surgically based therapeutic clinical trials.

PubMed

Chang, Susan; Vogelbaum, Michael; Lang, Frederick F; Haines, Stephen; Kunwar, Sandeep; Chiocca, E Antonio; Olivi, Alessandro; Quinones-Hinojosa, Alfredo; Parsa, Andrew; Warnick, Ronald

2007-04-01

We present guidelines to standardize the reporting of surgically based neuro-oncology trials. The guidelines are summarized in a checklist format that can be used as a framework from which to construct a surgically based trial. This manuscript follows and is taken in part from GNOSIS: Guidelines for neuro-oncology: Standards for investigational studies-reporting of phase 1 and phase 2 clinical trials [Chang SM, Reynolds SL, Butowski N, Lamborn KR, Buckner JC, Kaplan RS, Bigner DD (2005) Neuro-oncology 7:425-434].

NeuroElectro: a window to the world's neuron electrophysiology data

PubMed Central

Tripathy, Shreejoy J.; Savitskaya, Judith; Burton, Shawn D.; Urban, Nathaniel N.; Gerkin, Richard C.

2014-01-01

The behavior of neural circuits is determined largely by the electrophysiological properties of the neurons they contain. Understanding the relationships of these properties requires the ability to first identify and catalog each property. However, information about such properties is largely locked away in decades of closed-access journal articles with heterogeneous conventions for reporting results, making it difficult to utilize the underlying data. We solve this problem through the NeuroElectro project: a Python library, RESTful API, and web application (at http://neuroelectro.org) for the extraction, visualization, and summarization of published data on neurons' electrophysiological properties. Information is organized both by neuron type (using neuron definitions provided by NeuroLex) and by electrophysiological property (using a newly developed ontology). We describe the techniques and challenges associated with the automated extraction of tabular electrophysiological data and methodological metadata from journal articles. We further discuss strategies for how to best combine, normalize and organize data across these heterogeneous sources. NeuroElectro is a valuable resource for experimental physiologists attempting to supplement their own data, for computational modelers looking to constrain their model parameters, and for theoreticians searching for undiscovered relationships among neurons and their properties. PMID:24808858

Evaluating cognition in individuals with Huntington disease: Neuro-QoL cognitive functioning measures.

PubMed

Lai, Jin-Shei; Goodnight, Siera; Downing, Nancy R; Ready, Rebecca E; Paulsen, Jane S; Kratz, Anna L; Stout, Julie C; McCormack, Michael K; Cella, David; Ross, Christopher; Russell, Jenna; Carlozzi, Noelle E

2018-03-01

Cognitive functioning impacts health-related quality of life (HRQOL) for individuals with Huntington disease (HD). The Neuro-QoL includes two patient-reported outcome (PRO) measures of cognition-Executive Function (EF) and General Concerns (GC). These measures have not previously been validated for use in HD. The purpose of this analysis is to evaluate the reliability and validity of the Neuro-QoL Cognitive Function measures for use in HD. Five hundred ten individuals with prodromal or manifest HD completed the Neuro-QoL Cognition measures, two other PRO measures of HRQOL (WHODAS 2.0 and EQ5D), and a depression measure (PROMIS Depression). Measures of functioning The Total Functional Capacity and behavior (Problem Behaviors Assessment) were completed by clinician interview. Objective measures of cognition were obtained using clinician-administered Symbol Digit Modalities Test and the Stroop Test (Word, Color, and Interference). Self-rated, clinician-rated, and objective composite scores were developed. We examined the Neuro-QoL measures for reliability, convergent validity, discriminant validity, and known-groups validity. Excellent reliabilities (Cronbach's alphas ≥ 0.94) were found. Convergent validity was supported, with strong relationships between self-reported measures of cognition. Discriminant validity was supported by less robust correlations between self-reported cognition and other constructs. Prodromal participants reported fewer cognitive problems than manifest groups, and early-stage HD participants reported fewer problems than late-stage HD participants. The Neuro-QoL Cognition measures provide reliable and valid assessments of self-reported cognitive functioning for individuals with HD. Findings support the utility of these measures for assessing self-reported cognition.

Intramuscular pressure and torque during isometric, concentric and eccentric muscular activity

NASA Technical Reports Server (NTRS)

Styf, J.; Ballard, R.; Aratow, M.; Crenshaw, A.; Watenpaugh, D.; Hargens, A. R.

1995-01-01

Intramuscular pressures, electromyography (EMG) and torque generation during isometric, concentric and eccentric maximal isokinetic muscle activity were recorded in 10 healthy volunteers. Pressure and EMG activity were continuously and simultaneously measured side by side in the tibialis anterior and soleus muscles. Ankle joint torque and position were monitored continuously by an isokinetic dynamometer during plantar flexion and dorsiflexion of the foot. The increased force generation during eccentric muscular activity, compared with other muscular activity, was not accompanied by higher intramuscular pressure. Thus, this study demonstrated that eccentric muscular activity generated higher torque values for each increment of intramuscular pressure. Intramuscular pressures during antagonistic co-activation were significantly higher in the tibilis anterior muscle (42-46% of maximal agonistic activity) compared with the soleus muscle (12-29% of maximal agonistic activity) and was largely due to active recruitment of muscle fibers. In summary, eccentric muscular activity creates higher torque values with no additional increase of the intramuscular pressure compared with concentric and isometric muscular activity.

The Impact of Obesity on Back and Core Muscular Endurance in Firefighters

PubMed Central

Mayer, John M.; Nuzzo, James L.; Chen, Ren; Quillen, William S.; Verna, Joe L.; Miro, Rebecca; Dagenais, Simon

2012-01-01

The purpose of this study was to assess the relationships between obesity and measures of back and core muscular endurance in firefighters. Methods. A cross-sectional study was conducted in career firefighters without low back pain. Obesity measures included body mass index (BMI) and body fat percentage assessed with air displacement plethysmography. Muscular endurance was assessed with the Modified Biering Sorensen (back) and Plank (core) tests. Relationships were explored using t-tests and regression analyses. Results. Of the 83 participants enrolled, 24 (29%) were obese (BMI ≥ 30). Back and core muscular endurance was 27% lower for obese participants. Significant negative correlations were observed for BMI and body fat percentage with back and core endurance (r = −0.42 to −0.52). Stepwise regression models including one obesity measure (BMI, body fat percentage, and fat mass/fat-free mass), along with age and self-reported physical exercise, accounted for 17–19% of the variance in back muscular endurance and 29–37% of the variance in core muscular endurance. Conclusions. Obesity is associated with reduced back and core muscular endurance in firefighters, which may increase the risk of musculoskeletal injuries. Obesity should be considered along with back and core muscular endurance when designing exercise programs for back pain prevention in firefighters. PMID:23213491

Meaning of Muscular Dystrophy

MedlinePlus

... is very similar to Duchenne, except kids with Becker MD may not have problems until much later, when they're teenagers or adults. It takes a long time for their muscles to become weak. How Does a Kid Get Muscular Dystrophy? MD is not contagious (say: con-TAY-juss), ...

Muscular activity and its relationship to biomechanics and human performance

NASA Technical Reports Server (NTRS)

Ariel, Gideon

1994-01-01

The purpose of this manuscript is to address the issue of muscular activity, human motion, fitness, and exercise. Human activity is reviewed from the historical perspective as well as from the basics of muscular contraction, nervous system controls, mechanics, and biomechanical considerations. In addition, attention has been given to some of the principles involved in developing muscular adaptations through strength development. Brief descriptions and findings from a few studies are included. These experiments were conducted in order to investigate muscular adaptation to various exercise regimens. Different theories of strength development were studied and correlated to daily human movements. All measurement tools used represent state of the art exercise equipment and movement analysis. The information presented here is only a small attempt to understand the effects of exercise and conditioning on Earth with the objective of leading to greater knowledge concerning human responses during spaceflight. What makes life from nonliving objects is movement which is generated and controlled by biochemical substances. In mammals. the controlled activators are skeletal muscles and this muscular action is an integral process composed of mechanical, chemical, and neurological processes resulting in voluntary and involuntary motions. The scope of this discussion is limited to voluntary motion.

Linking of the quality of life in neurological disorders (Neuro-QoL) to the international classification of functioning, disability and health.

PubMed

Wong, Alex W K; Lau, Stephen C L; Cella, David; Lai, Jin-Shei; Xie, Guanli; Chen, Lidian; Chan, Chetwyn C H; Heinemann, Allen W

2017-09-01

The quality of life in neurological disorders (Neuro-QoL) is a U.S. National Institutes of Health initiative that produced a set of self-report measures of physical, mental, and social health experienced by adults or children who have a neurological condition or disorder. To describe the content of the Neuro-QoL at the item level using the World Health Organization's international classification of functioning, disability and health (ICF). We assessed the Neuro-QoL for its content coverage of functioning and disability relative to each of the four ICF domains (i.e., body functions, body structures, activities and participation, and environment). We used second-level ICF three-digit codes to classify items into categories within each ICF domain and computed the percentage of categories within each ICF domain that were represented in the Neuro-QoL items. All items of Neuro-QoL could be mapped to the ICF categories at the second-level classification codes. The activities and participation domain and the mental functions category of the body functions domain were the areas most often represented by Neuro-QoL. Neuro-QoL provides limited coverage of the environmental factors and body structure domains. Neuro-QoL measures map well to the ICF. The Neuro-QoL-ICF-mapped items provide a blueprint for users to select appropriate measures in ICF-based measurement applications.

NeuroLines: A Subway Map Metaphor for Visualizing Nanoscale Neuronal Connectivity.

PubMed

Al-Awami, Ali K; Beyer, Johanna; Strobelt, Hendrik; Kasthuri, Narayanan; Lichtman, Jeff W; Pfister, Hanspeter; Hadwiger, Markus

2014-12-01

We present NeuroLines, a novel visualization technique designed for scalable detailed analysis of neuronal connectivity at the nanoscale level. The topology of 3D brain tissue data is abstracted into a multi-scale, relative distance-preserving subway map visualization that allows domain scientists to conduct an interactive analysis of neurons and their connectivity. Nanoscale connectomics aims at reverse-engineering the wiring of the brain. Reconstructing and analyzing the detailed connectivity of neurons and neurites (axons, dendrites) will be crucial for understanding the brain and its development and diseases. However, the enormous scale and complexity of nanoscale neuronal connectivity pose big challenges to existing visualization techniques in terms of scalability. NeuroLines offers a scalable visualization framework that can interactively render thousands of neurites, and that supports the detailed analysis of neuronal structures and their connectivity. We describe and analyze the design of NeuroLines based on two real-world use-cases of our collaborators in developmental neuroscience, and investigate its scalability to large-scale neuronal connectivity data.

Upper Body Muscular Endurance Among Children 2-5 Years.

ERIC Educational Resources Information Center

Gabbard, Carl P.; And Others

The upper body muscular endurance of males and females 2-5 years of age was assessed, and relationships relative to sex, age, endurance and selected anthropometric measures were investigated. None of the relationships were found to be of practical predicative value; while upper body muscular strength increased with age, no significant differences…

Reflections on 50 Years of Neuroscience Nursing: Neuro-Oncology, Moving Forward by Looking Back.

PubMed

Reed, Marilyn E; Anthony, Patricia P; Rosenfeld, Priscilla B; Ligon, Brandi L; Doris, Estelle M; Fox, Sherry W

2018-06-01

During the past 50 years, there have been more than 100 articles published in the Journal of Neuroscience Nursing covering the topic of neuro-oncology. This article will explore the historical implications and milestones from these articles. The analysis highlights the scope and depth of the many articles as they relate to the advancements in neuro-oncology.

Intelligence and cognitive function in children and adolescents with spinal muscular atrophy.

PubMed

von Gontard, A; Zerres, K; Backes, M; Laufersweiler-Plass, C; Wendland, C; Melchers, P; Lehmkuhl, G; Rudnik-Schöneborn, S

2002-02-01

Spinal muscular atrophy is a chronic disease characterised by loss of motor function. The aim of the study was to analyse cognitive functions in a large group of patients with spinal muscular atrophy. It was hypothesised that their intelligence is comparable to controls, but not above average as previously postulated. Ninety-six children and adolescents with spinal muscular atrophy I-III, aged 6.0-18.11 years, 45 non-affected siblings and 59 healthy, matched controls were examined with one- (CPM/SPM), as well as multi-dimensional intelligence tests (Kaufman-ABC; Wechsler tests). The mean IQ measured with the CPM/SPM tests was 109.6 for the spinal muscular atrophy group, 107.3 for the sibs and 104.1 for the healthy controls (no significant difference). In the older children and adolescents (SPM only) the mean IQ was significantly higher for the spinal muscular atrophy patients (109.6) than for the controls (95.4). The standard score in the 'mental processing composite' scale of the Kaufman-ABC was identical in the spinal muscular atrophy group and controls (103.8). The cognitive profile was relatively homogeneous. However, the older children and adolescents did have a significantly higher verbal IQ (113.8) than controls (104.6) in the Wechsler tests. There were no significant differences in any of the tests among different grades of severity (spinal muscular atrophy types I-III). It can be concluded that children and adolescents with spinal muscular atrophy have a general intelligence in the normal range. By adolescence, environmentally mediated aspects of intelligence are higher in patients with spinal muscular atrophy. It could be speculated that the development of cognitive skills and knowledge is a creative way to compensate the many restrictions due to their physical handicap.

Willingness to Pay for a Newborn Screening Test for Spinal Muscular Atrophy.

PubMed

Lin, Pei-Jung; Yeh, Wei-Shi; Neumann, Peter J

2017-01-01

The current US mandatory newborn screening panel does not include spinal muscular atrophy, the most common fatal genetic disease among children. We assessed population preferences for newborn screening for spinal muscular atrophy, and how test preferences varied depending on immediate treatment implications. We conducted an online willingness-to-pay survey of US adults (n = 982). Respondents were asked to imagine being parents of a newborn. Each respondent was presented with two hypothetical scenarios following the spinal muscular atrophy screening test: current standard of care (no treatment available) and one of three randomly assigned scenarios (new treatment available to improve functioning, survival, or both). We used a bidding game to elicit willingness to pay for the spinal muscular atrophy test, and performed a two-part model to estimate median and mean willingness-to-pay values. Most respondents (79% to 87%) would prefer screening their newborns for spinal muscular atrophy. People expressed a willingness to pay for spinal muscular atrophy screening even without an available therapy (median: $142; mean: $253). Willingness to pay increased with treatment availability (median: $161 to $182; mean: $270 to $297) and respondent income. Most respondents considered test accuracy, treatment availability, and treatment effectiveness very important or important factors in deciding willingness to pay. Most people would prefer and would be willing to pay for testing their newborn for spinal muscular atrophy, even in the absence of direct treatment. People perceive the spinal muscular atrophy test more valuable if treatment were available to improve the newborn's functioning and survival. Despite preferences for the test information, adding spinal muscular atrophy to newborn screening programs remains controversial. Future studies are needed to determine how early detection may impact long-term patient outcomes. Copyright © 2016 Elsevier Inc. All rights reserved.

An Exploration of the Drive for Muscularity in Adolescent Boys and Girls.

ERIC Educational Resources Information Center

McCreary, Donald R.; Sasse, Doris K.

2000-01-01

Investigated the drive for muscularity among high school adolescents using the Drive for Muscularity Scale. Results indicated that the scale was reliable. High-drive students were mainly boys trying to gain weight and muscle mass. Drive related to poor self-esteem and higher depression levels among boys, but not girls. Drive for muscularity was…

LAMA2-related myopathy: Frequency among congenital and limb-girdle muscular dystrophies.

PubMed

Løkken, Nicoline; Born, Alfred Peter; Duno, Morten; Vissing, John

2015-10-01

Muscular dystrophy caused by LAMA2-gene mutations is an autosomal recessive disease typically presenting as a severe, early-onset congenital muscular dystrophy (CMD). However, milder cases with a limb-girdle type muscular dystrophy (LGMD) have been described. In this study, we assessed the frequency and phenotypic spectrum of LAMA2-related muscular dystrophy in CMD (n = 18) and LGMD2 (n = 128) cohorts identified in the last 15 years in eastern Denmark. The medical history, brain-MRI, muscle pathology, muscle laminin-α2 expression, and genetic analyses were assessed. Molecular genetics revealed 2 pathogenic LAMA2 mutations in 5 of 18 CMD and 3 of 128 LGMD patients, corresponding to a LAMA2-mutation frequency of 28% in the CMD and 2.3% in the LGMD cohorts, respectively. This study demonstrates a wide clinical spectrum of LAMA2-related muscular dystrophy and its prevalence in an LGMD2 cohort, which indicates that LAMA2 muscular dystrophy should be included in the LGMD2 nomenclature. © 2015 Wiley Periodicals, Inc.

Integrating RNA sequencing into neuro-oncology practice.

PubMed

Rogawski, David S; Vitanza, Nicholas A; Gauthier, Angela C; Ramaswamy, Vijay; Koschmann, Carl

2017-11-01

Malignant tumors of the central nervous system (CNS) cause substantial morbidity and mortality, yet efforts to optimize chemo- and radiotherapy have largely failed to improve dismal prognoses. Over the past decade, RNA sequencing (RNA-seq) has emerged as a powerful tool to comprehensively characterize the transcriptome of CNS tumor cells in one high-throughput step, leading to improved understanding of CNS tumor biology and suggesting new routes for targeted therapies. RNA-seq has been instrumental in improving the diagnostic classification of brain tumors, characterizing oncogenic fusion genes, and shedding light on intratumor heterogeneity. Currently, RNA-seq is beginning to be incorporated into regular neuro-oncology practice in the form of precision neuro-oncology programs, which use information from tumor sequencing to guide implementation of personalized targeted therapies. These programs show great promise in improving patient outcomes for tumors where single agent trials have been ineffective. As RNA-seq is a relatively new technique, many further applications yielding new advances in CNS tumor research and management are expected in the coming years. Copyright © 2017 Elsevier Inc. All rights reserved.

"The sixth sense": towards a history of muscular sensation.

PubMed

Smith, Roger

2011-01-01

This paper outlines the history of knowledge about the muscular sense and provides a bibliographic resource for further research. A range of different topics, questions and approaches have interrelated throughout this history, and the discussion clarifies this rather than presenting detailed research in any one area. Part I relates the origin of belief in a muscular sense to empiricist accounts of the contribution of the senses to knowledge from Locke, via the iddologues and other authors, to the second half of the nineteenth century. Analysis paid much attention to touch, first in the context of the theory of vision and then in its own right, which led to naming a distinct muscular sense. From 1800 to the present, there was much debate, the main lines of which this paper introduces, about the nature and function of what turned out to be a complex sense. A number of influential psycho-physiologists, notably Alexander Bain and Herbert Spencer, thought this sense the most primitive and primary of all, the origin of knowledge of world, causation and self as an active subject. Part II relates accounts of the muscular sense to the development of nervous physiology and of psychology. In the decades before 1900, the developing separation of philosophy, psychology and physiology as specialised disciplines divided up questions which earlier writers had discussed under the umbrella heading of muscular sensation. The term'kinaesthesia' came in 1880 and 'proprio-ception' in 1906. There was, all the same, a lasting interest in the argument that touch and muscular sensation are intrinsic to the existence of embodied being in the way the other senses are not. In the wider culture--the arts, sport, the psychophysiology of labour and so on--there were many ways in which people expressed appreciation of the importance of what the anatomist Charles Bell had called 'the sixth sense'.

Muscular system in interna of Peltogaster paguri (Rhizocephala: Peltogastridae).

PubMed

Miroliubov, Aleksei A

2017-03-01

Rhizocephalan parasites have a peculiar life cycle, and their adults lost almost all traits found usually in Crustacea. Despite some data on anatomy and ultrastructure of interna of Peltogastridae, some crucial aspects of morphology are still unknown. For example, there is only one mentioning of myocytes found in interna of Rhizocephalans (Sacculina carcini). So we aimed at studying the muscular system of the interna of Peltogaster paguri using serial histological sectioning and fluorescent staining (TRITC-labeled phalloidin) with confocal microscopy. Within the wall of the main trunk we found striated muscular fibers. The majority of these fibers form a unidirectional single spiral. There are additional small fibers that connect the coils of the large spiral. The density of muscular fibers is highest near the externa stalk, and the number of muscle fibers decreases towards the distal part of the main trunk. We suggest that such a muscular system could provide peristaltic movements of the main trunk and the transport of nutrients through the interna. Copyright © 2016 Elsevier Ltd. All rights reserved.

Yogic exercises and health--a psycho-neuro immunological approach.

PubMed

Kulkarni, D D; Bera, T K

2009-01-01

Relaxation potential of yogic exercises seems to play a vital role in establishing psycho-physical health in reversing the psycho-immunology of emotions under stress based on breath and body awareness. However, mechanism of yogic exercises for restoring health and fitness components operating through psycho-neuro-immunological pathways is unknown. Therefore, a hybrid model of human information processing-psycho-neuroendocrine (HIP-PNE) network has been proposed to reveal the importance of yogic information processing. This study focuses on two major pathways of information processing involving cortical and hypothalamo-pituitary-adrenal axis (HPA) interactions with a deep reach molecular action on cellular, neuro-humoral and immune system in reversing stress mediated diseases. Further, the proposed HIP-PNE model has ample of experimental potential for objective evaluation of yogic view of health and fitness.

Dasatinib as a treatment for Duchenne muscular dystrophy.

PubMed

Lipscomb, Leanne; Piggott, Robert W; Emmerson, Tracy; Winder, Steve J

2016-01-15

Identification of a systemically acting and universal small molecule therapy for Duchenne muscular dystrophy would be an enormous advance for this condition. Based on evidence gained from studies on mouse genetic models, we have identified tyrosine phosphorylation and degradation of β-dystroglycan as a key event in the aetiology of Duchenne muscular dystrophy. Thus, preventing tyrosine phosphorylation and degradation of β-dystroglycan presents itself as a potential therapeutic strategy. Using the dystrophic sapje zebrafish, we have investigated the use of tyrosine kinase and other inhibitors to treat the dystrophic symptoms in this model of Duchenne muscular dystrophy. Dasatinib, a potent and specific Src tyrosine kinase inhibitor, was found to decrease the levels of β-dystroglycan phosphorylation on tyrosine and to increase the relative levels of non-phosphorylated β-dystroglycan in sapje zebrafish. Furthermore, dasatinib treatment resulted in the improved physical appearance of the sapje zebrafish musculature and increased swimming ability as measured by both duration and distance of swimming of dasatinib-treated fish compared with control animals. These data suggest great promise for pharmacological agents that prevent the phosphorylation of β-dystroglycan on tyrosine and subsequent steps in the degradation pathway as therapeutic targets for the treatment of Duchenne muscular dystrophy. © The Author 2015. Published by Oxford University Press.

Engagement in muscular strengthening activities is associated with better sleep

PubMed Central

Loprinzi, Paul D.; Loenneke, Jeremy P.

2015-01-01

Few studies have examined whether engagement in muscular strengthening activities is associated with sleep duration, which was the purpose of this study. Data from the population-based 2005–2006 National Health and Nutrition Examination Survey were used, which included an analytic sample of 4386 adults (20–85 yrs). Sleep duration and engagement in muscle strengthening activities was self-reported. After adjustments (including aerobic-based physical activity), those engaging in muscular strength activities, compared to those not engaging in muscular strengthening activities, had an 19% increased odds of meeting sleep guidelines (7–8 h/night) (Odds Ratio = 1.19, 95% Confidence Interval: 1.01–1.38, P = 0.04). Promotion of muscular strengthening activities by clinicians should occur not only for improvements in other aspects of health (e.g., cardiovascular benefits), but also to help facilitate optimal sleep duration. PMID:26844170

The Identification of Carriers in Duchenne Muscular Dystrophy

PubMed Central

Monckton, George; Ludvigsen, Bernhard

1963-01-01

Following recurrent reports in the literature that it is possible to detect carriers of the gene for progressive muscular dystrophy (Duchenne), a survey of nine families was performed. No unequivocal results could be obtained in comparison of clinical data with circulation-time measurements or serum enzyme studies. It is concluded that carrier-labelling with respect to progressive muscular dystrophy is not yet at a satisfactory stage, although progress is being made. PMID:13935998

Biological, Psychological, and Sociocultural Factors Contributing to the Drive for Muscularity in Weight-Training Men

PubMed Central

Schneider, Catharina; Rollitz, Laura; Voracek, Martin; Hennig-Fast, Kristina

2016-01-01

The drive for muscularity and associated behaviors (e.g., exercising and dieting) are of growing importance for men in Western societies. In its extreme form, it can lead to body image concerns and harmful behaviors like over-exercising and the misuse of performance-enhancing substances. Therefore, investigating factors associated with the drive for muscularity, especially in vulnerable populations like bodybuilders and weight trainers can help identify potential risk and protective factors for body image problems. Using a biopsychosocial framework, the aim of the current study was to explore different factors associated with drive for muscularity in weight-training men. To this purpose, German-speaking male weight trainers (N = 248) completed an online survey to determine the extent to which biological, psychological, and sociocultural factors contribute to drive for muscularity and its related attitudes and behaviors. Using multiple regression models, findings showed that media ideal body internalization was the strongest positive predictor for drive for muscularity, while age (M = 25.9, SD = 7.4) held the strongest negative association with drive for muscularity. Dissatisfaction with muscularity, but not with body fat, was related to drive for muscularity. The fat-free mass index, a quantification of the actual degree of muscularity of a person, significantly predicted drive for muscularity-related behavior but not attitudes. Body-related aspects of self-esteem, but not global self-esteem, were significant negative predictors of drive for muscularity. Since internalization of media body ideals presented the highest predictive value for drive for muscularity, these findings suggest that media body ideal internalizations may be a risk factor for body image concerns in men, leading, in its most extreme form to disordered eating or muscle dysmorphia. Future research should investigate the relations between drive for muscularity, age, body composition

Drive for muscularity and disordered eating among French adolescent boys: a sociocultural model.

PubMed

Rodgers, Rachel F; Ganchou, Camille; Franko, Debra L; Chabrol, Henri

2012-06-01

The pursuit of muscularity is an important body image concern among boys which has been described within sociocultural models of risk for eating disorders. This study explored a sociocultural model of disordered eating in which drive for thinness and pursuit of muscularity were both pathways to disordered eating among French adolescent boys. A sample of 146 adolescents completed a questionnaire assessing drive for thinness, drive for muscularity, media-ideal internalization, appearance comparison, and sociocultural pressure. The model was a good fit to the data and both drive for thinness and the pursuit of muscularity were related to disordered eating. Furthermore, internalization and appearance comparison mediated the relationships between pressure to increase muscle and both drive for muscularity and drive for thinness. Longitudinal research could help clarify the role of the pursuit of muscularity in the development of disordered eating and extreme body shape changing behaviors. Copyright © 2012 Elsevier Ltd. All rights reserved.

The Role of Adiposity in the Association between Muscular Fitness and Cardiovascular Disease.

PubMed

Pérez-Bey, Alejandro; Segura-Jiménez, Víctor; Fernández-Santos, Jorge Del Rosario; Esteban-Cornejo, Irene; Gómez-Martínez, Sonia; Veiga, Oscar L; Marcos, Ascensión; Castro-Piñero, José

2018-05-11

To test the associations of muscular fitness and body mass index (BMI), individually and combined, with clustered cardiovascular disease risk factors in children and adolescents and to analyze the mediator role of BMI in the association between muscular fitness and clustered cardiovascular disease risk factors. In total, 239 children (113 girls) and 270 adolescents (128 girls) participated in this cross-sectional study. Height and weight were assessed, and BMI was calculated. A cardiovascular disease risk factors index (CVDRF-I) was created from the combination of the following variables: waist circumference, systolic blood pressure, triglycerides, high-density lipoprotein cholesterol, and glucose. Handgrip strength/weight and standing long jump tests were used to assess muscular fitness. A muscular fitness index was computed from the combination of both tests. Muscular fitness index was associated with CVDRF-I in children of both sexes and adolescent boys; however, these associations disappeared after accounting for BMI. BMI was associated with CVDRF-I in both children and adolescents, even after adjusting for muscular fitness (all P < .001). In male and female children and in adolescent boys, the association between muscular fitness and CVDRF-I was mediated by BMI (all P < .001). Because there was no association between muscular fitness and CVDRF-I in adolescent girls, the mediation hypothesis was discarded. BMI is an independent predictor of CVDRF-I in children and adolescents of both sexes. Conversely, the effect of muscular fitness on CVDRF-I seems to be fully mediated by BMI levels in male and female children and in adolescent boys. Copyright © 2018 Elsevier Inc. All rights reserved.

Implementation of Duchenne Muscular Dystrophy Care Considerations.

PubMed

Andrews, Jennifer G; Conway, Kristin; Westfield, Christina; Trout, Christina; Meaney, F John; Mathews, Katherine; Ciafaloni, Emma; Cunniff, Christopher; Fox, Deborah J; Matthews, Dennis; Pandya, Shree

2018-06-20

Duchenne muscular dystrophy (DMD) is an X-linked disorder characterized by progressive muscle weakness and multisystem involvement. Recent advances in management of individuals with DMD have prolonged survival. Lack of standardized care spurred an international collaboration to develop consensus-based care considerations for diagnosis and management. In this study, we evaluate adherence to considerations at selected sites. We collaborated with the Muscular Dystrophy Surveillance, Tracking, and Research Network. Our sample included males with DMD and Becker muscular dystrophy <21 years as of December 31, 2010, with 1 health care encounter on or after January 1, 2012. We collected data from medical records on encounters occurring January 1, 2012, through December 31, 2014. Adherence was determined when frequency of visits or assessments were at or above recommendations for selected care considerations. Our analytic sample included 299 individuals, 7% of whom (20/299) were classified as childhood-onset Becker muscular dystrophy. Adherence for neuromuscular and respiratory clinician visits was 65% for the cohort; neuromuscular assessments and corticosteroid side effect monitoring measures ranged from 16% to 68%. Adherence was 83% for forced vital capacity and ≤58% for other respiratory diagnostics. Cardiologist assessments and echocardiograms were found for at least 84%. Transition planning for education or health care was documented for 31% of eligible males. Medical records data were used to identify areas in which practice aligns with the care considerations. However, there remains inconsistency across domains and insufficiency in critical areas. More research is needed to explain this variability and identify reliable methods to measure outcomes. Copyright © 2018 by the American Academy of Pediatrics.

Evidence Report: Risk of Spaceflight Associated Neuro-ocular Syndrome (SANS)

NASA Technical Reports Server (NTRS)

Stenger, Michael B.; Tarver, William J.; Brunstetter, Tyson; Gibson, Charles Robert; Laurie, Steven S.; Lee, Stuart M. C.; Macias, Brandon R.; Mader, Thomas H.; Otto, Christian; Smith, Scott M.;

Neuro-Linguistic Programming, Matching Sensory Predicates, and Rapport.

ERIC Educational Resources Information Center

Schmedlen, George W.; And Others

A key task for the therapist in psychotherapy is to build trust and rapport with the client. Neuro-Linguistic Programming (NLP) practitioners believe that matching the sensory modality (representational system) of a client's predicates (verbs, adverbs, and adjectives) improves rapport. In this study, 16 volunteer subjects participated in two…

Man versus machine: comparison of radiologists' interpretations and NeuroQuant® volumetric analyses of brain MRIs in patients with traumatic brain injury.

PubMed

Ross, David E; Ochs, Alfred L; Seabaugh, Jan M; Shrader, Carole R

2013-01-01

NeuroQuant® is a recently developed, FDA-approved software program for measuring brain MRI volume in clinical settings. The purpose of this study was to compare NeuroQuant with the radiologist's traditional approach, based on visual inspection, in 20 outpatients with mild or moderate traumatic brain injury (TBI). Each MRI was analyzed with NeuroQuant, and the resulting volumetric analyses were compared with the attending radiologist's interpretation. The radiologist's traditional approach found atrophy in 10.0% of patients; NeuroQuant found atrophy in 50.0% of patients. NeuroQuant was more sensitive for detecting brain atrophy than the traditional radiologist's approach.

The drive for muscularity in men: media influences and objectification theory.

PubMed

Daniel, Samantha; Bridges, Sara K

2010-01-01

Presently, objectification theory has yielded mixed results when utilized to explain body image concerns in men. An online survey assessing internalization of media ideals, self-objectification, body surveillance, body shame, the drive for muscularity, and body mass index (BMI) was completed by 244 predominantly college-aged males. Path analyses were used to investigate relationships among these variables where it was hypothesized that objectification variables would mediate the relationship between internalization of media ideals and the drive for muscularity. Internalization of media ideals was the strongest predictor of the drive for muscularity, followed by BMI, though variables of objectification theory had no impact on the drive for muscularity contrary to hypotheses. The results suggest that objectification theory may not be applicable to men as it is currently measured. Copyright 2009 Elsevier Ltd. All rights reserved.

Expression and cellular localization of the transcription factor NeuroD1 in the developing and adult rat pineal gland.

PubMed

Castro, Analía E; Benitez, Sergio G; Farias Altamirano, Luz E; Savastano, Luis E; Patterson, Sean I; Muñoz, Estela M

2015-05-01

Circadian rhythms govern many aspects of mammalian physiology. The daily pattern of melatonin synthesis and secretion is one of the classic examples of circadian oscillations. It is mediated by a class of neuroendocrine cells known as pinealocytes which are not yet fully defined. An established method to evaluate functional and cytological characters is through the expression of lineage-specific transcriptional regulators. NeuroD1 is a basic helix-loop-helix transcription factor involved in the specification and maintenance of both endocrine and neuronal phenotypes. We have previously described developmental and adult regulation of NeuroD1 mRNA in the rodent pineal gland. However, the transcript levels were not influenced by the elimination of sympathetic input, suggesting that any rhythmicity of NeuroD1 might be found downstream of transcription. Here, we describe NeuroD1 protein expression and cellular localization in the rat pineal gland during development and the daily cycle. In embryonic and perinatal stages, protein expression follows the mRNA pattern and is predominantly nuclear. Thereafter, NeuroD1 is mostly found in pinealocyte nuclei in the early part of the night and in cytoplasm during the day, a rhythm maintained into adulthood. Additionally, nocturnal nuclear NeuroD1 levels are reduced after sympathetic disruption, an effect mimicked by the in vivo administration of α- and β-adrenoceptor blockers. NeuroD1 phosphorylation at two sites, Ser(274) and Ser(336) , associates with nuclear localization in pinealocytes. These data suggest that NeuroD1 influences pineal phenotype both during development and adulthood, in an autonomic and phosphorylation-dependent manner. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Dystrophin quantification and clinical correlations in Becker muscular dystrophy: implications for clinical trials.

PubMed

Anthony, Karen; Cirak, Sebahattin; Torelli, Silvia; Tasca, Giorgio; Feng, Lucy; Arechavala-Gomeza, Virginia; Armaroli, Annarita; Guglieri, Michela; Straathof, Chiara S; Verschuuren, Jan J; Aartsma-Rus, Annemieke; Helderman-van den Enden, Paula; Bushby, Katherine; Straub, Volker; Sewry, Caroline; Ferlini, Alessandra; Ricci, Enzo; Morgan, Jennifer E; Muntoni, Francesco

2011-12-01

Duchenne muscular dystrophy is caused by mutations in the DMD gene that disrupt the open reading frame and prevent the full translation of its protein product, dystrophin. Restoration of the open reading frame and dystrophin production can be achieved by exon skipping using antisense oligonucleotides targeted to splicing elements. This approach aims to transform the Duchenne muscular dystrophy phenotype to that of the milder disorder, Becker muscular dystrophy, typically caused by in-frame dystrophin deletions that allow the production of an internally deleted but partially functional dystrophin. There is ongoing debate regarding the functional properties of the different internally deleted dystrophins produced by exon skipping for different mutations; more insight would be valuable to improve and better predict the outcome of exon skipping clinical trials. To this end, we have characterized the clinical phenotype of 17 patients with Becker muscular dystrophy harbouring in-frame deletions relevant to on-going or planned exon skipping clinical trials for Duchenne muscular dystrophy and correlated it to the levels of dystrophin, and dystrophin-associated protein expression. The cohort of 17 patients, selected exclusively on the basis of their genotype, included 4 asymptomatic, 12 mild and 1 severe patient. All patients had dystrophin levels of >40% of control and significantly higher dystrophin (P = 0.013), β-dystroglycan (P = 0.025) and neuronal nitric oxide synthase (P = 0.034) expression was observed in asymptomatic individuals versus symptomatic patients with Becker muscular dystrophy. Furthermore, grouping the patients by deletion, patients with Becker muscular dystrophy with deletions with an end-point of exon 51 (the skipping of which could rescue the largest group of Duchenne muscular dystrophy deletions) showed significantly higher dystrophin levels (P = 0.034) than those with deletions ending with exon 53. This is the first quantitative study on both

Dystrophin quantification and clinical correlations in Becker muscular dystrophy: implications for clinical trials

PubMed Central

Anthony, Karen; Cirak, Sebahattin; Torelli, Silvia; Tasca, Giorgio; Feng, Lucy; Arechavala-Gomeza, Virginia; Armaroli, Annarita; Guglieri, Michela; Straathof, Chiara S.; Verschuuren, Jan J.; Aartsma-Rus, Annemieke; Helderman-van den Enden, Paula; Bushby, Katherine; Straub, Volker; Sewry, Caroline; Ferlini, Alessandra; Ricci, Enzo; Morgan, Jennifer E.

2011-01-01

Duchenne muscular dystrophy is caused by mutations in the DMD gene that disrupt the open reading frame and prevent the full translation of its protein product, dystrophin. Restoration of the open reading frame and dystrophin production can be achieved by exon skipping using antisense oligonucleotides targeted to splicing elements. This approach aims to transform the Duchenne muscular dystrophy phenotype to that of the milder disorder, Becker muscular dystrophy, typically caused by in-frame dystrophin deletions that allow the production of an internally deleted but partially functional dystrophin. There is ongoing debate regarding the functional properties of the different internally deleted dystrophins produced by exon skipping for different mutations; more insight would be valuable to improve and better predict the outcome of exon skipping clinical trials. To this end, we have characterized the clinical phenotype of 17 patients with Becker muscular dystrophy harbouring in-frame deletions relevant to on-going or planned exon skipping clinical trials for Duchenne muscular dystrophy and correlated it to the levels of dystrophin, and dystrophin-associated protein expression. The cohort of 17 patients, selected exclusively on the basis of their genotype, included 4 asymptomatic, 12 mild and 1 severe patient. All patients had dystrophin levels of >40% of control and significantly higher dystrophin (P = 0.013), β-dystroglycan (P = 0.025) and neuronal nitric oxide synthase (P = 0.034) expression was observed in asymptomatic individuals versus symptomatic patients with Becker muscular dystrophy. Furthermore, grouping the patients by deletion, patients with Becker muscular dystrophy with deletions with an end-point of exon 51 (the skipping of which could rescue the largest group of Duchenne muscular dystrophy deletions) showed significantly higher dystrophin levels (P = 0.034) than those with deletions ending with exon 53. This is the first quantitative

Toward an operational neuroethical risk analysis and mitigation paradigm for emerging neuroscience and technology (neuroS/T).

PubMed

Giordano, James

2017-01-01

Research in neuroscience and neurotechnology (neuroS/T) is progressing at a rapid pace with translational applications both in medicine, and more widely in the social milieu. Current and projected neuroS/T research and its applications evoke a number of neuroethicolegal and social issues (NELSI). This paper defines inherent and derivative NELSI of current and near-term neuroS/T development and engagement, and provides an overview of our group's ongoing work to develop a systematized approach to their address. Our proposed operational neuroethical risk assessment and mitigation paradigm (ONRAMP) is presented, which entails querying, framing, and modeling patterns and trajectories of neuroS/T research and translational uses, and the NELSI generated by such advancements and their applications. Extant ethical methods are addressed, with suggestion toward possible revision or re-formulation to meet the needs and exigencies fostered by neuroS/T and resultant NELSI in multi-cultural contexts. The relevance and importance of multi-disciplinary expertise in focusing upon NELSI is discussed, and the need for neuroethics education toward cultivating such a cadre of expertise is emphasized. Copyright © 2016 Elsevier Inc. All rights reserved.

Genetics and emerging treatments for Duchenne and Becker muscular dystrophy.

PubMed

Wein, Nicolas; Alfano, Lindsay; Flanigan, Kevin M

2015-06-01

Mutations in the DMD gene result in Duchenne or Becker muscular dystrophy due to absent or altered expression of the dystrophin protein. The more severe Duchenne muscular dystrophy typically presents around ages 2 to 5 with gait disturbance, and historically has led to the loss of ambulation by age 12. It is important for the practicing pediatrician, however, to be aware of other presenting signs, such as delayed motor or cognitive milestones, or elevated serum transaminases. Becker muscular dystrophy is milder, often presenting after age 5, with ambulation frequently preserved past 20 years and sometimes into late decades. Copyright © 2015 Elsevier Inc. All rights reserved.

Improved Anatomical Specificity of Non-invasive Neuro-stimulation by High Frequency (5 MHz) Ultrasound

NASA Astrophysics Data System (ADS)

Li, Guo-Feng; Zhao, Hui-Xia; Zhou, Hui; Yan, Fei; Wang, Jing-Yao; Xu, Chang-Xi; Wang, Cong-Zhi; Niu, Li-Li; Meng, Long; Wu, Song; Zhang, Huai-Ling; Qiu, Wei-Bao; Zheng, Hai-Rong

2016-04-01

Low frequency ultrasound (<1 MHz) has been demonstrated to be a promising approach for non-invasive neuro-stimulation. However, the focal width is limited to be half centimeter scale. Minimizing the stimulation region with higher frequency ultrasound will provide a great opportunity to expand its application. This study first time examines the feasibility of using high frequency (5 MHz) ultrasound to achieve neuro-stimulation in brain, and verifies the anatomical specificity of neuro-stimulation in vivo. 1 MHz and 5 MHz ultrasound stimulation were evaluated in the same group of mice. Electromyography (EMG) collected from tail muscles together with the motion response videos were analyzed for evaluating the stimulation effects. Our results indicate that 5 MHz ultrasound can successfully achieve neuro-stimulation. The equivalent diameter (ED) of the stimulation region with 5 MHz ultrasound (0.29 ± 0.08 mm) is significantly smaller than that with 1 MHz (0.83 ± 0.11 mm). The response latency of 5 MHz ultrasound (45 ± 31 ms) is also shorter than that of 1 MHz ultrasound (208 ± 111 ms). Consequently, high frequency (5 MHz) ultrasound can successfully activate the brain circuits in mice. It provides a smaller stimulation region, which offers improved anatomical specificity for neuro-stimulation in a non-invasive manner.

Encouraging Subspecialty Practice by Constructively Influencing Trainees Early in their Careers Will Improve Advocacy for Neuro-Ophthalmology among Nigerian Ophthalmologists

PubMed Central

Ogun, Olufunmilola A.

2016-01-01

This study was conducted to assess the current knowledge, attitude, and perception of Nigerian ophthalmologists toward neuro-ophthalmology; identify barriers to the uptake of neuro-ophthalmology as a desired subspecialty; and make recommendations to improve interest in neuro-ophthalmology training. This was a cross-sectional survey of ophthalmology consultants and trainees from the six geopolitical zones of Nigeria, who were attending a national ophthalmology conference. All consenting respondents voluntarily completed a validated self-administered questionnaire. There were 107 respondents comprising 56 males and 51 females. Majority (54.2%) of respondents were aged 40 years and younger. Almost half (47.8%) worked at tertiary level, public health institutions. Only 10.3% worked in private practice. Neuro-ophthalmology exposure was short and occurred mainly during residency (65.7%), while 15% had no exposure at all. Most (80.4%) indicated only nominal interest in neuro-ophthalmology, while only 4.6% indicated a desire to specialize in the field. Financial constraint was the main obstacle to the pursuit of subspecialty training. A total of 86% of respondents admitted that full (34%) or partial (52%) Funding would motivate them to pursue the training. Among respondents desiring part sponsorship, more than half were willing to augment such sponsorship with personal funds. In conclusion, career interest in neuro-ophthalmology is very low among Nigerian ophthalmologists. Late and limited exposure to neuro-ophthalmology during medical training may be contributing factors. Early exposure to neuro-ophthalmology during medical school rotations, coupled with the provision of sponsored subspecialty training opportunities, will serve to increase enrollment in the field. PMID:29349319

Three Forms of Neuro-Realism: Explaining the Persistence of the "Uncritically Real" in Popular Neuroscience News

ERIC Educational Resources Information Center

Gruber, David R.

2017-01-01

Neuro-realism is a widely cited concept describing a textual phenomenon in popular science news wherein brain research uncritically validates or invalidates the "realness" of particular beliefs or practices. Currently, no research on neuro-realism examines the variable rhetorical roles of such statements, that is, how they support…

Brillouin spectroscopy reveals changes in muscular viscoelasticity in Drosophila POMT mutants

NASA Astrophysics Data System (ADS)

Meng, Zhaokai; Baker, Ryan; Panin, Vladislav M.; Yakovlev, Vladislav V.

2015-03-01

Muscular dystrophy (MD) is a group of muscle diseases that induce weakness in skeletal muscle and cause progressive muscle degeneration. The muscular mechanical properties (i.e., viscoelasticity), however, have not been thoroughly examined before and after MD. On the other hand, Brillouin spectroscopy (BS) provides a non-invasive approach to probing the local sound speed within a small volume. Moreover, recent advances in background-free Brillouin spectroscopy enable investigators to imaging not only transparent samples, but also turbid ones. In this study, we investigated the mechanical properties of muscles while employing Drosophila model of dystroglycanopathies, human congenital muscular dystrophies resulting from abnormal glycosylation of alphadystroglycan. Specifically, we analyzed larval abdominal muscles of Drosophila with mutations in protein Omannosyltransferase (POMT) genes. As a comparison, we have also examined muscular tissues dissected from wildtype Drosophila. The Brillouin spectra were obtained by a background free VIPA (virtually imaged phased array) spectrometer described in the previous report. As a reference, the Raman spectra were also acquired for each test. Our current results indicated that POMT defects cause changes in muscle elasticity, which suggests that muscular dystrophy conditions may be also associated with abnormalities in muscle elastic properties.

Platinum nanoparticles for the photothermal treatment of Neuro 2A cancer cells.

PubMed

Manikandan, M; Hasan, Nazim; Wu, Hui-Fen

2013-07-01

This study demonstrates the effective synthesis of five different sized/shaped Pt NPs, within a narrow size regime of 1-21 nm using a modified methodology and the toxicity/biocompatibility of Pt NPs on Neuro 2A cancer cells was investigated elaborately by using light microscopic observations, tryphan blue exclusion assay, MTT assay and ICP-MS. The Pt NPs-C with sizes 5-6 nm showed superior non-cytotoxic property compared to the other four Pt NPs. These non-cytotoxic Pt NPs were employed for successful photothermal treatment of Neuro 2A cell lines using near-IR 1064 nm of laser irradiation. The Pt NPs-C could generate a 9 °C increase in temperature leading to effective photothermal killing of cancer cells. The MALDI-MS was used to prove the possibility of apoptosis related triggering of cell death in the presence of the Pt NPs. The results confirm that the current approach is an effective platform for in vivo treatment of neuro cancer cells. Copyright © 2013 Elsevier Ltd. All rights reserved.

NeuroPG: open source software for optical pattern generation and data acquisition

PubMed Central

Avants, Benjamin W.; Murphy, Daniel B.; Dapello, Joel A.; Robinson, Jacob T.

2015-01-01

Patterned illumination using a digital micromirror device (DMD) is a powerful tool for optogenetics. Compared to a scanning laser, DMDs are inexpensive and can easily create complex illumination patterns. Combining these complex spatiotemporal illumination patterns with optogenetics allows DMD-equipped microscopes to probe neural circuits by selectively manipulating the activity of many individual cells or many subcellular regions at the same time. To use DMDs to study neural activity, scientists must develop specialized software to coordinate optical stimulation patterns with the acquisition of electrophysiological and fluorescence data. To meet this growing need we have developed an open source optical pattern generation software for neuroscience—NeuroPG—that combines, DMD control, sample visualization, and data acquisition in one application. Built on a MATLAB platform, NeuroPG can also process, analyze, and visualize data. The software is designed specifically for the Mightex Polygon400; however, as an open source package, NeuroPG can be modified to incorporate any data acquisition, imaging, or illumination equipment that is compatible with MATLAB’s Data Acquisition and Image Acquisition toolboxes. PMID:25784873

A prospective investigation of interpersonal influences on the pursuit of muscularity in late adolescent boys and girls.

PubMed

Shomaker, Lauren B; Furman, Wyndol

2010-04-01

This project examined whether interpersonal pressure to be muscular predicted late adolescents' pursuit of muscularity. Participants were 199 adolescents (16-19 years), mothers (n = 175), and friends (n = 159), assessed at two annual times. Pressure to be muscular was assessed with adolescents', mothers', and friends' reports of their relationships. Adolescents reported pressure from fathers and romantic partners, appearance satisfaction, disordered eating, and pursuit of muscularity. Adolescents', mothers', and friends' reports of pressure related to pursuit of muscularity at both times. Adolescents' perceptions and mothers' reports prospectively predicted pursuit of muscularity. Findings highlight the relevance of relationships to pursuit of muscularity in late adolescents.

Evolutionary Local Search of Fuzzy Rules through a novel Neuro-Fuzzy encoding method.

PubMed

Carrascal, A; Manrique, D; Ríos, J; Rossi, C

2003-01-01

This paper proposes a new approach for constructing fuzzy knowledge bases using evolutionary methods. We have designed a genetic algorithm that automatically builds neuro-fuzzy architectures based on a new indirect encoding method. The neuro-fuzzy architecture represents the fuzzy knowledge base that solves a given problem; the search for this architecture takes advantage of a local search procedure that improves the chromosomes at each generation. Experiments conducted both on artificially generated and real world problems confirm the effectiveness of the proposed approach.

Men, Muscles, and Eating Disorders: an Overview of Traditional and Muscularity-Oriented Disordered Eating.

PubMed

Lavender, Jason M; Brown, Tiffany A; Murray, Stuart B

2017-06-01

There is growing recognition that eating disorder (ED) symptoms, particularly those of a muscularity-oriented nature, are more common in men than previously understood. The purpose of the current review is to describe contemporary directions and implications of research on traditional and muscularity-oriented ED symptoms among males. Evidence indicates that ED symptoms occur in a substantial minority of men. Importantly, recent research has focused on muscularity-oriented body image and disordered eating in males, demonstrating the prevalence, correlates, and consequences of maladaptive muscularity-oriented attitudes and behaviors. A growing number of assessments are available to measure these constructs in males, and preliminary treatment considerations have begun to be addressed in the literature. Research on male EDs and body image is increasingly focusing on muscularity-oriented manifestations. Continued empirical work will be critical to improve our understanding of the onset, maintenance, and treatment of muscularity-oriented disordered eating in males.

Porcine models of muscular dystrophy

USDA-ARS?s Scientific Manuscript database

Duchenne muscular dystrophy is a progressive, fatal, X-linked disease caused by a failure to accumulate the cytoskeletal protein, dystrophin. This disease is modeled by a variety of animal models including several fish models, mice, rats, and dogs. While these models have contributed substantially t...

Analysis and prediction of flow from local source in a river basin using a Neuro-fuzzy modeling tool.

PubMed

Aqil, Muhammad; Kita, Ichiro; Yano, Akira; Nishiyama, Soichi

2007-10-01

Traditionally, the multiple linear regression technique has been one of the most widely used models in simulating hydrological time series. However, when the nonlinear phenomenon is significant, the multiple linear will fail to develop an appropriate predictive model. Recently, neuro-fuzzy systems have gained much popularity for calibrating the nonlinear relationships. This study evaluated the potential of a neuro-fuzzy system as an alternative to the traditional statistical regression technique for the purpose of predicting flow from a local source in a river basin. The effectiveness of the proposed identification technique was demonstrated through a simulation study of the river flow time series of the Citarum River in Indonesia. Furthermore, in order to provide the uncertainty associated with the estimation of river flow, a Monte Carlo simulation was performed. As a comparison, a multiple linear regression analysis that was being used by the Citarum River Authority was also examined using various statistical indices. The simulation results using 95% confidence intervals indicated that the neuro-fuzzy model consistently underestimated the magnitude of high flow while the low and medium flow magnitudes were estimated closer to the observed data. The comparison of the prediction accuracy of the neuro-fuzzy and linear regression methods indicated that the neuro-fuzzy approach was more accurate in predicting river flow dynamics. The neuro-fuzzy model was able to improve the root mean square error (RMSE) and mean absolute percentage error (MAPE) values of the multiple linear regression forecasts by about 13.52% and 10.73%, respectively. Considering its simplicity and efficiency, the neuro-fuzzy model is recommended as an alternative tool for modeling of flow dynamics in the study area.

Diagnostic value of neuro-ophthalmological signs in cases of Chiari I malformation.

PubMed

Bekerman, Inessa; Sigal, Tal; Kimiagar, Itzhak; Almer, Zina Evy; Vaiman, Michael

2016-12-01

Our purpose was to evaluate the diagnostic value of measuring diameters of optic nerve sheath (ONSD), presence/absence of papilledema, tortuosity of the optic nerve, flattening of the posterior sclera, and intraocular protrusion of the prelaminar optic nerve for intracranial pressure assessment in cases of Chiari I malformation. In a retrospective study, MRI data of 37 consecutive pediatric patients with Chiari malformation and data of 400 patients without intracranial pathology were compared and analyzed. ONSDs were measured at the point where the ophthalmic artery crosses the optic nerve (anatomical landmark). The correlation analysis was performed with clinical findings, gender, age, papilledema, and other neuro-ophthalmological findings. ONSD was enlarged in 38 % of cases of Chiari malformation. The enlargement was bilateral, no correlation with age or gender was found (p = 0.67 and p = 0.76, respectively). The presence of papilledema was detected in 19 % of cases presenting less valuable diagnostic sign if compared with ONSD. The tortuosity of the optic nerve was found in 22 % of cases, but in three patients, it was unilateral. All patients with enlarged ONSD and other neuro-ophthalmological signs present were treated surgically, while most of the patients without these signs (20/23) were treated conservatively. In majority of pediatric cases of Chiari malformation, the ONSD is not enlarged and other neuro-ophthalmological signs are not present. Detecting the enlarged ONSD and other neuro-ophthalmological signs in cases of Chiari malformation may indicate the elevated intracranial pressure and necessity for urgent surgical intervention.

Predicting muscularity-related behavior, emotions, and cognitions in men: The role of psychological need thwarting, drive for muscularity, and mesomorphic internalization.

PubMed

Edwards, Christian; Tod, David; Molnar, Gyozo; Markland, David

2016-09-01

We examine the relationships that internalization, need thwarting (NT), and drive for muscularity (DFM), along with their interactions, had with weightlifting, muscle dissatisfaction (MD), and muscle-related-worry (MRW). A sample of 552 men (MAge=20.5 years, SD=3.1) completed the Psychological Need Thwarting Scale, the Internalization subscale of the male version of the Sociocultural Attitudes Towards Appearance Questionnaire, the Drive for Muscularity Scale-Attitudes subscale, the Male Body Attitudes Scale-Muscularity subscale, the Body Change Inventory-Worry subscale, and an inventory assessing weightlifting behavior. DFM significantly predicted weightlifting, MRW, and MD. Internalization significantly predicted weightlifting and MRW. NT significantly predicted weightlifting and MD, and its relationship with MRW approached significance. The interaction terms did not predict weightlifting or MRW. The NT/DFM and NT/Internalization interaction terms predicted MD. These results highlight the role of NT in predicting appearance variables in men. Copyright © 2016 Elsevier Ltd. All rights reserved.

NeuroMatic: An Integrated Open-Source Software Toolkit for Acquisition, Analysis and Simulation of Electrophysiological Data

PubMed Central

Rothman, Jason S.; Silver, R. Angus

2018-01-01

Acquisition, analysis and simulation of electrophysiological properties of the nervous system require multiple software packages. This makes it difficult to conserve experimental metadata and track the analysis performed. It also complicates certain experimental approaches such as online analysis. To address this, we developed NeuroMatic, an open-source software toolkit that performs data acquisition (episodic, continuous and triggered recordings), data analysis (spike rasters, spontaneous event detection, curve fitting, stationarity) and simulations (stochastic synaptic transmission, synaptic short-term plasticity, integrate-and-fire and Hodgkin-Huxley-like single-compartment models). The merging of a wide range of tools into a single package facilitates a more integrated style of research, from the development of online analysis functions during data acquisition, to the simulation of synaptic conductance trains during dynamic-clamp experiments. Moreover, NeuroMatic has the advantage of working within Igor Pro, a platform-independent environment that includes an extensive library of built-in functions, a history window for reviewing the user's workflow and the ability to produce publication-quality graphics. Since its original release, NeuroMatic has been used in a wide range of scientific studies and its user base has grown considerably. NeuroMatic version 3.0 can be found at http://www.neuromatic.thinkrandom.com and https://github.com/SilverLabUCL/NeuroMatic. PMID:29670519

Thinness and muscularity internalization: Associations with disordered eating and muscle dysmorphia in men.

PubMed

Klimek, Patrycja; Murray, Stuart B; Brown, Tiffany; Gonzales Iv, Manuel; Blashill, Aaron J

2018-04-01

The tripartite influence model of body image identifies internalization of societal body ideals as a risk factor for developing body dissatisfaction, and subsequent disordered eating behavior. In men, internalization of two dimensions of body image ideals, thinness and muscularity, is associated with body dissatisfaction and eating concerns. However, it is unknown how thinness and muscularity internalization interact in predicting muscle dysmorphia and disordered eating in men. Data were collected online from 180 undergraduate men, with ages ranging from 18 to 33 years (19.6, SD = 2.6). Regression models were used to test the interactive effects of thinness and muscularity internalization on (a) muscle dysmorphia symptoms and (b) disordered eating. Subsequent simple slope analyses probed effects at the mean, and ±1 standard deviation of thinness internalization. Muscularity and thinness internalization were independently positively related to muscle dysmorphia symptoms and disordered eating. Additionally, a significant interaction revealed that muscularity internalization was increasingly related to muscle dysmorphia symptoms as thinness internalization decreased. Men who internalized the muscular ideal had higher levels of muscle dysmorphia when they did not highly internalize the thin ideal. However, greater internalization of both the muscularity and thin ideal independently may be most relevant in the development of disordered eating in men. Future research is needed to explore variability in experiences of muscle dysmorphia compared with disordered eating in males. © 2018 Wiley Periodicals, Inc.

Determinants of the epithelial-muscular axis on embryonic stem cell-derived gut-like structures.

PubMed

Luo, Yi; Takaki, Miyako; Misawa, Hiromi; Matsuyoshi, Hiroko; Sasahira, Tomonori; Chihara, Yoshitomo; Fujii, Kiyomu; Ohmori, Hitoshi; Kuniyasu, Hiroki

2010-01-01

Dome-like structures with epithelial-muscular layers resembling the gut have been derived from mouse embryonic stem (ES) cells. These domes have been reported to show spontaneous contractions and are called ES gut. In the present study, we examined the epithelial-muscular axis of these domes by detecting differentiation markers. A normal epithelial-muscular axis was exhibited in the domes with spontaneous motility, whereas the domes without spontaneous motility showed either an inverted or obscure axis. To investigate the factors affecting the epithelial-muscular axis, we examined the expression of hedgehog signaling factors in the domes. Expression of hedgehog family factors was detected in the epithelial components of the domes with motility, whereas this expression was inverted or obscure in the domes without motility. Out of the 25 domes, 10 of the 10 motility (+) domes showed a normal epithelial-muscular axis, whereas 14 of the 15 motility (-) domes lacked a normal epithelial-muscular axis. This implies that activin A upregulated the expression of sonic hedgehog and intestinal alkaline phosphatase in the embryoid bodies. These findings suggest that the motility of the ES gut depends on the domes' epithelial-muscular axis. Copyright © 2010 S. Karger AG, Basel.

A Prospective Investigation of Interpersonal Influences on the Pursuit of Muscularity in Late Adolescent Boys and Girls

PubMed Central

Shomaker, Lauren B.; Furman, Wyndol

2010-01-01

This project examined whether interpersonal pressure to be muscular predicted late adolescents’ pursuit of muscularity. Participants were 199 adolescents (16–19 years), mothers (n=175), and friends (n=159), assessed at two annual times. Pressure to be muscular was assessed with adolescents’, mothers’, and friends’ reports of their relationships. Adolescents reported pressure from fathers and romantic partners, appearance satisfaction, disordered eating, and pursuit of muscularity. Adolescents,’ mothers’, and friends’ reports of pressure related to pursuit of muscularity at both times. Adolescents’ perceptions and mothers’ reports prospectively predicted pursuit of muscularity. Findings highlight the relevance of relationships to pursuit of muscularity in late adolescents. PMID:20348360

Response assessment in neuro-oncology.

PubMed

Quant, Eudocia C; Wen, Patrick Y

2011-02-01

Accuracy and reproducibility in determining response to therapy and tumor progression can be difficult to achieve for nervous system tumors. Current response criteria vary depending on the pathology and have several limitations. Until recently, the most widely used criteria for gliomas were "Macdonald criteria," based on two-dimensional tumor measurements on neuroimaging studies. However, the Response Assessment in Neuro-Oncology (RANO) Working Group has published new recommendations in high-grade gliomas and is working on recommendations for other nervous system tumors. This article reviews current response criteria for high-grade glioma, low-grade glioma, brain metastasis, meningioma, and schwannoma.

Learning about Spinal Muscular Atrophy

MedlinePlus

... causes the disorder. Top of page NHGRI Clinical Research on Spinal Muscular Atrophy Currently, NHGRI is not conducting studies on SMA. The National Institutes of Health is conducting clinical trials identified as enrolling individuals with SMA: Quantitative Analysis of SMN1 and SMN2 Gene Based on ...

Some Dynamics of Personality Development in Boys Suffering from Muscular Dystrophy

ERIC Educational Resources Information Center

Mearig, Judith S.

1973-01-01

Discussed are personality aspects of Duchenne or pseudohypertrophic muscular dystrophy, a progressive wasting of muscular tissue, which afflicts only boys, and usually has its noticeable onset before the age of 6 years; and described is the development of three male dystrophic siblings. (DB)

[Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy].

PubMed

Silva, Helga Cristina Almeida da; Hiray, Marcia; Vainzof, Mariz; Schmidt, Beny; Oliveira, Acary Souza Bulle; Amaral, José Luiz Gomes do

2017-05-31

Duchenne/Becker muscular dystrophy affects skeletal muscles and leads to progressive muscle weakness and risk of atypical anesthetic reactions following exposure to succinylcholine or halogenated agents. The aim of this report is to describe the investigation and diagnosis of a patient with Becker muscular dystrophy and review the care required in anesthesia. Male patient, 14 years old, referred for hyperCKemia (chronic increase of serum creatine kinase levels - CK), with CK values of 7,779-29,040IU.L -1 (normal 174IU.L -1 ). He presented with a discrete delay in motor milestones acquisition (sitting at 9 months, walking at 18 months). He had a history of liver transplantation. In the neurological examination, the patient showed difficulty in walking on one's heels, myopathic sign (hands supported on the thighs to stand), high arched palate, calf hypertrophy, winged scapulae, global muscle hypotonia and arreflexia. Spirometry showed mild restrictive respiratory insufficiency (forced vital capacity: 77% of predicted). The in vitro muscle contracture test in response to halothane and caffeine was normal. Muscular dystrophy analysis by Western blot showed reduced dystrophin (20% of normal) for both antibodies (C and N-terminal), allowing the diagnosis of Becker muscular dystrophy. On preanesthetic assessment, the history of delayed motor development, as well as clinical and/or laboratory signs of myopathy, should encourage neurological evaluation, aiming at diagnosing subclinical myopathies and planning the necessary care to prevent anesthetic complications. Duchenne/Becker muscular dystrophy, although it does not increase susceptibility to MH, may lead to atypical fatal reactions in anesthesia. Copyright © 2017 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

Muscular Dystrophies at Different Ages: Metabolic and Endocrine Alterations

PubMed Central

Cruz Guzmán, Oriana del Rocío; Chávez García, Ana Laura; Rodríguez-Cruz, Maricela

2012-01-01

Common metabolic and endocrine alterations exist across a wide range of muscular dystrophies. Skeletal muscle plays an important role in glucose metabolism and is a major participant in different signaling pathways. Therefore, its damage may lead to different metabolic disruptions. Two of the most important metabolic alterations in muscular dystrophies may be insulin resistance and obesity. However, only insulin resistance has been demonstrated in myotonic dystrophy. In addition, endocrine disturbances such as hypogonadism, low levels of testosterone, and growth hormone have been reported. This eventually will result in consequences such as growth failure and delayed puberty in the case of childhood dystrophies. Other consequences may be reduced male fertility, reduced spermatogenesis, and oligospermia, both in childhood as well as in adult muscular dystrophies. These facts all suggest that there is a need for better comprehension of metabolic and endocrine implications for muscular dystrophies with the purpose of developing improved clinical treatments and/or improvements in the quality of life of patients with dystrophy. Therefore, the aim of this paper is to describe the current knowledge about of metabolic and endocrine alterations in diverse types of dystrophinopathies, which will be divided into two groups: childhood and adult dystrophies which have different age of onset. PMID:22701119

Neuro-Linguistic Programming Treatment for Anxiety: Magic or Myth?

ERIC Educational Resources Information Center

Krugman, Martin; And Others

1985-01-01

Compared neuro-linguistic programing treatment for anxiety with self-control desensitization of equal duration and a waiting-list control group in treating public speaking anxiety. Results indicated that neither treatment was more effective in reducing anxiety than merely waiting for one hour. (Author/MCF)

Muscular forearm activation in hand-grip tasks with superimposition of mechanical vibrations.

PubMed

Fattorini, L; Tirabasso, A; Lunghi, A; Di Giovanni, R; Sacco, F; Marchetti, E

2016-02-01

The purpose of this paper is to evaluate the muscular activation of the forearm, with or without vibration stimuli at different frequencies while performing a grip tasks of 45s at various level of exerted force. In 16 individuals, 9 females and 7 males, the surface electromyogram (EMG) of extensor carpi radialis longus and the flexor carpi ulnari muscles were assessed. At a short latency from onset EMG, RMS and the level of MU synchronization were assessed to evaluate the muscular adaptations. Whilst a trend of decay of EMG Median frequency (MDFd) was employed as an index of muscular fatigue. Muscular tasks consists of the grip of an instrumented handle at a force level of 20%, 30%, 40%, 60% of the maximum voluntary force. Vibration was supplied by a shaker to the hand in mono-frequential waves at 20, 30, 33 and 40Hz. In relation to EMG, RMS and MU synchronization, the muscular activation does not seem to change with the superimposition of the mechanical vibrations, on the contrary a lower MDFd was observed at 33Hz than in absence of vibration. This suggests an early muscular fatigue induced by vibration due to the fact that 33Hz is a resonance frequency for the hand-arm system. Copyright © 2015 Elsevier Ltd. All rights reserved.

Bortezomib partially improves laminin α2 chain-deficient muscular dystrophy.

PubMed

Körner, Zandra; Fontes-Oliveira, Cibely C; Holmberg, Johan; Carmignac, Virginie; Durbeej, Madeleine

2014-05-01

Congenital muscular dystrophy, caused by mutations in LAMA2 (the gene encoding laminin α2 chain), is a severe and incapacitating disease for which no therapy is yet available. We have recently demonstrated that proteasome activity is increased in laminin α2 chain-deficient muscle and that treatment with the nonpharmaceutical proteasome inhibitor MG-132 reduces muscle pathology in laminin α2 chain-deficient dy(3K)/dy(3K) mice. Here, we explore the use of the selective and therapeutic proteasome inhibitor bortezomib (currently used for treatment of relapsed multiple myeloma and mantle cell lymphoma) in dy(3K)/dy(3K) mice and in congenital muscular dystrophy type 1A muscle cells. Outcome measures included quantitative muscle morphology, gene and miRNA expression analyses, proteasome activity, motor activity, and survival. Bortezomib improved several histological hallmarks of disease, partially normalized miRNA expression (miR-1 and miR-133a), and enhanced body weight, locomotion, and survival of dy(3K)/dy(3K) mice. In addition, bortezomib reduced proteasome activity in congenital muscular dystrophy type 1A myoblasts and myotubes. These findings provide evidence that the proteasome inhibitor bortezomib partially reduces laminin α2 chain-deficient muscular dystrophy. Investigation of the clinical efficacy of bortezomib administration in congenital muscular dystrophy type 1A clinical trials may be warranted. Copyright © 2014 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

A preliminary study of muscular artifact cancellation in single-channel EEG.

PubMed

Chen, Xun; Liu, Aiping; Peng, Hu; Ward, Rabab K

2014-10-01

Electroencephalogram (EEG) recordings are often contaminated with muscular artifacts that strongly obscure the EEG signals and complicates their analysis. For the conventional case, where the EEG recordings are obtained simultaneously over many EEG channels, there exists a considerable range of methods for removing muscular artifacts. In recent years, there has been an increasing trend to use EEG information in ambulatory healthcare and related physiological signal monitoring systems. For practical reasons, a single EEG channel system must be used in these situations. Unfortunately, there exist few studies for muscular artifact cancellation in single-channel EEG recordings. To address this issue, in this preliminary study, we propose a simple, yet effective, method to achieve the muscular artifact cancellation for the single-channel EEG case. This method is a combination of the ensemble empirical mode decomposition (EEMD) and the joint blind source separation (JBSS) techniques. We also conduct a study that compares and investigates all possible single-channel solutions and demonstrate the performance of these methods using numerical simulations and real-life applications. The proposed method is shown to significantly outperform all other methods. It can successfully remove muscular artifacts without altering the underlying EEG activity. It is thus a promising tool for use in ambulatory healthcare systems.

Emerging strategies for cell and gene therapy of the muscular dystrophies

PubMed Central

Muir, Lindsey A.; Chamberlain, Jeffrey S.

2016-01-01

The muscular dystrophies are a heterogeneous group of over 40 disorders that are characterised by muscle weakness and wasting. The most common are Duchenne muscular dystrophy and Becker muscular dystrophy, which result from mutations within the gene encoding dystrophin; myotonic dystrophy type 1, which results from an expanded trinucleotide repeat in the myotonic dystrophy protein kinase gene; and facioscapulohumeral dystrophy, which is associated with contractions in the subtelomeric region of human chromosome 1. Currently the only treatments involve clinical management of symptoms, although several promising experimental strategies are emerging. These include gene therapy using adeno-associated viral, lentiviral and adenoviral vectors and nonviral vectors, such as plasmid DNA. Exon-skipping and cell-based therapies have also shown promise in the effective treatment and regeneration of dystrophic muscle. The availability of numerous animal models for Duchenne muscular dystrophy has enabled extensive testing of a wide range of therapeutic approaches for this type of disorder. Consequently, we focus here on the therapeutic developments for Duchenne muscular dystrophy as a model of the types of approaches being considered for various types of dystrophy. We discuss the advantages and limitations of each therapeutic strategy, as well as prospects and recent successes in the context of future clinical applications. PMID:19555515

Global cross-station assessment of neuro-fuzzy models for estimating daily reference evapotranspiration

NASA Astrophysics Data System (ADS)

Shiri, Jalal; Nazemi, Amir Hossein; Sadraddini, Ali Ashraf; Landeras, Gorka; Kisi, Ozgur; Fard, Ahmad Fakheri; Marti, Pau

2013-02-01

SummaryAccurate estimation of reference evapotranspiration is important for irrigation scheduling, water resources management and planning and other agricultural water management issues. In the present paper, the capabilities of generalized neuro-fuzzy models were evaluated for estimating reference evapotranspiration using two separate sets of weather data from humid and non-humid regions of Spain and Iran. In this way, the data from some weather stations in the Basque Country and Valencia region (Spain) were used for training the neuro-fuzzy models [in humid and non-humid regions, respectively] and subsequently, the data from these regions were pooled to evaluate the generalization capability of a general neuro-fuzzy model in humid and non-humid regions. The developed models were tested in stations of Iran, located in humid and non-humid regions. The obtained results showed the capabilities of generalized neuro-fuzzy model in estimating reference evapotranspiration in different climatic zones. Global GNF models calibrated using both non-humid and humid data were found to successfully estimate ET0 in both non-humid and humid regions of Iran (the lowest MAE values are about 0.23 mm for non-humid Iranian regions and 0.12 mm for humid regions). non-humid GNF models calibrated using non-humid data performed much better than the humid GNF models calibrated using humid data in non-humid region while the humid GNF model gave better estimates in humid region.

Cytotoxic Effects of Ochratoxin A in Neuro-2a Cells: Role of Oxidative Stress Evidenced by N-acetylcysteine.

PubMed

Bhat, Pratiksha V; Pandareesh; Khanum, Farhath; Tamatam, Anand

2016-01-01

Ochratoxin-A (OTA), is toxic secondary metabolite and is found to be a source of vast range of toxic effects like hepatotoxicity, nephrotoxicity. However, the information available currently regarding neurotoxic effects exerted by OTA is scanty. Hence, the present study was aimed to evaluate the neurotoxic effects of OTA and the possible mechanisms of toxicity as well as the role of cytotoxic oxidative stress on neuronal (Neuro-2a) cell line was evaluated in vitro. Results of the MTT and LDH assay showed that, OTA induced dose-dependent cell death in Neuro-2a cells and EC50 value was determined as 500 nM. OTA induced high levels of reactive oxygen species (ROS) and elevated levels of malondialdehyde, also loss of mitochondrial membrane potential was observed in a dose depended manner. Effects of OTA on ROS induced chromosomal DNA damage was assessed by Comet assay and plasmid DNA damage assay in which increase in DNA damage was observed in Neuro-2a cells by increasing the OTA concentration. Further western blotting analysis of OTA treated Neuro-2a cells indicated elevated expression levels of c-Jun, JNK3 and cleaved caspase-3 leading to apoptotic cell death. Other hand realtime-Q-PCR analysis clearly indicates the suppressed expression of neuronal biomarker genes including AChE, BDNF, TH and NOS2. Further N-acetylcysteine (NAC) pretreatment to Neuro-2a cells followed by OTA treatment clearly evidenced that, the significant reversal of toxic effects exerted by OTA on Neuro-2a cells. In the present study, results illustrate that ROS a principle event in oxidative stress was elevated by OTA toxicity in Neuro-2a cells. However, further in vivo, animal studies are in need to conclude the present study reports and the use of NAC as a remedy for OTA induced neuronal stress.

The pursuit of muscularity among adolescent boys in Fiji and Tonga.

PubMed

Ricciardelli, Lina A; McCabe, Marita P; Mavoa, Helen; Fotu, Kalesita; Goundar, Ramneek; Schultz, Jimaima; Waqa, Gade; Swinburn, Boyd A

2007-12-01

The desire for muscularity is tied to Western views of the male gender role, which prescribe that men be strong, physically fit and athletically successful. Although, these ideals have been primarily studied among Western adolescent boys, there is emerging evidence that the same ideals are valued and promoted among males from the Pacific Islands. The aim of the present study was to examine body image concerns associated with muscularity and the reasons for these concerns among Fijian and Tongan adolescent boys. Semi-structured interviews were conducted with 24 Indigenous Fijian, 24 Indo-Fijian, and 24 Tongan boys aged between 13 and 20 years. A thematic analysis of boys' narratives showed that the pursuit of muscularity was a dominant theme for many boys. Boys' reasons for pursing muscularity included the attainment of strength and fitness, sporting performance, physical work, dominance, and health. These findings are examined in relation to previous research with Western adolescent boys.

Guiding Principles for a Pediatric Neurology ICU (neuroPICU) Bedside Multimodal Monitor

PubMed Central

Eldar, Yonina C.; Gopher, Daniel; Gottlieb, Amihai; Lammfromm, Rotem; Mangat, Halinder S; Peleg, Nimrod; Pon, Steven; Rozenberg, Igal; Schiff, Nicholas D; Stark, David E; Yan, Peter; Pratt, Hillel; Kosofsky, Barry E

2016-01-01

Summary Background Physicians caring for children with serious acute neurologic disease must process overwhelming amounts of physiological and medical information. Strategies to optimize real time display of this information are understudied. Objectives Our goal was to engage clinical and engineering experts to develop guiding principles for creating a pediatric neurology intensive care unit (neuroPICU) monitor that integrates and displays data from multiple sources in an intuitive and informative manner. Methods To accomplish this goal, an international group of physicians and engineers communicated regularly for one year. We integrated findings from clinical observations, interviews, a survey, signal processing, and visualization exercises to develop a concept for a neuroPICU display. Results Key conclusions from our efforts include: (1) A neuroPICU display should support (a) rapid review of retrospective time series (i.e. cardiac, pulmonary, and neurologic physiology data), (b) rapidly modifiable formats for viewing that data according to the specialty of the reviewer, and (c) communication of the degree of risk of clinical decline. (2) Specialized visualizations of physiologic parameters can highlight abnormalities in multivariable temporal data. Examples include 3-D stacked spider plots and color coded time series plots. (3) Visual summaries of EEG with spectral tools (i.e. hemispheric asymmetry and median power) can highlight seizures via patient-specific “fingerprints.” (4) Intuitive displays should emphasize subsets of physiology and processed EEG data to provide a rapid gestalt of the current status and medical stability of a patient. Conclusions A well-designed neuroPICU display must present multiple datasets in dynamic, flexible, and informative views to accommodate clinicians from multiple disciplines in a variety of clinical scenarios. PMID:27437048

Cardio-Muscular Conditioner

NASA Technical Reports Server (NTRS)

1993-01-01

In the mid-sixties, Gary Graham, a Boeing designer, developed a cardiovascular conditioner for a planned Air Force orbiting laboratory. After the project was cancelled, Graham participated in space station conditioning studies for the Skylab program. Twenty years later, he used this expertise to develop the Shuttle 2000-1, a physical therapy and athletic development conditioner, available through Contemporary Designs. The machine is used by football teams, sports clinics and medical rehabilitation centers. Cardiovascular fitness and muscular strength development are promoted through both kinetic and plyometric exercises.

Eteplirsen in the treatment of Duchenne muscular dystrophy

PubMed Central

Lim, Kenji Rowel Q; Maruyama, Rika; Yokota, Toshifumi

2017-01-01

Duchenne muscular dystrophy is a fatal neuromuscular disorder affecting around one in 3,500–5,000 male births that is characterized by progressive muscular deterioration. It is inherited in an X-linked recessive fashion and is caused by loss-of-function mutations in the DMD gene coding for dystrophin, a cytoskeletal protein that stabilizes the plasma membrane of muscle fibers. In September 2016, the US Food and Drug Administration granted accelerated approval for eteplirsen (or Exondys 51), a drug that acts to promote dystrophin production by restoring the translational reading frame of DMD through specific skipping of exon 51 in defective gene variants. Eteplirsen is applicable for approximately 14% of patients with DMD mutations. This article extensively reviews and discusses the available information on eteplirsen to date, focusing on pharmacological, efficacy, safety, and tolerability data from preclinical and clinical trials. Issues faced by eteplirsen, particularly those relating to its efficacy, will be identified. Finally, the place of eteplirsen and exon skipping as a general therapeutic strategy in Duchenne muscular dystrophy treatment will be discussed. PMID:28280301

Vision rehabilitation for visual-vestibular dysfunction: the role of the neuro-optometrist.

PubMed

Cohen, Allen H

2013-01-01

This article discusses, in a clinically relevant format, the importance of including a neuro-optometrist as a member of the management team for patients with balance disorders. To review the importance of vision and visual processing for maintaining a sense of balance and equilibrium and the role of the neuro-optometrist in the overall rehabilitation of patients with balance disorders Dizziness, balance problems and the sensation that the space world is moving (vertigo) are one of the most commonly reported problems in general medical practice. Persons with a central nervous system injury or other idiopathic causes of visual processing problems or who have functional vision problems that are not adequately managed, often experience extreme difficulty with balance and movement, as well as with their perception of space. Consequently, the patient often experiences difficulty functioning in an environment with excessive visual stimulation such as a grocery store or shopping mall. Symptoms of disequilibrium, vestibular and balance problems are commonly a result of VOR disturbance secondary to an inner ear problem and an unstable binocularity. The combination of neuro-optomertic rehabilitative therapy and balance therapy will result in a is an effective treatment for reducing or resolving these symptoms.

Ramus marginalis mandibulae nervus facialis palsy in hemifacial microsomia.

PubMed

Silvestri, A; Mariani, G; Vernucci, R A

2008-12-01

The paralysis of the ramus marginalis mandibulae nervus facialis may occur in Hemifacial Microsomia (HM); the combination of both HM and palsy contributes to an elongation of the mandibular body. This study explores a possible correlation between neurological deficit, muscular atony, and structural deficiency. Of 58 patients with HM who had come to the University of Rome (Sapienza) Pre-surgical Orthodontics Unit, 4 patients were afflicted with Hemifacial Microsomia and ramus marginalis mandibulae nervus palsy; these patients underwent physical, neurological, opthamologic and systemic examinations. The results were then analysed in order to determine a possible correlation between neuro-muscular and structural deficit. Electroneurographic and electromyographic examinations were performed to estimate facial nerve and muscles involvement. Neuroelectrographic exam showed a damage of the nervous motor fibres of the facial nerve ipsilateral to HM, with an associated damage of the muscular function, while neuro-muscular functions on the healthy side were normal. The peripheral nervous and muscular deficits affect the function of facial soft tissues and the growth of mandibular body with an asymmetry characterised by a hypodevelopment of the ramus (due to the HM) and by an elongation of the mandibular body (due to ramus marginalis mandibulae nerve palsy), so that the chin deviation is contralateral to HM. In these forms, a neurological examination is necessary to assess the neurological damage on the HM side. Neuromuscular deficiency can also contribute to a relapse tendency after a surgical-orthodontic treatment.

Experiments on Factors That Influence Muscular Function in Man.

DTIC Science & Technology

1983-05-25

of phentolamine or phenylephrine did not affect the increase in mean arterial blood pressure , which reached the same high levels at fatigue in all...CLASSIFICATION OF THIS PAGE(Whon Date Elrered) same when the contractions result in muscular fatigue, at which time the mean . blood pressure is the same as it is...arterial blood pressure of bout 150 mmHg at the point of muscular fatigue. Clearly, some vaso- constrictp> response was intervening since the blood flow

NeuroAIDS, drug abuse, and inflammation: building collaborative research activities.

PubMed

Berman, Joan W; Carson, Monica J; Chang, Linda; Cox, Brian M; Fox, Howard S; Gonzalez, R Gilberto; Hanson, Glen R; Hauser, Kurt F; Ho, Wen-Zhe; Hong, Jau-Shyong; Major, Eugene O; Maragos, William F; Masliah, Eliezer; McArthur, Justin C; Miller, Diane B; Nath, Avindra; O'Callaghan, James P; Persidsky, Yuri; Power, Christopher; Rogers, Thomas J; Royal, Walter

2006-12-01

Neurological complications of human immunodeficiency virus (HIV) infection are a public health problem despite the availability of active antiretroviral therapies. The neuropathogenesis of HIV infection revolves around a complex cascade of events that include viral infection and glial immune activation, monocyte-macrophage brain infiltration, and secretion of a host of viral and cellular inflammatory and neurotoxic molecules. Although there is evidence that HIV-infected drug abusers experience more severe neurological disease, the biological basis for this finding is unknown. A scientific workshop organized by the National Institute on Drug Abuse (NIDA) was held on March 23-24, 2006 to address this question. The goal of the meeting was to bring together basic science and clinical researchers who are experts in NeuroAIDS, glial immunity, drugs of abuse, and/or pharmacology in order to find new approaches to understanding interactions between drug abuse and neuroAIDS. The format of the meeting was designed to stimulate open discussion and forge new multidisciplinary research collaborations. This report includes transcripts of active discussions and short presentations from invited participants. The presentations were separated into sections that included: Glial Biology, Inflammation, and HIV; Pharmacology, Neurotoxicology, and Neuroprotection; NeuroAIDS and Virology; and Virus-Drug and Immune-Drug Interactions. Research priorities were identified. Additional information about this meeting is available through links from the NIDA AIDS Research Program website ( http://www.nida.nih.gov/about/organization/arp/arp-websites.htm ).

Muscular strength is associated with self-esteem in college men but not women.

PubMed

Ciccolo, Joseph T; SantaBarbara, Nicholas J; Dunsiger, Shira I; Busch, Andrew M; Bartholomew, John B

2016-12-01

Muscular strength is a well-known predictor of morbidity and mortality. Similarly, self-esteem is a predictor of health and well-being. The relationship between these two variables, however, is currently unknown. This study examined the cross-sectional relationship between maximal muscular strength (i.e. handgrip and one-repetition-maximum (1-RM) squat) and global self-esteem in 126 college students. Significant correlations were found between both measures of muscular strength and self-esteem. Further analyses revealed that these relationships were only significant for men. Based on these results, additional research is needed to further explore the relationship between muscular strength and self-esteem, especially in other demographic groups and longitudinally. © The Author(s) 2015.

Advanced Gene Therapy for Treatment of Cardiomyopathy and Respiratory Insufficiency in Duchenne Muscular Dystrophy

DTIC Science & Technology

2014-09-01

TITLE: Advanced Gene Therapy for Treatment of Cardiomyopathy and Respiratory Insufficiency in Duchenne Muscular Dystrophy PRINCIPAL...Advanced Gene Therapy for Treatment of Cardiomyopathy and Respiratory Insufficiency in Duchenne Muscular Dystrophy 5a. CONTRACT NUMBER 5b. GRANT...effective recombinant AAV vector serotype 9 delivery system for the treatment of cardiorespiratory dysfunction in Duchenne Muscular Dystrophy . 2

Autonomic dysfunction in muscular dystrophy: a theoretical framework for muscle reflex involvement

PubMed Central

Smith, Scott A.; Downey, Ryan M.; Williamson, Jon W.; Mizuno, Masaki

2014-01-01

Muscular dystrophies are a heterogeneous group of genetically inherited disorders whose most prominent clinical feature is progressive degeneration of skeletal muscle. In several forms of the disease, the function of cardiac muscle is likewise affected. The primary defect in this group of diseases is caused by mutations in myocyte proteins important to cellular structure and/or performance. That being stated, a growing body of evidence suggests that the development of autonomic dysfunction may secondarily contribute to the generation of skeletal and cardio-myopathy in muscular dystrophy. Indeed, abnormalities in the regulation of both sympathetic and parasympathetic nerve activity have been reported in a number of muscular dystrophy variants. However, the mechanisms mediating this autonomic dysfunction remain relatively unknown. An autonomic reflex originating in skeletal muscle, the exercise pressor reflex, is known to contribute significantly to the control of sympathetic and parasympathetic activity when stimulated. Given the skeletal myopathy that develops with muscular dystrophy, it is logical to suggest that the function of this reflex might also be abnormal with the pathogenesis of disease. As such, it may contribute to or exacerbate the autonomic dysfunction that manifests. This possibility along with a basic description of exercise pressor reflex function in health and disease are reviewed. A better understanding of the mechanisms that possibly underlie autonomic dysfunction in muscular dystrophy may not only facilitate further research but could also lead to the identification of new therapeutic targets for the treatment of muscular dystrophy. PMID:24600397

Sapphire implant based neuro-complex for deep-lying brain tumors phototheranostics

NASA Astrophysics Data System (ADS)

Sharova, A. S.; Maklygina, YU S.; Yusubalieva, G. M.; Shikunova, I. A.; Kurlov, V. N.; Loschenov, V. B.

2018-01-01

The neuro-complex as a combination of sapphire implant optical port and osteoplastic biomaterial "Collapan" as an Aluminum phthalocyanine nanoform photosensitizer (PS) depot was developed within the framework of this study. The main goals of such neuro-complex are to provide direct access of laser radiation to the brain tissue depth and to transfer PS directly to the pathological tissue location that will allow multiple optical phototheranostics of the deep-lying tumor region without repeated surgical intervention. The developed complex spectral-optical properties research was carried out by photodiagnostics method using the model sample: a brain tissue phantom. The optical transparency of sapphire implant allows obtaining a fluorescent signal with high accuracy, comparable to direct measurement "in contact" with the tissue.

Molecular markers in pediatric neuro-oncology.

PubMed

Ichimura, Koichi; Nishikawa, Ryo; Matsutani, Masao

2012-09-01

Pediatric molecular neuro-oncology is a fast developing field. A multitude of molecular profiling studies in recent years has unveiled a number of genetic abnormalities unique to pediatric brain tumors. It has now become clear that brain tumors that arise in children have distinct pathogenesis and biology, compared with their adult counterparts, even for those with indistinguishable histopathology. Some of the molecular features are so specific to a particular type of tumors, such as the presence of the KIAA1549-BRAF fusion gene for pilocytic astrocytomas or SMARCB1 mutations for atypical teratoid/rhabdoid tumors, that they could practically serve as a diagnostic marker on their own. Expression profiling has resolved the existence of 4 molecular subgroups in medulloblastomas, which positively translated into improved prognostication for the patients. The currently available molecular markers, however, do not cover all tumors even within a single tumor entity. The molecular pathogenesis of a large number of pediatric brain tumors is still unaccounted for, and the hierarchy of tumors is likely to be more complex and intricate than currently acknowledged. One of the main tasks of future molecular analyses in pediatric neuro-oncology, including the ongoing genome sequencing efforts, is to elucidate the biological basis of those orphan tumors. The ultimate goal of molecular diagnostics is to accurately predict the clinical and biological behavior of any tumor by means of their molecular characteristics, which is hoped to eventually pave the way for individualized treatment.

Molecular markers in pediatric neuro-oncology

PubMed Central

Ichimura, Koichi; Nishikawa, Ryo; Matsutani, Masao

2012-01-01

Pediatric molecular neuro-oncology is a fast developing field. A multitude of molecular profiling studies in recent years has unveiled a number of genetic abnormalities unique to pediatric brain tumors. It has now become clear that brain tumors that arise in children have distinct pathogenesis and biology, compared with their adult counterparts, even for those with indistinguishable histopathology. Some of the molecular features are so specific to a particular type of tumors, such as the presence of the KIAA1549-BRAF fusion gene for pilocytic astrocytomas or SMARCB1 mutations for atypical teratoid/rhabdoid tumors, that they could practically serve as a diagnostic marker on their own. Expression profiling has resolved the existence of 4 molecular subgroups in medulloblastomas, which positively translated into improved prognostication for the patients. The currently available molecular markers, however, do not cover all tumors even within a single tumor entity. The molecular pathogenesis of a large number of pediatric brain tumors is still unaccounted for, and the hierarchy of tumors is likely to be more complex and intricate than currently acknowledged. One of the main tasks of future molecular analyses in pediatric neuro-oncology, including the ongoing genome sequencing efforts, is to elucidate the biological basis of those orphan tumors. The ultimate goal of molecular diagnostics is to accurately predict the clinical and biological behavior of any tumor by means of their molecular characteristics, which is hoped to eventually pave the way for individualized treatment. PMID:23095836

Mesial Temporal Sclerosis: Accuracy of NeuroQuant versus Neuroradiologist.

PubMed

Azab, M; Carone, M; Ying, S H; Yousem, D M

2015-08-01

We sought to compare the accuracy of a volumetric fully automated computer assessment of hippocampal volume asymmetry versus neuroradiologists' interpretations of the temporal lobes for mesial temporal sclerosis. Detecting mesial temporal sclerosis (MTS) is important for the evaluation of patients with temporal lobe epilepsy as it often guides surgical intervention. One feature of MTS is hippocampal volume loss. Electronic medical record and researcher reports of scans of patients with proved mesial temporal sclerosis were compared with volumetric assessment with an FDA-approved software package, NeuroQuant, for detection of mesial temporal sclerosis in 63 patients. The degree of volumetric asymmetry was analyzed to determine the neuroradiologists' threshold for detecting right-left asymmetry in temporal lobe volumes. Thirty-six patients had left-lateralized MTS, 25 had right-lateralized MTS, and 2 had bilateral MTS. The estimated accuracy of the neuroradiologist was 72.6% with a κ statistic of 0.512 (95% CI, 0.315-0.710) [moderate agreement, P < 3 × 10(-6)]), whereas the estimated accuracy of NeuroQuant was 79.4% with a κ statistic of 0.588 (95% CI, 0.388-0.787) [moderate agreement, P < 2 × 10(-6)]). This discrepancy in accuracy was not statistically significant. When at least a 5%-10% volume discrepancy between temporal lobes was present, the neuroradiologists detected it 75%-80% of the time. As a stand-alone fully automated software program that can process temporal lobe volume in 5-10 minutes, NeuroQuant compares favorably with trained neuroradiologists in predicting the side of mesial temporal sclerosis. Neuroradiologists can often detect even small temporal lobe volumetric changes visually. © 2015 by American Journal of Neuroradiology.

e100 NeuroRobotic system.

PubMed

Stein, Joel

2009-01-01

Therapy incorporating the repeated practice of motor tasks has been found to enhance motor function after stroke. This type of therapy may be facilitated by robotic devices and several such devices are being developed for use in rehabilitation. The Myomo e100 NeuroRobotic system is a novel device developed to provide assistance during elbow movements in stroke survivors. The device uses surface electromyographic signals to control a powered elbow orthosis. Data from a pilot study reveals that the device can be used successfully by stroke survivors and suggests that it may be effective in helping to restore motor control after stroke. Further studies are needed to confirm these preliminary results.

Relationship between metabolic cost and muscular coactivation across running speeds.

PubMed

Moore, Isabel S; Jones, Andrew M; Dixon, Sharon J

2014-11-01

Muscular coactivation can help stabilise a joint, but contrasting results in previous gait studies highlight that it is not clear whether this is metabolically beneficial. The aim was to assess the relationship between the metabolic cost of running and muscular coactivation across different running speeds, in addition to assessing the reliability and precision of lower limb muscular coactivation. Eleven female recreational runners visited the laboratory on two separate occasions. On both occasions subjects ran at three speeds (9.1, 11 and 12 km h(-1)) for six minutes each. Oxygen consumption and electromyographic data were simultaneously recorded during the final two minutes of each speed. Temporal coactivations of lower limb muscles during the stance phase were calculated. Five muscles were assessed: rectus femoris, vastus lateralis, biceps femoris, tibialis anterior and gastrocnemius lateralis. Nonparametric correlations revealed at least one significant, positive association between lower limb muscular coactivation and the metabolic cost of running for each speed. The length of tibialis anterior activation and muscular coactivation of the biceps femoris-tibialis anterior and gastrocnemius lateralis-tibialis anterior decreased with speed. These results show that longer coactivations of the proximal (rectus femoris-biceps femoris and vastus lateralis-biceps femoris) and leg extensor (rectus femoris-gastrocnemius lateralis) muscles were related to a greater metabolic cost of running, which could be detrimental to performance. The decrease in coactivation in the flexor and distal muscles at faster speeds occurs due to the shorter duration of tibialis anterior activation as speed increases, yet stability may be maintained. Copyright © 2013 Sports Medicine Australia. Published by Elsevier Ltd. All rights reserved.

Disparities in the diagnostic process of Duchenne and Becker muscular dystrophy.

PubMed

Holtzer, Caleb; Meaney, F John; Andrews, Jennifer; Ciafaloni, Emma; Fox, Deborah J; James, Katherine A; Lu, Zhenqiang; Miller, Lisa; Pandya, Shree; Ouyang, Lijing; Cunniff, Christopher

2011-11-01

To determine whether sociodemographic factors are associated with delays at specific steps in the diagnostic process of Duchenne and Becker muscular dystrophy. We examined abstracted medical records for 540 males from population-based surveillance sites in Arizona, Colorado, Georgia, Iowa, and western New York. We used linear regressions to model the association of three sociodemographic characteristics with age at initial medical evaluation, first creatine kinase measurement, and earliest DNA analysis while controlling for changes in the diagnostic process over time. The analytical dataset included 375 males with information on family history of Duchenne and Becker muscular dystrophy, neighborhood poverty levels, and race/ethnicity. Black and Hispanic race/ethnicity predicted older ages at initial evaluation, creatine kinase measurement, and DNA testing (P < 0.05). A positive family history of Duchenne and Becker muscular dystrophy predicted younger ages at initial evaluation, creatine kinase measurement and DNA testing (P < 0.001). Higher neighborhood poverty was associated with earlier ages of evaluation (P < 0.05). Racial and ethnic disparities in the diagnostic process for Duchenne and Becker muscular dystrophy are evident even after adjustment for family history of Duchenne and Becker muscular dystrophy and changes in the diagnostic process over time. Black and Hispanic children are initially evaluated at older ages than white children, and the gap widens at later steps in the diagnostic process.

Validating Neuro-QoL short forms and targeted scales with people who have multiple sclerosis.

PubMed

Miller, Deborah M; Bethoux, Francois; Victorson, David; Nowinski, Cindy J; Buono, Sarah; Lai, Jin-Shei; Wortman, Katy; Burns, James L; Moy, Claudia; Cella, David

2016-05-01

Multiple sclerosis (MS) is a chronic, progressive, and disabling disease of the central nervous system with dramatic variations in the combination and severity of symptoms it can produce. The lack of reliable disease-specific health-related quality of life (HRQL) measures for use in clinical trials prompted the development of the Neurology Quality of Life (Neuro-QOL) instrument, which includes 13 scales that assess physical, emotional, cognitive, and social domains, for use in a variety of neurological illnesses. The objective of this research paper is to conduct an initial assessment of the reliability and validation of the Neuro-QOL short forms (SFs) in MS. We assessed reliability, concurrent validity, known groups validity, and responsiveness between cross-sectional and longitudinal data in 161 recruited MS patients. Internal consistency was high for all measures (α = 0.81-0.95) and ICCs were within the acceptable range (0.76-0.91); concurrent and known groups validity were highest with the Global HRQL question. Longitudinal assessment was limited by the lack of disease progression in the group. The Neuro-QOL SFs demonstrate good internal consistency, test-re-test reliability, and concurrent and known groups validity in this MS population, supporting the validity of Neuro-QOL in adults with MS. © The Author(s), 2015.

Ultrasound neuro-modulation chip: activation of sensory neurons in Caenorhabditis elegans by surface acoustic waves.

PubMed

Zhou, Wei; Wang, Jingjing; Wang, Kaiyue; Huang, Bin; Niu, Lili; Li, Fei; Cai, Feiyan; Chen, Yan; Liu, Xin; Zhang, Xiaoyan; Cheng, Hankui; Kang, Lijun; Meng, Long; Zheng, Hairong

2017-05-16

Ultrasound neuro-modulation has gained increasing attention as a non-invasive method. In this paper, we present an ultrasound neuro-modulation chip, capable of initiating reversal behaviour and activating neurons of C. elegans under the stimulation of a single-shot, short-pulsed ultrasound. About 85.29% ± 6.17% of worms respond to the ultrasound stimulation exhibiting reversal behaviour. Furthermore, the worms can adapt to the ultrasound stimulation with a lower acoustic pulse duration of stimulation. In vivo calcium imaging shows that the activity of ASH, a polymodal sensory neuron in C. elegans, can be directly evoked by the ultrasound stimulation. On the other hand, AFD, a thermal sensitive neuron, cannot be activated by the ultrasound stimulation using the same parameter and the temperature elevation during the stimulation process is relatively small. Consistent with the calcium imaging results, the tax-4 mutants, which are insensitive to temperature increase, do not show a significant difference in avoidance probability compared to the wild type. Therefore, the mechanical effects induced by ultrasound are the main reason for neural and behavioural modulation of C. elegans. With the advantages of confined acoustic energy on the surface, compatible with standard calcium imaging, this neuro-modulation chip could be a powerful tool for revealing the molecular mechanisms of ultrasound neuro-modulation.

Intramuscular renin-angiotensin system is activated in human muscular dystrophy.

PubMed

Sun, Guilian; Haginoya, Kazuhiro; Dai, Hongmei; Chiba, Yoko; Uematsu, Mitsugu; Hino-Fukuyo, Naomi; Onuma, Akira; Iinuma, Kazuie; Tsuchiya, Shigeru

2009-05-15

To investigate the role of the muscular renin-angiotensin system (RAS) in human muscular dystrophy, we used immunohistochemistry and Western blotting to examine the cellular localization of angiotensin-converting enzyme (ACE), the angiotensin II type 1 receptor (AT1) and the angiotensin II type 2 receptor (AT2) in muscle biopsies from patients with Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), and congenital muscular dystrophy (CMD). In normal muscle, ACE was expressed in vascular endothelial cells and neuromuscular junctions (NMJs), whereas AT1 was immunolocalized to the smooth muscle cells of blood vessels and intramuscular nerve twigs. AT2 was immunolocalized in the smooth muscle cells of blood vessels. These findings suggest that the RAS has a functional role in peripheral nerves and NMJs. ACE and AT1, but AT2 immunoreactivity were increased markedly in dystrophic muscle as compared to controls. ACE and the AT1 were strongly expressed in the cytoplasm and nuclei of regenerating muscle fibers, fibroblasts, and in macrophages infiltrating necrotic fibers. Double immunolabeling revealed that activated fibroblasts in the endomysium and perimysium of DMD and CMD muscle were positive for ACE and AT1. Triple immunolabeling demonstrated that transforming growth factor-beta1 (TGF-beta1) and ACE were colocalized on the cytoplasm of activated fibroblasts in dystrophic muscle. Furthermore, Western blotting showed increases in the expression of AT1 and TGF-beta1 protein in dystrophic muscle, which coincided with our immunohistochemical results. The overexpression of ACE and AT1 in dystrophic muscle would likely result in the increased production of Ang II, which may act on these cells in an autocrine manner via AT1. The activation of AT1 may induce fibrous tissue formation through overexpression of TGF-beta1, which potently activates fibrogenesis and suppresses regeneration. In conclusion, our results imply that the intramuscular RAS-TGF-beta1 pathway

HyFIS: adaptive neuro-fuzzy inference systems and their application to nonlinear dynamical systems.

PubMed

Kim, J; Kasabov, N

1999-11-01

This paper proposes an adaptive neuro-fuzzy system, HyFIS (Hybrid neural Fuzzy Inference System), for building and optimising fuzzy models. The proposed model introduces the learning power of neural networks to fuzzy logic systems and provides linguistic meaning to the connectionist architectures. Heuristic fuzzy logic rules and input-output fuzzy membership functions can be optimally tuned from training examples by a hybrid learning scheme comprised of two phases: rule generation phase from data; and rule tuning phase using error backpropagation learning scheme for a neural fuzzy system. To illustrate the performance and applicability of the proposed neuro-fuzzy hybrid model, extensive simulation studies of nonlinear complex dynamic systems are carried out. The proposed method can be applied to an on-line incremental adaptive learning for the prediction and control of nonlinear dynamical systems. Two benchmark case studies are used to demonstrate that the proposed HyFIS system is a superior neuro-fuzzy modelling technique.

Meeting the Assistive Technology Needs of Students with Duchenne Muscular Dystrophy

ERIC Educational Resources Information Center

Heller, Kathryn Wolff; Mezei, Peter J.; Avant, Mary Jane Thompson

2009-01-01

Students with Duchenne muscular dystrophy (DMD) have a degenerative disease that requires ongoing changes in assistive technology (AT). The AT team needs to be knowledgeable about the disease and its progression in order to meet these students' changing needs in a timely manner. The unique needs of students with Duchenne muscular dystrophy in…

Investigation of Poor Academic Achievement in Children with Duchenne Muscular Dystrophy

ERIC Educational Resources Information Center

Hinton, V. J.; De Vivo, D. C.; Fee, R.; Goldstein, E.; Stern, Y.

2004-01-01

Duchenne Muscular Dystrophy (DMD) is a neurogenetic developmental disorder that presents with progressive muscular weakness. It is caused by a mutation in a gene that results in the absence of specific products that normally localize to muscle cells and the central nervous system (CNS). The majority of affected individuals have IQs within the…

[Clinical and therapeutic differences in neuro-ophthalmological involvement secondary to syphilis].

PubMed

Crespo-Burillo, J A; Gil-Perez, D; Alarcia-Alejos, R; Hernando-Quintana, N; Garcia-Rubio, S; Martin-Martinez, J

2014-09-16

INTRODUCTION. There are many forms of neuro-ophthalmological involvement secondary to syphilis, and not all of them are well known. Our aim is to determine the clinical and therapeutic differences in these patients. CASE REPORTS. Our sample included eight patients diagnosed with an ocular and neuro-ophthalmological disorder due to syphilis over the years 2012 and 2013. Five of them presented uveitis, pan-eveitis being the most frequent, with three cases. Two cases presented papilloedema and another displayed retrobulbar optic neuropathy. A total of 62.5% were diagnosed with neurosyphilis, the presence of which was related with compromise of the optic nerve (p = 0.035). None of them gave positive for VDRL in cerebrospinal fluid and they were diagnosed by the presence of FTA antibodies together with high protein levels in cerebrospinal fluid, lymphocytic pleocytosis or intrathecal synthesis of antibodies. In the absence of uveitis, diagnosis was delayed by a mean time of 2.6 months (p = 0.047). All the patients, except one who required a vitrectomy, progressed favourably with intravenous antibiotic therapy. CONCLUSIONS. In cases of neuro-ophthalmological compromise, whether inflammatory or non-inflammatory, the physician must bear syphilis in mind as a potential causation in order to avoid delays in the diagnosis, since early well-tailored treatment can prevent permanent loss of sight.

POTENTIAL DEVELOPMENTAL TOXICITY OF ANATOXIN-A, A CYANOBACTERIAL TOXIN

EPA Science Inventory

Anatoxin-a acts as a neuro-muscular blocking agent. Acute toxicity is characterized by rapid onset of paralysis, tremors, convulsions, and death. Human exposures may occur from recreational water activities and dietary supplements, but are primarily through drinking water. The...

Endoscopy in neuro-otologic surgery.

PubMed

Wackym, Phillip A; King, Wesley A; Meyer, Glenn A; Poe, Dennis S

2002-04-01

Endoscopy offers several distinct advantages over the operating microscope during neuro-otologic surgery that make it an excellent adjunctive tool to the microscope or independent modality during cranial base surgery. The high magnification gives excellent definition of perforating blood vessels, cranial nerves, and neural structures, which in many cases is superior to that achieved with the microscope. Furthermore, the use of angled or flexible endoscopes allows one to look around corners and behind anatomic structures blocking the view seen via a 0 degree microscope. Endoscopy also has the theoretical advantage that a less invasive operative procedure is required, which should reduce the operative morbidity. Several notable disadvantages of endoscopy include the problems associated with blood soiling the endoscope, making visualization difficult or impossible, the lack of readily available instrumentation designed specifically for endoscopic neuro-otology, and the poor overview of the operative field. This last point is an important one because the endoscope is placed adjacent to the lesion and does not allow one to look backward to prevent [figure: see text] injury to structures next to the shaft of the telescope. Furthermore, the surgeon must be cognizant of potential thermal injury to structures caused by the heat generated by the light source. The present endoscopic technology limits the image that the surgeon sees to two dimensions, which results in certain unique problems when operating in a three-dimensional milieu. Because of this, there is a steep learning curve to acquire endoscopic dexterity and three-dimensional orientation. Finally, bimanual operation requires the use of an articulated endoscope holder or the commitment of the co-surgeon to hold the endoscope. One of the limitations of the operative microscope is that the angle of view is determined by the distance of the lens to the skull, retractor, or obstructing tissue, which is a function of the

Energy-saving technology of vector controlled induction motor based on the adaptive neuro-controller

NASA Astrophysics Data System (ADS)

Engel, E.; Kovalev, I. V.; Karandeev, D.

2015-10-01

The ongoing evolution of the power system towards a Smart Grid implies an important role of intelligent technologies, but poses strict requirements on their control schemes to preserve stability and controllability. This paper presents the adaptive neuro-controller for the vector control of induction motor within Smart Gird. The validity and effectiveness of the proposed energy-saving technology of vector controlled induction motor based on adaptive neuro-controller are verified by simulation results at different operating conditions over a wide speed range of induction motor.

Childhood cardiorespiratory fitness, muscular fitness and adult measures of glucose homeostasis.

PubMed

Fraser, Brooklyn J; Blizzard, Leigh; Schmidt, Michael D; Juonala, Markus; Dwyer, Terence; Venn, Alison J; Magnussen, Costan G

2018-02-14

To assess whether childhood cardiorespiratory fitness (CRF) and muscular fitness phenotypes (strength, power, endurance) predict adult glucose homeostasis measures. Prospective longitudinal study. Study examining participants who had physical fitness measured in childhood (aged 7-15 years) and who attended follow-up clinics approximately 20 years later and provided a fasting blood sample which was tested for glucose and insulin. Physical fitness measurements included muscular strength (right and left grip, shoulder flexion, shoulder and leg extension), power (standing long jump distance) and endurance (number of push-ups in 30s), and CRF (1.6km run duration). In adulthood, fasting glucose and insulin levels were used to derive glucose homeostasis measures of insulin resistance (HOMA2-IR) and beta cell function (HOMA2-β). A standard deviation increase in childhood CRF or muscular strength (males) was associated with fasting glucose (CRF: β=-0.06mmol/L), fasting insulin (CRF: β=-0.73mU/L; strength: β=-0.40mU/L), HOMA2-IR (CRF: β=-0.06; strength: β=-0.05) and HOMA2-β (CRF: β=-3.06%; strength: β=-2.62%) in adulthood, independent of the alternative fitness phenotype (all p<0.01). Adjustment for childhood waist circumference reduced the effect by 17-35% for CRF and 0-15% for muscular strength (males) and statistical significance remained for all associations expect between CRF, fasting glucose and HOMA2-β (p>0.06). CRF and muscular fitness in childhood were inversely associated with measures of fasting insulin, insulin resistance and beta cell function in adulthood. Childhood CRF and muscular fitness could both be potential independent targets for strategies to help reduce the development of adverse glucose homeostasis. Copyright © 2018 Sports Medicine Australia. Published by Elsevier Ltd. All rights reserved.

Friend or foe: the dichotomous impact of T cells on neuro-de/re-generation during aging.

PubMed

Coder, Brandon; Wang, Weikan; Wang, Liefeng; Wu, Zhongdao; Zhuge, Qichuan; Su, Dong-Ming

2017-01-24

The interaction between T cells and the central nervous system (CNS) in homeostasis and injury has been recognized being both pathogenic (CD4+ T-helper 1 - Th1, Th17 and γδT) and ameliorative (Th2 and regulatory T cells - Tregs). However, in-depth studies aimed to elucidate the precise in the aged microenvironment and the dichotomous role of Tregs have just begun and many aspects remain unclear. This is due, not only to a mutual dependency and reciprocal causation of alterations and diseases between the nervous and T cell immune systems, but also to an inconsistent aging of the two systems, which dynamically changes with CNS injury/recovery and/or aging process. Cellular immune system aging, particularly immunosenescence and T cell aging initiated by thymic involution - sources of chronic inflammation in the elderly (termed inflammaging), potentially induces an acceleration of brain aging and memory loss. In turn, aging of the brain via neuro-endocrine-immune network drives total body systemic aging, including that of the immune system. Therefore, immunotherapeutics including vaccination and "protective autoimmunity" provide promising means to rejuvenate neuro-inflammatory disorders and repair CNS acute injury and chronic neuro-degeneration. We review the current understanding and recent discoveries linking the aging immune system with CNS injury and neuro-degeneration. Additionally, we discuss potential recovery and rejuvenation strategies, focusing on targeting the aging T cell immune system in an effort to alleviate acute brain injury and chronic neuro-degeneration during aging, via the "thymus-inflammaging-neurodegeneration axis".

Friend or foe: the dichotomous impact of T cells on neuro-de/re-generation during aging

PubMed Central

Wang, Liefeng; Wu, Zhongdao; Zhuge, Qichuan; Su, Dong-Ming

2017-01-01

The interaction between T cells and the central nervous system (CNS) in homeostasis and injury has been recognized being both pathogenic (CD4+ T-helper 1 - Th1, Th17 and γδT) and ameliorative (Th2 and regulatory T cells - Tregs). However, in-depth studies aimed to elucidate the precise in the aged microenvironment and the dichotomous role of Tregs have just begun and many aspects remain unclear. This is due, not only to a mutual dependency and reciprocal causation of alterations and diseases between the nervous and T cell immune systems, but also to an inconsistent aging of the two systems, which dynamically changes with CNS injury/recovery and/or aging process. Cellular immune system aging, particularly immunosenescence and T cell aging initiated by thymic involution - sources of chronic inflammation in the elderly (termed inflammaging), potentially induces an acceleration of brain aging and memory loss. In turn, aging of the brain via neuro-endocrine-immune network drives total body systemic aging, including that of the immune system. Therefore, immunotherapeutics including vaccination and “protective autoimmunity” provide promising means to rejuvenate neuro-inflammatory disorders and repair CNS acute injury and chronic neuro-degeneration. We review the current understanding and recent discoveries linking the aging immune system with CNS injury and neuro-degeneration. Additionally, we discuss potential recovery and rejuvenation strategies, focusing on targeting the aging T cell immune system in an effort to alleviate acute brain injury and chronic neuro-degeneration during aging, via the “thymus-inflammaging-neurodegeneration axis”. PMID:27738345

Neuro-fuzzy control of structures using acceleration feedback

NASA Astrophysics Data System (ADS)

Schurter, Kyle C.; Roschke, Paul N.

2001-08-01

This paper described a new approach for the reduction of environmentally induced vibration in constructed facilities by way of a neuro-fuzzy technique. The new control technique is presented and tested in a numerical study that involves two types of building models. The energy of each building is dissipated through magnetorheological (MR) dampers whose damping properties are continuously updated by a fuzzy controller. This semi-active control scheme relies on the development of a correlation between the accelerations of the building (controller input) and the voltage applied to the MR damper (controller output). This correlation forms the basis for the development of an intelligent neuro-fuzzy control strategy. To establish a context for assessing the effectiveness of the semi-active control scheme, responses to earthquake excitation are compared with passive strategies that have similar authority for control. According to numerical simulation, MR dampers are less effective control mechanisms than passive dampers with respect to a single degree of freedom (DOF) building model. On the other hand, MR dampers are predicted to be superior when used with multiple DOF structures for reduction of lateral acceleration.

Dystrophin Immunity in Duchenne’s Muscular Dystrophy

PubMed Central

Mendell, Jerry R.; Campbell, Katherine; Rodino-Klapac, Louise; Sahenk, Zarife; Shilling, Chris; Lewis, Sarah; Bowles, Dawn; Gray, Steven; Li, Chengwen; Galloway, Gloria; Malik, Vinod; Coley, Brian; Clark, K. Reed; Li, Juan; Xiao, Xiao; Samulski, Jade; McPhee, Scott W.; Samulski, R. Jude; Walker, Christopher M.

2010-01-01

SUMMARY We report on delivery of a functional dystrophin transgene to skeletal muscle in six patients with Duchenne’s muscular dystrophy. Dystrophin-specific T cells were detected after treatment, providing evidence of transgene expression even when the functional protein was not visualized in skeletal muscle. Circulating dystrophin-specific T cells were unexpectedly detected in two patients before vector treatment. Revertant dystrophin fibers, which expressed functional, truncated dystrophin from the deleted endogenous gene after spontaneous in-frame splicing, contained epitopes targeted by the autoreactive T cells. The potential for T-cell immunity to self and nonself dystrophin epitopes should be considered in designing and monitoring experimental therapies for this disease. (Funded by the Muscular Dystrophy Association and others; ClinicalTrials.gov number, NCT00428935.) PMID:20925545

Early-onset facioscapulohumeral muscular dystrophy type 1 with some atypical features.

PubMed

Dorobek, Małgorzata; van der Maarel, Silvère M; Lemmers, Richard J L F; Ryniewicz, Barbara; Kabzińska, Dagmara; Frants, Rune R; Gawel, Malgorzata; Walecki, Jerzy; Hausmanowa-Petrusewicz, Irena

2015-04-01

Facioscapulohumeral muscular dystrophy cases with facial weakness before the age of 5 and signs of shoulder weakness by the age of 10 are defined as early onset. Contraction of the D4Z4 repeat on chromosome 4q35 is causally related to facioscapulohumeral muscular dystrophy type 1, and the residual size of the D4Z4 repeat shows a roughly inverse correlation with the severity of the disease. Contraction of the D4Z4 repeat on chromosome 4q35 is believed to induce a local change in chromatin structure and consequent transcriptional deregulation of 4qter genes. We present early-onset cases in the Polish population that amounted to 21% of our total population with facioscapulohumeral muscular dystrophy. More than 27% of them presented with severe phenotypes (wheelchair dependency). The residual D4Z4 repeat sizes ranged from 1 to 4 units. In addition, even within early-onset facioscapulohumeral muscular dystrophy type 1 phenotypes, some cases had uncommon features (head drop, early disabling contractures, progressive ptosis, and respiratory insufficiency and cardiomyopathy). © The Author(s) 2014.

Zebrafish models flex their muscles to shed light on muscular dystrophies.

PubMed

Berger, Joachim; Currie, Peter D

2012-11-01

Muscular dystrophies are a group of genetic disorders that specifically affect skeletal muscle and are characterized by progressive muscle degeneration and weakening. To develop therapies and treatments for these diseases, a better understanding of the molecular basis of muscular dystrophies is required. Thus, identification of causative genes mutated in specific disorders and the study of relevant animal models are imperative. Zebrafish genetic models of human muscle disorders often closely resemble disease pathogenesis, and the optical clarity of zebrafish embryos and larvae enables visualization of dynamic molecular processes in vivo. As an adjunct tool, morpholino studies provide insight into the molecular function of genes and allow rapid assessment of candidate genes for human muscular dystrophies. This unique set of attributes makes the zebrafish model system particularly valuable for the study of muscle diseases. This review discusses how recent research using zebrafish has shed light on the pathological basis of muscular dystrophies, with particular focus on the muscle cell membrane and the linkage between the myofibre cytoskeleton and the extracellular matrix.

Rimmed vacuoles in Becker muscular dystrophy have similar features with inclusion myopathies.

PubMed

Momma, Kazunari; Noguchi, Satoru; Malicdan, May Christine V; Hayashi, Yukiko K; Minami, Narihiro; Kamakura, Keiko; Nonaka, Ikuya; Nishino, Ichizo

2012-01-01

Rimmed vacuoles in myofibers are thought to be due to the accumulation of autophagic vacuoles, and can be characteristic in certain myopathies with protein inclusions in myofibers. In this study, we performed a detailed clinical, molecular, and pathological characterization of Becker muscular dystrophy patients who have rimmed vacuoles in muscles. Among 65 Becker muscular dystrophy patients, we identified 12 patients who have rimmed vacuoles and 11 patients who have deletions in exons 45-48 in DMD gene. All patients having rimmed vacuoles showed milder clinical features compared to those without rimmed vacuoles. Interestingly, the rimmed vacuoles in Becker muscular dystrophy muscles seem to represent autophagic vacuoles and are also associated with polyubiquitinated protein aggregates. These findings support the notion that rimmed vacuoles can appear in Becker muscular dystrophy, and may be related to the chronic changes in muscle pathology induced by certain mutations in the DMD gene.

Comparative study on the muscular load of the arms using hair driers.

PubMed

Harada, H; Katsuura, T; Kikuchi, Y

1995-12-01

The purpose of the present study was to evaluate the muscular load of the arm when combing the hair using different "kuru-kuru" type of hair driers. Ten female students (20-24 years old) volunteered as subjects. Five combing patterns were conducted as follows: 1) comb outer layer of right side of hair using right hand, 2) comb outer layer of left side of hair using right hand, 3) comb inner layer of left side of hair using right hand, 4) comb outer layer of back hair using right hand, and 5) comb inner layer of right side of hair using left hand. Surface EMGs were recorded from M. flexor carpi ulnaris, M. brachioradialis, M. biceps brachii, M. triceps brachii, M. deltoideus and M. trapezius of both sides of body. Integrated EMGs (iEMGs) were used to evaluate muscular load for each of the seven different types of hair driers used. The relationship between iEMGs and weight, center of gravity, diameter, length, and circumference of each hair drier were examined. The weight of hair driers tended to be the effective factor on the muscular load. Muscular load also had a tendency to be affected by the shape of the grips. With regard to the hand size, the longer the thumb length, the smaller is the muscular load. It was suggested that a relatively large diameter of the bulb-shaped grip of the drier gave a smaller muscular load among the hair driers examined in the present experiment.

Usefulness of Neuro-Fuzzy Models' Application for Tobacco Control

NASA Astrophysics Data System (ADS)

Petrovic-Lazarevic, Sonja; Zhang, Jian Ying

2007-12-01

The paper presents neuro-fuzzy models' application appropriate for tobacco control: the fuzzy control model, Adaptive Network Based Fuzzy Inference System, Evolving Fuzzy Neural Network models, and EVOlving POLicies. We propose further the use of Fuzzy Casual Networks to help tobacco control decision makers develop policies and measure their impact on social regulation.

[Communicating effectively: neuro-linguistic programming in the psychiatric interview].

PubMed

Ducasse, Déborah; Fond, Guillaume

2014-01-01

Neuro-linguistic programming is a set of practices and knowledge which seeks to "model" and then imitate the best communication practices. Applying the key concepts to the care relationship in mental health care helps to improve the quality of the contact, the clarity of the communication and to create an openness to change.

Neuro-Linguistic Programming: Developing Effective Communication in the Classroom.

ERIC Educational Resources Information Center

Torres, Cresencio; Katz, Judy H.

Neuro-Linguistic Programming (NLP) is a method that teachers can use to increase their communication effectiveness by matching their communication patterns with those of their students. The basic premise of NLP is that people operate and make sense of their experience through information received from the world around them. This information is…

Serum creatinine level: a supplemental index to distinguish Duchenne muscular dystrophy from Becker muscular dystrophy.

PubMed

Zhang, Huili; Zhu, Yuling; Sun, Yiming; Liang, Yingyin; Li, Yaqin; Zhang, Yu; Deng, Langhui; Wen, Xingxuan; Zhang, Cheng

2015-01-01

To improve assessment of dystrophinopathy, the aim of this study was to identify whether serum creatinine (Crn) level reflects disease severity. Biochemical, Vignos score, and genetic data were collected on 212 boys with dystrophinopathy. Serum Crn level had a strong inverse correlation with Vignos score by simple correlation (r = -0.793) and partial correlation analysis after adjustment for age, height, and weight (r = -0.791; both P < 0.01). Serum Crn level was significantly higher in patients with in-frame than out-of-frame mutations (Z = -4.716,  P < 0.01) and in Becker muscular dystrophy (BMD) patients than Duchenne muscular dystrophy (DMD) patients at ages 4, 5, 7, and 9 yr (all P < 0.0125). After adjusting for age, height, and weight, BMD patients still had a significantly higher serum Crn level than DMD patients (β = 7.140,  t = 6.277,  P < 0.01). Serum Crn level reflected disease severity and may serve as a supplemental index to distinguish DMD from BMD in clinical practice.

Effects of Partner's Improvisational Resistance Training on dancers' muscular strength.

PubMed

Vetter, Rheba E; Dorgo, Sandor

2009-05-01

The purpose of this study was to observe the effects of Partner's Improvisational Resistance Training (PIRT) on muscular strength, body circumference, and body fat percentage in 10 female college-age dancers in comparison with 8 female dancers in a control group. The PIRT program, based on the concepts of manual resistance training, is the application of contact improvisation in a systematic strength development program, which proposes a way of contextualizing muscular strength development within the dance class. The program lasted 8 weeks, meeting 3 times weekly for 60-minute sessions. The muscular strength pre- and posttests included 1-repetition maximum (1RM) for leg extension, leg flexion, leg press, bench press, lat pulldown, back extension, and modified sit-up. Hydrostatic weighing for body composition and circumference measures on the waist, hip, shoulder, upper arm, and thigh were made pre- and posttest analyses. There were no significant pretest differences between the groups for age, height, body weight, body fat percentage, any of the circumference measures, or 5 of the 7 muscular strength measures. At posttest, neither group showed significant changes in total body weight, body fat percentage, or lean body weight. The experimental group showed significant decrements in the waist and hip circumference measures, and all other body circumference changes were nonsignificant. The experimental group showed significant changes from pretest to posttest for all seven 1RM strength measures and greater absolute and relative strength improvements in 5 measures compared with the control group. Thus, the 8-week PIRT program for female dancers was found effective in improving overall muscular strength and decreasing circumference in the waist-hip region, but it did not elicit significant changes in body composition.

[Motor function evaluation in merosin-deficient congenital muscular dystrophy children].

PubMed

Rocco, Fernanda M; Luz, Fernanda H Gianini; Rossato, Alexsander Junquera; Fernandes, Antônio Carlos; Oliveira, Acary S B; Betetas, Javier Toledano; Zanoteli, Edmar

2005-06-01

Congenital muscular dystrophy (CMD) is a heterogeneous group of disorders characterized by early onset of hypotonia and weakness. Almost 50% of the cases are caused by primary deficiency of a protein named merosin (MD), and present a homogenous phenotype with a severe motor and respiratory involvement. Eleven children with clinical and histological diagnosis of CMD-MD, aged of 3 to 15 years, were studied using the manual muscle testing (Medical Research Council), goniometric analysis, motor ability and day life activities (Barthel index) scales, with the objective to characterize the main motor function limitations. The muscular groups most affected were cervical flexors, paravertebral and proximal portions of limbs. The muscular groups of upper limbs were as affected as the lower limbs, and the extensors were more affected than the flexors groups. All children had severe muscular retractions on the hip, knee and elbow. Other frequent deformities were scoliosis and equinus-varum feet. No children presented the motor ability to walk, stand up and crawl; and all of them were classified as dependents or semi-dependents in the day life activities scale. Our findings confirm the severe and diffuse involvement of skeletal muscle in CMD-MD patients, producing serious motor limitations and deformities.

Contextualizing neuro-collaborations: reflections on a transdisciplinary fMRI lie detection experiment

PubMed Central

Littlefield, Melissa M.; Fitzgerald, Des; Knudsen, Kasper; Tonks, James; Dietz, Martin J.

2014-01-01

Recent neuroscience initiatives (including the E.U.’s Human Brain Project and the U.S.’s BRAIN Initiative) have reinvigorated discussions about the possibilities for transdisciplinary collaboration between the neurosciences, the social sciences, and the humanities. As STS scholars have argued for decades, however, such inter- and transdisciplinary collaborations are potentially fraught with tensions between researchers. This essay build on such claims by arguing that the tensions of transdisciplinary research also exist within researchers’ own experiences of working between disciplines - a phenomenon that we call “disciplinary double consciousness” (DDC). Building on previous work that has characterized similar spaces (and especially on the Critical Neuroscience literature), we argue that “neuro-collaborations” inevitably engage researchers in DDC - a phenomenon that allows us to explore the useful dissonance that researchers can experience when working between a “home” discipline and a secondary discipline. Our case study is a five-year research project in functional magnetic resonance imaging (fMRI) lie detection involving a transdisciplinary research team made up of social scientists, a neuroscientist, and a humanist. In addition to theorizing neuro-collaborations from the inside-out, this essay presents practical suggestions for developing transdisciplinary infrastructures that could support future neuro-collaborations. PMID:24744713

[Functional neuro-navigation and intraoperative magnetic resonance imaging for the resection of gliomas involving eloquent language structures].

PubMed

Chen, Xiao-lei; Xu, Bai-nan; Wang, Fei; Meng, Xiang-hui; Zhang, Jun; Jiang, Jin-li; Yu, Xin-guang; Zhou, Ding-biao

2011-08-01

To explore the clinical value of functional neuro-navigation and high-field-strength intraoperative magnetic resonance imaging (iMRI) for the resection of intracerebral gliomas involving eloquent language structures. From April 2009 to April 2010, 48 patients with intracerebral gliomas involving eloquent language structures, were operated with functional neuro-navigation and iMRI. Blood oxygen level dependent functional MRI (BOLD-fMRI) was used to depict both Broca and Wernicke cortex, while diffusion tensor imaging (DTI) based fiber tracking was used to delineate arcuate fasciculus. The reconstructed language structures were integrated into a navigation system, so that intra-operative microscopic-based functional neuro-navigation could be achieved. iMRI was used to update the images for both language structures and residual tumors. All patients were evaluated for language function pre-operatively and post-operatively upon short-term and long-term follow-up. In all patients, functional neuro-navigation and iMRI were successfully achieved. In 38 cases (79.2%), gross total resection was accomplished, while in the rest 10 cases (20.8%), subtotal resection was achieved. Only 1 case (2.1%) developed long-term (more than 3 months) new language function deficits at post-operative follow-up. No peri-operative mortality was recorded. With functional neuro-navigation and iMRI, the eloquent structures for language can be precisely located, while the resection size can be accurately evaluated intra-operatively. This technique is safe and helpful for preservation of language function.

NeuroLex.org: an online framework for neuroscience knowledge

PubMed Central

Larson, Stephen D.; Martone, Maryann E.

2013-01-01

The ability to transmit, organize, and query information digitally has brought with it the challenge of how to best use this power to facilitate scientific inquiry. Today, few information systems are able to provide detailed answers to complex questions about neuroscience that account for multiple spatial scales, and which cross the boundaries of diverse parts of the nervous system such as molecules, cellular parts, cells, circuits, systems and tissues. As a result, investigators still primarily seek answers to their questions in an increasingly densely populated collection of articles in the literature, each of which must be digested individually. If it were easier to search a knowledge base that was structured to answer neuroscience questions, such a system would enable questions to be answered in seconds that would otherwise require hours of literature review. In this article, we describe NeuroLex.org, a wiki-based website and knowledge management system. Its goal is to bring neurobiological knowledge into a framework that allows neuroscientists to review the concepts of neuroscience, with an emphasis on multiscale descriptions of the parts of nervous systems, aggregate their understanding with that of other scientists, link them to data sources and descriptions of important concepts in neuroscience, and expose parts that are still controversial or missing. To date, the site is tracking ~25,000 unique neuroanatomical parts and concepts in neurobiology spanning experimental techniques, behavioral paradigms, anatomical nomenclature, genes, proteins and molecules. Here we show how the structuring of information about these anatomical parts in the nervous system can be reused to answer multiple neuroscience questions, such as displaying all known GABAergic neurons aggregated in NeuroLex or displaying all brain regions that are known within NeuroLex to send axons into the cerebellar cortex. PMID:24009581

Clinical and genetic diversity of SMN1-negative proximal spinal muscular atrophies

PubMed Central

Jordanova, Albena

2014-01-01

Hereditary spinal muscular atrophy is a motor neuron disorder characterized by muscle weakness and atrophy due to degeneration of the anterior horn cells of the spinal cord. Initially, the disease was considered purely as an autosomal recessive condition caused by loss-of-function SMN1 mutations on 5q13. Recent developments in next generation sequencing technologies, however, have unveiled a growing number of clinical conditions designated as non-5q forms of spinal muscular atrophy. At present, 16 different genes and one unresolved locus are associated with proximal non-5q forms, having high phenotypic variability and diverse inheritance patterns. This review provides an overview of the current knowledge regarding the phenotypes, causative genes, and disease mechanisms associated with proximal SMN1-negative spinal muscular atrophies. We describe the molecular and cellular functions enriched among causative genes, and discuss the challenges in the post-genomics era of spinal muscular atrophy research. PMID:24970098

Widespread occurrence of neuro-active pharmaceuticals and metabolites in 24 Minnesota rivers and wastewaters

USGS Publications Warehouse

Writer, Jeffrey; Ferrer, Imma; Barber, Larry B.; Thurman, E. Michael

2013-01-01

Concentrations of 17 neuro-active pharmaceuticals and their major metabolites (bupropion, hydroxy-bupropion, erythro-hydrobupropion, threo-hydrobupropion, carbamazepine, 10,11,-dihydro-10,11,-dihydroxycarbamazepine, 10-hydroxy-carbamazepine, citalopram, N-desmethyl-citalopram, fluoxetine, norfluoxetine, gabapentin, lamotrigine, 2-N-glucuronide-lamotrigine, oxcarbazepine, venlafaxine and O-desmethyl-venlafaxine), were measured in treated wastewater and receiving surface waters from 24 locations across Minnesota, USA. The analysis of upstream and downstream sampling sites indicated that the wastewater treatment plants were the major source of the neuro-active pharmaceuticals and associated metabolites in surface waters of Minnesota. Concentrations of parent compound and the associated metabolite varied substantially between treatment plants (concentrations ± standard deviation of the parent compound relative to its major metabolite) as illustrated by the following examples; bupropion and hydrobupropion 700 ± 1000 ng L−1, 2100 ± 1700 ng L−1, carbamazepine and 10-hydroxy-carbamazepine 480 ± 380 ng L−1, 360 ± 400 ng L−1, venlafaxine and O-desmethyl-venlafaxine 1400 ± 1300 ng L−1, 1800 ± 2300 ng L−1. Metabolites of the neuro-active compounds were commonly found at higher or comparable concentrations to the parent compounds in wastewater effluent and the receiving surface water. Neuro-active pharmaceuticals and associated metabolites were detected only sporadically in samples upstream from the effluent outfall. Metabolite to parent ratios were used to evaluate transformation, and we determined that ratios in wastewater were much lower than those reported in urine, indicating that the metabolites are relatively more labile than the parent compounds in the treatment plants and in receiving waters. The widespread occurrence of neuro-active pharmaceuticals and metabolites in Minnesota effluents and surface waters indicate that

Muscle-Derived Proteins as Serum Biomarkers for Monitoring Disease Progression in Three Forms of Muscular Dystrophy

PubMed Central

Burch, Peter M.; Pogoryelova, Oksana; Goldstein, Richard; Bennett, Donald; Guglieri, Michela; Straub, Volker; Bushby, Kate; Lochmüller, Hanns; Morris, Carl

2015-01-01

Abstract Background: Identifying translatable, non-invasive biomarkers of muscular dystrophy that better reflect the disease pathology than those currently available would aid the development of new therapies, the monitoring of disease progression and the response to therapy. Objective: The goal of this study was to evaluate a panel of serum protein biomarkers with the potential to specifically detect skeletal muscle injury. Method: Serum concentrations of skeletal troponin I (sTnI), myosin light chain 3 (Myl3), fatty acid binding protein 3 (FABP3) and muscle-type creatine kinase (CKM) proteins were measured in 74 Duchenne muscular dystrophy (DMD), 38 Becker muscular dystrophy (BMD) and 49 Limb-girdle muscular dystrophy type 2B (LGMD2B) patients and 32 healthy controls. Results: All four proteins were significantly elevated in the serum of these three muscular dystrophy patient populations when compared to healthy controls, but, interestingly, displayed different profiles depending on the type of muscular dystrophy. Additionally, the effects of patient age, ambulatory status, cardiac function and treatment status on the serum concentrations of the proteins were investigated. Statistical analysis revealed correlations between the serum concentrations and certain clinical endpoints including forced vital capacity in DMD patients and the time to walk ten meters in LGMD2B patients. Serum concentrations of these proteins were also elevated in two preclinical models of muscular dystrophy, the mdx mouse and the golden-retriever muscular dystrophy dog. Conclusions: These proteins, therefore, are potential muscular dystrophy biomarkers for monitoring disease progression and therapeutic response in both preclinical and clinical studies. PMID:26870665

Genetics Home Reference: Fukuyama congenital muscular dystrophy

MedlinePlus

... Fujii T, Aiba H, Toda T. Seizure-genotype relationship in Fukuyama-type congenital muscular dystrophy. Brain Dev. ... for Links Data Files & API Site Map Subscribe Customer Support USA.gov Copyright Privacy Accessibility FOIA Viewers & ...

[Current status and future prospects of research on Fukuyama muscular dystrophy].

PubMed

Toda, Tatsushi

2015-08-01

Fukuyama congenital muscular dystrophy(FCMD) is a second common childhood muscular dystrophy in Japan. All FCMD patients have ancestral insertion of the SVA retrotransposal element into fukutin. We show that aberrant mRNA splicing induced by SVA exon-trapping caused FCMD. Introduction of 3 cocktailed antisense oligonucleotides(AONs) targeting around these splice sites prevented pathogenic splicing in FCMD patient cells and model mice, and normalized protein production and functions of Fukutin as well as O-glycosylation of α-dystroglycan. We show the promise of splicing modulation therapy as the first radical clinical treatment for FCMD in the near future. We also show that fukutin is prerequisite to ameliorate muscular dystrophic phenotype by myofiber-selective LARGE expression. Recent advances in FCMD are discussed.

Effects of Passive Physical Exercise on Peripheral Vision in Muscular Dystrophic Children.

ERIC Educational Resources Information Center

Eickelberg, Warren; And Others

1983-01-01

The effects of passive exercise of the extremities on peripheral vision of muscular dystrophic children aged 9 to 13 years was investigated. Compared to control subjects, those who experienced six minutes of passive exercise evidenced increased peripheral vision. Curriculum revisions for muscular dystrophic children indicate the importance of…

GABA and GABA-Alanine from the Red Microalgae Rhodosorus marinus Exhibit a Significant Neuro-Soothing Activity through Inhibition of Neuro-Inflammation Mediators and Positive Regulation of TRPV1-Related Skin Sensitization

PubMed Central

Scandolera, Amandine; Hubert, Jane; Humeau, Anne; Lambert, Carole; De Bizemont, Audrey; Winkel, Chris; Kaouas, Abdelmajid; Renault, Jean-Hugues; Reynaud, Romain

2018-01-01

The aim of the present study was to investigate the neuro-soothing activity of a water-soluble hydrolysate obtained from the red microalgae Rhodosorus marinus Geitler (Stylonemataceae). Transcriptomic analysis performed on ≈100 genes related to skin biological functions firstly revealed that the crude Rhodosorus marinus extract was able to significantly negatively modulate specific genes involved in pro-inflammation (interleukin 1α encoding gene, IL1A) and pain detection related to tissue inflammation (nerve growth factor NGF and its receptor NGFR). An in vitro model of normal human keratinocytes was then used to evaluate the ability of the Rhodosorus marinus extract to control the release of neuro-inflammation mediators under phorbol myristate acetate (PMA)-induced inflammatory conditions. The extract incorporated at 1% and 3% significantly inhibited the release of IL-1α and NGF secretion. These results were confirmed in a co-culture system of reconstructed human epithelium and normal human epidermal keratinocytes on which a cream formulated with the Rhodosorus marinus extract at 1% and 3% was topically applied after systemic induction of neuro-inflammation. Finally, an in vitro model of normal human astrocytes was developed for the evaluation of transient receptor potential vanilloid 1 (TRPV1) receptor modulation, mimicking pain sensing related to neuro-inflammation as observed in sensitive skins. Treatment with the Rhodosorus marinus extract at 1% and 3% significantly decreased PMA-mediated TRPV1 over-expression. In parallel with these biological experiments, the crude Rhodosorus marinus extract was fractionated by centrifugal partition chromatography (CPC) and chemically profiled by a recently developed 13C NMR-based dereplication method. The CPC-generated fractions as well as pure metabolites were tested again in vitro in an attempt to identify the biologically active constituents involved in the neuro-soothing activity of the Rhodosorus marinus extract

NeuroMind: Past, present, and future.

PubMed

Kubben, Pieter L

2017-01-01

This narrative report describes the underlying rationale and technical developments of NeuroMind, a mobile clinical decision support system for neurosurgery. From the perspective of a neurosurgeon - (app) developer it explains how technical progress has shaped the world's "most rated and highest rated" neurosurgical mobile application, with particular attention for operating system diversity on mobile hardware, cookbook medicine, regulatory affairs (in particular regarding software as a medical device), and new developments in the field of clinical data science, machine learning, and predictive analytics. Finally, the concept of "computational neurosurgery" is introduced as a vehicle to reach new horizons in neurosurgery.

Maximalist vs. minimalist shoes: dose-effect response of elastic compression on muscular oscillations.

PubMed

Gellaerts, Jules; Pirard, Maxime; Muzic, Jessie; Peseux, Maxime; Ménétrier, Arnaud

2017-10-01

The aim of this study was to establish whether maximalist shoes engender fewer muscular oscillations than minimalist shoes and determine to what extent these shoes, when combined with elastic compression (EC), help reduce muscle oscillations. For that purpose, we tested the effects of various levels of compression on the muscular oscillations in maximalist and minimalist footwear. Eleven volunteers executed 16 one-minute passages on a flat treadmill in a randomized order: maximalists or minimalists, walking (6 km/h) or running (10 km/h), without EC (control condition [CON]) or with EC applying different pressures (9.6 mmHg, 14.5 mmHg and 20.4 mmHg). The muscular oscillations were measured on both thighs, on the rectus femoris and on the vastus medialis with tri-axial accelerometers. Muscular oscillations are lower in maximalist shoes than in minimalist shoes, for both walking to 6 km/h and running to 10 km/h (P<0.05). Oscillations are also reduced with EC (P<0.05). This decrease is most marked when the pressure exercised by the EC is increased. Increased compression with minimalist shoes reduces muscular oscillations as much as maximalist shoes, when combined with lower compression.

Impact of a supervised worksite exercise program on back and core muscular endurance in firefighters.

PubMed

Mayer, John M; Quillen, William S; Verna, Joe L; Chen, Ren; Lunseth, Paul; Dagenais, Simon

2015-01-01

Low back pain is a leading cause of disability in firefighters and is related to poor muscular endurance. This study examined the impact of supervised worksite exercise on back and core muscular endurance in firefighters. A cluster randomized controlled trial was used for this study. The study occurred in fire stations of a municipal fire department (Tampa, Florida). Subjects were 96 full-duty career firefighters who were randomly assigned by fire station to exercise (n = 54) or control (n = 42) groups. Exercise group participants completed a supervised exercise targeting the back and core muscles while on duty, two times per week for 24 weeks, in addition to their usual fitness regimen. Control group participants continued their usual fitness regimen. Back and core muscular endurance was assessed with the Biering-Sorensen test and plank test, respectively. Changes in back and core muscular endurance from baseline to 24 weeks were compared between groups using analysis of covariance and linear mixed effects models. After 24 weeks, the exercise group had 12% greater (p = .021) back muscular endurance and 21% greater (p = .0006) core muscular endurance than did the control group. The exercise intervention did not disrupt operations or job performance. A supervised worksite exercise program was safe and effective in improving back and core muscular endurance in firefighters, which could protect against future low back pain.

Imaging of neuro-cognitive performance in extreme Environments—A (p)review

NASA Astrophysics Data System (ADS)

Schneider, Stefan; Bubeev, Juri A.; Choukèr, Alexander; Morukov, Boris; Johannes, Bernd; Strüder, Heiko K.

2012-12-01

Living in extreme environments is accompanied by a number of stressors, which can be classified either as physiological stressors (e.g. microgravity, missing sunlight) or psychological stressors (e.g. confinement). From earth bound studies a negative impact of stress on mental health and cognitive performance is well known and both factors might impair mission success and mission safety during longer inhabitation of space. Accordingly there is the need to identify adequate countermeasures. Nevertheless causal research of neuro-cognitive impairments in space remains speculative due to missing possibilities of brain imaging. Furthermore the reliability of current psychological tests used to assess and monitor cognitive performance in extreme environments seems to be vulnerable due to a lack of compliance. With on-going plans of international space agencies to send people to moon and/or mars, this manuscript aims to summarize and review research attempts of the past two decades and to identify methodological shortcomings. Finally, following the guideline that research has no legacy for its own but must serve the self-concept and well-being of man, this manuscript presents a number of recommendations to enhance future neuro-cognitive research in extreme environments. A deeper insight into neuro-cognitive coherence is not only desirable to understand the effects of stress on mental health, which seems to be a major issue for our current society, and to develop adequate countermeasures but will also help to maintain and improve mission success and mission safety in manned space flight.

Spatial modeling of environmental vulnerability of marine finfish aquaculture using GIS-based neuro-fuzzy techniques.

PubMed

Navas, Juan Moreno; Telfer, Trevor C; Ross, Lindsay G

2011-08-01

Combining GIS with neuro-fuzzy modeling has the advantage that expert scientific knowledge in coastal aquaculture activities can be incorporated into a geospatial model to classify areas particularly vulnerable to pollutants. Data on the physical environment and its suitability for aquaculture in an Irish fjard, which is host to a number of different aquaculture activities, were derived from a three-dimensional hydrodynamic and GIS models. Subsequent incorporation into environmental vulnerability models, based on neuro-fuzzy techniques, highlighted localities particularly vulnerable to aquaculture development. The models produced an overall classification accuracy of 85.71%, with a Kappa coefficient of agreement of 81%, and were sensitive to different input parameters. A statistical comparison between vulnerability scores and nitrogen concentrations in sediment associated with salmon cages showed good correlation. Neuro-fuzzy techniques within GIS modeling classify vulnerability of coastal regions appropriately and have a role in policy decisions for aquaculture site selection. Copyright © 2011 Elsevier Ltd. All rights reserved.

The Relationship between Selected Body Composition Variables and Muscular Endurance in Women

ERIC Educational Resources Information Center

Esco, Michael R.; Olson, Michele S.; Williford, Henry N.

2010-01-01

The primary purpose of this study was to determine if muscular endurance is affected by referenced waist circumference groupings, independent of body mass and subcutaneous abdominal fat, in women. This study also explored whether selected body composition measures were associated with muscular endurance. Eighty-four women were measured for height,…

Innovative neuro-fuzzy system of smart transport infrastructure for road traffic safety

NASA Astrophysics Data System (ADS)

Beinarovica, Anna; Gorobetz, Mikhail; Levchenkov, Anatoly

2017-09-01

The proposed study describes applying of neural network and fuzzy logic in transport control for safety improvement by evaluation of accidents’ risk by intelligent infrastructure devices. Risk evaluation is made by following multiple-criteria: danger, changeability and influence of changes for risk increasing. Neuro-fuzzy algorithms are described and proposed for task solution. The novelty of the proposed system is proved by deep analysis of known studies in the field. The structure of neuro-fuzzy system for risk evaluation and mathematical model is described in the paper. The simulation model of the intelligent devices for transport infrastructure is proposed to simulate different situations, assess the risks and propose the possible actions for infrastructure or vehicles to minimize the risk of possible accidents.

Kinematics, muscular activity and propulsion in gopher snakes

PubMed

Moon; Gans

1998-10-01

Previous studies have addressed the physical principles and muscular activity patterns underlying terrestrial lateral undulation in snakes, but not the mechanism by which muscular activity produces curvature and propulsion. In this study, we used synchronized electromyography and videography to examine the muscular basis and propulsive mechanism of terrestrial lateral undulation in gopher snakes Pituophis melanoleucus affinis. Specifically, we used patch electrodes to record from the semispinalis, longissimus dorsi and iliocostalis muscles in snakes pushing against one or more pegs. Axial bends propagate posteriorly along the body and contact the pegs at or immediately posterior to an inflection of curvature, which then reverses anterior to the peg. The vertebral column bends broadly around a peg, whereas the body wall bends sharply and asymmetrically around the anterior surface of the peg. The epaxial muscles are always active contralateral to the point of contact with a peg; they are activated slightly before or at the point of maximal convexity and deactivated variably between the inflection point and the point of maximal concavity. This pattern is consistent with muscular shortening and the production of axial bends, although variability in the pattern indicates that other muscles may affect the mechanics of the epaxial muscles. The kinematic and motor patterns in snakes crawling against experimentally increased drag indicated that forces are produced largely by muscles that are active in the axial bend around each peg, rather than by distant muscles from which the forces might be transmitted by connective tissues. At each point of force exertion, the propulsive mechanism of terrestrial lateral undulation may be modeled as a type of cam-follower, in which continuous bending of the trunk around the peg produces translation of the snake.

Prediction of specific depressive symptom clusters in youth with epilepsy: The NDDI-E-Y versus Neuro-QOL SF.

PubMed

Kellermann, Tanja S; Mueller, Martina; Carter, Emma G; Brooks, Byron; Smith, Gigi; Kopp, Olivia J; Wagner, Janelle L

2017-08-01

Proper assessment and early identification of depressive symptoms are essential to initiate treatment and minimize the risk for poor outcomes in youth with epilepsy (YWE). The current study examined the predictive utility of the Neurological Disorders Depression Inventory-Epilepsy for Youth (NDDI-E-Y) and the Neuro-QOL Depression Short Form (Neuro-QOL SF) in explaining variance in overall depressive symptoms and specific symptom clusters on the gold standard Children's Depression Inventory-2 (CDI-2). Cross-sectional study examining 99 YWE (female 68, mean age 14.7 years) during a routine epilepsy visit, who completed self-report measures of depressive symptoms, including the NDDI-E-Y, CDI-2, and the Neuro-QOL SF. Caregivers completed a measure of seizure severity. All sociodemographic and medical information was evaluated through electronic medical record review. After accounting for seizure and demographic variables, the NDDI-E-Y accounted for 45% of the variance in the CDI-2 Total score and the CDI-2 Ineffectiveness subscale. Furthermore, the NDDI-E-Y predicted CDI-2 Total scores and subscales similarly, with the exception of explaining significantly more variance in the CDI-2 Ineffectiveness subscale compared to the Negative Mood subscale. The NDDI-E-Y explained greater variance compared to Neuro-QOL SF across the Total (48% vs. 37%) and all CDI-2 subscale scores; however, the NDDI-E-Y emerged as a stronger predictor of only CDI-2 Ineffectiveness. Both the NDDI-E-Y and Neuro-QOL SF accounted for the lowest amount of variance in CDI-2 Negative Mood. Sensitivity was poor for the Neuro-QOL SF in predicting high versus low CDI-2 scores. The NDDI-E-Y has strong psychometrics and can be easily integrated into routine epilepsy care for quick, brief screening of depressive symptoms in YWE. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

Repair of an inguinoscrotal hernia in a patient with Becker muscular dystrophy.

PubMed

Tatulli, F; Caraglia, A; Delcuratolo, A; Cassano, S; Chetta, G S

2017-01-01

Inguinal hernia repairs are routinely performed as outpatient procedures in most patients, whereas a few require admission due to clinical or social peculiarities. Muscular dystrophies are inherited disorders characterized by progressive muscle wasting and weakness. In case of surgery there is no definite recommendation for either general or regional anesthesia. This contribution regards a 48 y. o. male patient diagnosed with Becker Muscular Dystrophy by muscle biopsy 10 years earlier. He had a left-sided sizable inguinoscrotal hernia with repeat episodes of incarceration. An elective mesh repair with suction drainage was accomplished under selective spinal anesthesia. The post-operative course was uneventful. A few inguinal hernia repairs require admission due to peculiarities such as extensive scrotal hernias requiring suction drainage. Muscular dystrophies are inherited disorders with no cure and no two dystrophy patients are exactly alike, therefore the health issues will be different for each individual. In case of surgery there is no definite recommendation for either general or regional anesthesia. This contribution regards the successful elective mesh repair with suction drainage of a large left-sided inguino-scrotal hernia in a 48 y. o. male patient affected by Becker muscular dystrophy by selective spinal anesthesia obtained by 10 milligrams of hyperbaric bupivacaine. Effective mesh repair with suction drainage of large inguinal hernias under spinal anesthesia can be achieved in patients affected by muscular dystrophy.

Derivation of new diagnostic reference levels for neuro-paediatric computed tomography examinations in Switzerland.

PubMed

Wagner, Franca; Bize, Julie; Racine, Damien; Le Coultre, Regis; Francis, Verdun; Trueb, Philipp; Treier, Reto

2018-05-22

Definition of new national diagnostic reference levels (DRLs) for volume computed tomography dose index (CTDIvol) and dose length product (DLP) for neuro-paediatric CT examinations depending on the medical indication. Methods: Paediatric cranial CT data sets acquired between January 2013 and December 2016 were retrospectively collected between July 2016 and March 2017 from the eight largest university and cantonal hospitals that perform most of the neuro-paediatric CTs in Switzerland. A consensus review of CTDIvol and DLP was undertaken for the three defined anatomical regions: brain, facial bone and petrous bone; each with and without contrast medium application. All indications for cranial CT imaging in paediatrics were assigned to one of these three regions. Descriptive statistical analysis of the distribution of the median values for CTDIvol and DLP yielded minimum, maximum, 25th percentile (1st quartile), median (2nd quartile) and 75th percentile (3rd quartile). New DRLs for neuro-paediatric CT examinations in Switzerland were based on the 75th percentiles of the distributions of the median values of all eight centres. Where appropriate, values were rounded such that the DRLs increase or at least remain constant as the age of the patient increases. Results: Our results revealed DRLs for CTDIvol and DLP up to 20% lower than the DRLs used so far in Switzerland and elsewhere in Europe. Conclusions: This study provides Swiss neuro-paediatric CT DRL values to establish optimum conditions for paediatric cranial CT examinations. Periodic national updates of DRLs, following international comparisons, are essential. . Creative Commons Attribution license.

Nanolipodendrosome-loaded glatiramer acetate and myogenic differentiation 1 as augmentation therapeutic strategy approaches in muscular dystrophy.

PubMed

Afzal, Ehsan; Zakeri, Saba; Keyhanvar, Peyman; Bagheri, Meisam; Mahjoubi, Parvin; Asadian, Mahtab; Omoomi, Nogol; Dehqanian, Mohammad; Ghalandarlaki, Negar; Darvishmohammadi, Tahmineh; Farjadian, Fatemeh; Golvajoee, Mohammad Sadegh; Afzal, Shadi; Ghaffari, Maryam; Cohan, Reza Ahangari; Gravand, Amin; Ardestani, Mehdi Shafiee

2013-01-01

[Corrected] Muscular dystrophies consist of a number of juvenile and adult forms of complex disorders which generally cause weakness or efficiency defects affecting skeletal muscles or, in some kinds, other types of tissues in all parts of the body are vastly affected. In previous studies, it was observed that along with muscular dystrophy, immune inflammation was caused by inflammatory cells invasion - like T lymphocyte markers (CD8+/CD4+). Inflammatory processes play a major part in muscular fibrosis in muscular dystrophy patients. Additionally, a significant decrease in amounts of two myogenic recovery factors (myogenic differentation 1 [MyoD] and myogenin) in animal models was observed. The drug glatiramer acetate causes anti-inflammatory cytokines to increase and T helper (Th) cells to induce, in an as yet unknown mechanism. MyoD recovery activity in muscular cells justifies using it alongside this drug. In this study, a nanolipodendrosome carrier as a drug delivery system was designed. The purpose of the system was to maximize the delivery and efficiency of the two drug factors, MyoD and myogenin, and introduce them as novel therapeutic agents in muscular dystrophy phenotypic mice. The generation of new muscular cells was analyzed in SW1 mice. Then, immune system changes and probable side effects after injecting the nanodrug formulations were investigated. The loaded lipodendrimer nanocarrier with the candidate drug, in comparison with the nandrolone control drug, caused a significant increase in muscular mass, a reduction in CD4+/CD8+ inflammation markers, and no significant toxicity was observed. The results support the hypothesis that the nanolipodendrimer containing the two candidate drugs will probably be an efficient means to ameliorate muscular degeneration, and warrants further investigation.

Can This Marriage Be Saved? The Future of "Neuro-Education"

ERIC Educational Resources Information Center

Schrag, Francis

2013-01-01

Neuro-education, a new frontier for educational researchers, has its passionate advocates and equally passionate detractors. Some philosophers, including Noel Purdy and Hugh Morrison, Andrew Davis, and Ralph Schumacher, have argued that the entire enterprise is misguided. I evaluate and challenge their arguments. This permits me to articulate my…

Advances in genetic therapeutic strategies for Duchenne muscular dystrophy.

PubMed

Guiraud, Simon; Chen, Huijia; Burns, David T; Davies, Kay E

2015-12-01

What is the topic of this review? This review highlights recent progress in genetically based therapies targeting the primary defect of Duchenne muscular dystrophy. What advances does it highlight? Over the last two decades, considerable progress has been made in understanding the mechanisms underlying Duchenne muscular dystrophy, leading to the development of genetic therapies. These include manipulation of the expression of the gene or related genes, the splicing of the gene and its translation, and replacement of the gene using viral approaches. Duchenne muscular dystrophy is a lethal X-linked disorder caused by mutations in the dystrophin gene. In the absence of the dystrophin protein, the link between the cytoskeleton and extracellular matrix is destroyed, and this severely compromises the strength, flexibility and stability of muscle fibres. The devastating consequence is progressive muscle wasting and premature death in Duchenne muscular dystrophy patients. There is currently no cure, and despite exhaustive palliative care, patients are restricted to a wheelchair by the age of 12 years and usually succumb to cardiac or respiratory complications in their late 20s. This review provides an update on the current genetically based therapies and clinical trials that target or compensate for the primary defect of this disease. These include dystrophin gene-replacement strategies, genetic modification techniques to restore dystrophin expression, and modulation of the dystrophin homologue, utrophin, as a surrogate to re-establish muscle function. © 2015 The Authors. Experimental Physiology published by John Wiley & Sons Ltd on behalf of The Physiological Society.

Influence of Manual Labor at Work on Muscular Fitness and Its Relationship With Work Performance.

PubMed

Ryan, Eric D; Thompson, Brennan J; Sobolewski, Eric J

2016-10-01

The present study examined the influence of workplace manual labor on measures of muscular fitness, with a secondary aim to investigate the relationship between muscular fitness and work performance in blue-collar (BC) workers. Leg extension isokinetic strength at slow and fast velocities, hamstring and hip-flexor flexibility, and low back muscular endurance were examined in young and older BC workers and white-collar (WC) controls, while work performance was examined in the BC cohort. There were no differences in muscular fitness variables between BC and WC groups; however, the older men had lower low back muscular endurance (-43.0%) and strength at slow (-9.4%) and fast (-12.7%) velocities. Work performance was associated with strength at fast velocities (r = 0.633) in the older BC workers. Leg strength may influence work performance, with higher velocities becoming more important in older workers.

Quality of life research in neuro-oncology: a quantitative comparison.

PubMed

Klein, Elizabeth; Altshuler, David; Hallock, Abhirami; Szerlip, Nicholas

2014-01-01

Our understanding of the biology of neuro-oncologic disease has improved vastly over time, however overall patient survival remains relatively poor. Our goal as clinicians, therefore, should be to ensure that the quality of life (QOL) in that survival time is optimized. Here we review neuro-oncology QOL abstracts presented at major oncology conferences and the published literature to make a quantitative comparison to other common cancer subtypes. First, all abstracts presented at major oncology meetings from 2008 to 2012 were reviewed and filtered to find those related to QOL in CNS, breast, lung, and prostate cancer. Next, a Medline search was performed to identify all QOL papers published from 2003 to 2012 for the same cancer subtypes. The results were compared as absolute values and percentages. The average percentage of CNS QOL-related abstracts presented at ASCO and ASTRO over the last 5 years was 4.9 %, compared to 6.4 % for breast, 4.4 % for lung, and 6.1 % for prostate. There is a significant difference in total percentage of QOL abstracts over the time period when comparing CNS to breast and prostate, but not lung (p < 0.05). The Medline search revealed an average of 25.2 publications per year for CNS cancer, compared to 146.2 for breast, 39.3 for lung, and 64.2 for prostate. When looking at trends over time, publications in CNS cancer have not been as prolific and have not increased as rapidly as publications in breast and lung cancer, indicating that QOL is underrepresented in neuro-oncology research. We need to improve this by standardizing QOL measures and including them in every outcome study.

Emerging genetic therapies to treat Duchenne muscular dystrophy

PubMed Central

Nelson, Stanley F.; Crosbie, Rachelle H.; Miceli, M. Carrie; Spencer, Melissa J.

2010-01-01

Purpose of review Duchenne muscular dystrophy is a progressive muscle degenerative disease caused by dystrophin mutations. The purpose of this review is to highlight two emerging therapies designed to repair the primary genetic defect, called `exon skipping' and `nonsense codon suppression'. Recent findings A drug, PTC124, was identified that suppresses nonsense codon translation termination. PTC124 can lead to restoration of some dystrophin expression in human Duchenne muscular dystrophy muscles with mutations resulting in premature stops. Two drugs developed for exon skipping, PRO051 and AVI-4658, result in the exclusion of exon 51 from mature mRNA. They can restore the translational reading frame to dystrophin transcripts from patients with a particular subset of dystrophin gene deletions and lead to some restoration of dystrophin expression in affected boys' muscle in vivo. Both approaches have concluded phase I trials with no serious adverse events. Summary These novel therapies that act to correct the primary genetic defect of dystrophin deficiency are among the first generation of therapies tailored to correct specific mutations in humans. Thus, they represent paradigm forming approaches to personalized medicine with the potential to lead to life changing treatment for those affected by Duchenne muscular dystrophy. PMID:19745732

Muscle MRI findings in facioscapulohumeral muscular dystrophy.

PubMed

Gerevini, Simonetta; Scarlato, Marina; Maggi, Lorenzo; Cava, Mariangela; Caliendo, Giandomenico; Pasanisi, Barbara; Falini, Andrea; Previtali, Stefano Carlo; Morandi, Lucia

2016-03-01

Facioscapulohumeral muscular dystrophy (FSHD) is characterized by extremely variable degrees of facial, scapular and lower limb muscle involvement. Clinical and genetic determination can be difficult, as molecular analysis is not always definitive, and other similar muscle disorders may have overlapping clinical manifestations. Whole-body muscle MRI examination for fat infiltration, atrophy and oedema was performed to identify specific patterns of muscle involvement in FSHD patients (30 subjects), and compared to a group of control patients (23) affected by other myopathies (NFSHD). In FSHD patients, we detected a specific pattern of muscle fatty replacement and atrophy, particularly in upper girdle muscles. The most frequently affected muscles, including paucisymptomatic and severely affected FSHD patients, were trapezius, teres major and serratus anterior. Moreover, asymmetric muscle involvement was significantly higher in FSHD as compared to NFSHD patients. In conclusion, muscle MRI is very sensitive for identifying a specific pattern of involvement in FSHD patients and in detecting selective muscle involvement of non-clinically testable muscles. Muscle MRI constitutes a reliable tool for differentiating FSHD from other muscular dystrophies to direct diagnostic molecular analysis, as well as to investigate FSHD natural history and follow-up of the disease. Muscle MRI identifies a specific pattern of muscle involvement in FSHD patients. Muscle MRI may predict FSHD in asymptomatic and severely affected patients. Muscle MRI of upper girdle better predicts FSHD. Muscle MRI may differentiate FSHD from other forms of muscular dystrophy. Muscle MRI may show the involvement of non-clinical testable muscles.

Correlations of frontal lip-line canting with craniofacial morphology and muscular activity.

PubMed

Cho, Jin-Hyoung; Kim, Eun-Jung; Kim, Byeong-Chae; Cho, Ki-Hyun; Lee, Ki-Heon; Hwang, Hyeon-Shik

2007-09-01

The purpose of this study was to investigate factors affecting lip-line canting by using musculoskeletal analyses. Fifty-six adults with lip-line canting were selected as subjects. They were divided into 3 groups according to the changes of lip line during smiling: increasing (group I), decreasing (group D), and minimal (group M). Lip-line canting at rest was correlated to craniofacial morphology and muscular activity: Regarding craniofacial morphology, various craniofacial measurements in lateral and frontal cephalograms were used, including inclination of the tongue blade placed across both first molars. The zygomaticus major was the focus of the measurement of muscular activity affecting lip-line canting, and its activity during smiling was evaluated by using a needle electrode. In group I, lip-line canting at rest showed a significant correlation with the right-left (R/L) difference of muscular activity, but no significant correlation with the measurements of craniofacial morphology. In group D, lip-line canting showed a positive correlation with the measurements of craniofacial morphology, such as the inclination of the tongue blade, and a negative correlation with the R/L difference of muscular activity. In group M, lip-line canting showed no significant correlation with the R/L difference of muscular activity, but a significant correlation with inclination of the tongue blade. The results indicate that lip-line canting is caused by craniofacial morphology when the change of lip-line canting during smiling is minimal, whereas lip-line canting is affected by the R/L difference of muscular activity in addition to craniofacial morphology when the cant of lip line markedly changes during smiling. The findings suggest that the cause of lip-line canting can be identified easily by the change of canting during smiling, without complicated musculoskeletal analyses.

Fat embolism after fractures in Duchenne muscular dystrophy: an underdiagnosed complication? A systematic review.

PubMed

Feder, David; Koch, Miriam Eva; Palmieri, Beniamino; Fonseca, Fernando Luiz Affonso; Carvalho, Alzira Alves de Siqueira

2017-01-01

Duchenne muscular dystrophy is the most frequent lethal genetic disease. Several clinical trials have established both the beneficial effect of steroids in Duchenne muscular dystrophy and the well-known risk of side effects associated with their daily use. For many years it has been known that steroids associated with ambulation loss lead to obesity and also damage the bone structure resulting in the bone density reduction and increased incidence of bone fractures and fat embolism syndrome, an underdiagnosed complication after fractures. Fat embolism syndrome is characterized by consciousness disturbance, respiratory failure and skin rashes. The use of steroids in Duchenne muscular dystrophy may result in vertebral fractures, even without previous trauma. Approximately 25% of patients with Duchenne muscular dystrophy have a long bone fracture, and 1% to 22% of fractures have a chance to develop fat embolism syndrome. As the patients with Duchenne muscular dystrophy have progressive cardiac and respiratory muscle dysfunction, the fat embolism may be unnoticed clinically and may result in increased risk of death and major complications. Different treatments and prevention measures of fat embolism have been proposed; however, so far, there is no efficient therapy. The prevention, early diagnosis and adequate symptomatic treatment are of paramount importance. The fat embolism syndrome should always be considered in patients with Duchenne muscular dystrophy presenting with fractures, or an unexplained and sudden worsening of respiratory and cardiac symptoms.

Fat embolism after fractures in Duchenne muscular dystrophy: an underdiagnosed complication? A systematic review

PubMed Central

Feder, David; Koch, Miriam Eva; Palmieri, Beniamino; Fonseca, Fernando Luiz Affonso; Carvalho, Alzira Alves de Siqueira

2017-01-01

Duchenne muscular dystrophy is the most frequent lethal genetic disease. Several clinical trials have established both the beneficial effect of steroids in Duchenne muscular dystrophy and the well-known risk of side effects associated with their daily use. For many years it has been known that steroids associated with ambulation loss lead to obesity and also damage the bone structure resulting in the bone density reduction and increased incidence of bone fractures and fat embolism syndrome, an underdiagnosed complication after fractures. Fat embolism syndrome is characterized by consciousness disturbance, respiratory failure and skin rashes. The use of steroids in Duchenne muscular dystrophy may result in vertebral fractures, even without previous trauma. Approximately 25% of patients with Duchenne muscular dystrophy have a long bone fracture, and 1% to 22% of fractures have a chance to develop fat embolism syndrome. As the patients with Duchenne muscular dystrophy have progressive cardiac and respiratory muscle dysfunction, the fat embolism may be unnoticed clinically and may result in increased risk of death and major complications. Different treatments and prevention measures of fat embolism have been proposed; however, so far, there is no efficient therapy. The prevention, early diagnosis and adequate symptomatic treatment are of paramount importance. The fat embolism syndrome should always be considered in patients with Duchenne muscular dystrophy presenting with fractures, or an unexplained and sudden worsening of respiratory and cardiac symptoms. PMID:29066903

Theories and control models and motor learning: clinical applications in neuro-rehabilitation.

PubMed

Cano-de-la-Cuerda, R; Molero-Sánchez, A; Carratalá-Tejada, M; Alguacil-Diego, I M; Molina-Rueda, F; Miangolarra-Page, J C; Torricelli, D

2015-01-01

In recent decades there has been a special interest in theories that could explain the regulation of motor control, and their applications. These theories are often based on models of brain function, philosophically reflecting different criteria on how movement is controlled by the brain, each being emphasised in different neural components of the movement. The concept of motor learning, regarded as the set of internal processes associated with practice and experience that produce relatively permanent changes in the ability to produce motor activities through a specific skill, is also relevant in the context of neuroscience. Thus, both motor control and learning are seen as key fields of study for health professionals in the field of neuro-rehabilitation. The major theories of motor control are described, which include, motor programming theory, systems theory, the theory of dynamic action, and the theory of parallel distributed processing, as well as the factors that influence motor learning and its applications in neuro-rehabilitation. At present there is no consensus on which theory or model defines the regulations to explain motor control. Theories of motor learning should be the basis for motor rehabilitation. The new research should apply the knowledge generated in the fields of control and motor learning in neuro-rehabilitation. Copyright © 2011 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

Berberine attenuates depressive-like behaviors by suppressing neuro-inflammation in stressed mice.

PubMed

Liu, Ya-Min; Niu, Le; Wang, Lin-Lin; Bai, Li; Fang, Xiao-Yan; Li, Yu-Cheng; Yi, Li-Tao

2017-09-01

Berberine, the major constituent alkaloid originally from the famous Chinese herb Huanglian (Coptis chinensis), has been shown to exert antidepressant-like effects in rodents. However, it is still not clear the involvement of neuro-inflammation suppression in the effects of berberine. The purpose of this study was to determine whether berberine affects the neuro-inflammation system in mice induced by chronic unpredictable mild stress (CUMS). Berberine was orally administrated in normal or CUMS mice for successive four weeks. Behavioral evaluation showed that berberine prevented the depressive deficits both in sucrose preference test and novelty-suppressed feeding test. The elevation of hippocampal pro-inflammatory cytokines such as interleukin-1β (IL-1β), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α), as well as the activation of microglia were decreased by berberine. In addition, chronic berberine treatment inhibited nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB) signaling pathway as the phosphorylated proteins of NF-κB, IκB kinase (IKK)α and IKKβ in the hippocampus were suppressed after berberine administration. Furthermore, inducible nitric oxide synthase (iNOS), one downstream target of NF-κB signaling pathway was also inhibited by berberine. In conclusion, these findings suggest that administration of berberine could prevent depressive-like behaviors in CUMS mice by suppressing neuro-inflammation in the hippocampus. Copyright © 2017 Elsevier Inc. All rights reserved.

Impact of provider level, training and gender on the utilization of palliative care and hospice in neuro-oncology: a North-American survey.

PubMed

Walbert, Tobias; Glantz, Michael; Schultz, Lonni; Puduvalli, Vinay K

2016-01-01

Specialized palliative care (PC) services have emerged to address symptoms and provide end-of-life management for patients with brain tumors. The utilization patterns of PC in neuro-oncology are unknown. A 22-question survey was distributed to participants of the society for neuro-oncology annual meeting 2012 (n = 4487). Nonparametric methods including Wilcoxon two-sample and Kruskal-Wallis tests were used to assess differences in responses. 239 (5.3 %) evaluable responses were received; 79 % of respondents were physicians, and 17 % were nurses or midlevel providers. Forty-seven percent were medical or neuro-oncologists, 31 % neurosurgeons and 11 % radiation oncologists. Forty percent had no formal training in PC, 57 % had some formal training and 3 % completed a PC fellowship. Seventy-nine percent practiced in an academic setting. Of the respondents, 57 % referred patients to PC when symptoms required treatment and 18 % at end of life. Only 51 % of all providers felt comfortable dealing with end-of-life issues and symptoms, while 33 % did not. Fifty-one percent preferred a service named "Supportive Care" rather than "Palliative Care" (MDs > midlevel providers, p < 0.001), and 32 % felt that patient expectations for ongoing therapy hindered their ability to make PC referrals. Female gender, formal training in neuro-oncology and PC, and medical versus surgical neuro-oncology training were significantly associated with hospice referral, comfort in dealing with end-of-life issues, and ease of access to PC services. Provider level, specialty, gender, training in PC and neuro-oncology have significant impact on the utilization of PC and hospice in neuro-oncology.

Muscularity as a function of species, sex and age in small mammals

NASA Technical Reports Server (NTRS)

Pace, N.; Rahlmann, D. F.; Smith, A. H.

1984-01-01

Changes in the body skeletal muscle mass SMM (measured as a function of the ratio between the body creatine mass and the fat-free muscle creatine), and in muscularity (expressed as the ratio of SMM to fat-free body mass) were studied as functions of age, sex, and species in mouse, rat, hamster, guinea pig, and rabbit. Six animals of each sex were examined in eight age cohorts ranging from 1 to 24 months. Both species and age factors affect SMM. Strong sexual dimorphism in the SMM changes with age was displayed by mouse, rat, and guinea pig, whereas the hamster and rabbit were statistically monomorphic. The mouse, rat, and hamster attain a maximal SMM at about 1 year of age, whereas in the guinea pig and rabbit the decrease in SMM starts after 2 years. The value of muscularity reached a peak at age of 2-3 months in all animals of both sexes, with a pronounced difference among the species. The mouse emerged as the most muscular, while the guinea pig the least muscular, of all species.

Cognitive and Neurobehavioral Profile in Boys With Duchenne Muscular Dystrophy.

PubMed

Banihani, Rudaina; Smile, Sharon; Yoon, Grace; Dupuis, Annie; Mosleh, Maureen; Snider, Andrea; McAdam, Laura

2015-10-01

Duchenne muscular dystrophy is a progressive neuromuscular condition that has a high rate of cognitive and learning disabilities as well as neurobehavioral disorders, some of which have been associated with disruption of dystrophin isoforms. Retrospective cohort of 59 boys investigated the cognitive and neurobehavioral profile of boys with Duchenne muscular dystrophy. Full-scale IQ of < 70 was seen in 27%; learning disability in 44%, intellectual disability in 19%; attention-deficit/hyperactivity disorder in 32%; autism spectrum disorders in 15%; and anxiety in 27%. Mutations affecting Dp260 isoform and 5'untranslated region of Dp140 were observed in 60% with learning disability, 50% intellectual disability, 77% with autism spectrum disorders, and 94% with anxiety. No statistically significant correlation was noted between comorbidities and dystrophin isoforms; however, there is a trend of cumulative loss of dystrophin isoforms with declining full-scale IQ. Enhanced psychology testing to include both cognitive and neurobehavioral disorders is recommended for all individuals with Duchenne muscular dystrophy. © The Author(s) 2015.

Secondary Conditions Among Males With Duchenne or Becker Muscular Dystrophy.

PubMed

Latimer, Rebecca; Street, Natalie; Conway, Kristin Caspers; James, Kathy; Cunniff, Christopher; Oleszek, Joyce; Fox, Deborah; Ciafaloni, Emma; Westfield, Christina; Paramsothy, Pangaja

2017-06-01

Duchenne and Becker muscular dystrophy are X-linked neuromuscular disorders characterized by progressive muscle degeneration. Despite the involvement of multiple systems, secondary conditions among affected males have not been comprehensively described. Two hundred nine caregivers of affected males (aged 3-31 years) identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network completed a mailed survey that included questions about secondary conditions impacting multiple body functions. The 5 most commonly reported conditions in males with Duchenne were cognitive deficits (38.4%), constipation (31.7%), anxiety (29.3%), depression (27.4%), and obesity (19.5%). Higher frequencies of anxiety, depression, and kidney stones were found among nonambulatory males compared to ambulatory males. Attention-deficit hyperactivity disorder (ADHD) was more common in ambulatory than nonambulatory males. These data support clinical care recommendations for monitoring of patients with Duchenne or Becker muscular dystrophy by a multidisciplinary team to prevent and treat conditions that may be secondary to the diagnosis.

Secondary conditions among males with Duchenne or Becker muscular dystrophy

PubMed Central

Latimer, Rebecca; Street, Natalie; Conway, Kristin Caspers; James, Kathy; Cunniff, Christopher; Oleszek, Joyce; Fox, Deborah; Ciafaloni, Emma; Westfield, Christina; Paramsothy, Pangaja

2017-01-01

Duchenne and Becker muscular dystrophy are X-linked neuromuscular disorders characterized by progressive muscle degeneration. Despite the involvement of multiple systems, secondary conditions among affected males have not been comprehensively described. Two hundred and nine caregivers of affected males (aged 3–31 years) identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network completed a mailed survey that included questions about secondary conditions impacting multiple body functions. The five most commonly reported conditions in males with Duchenne were cognitive deficits (38.4%), constipation (31.7%), anxiety (29.3%), depression (27.4%), and obesity (19.5%). Higher frequencies of anxiety, depression, and kidney stones, were found among non-ambulatory males compared to ambulatory males. Attention deficit hyperactivity disorder was more common in ambulatory than non-ambulatory males. These data support clinical care recommendations for monitoring of patients with Duchenne or Becker muscular dystrophy by a multidisciplinary team to prevent and treat conditions that may be secondary to the diagnosis. PMID:28393671

Drive for muscularity and social physique anxiety mediate the perceived ideal physique muscle dysmorphia relationship.

PubMed

Thomas, Adam; Tod, David A; Edwards, Christian J; McGuigan, Michael R

2014-12-01

This study examined the mediating role of drive for muscularity and social physique anxiety (SPA) in the perceived muscular male ideal physique and muscle dysmorphia relationship in weight training men. Men (N = 146, mean ± SD; age, 22.8 ± 5.0 years; weight, 82.0 ± 11.1 kg; height, 1.80 ± 0.07 m; body mass index, 25.1 ± 3.0) who participated in weight training completed validated questionnaires measuring drive for muscularity, SPA, perceived muscular male ideal physique, global muscle dysmorphia, and several characteristics of muscle dysmorphia (exercise dependence, diet manipulation, concerns about size/symmetry, physique protection behavior, and supplementation). Perceived ideal physique was an independent predictor of muscle dysmorphia measures except physique protection (coefficients = 0.113-0.149, p ≤ 0.05). Perceived ideal physique also predicted muscle dysmorphia characteristics (except physique protection and diet) through the indirect drive for muscularity pathway (coefficients = 0.055-0.116, p ≤ 0.05). Perceived ideal physique also predicted size/symmetry concerns and physique protection through the indirect drive for muscularity and SPA pathway (coefficients = 0.080-0.025, p ≤ 0.05). These results extend current research by providing insights into the way correlates of muscle dysmorphia interact to predict the condition. The results also highlight signs (e.g., anxiety about muscularity) that strength and conditioning coaches can use to identify at-risk people who may benefit from being referred for psychological assistance.

Decreased Insulin Receptors but Normal Glucose Metabolism in Duchenne Muscular Dystrophy

NASA Astrophysics Data System (ADS)

de Pirro, Roberto; Lauro, Renato; Testa, Ivano; Ferretti, Ginofabrizio; de Martinis, Carlo; Dellantonio, Renzo

1982-04-01

Compared to matched controls, 17 patients with Duchenne muscular dystrophy showed decreased insulin binding to monocytes due to decreased receptor concentration. These patients showed no signs of altered glucose metabolism and retrospective analysis of the clinical records of a further 56 such patients revealed no modification in carbohydrate metabolism. These data suggest that reduced insulin receptor number does not produce overt modifications of glucose metabolism in Duchenne muscular dystrophy.

Nanolipodendrosome-loaded glatiramer acetate and myogenic differentiation 1 as augmentation therapeutic strategy approaches in muscular dystrophy

PubMed Central

Afzal, Ehsan; Zakeri, Saba; Keyhanvar, Peyman; Bagheri, Meisam; Mahjoubi, Parvin; Asadian, Mahtab; Omoomi, Nogol; Dehqanian, Mohammad; Ghalandarlaki, Negar; Darvishmohammadi, Tahmineh; Farjadian, Fatemeh; Golvajoee, Mohammad Sadegh; Afzal, Shadi; Ghaffari, Maryam; Cohan, Reza Ahangari; Gravand, Amin; Ardestani, Mehdi Shafiee

2013-01-01

Backgrond Muscular dystrophies consist of a number of juvenile and adult forms of complex disorders which generally cause weakness or efficiency defects affecting skeletal muscles or, in some kinds, other types of tissues in all parts of the body are vastly affected. In previous studies, it was observed that along with muscular dystrophy, immune inflammation was caused by inflammatory cells invasion – like T lymphocyte markers (CD8+/CD4+). Inflammatory processes play a major part in muscular fibrosis in muscular dystrophy patients. Additionally, a significant decrease in amounts of two myogenic recovery factors (myogenic differentation 1 [MyoD] and myogenin) in animal models was observed. The drug glatiramer acetate causes anti-inflammatory cytokines to increase and T helper (Th) cells to induce, in an as yet unknown mechanism. MyoD recovery activity in muscular cells justifies using it alongside this drug. Methods In this study, a nanolipodendrosome carrier as a drug delivery system was designed. The purpose of the system was to maximize the delivery and efficiency of the two drug factors, MyoD and myogenin, and introduce them as novel therapeutic agents in muscular dystrophy phenotypic mice. The generation of new muscular cells was analyzed in SW1 mice. Then, immune system changes and probable side effects after injecting the nanodrug formulations were investigated. Results The loaded lipodendrimer nanocarrier with the candidate drug, in comparison with the nandrolone control drug, caused a significant increase in muscular mass, a reduction in CD4+/CD8+ inflammation markers, and no significant toxicity was observed. The results support the hypothesis that the nanolipodendrimer containing the two candidate drugs will probably be an efficient means to ameliorate muscular degeneration, and warrants further investigation. PMID:23966782

Venous pump of the calf: a study of venous and muscular pressures.

PubMed

Alimi, Y S; Barthelemy, P; Juhan, C

1994-11-01

Little data are available concerning the relation between the muscular pumping mechanism and the variation of superficial and deep venous pressure during normal action of the calf pump; therefore we undertook this study to determine the pressure values in three compartments of the calf and in the deep and the superficial venous system and to establish correlation between muscular and venous pressure. Nine healthy young women with a mean age of 23 years (range 19 to 28 years) were examined. In the same calf, a muscular catheter was placed in the deep posterior compartment (DPC), in the superficial posterior compartment (SPC), and in the anterior tibial compartment (ATC), and a vascular catheter was placed in the popliteal vein and in the greater saphenous vein (GSV). The five lines of pressure were simultaneously recorded in the following situations: at rest, during Valsalva maneuver, foot flexion, and foot extension. The situation was studied with the patient in the following positions: decubitus, sitting, standing, and squatting. A final continuous recording was carried out after the patient had been walking for 5 minutes. Mean values with standard errors of muscular and venous pressure were established in each situation. At rest and during Valsalva maneuver, the muscular pressures did not vary, whereas venous pressures increased significantly when the patient was sitting and standing. On the other hand, squatting was associated with a rise in the muscular and vein pressures. Foot flexion entailed a significant increase in the ATC pressure and a rise in the GSV pressure, whereas foot extension caused the DPC pressure to rise without venous pressure modifications. Walking was associated with an alternating increase in the DPC, SPC, GSV and popliteal vein pressures when the foot was compressed to floor followed by a significant decrease when the foot pressure was released. The variations in the deep and superficial venous pressures when the patient is sitting and

Cardiomyopathy in becker muscular dystrophy: Overview.

PubMed

Ho, Rady; Nguyen, My-Le; Mather, Paul

2016-06-26

Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. Cardiac involvement in BMD has been described and cardiomyopathy represents the number one cause of death in these patients. In this paper, the pathophysiology, clinical evaluations and management of cardiomyopathy in patients with BMD will be discussed.

Complementary and alternative medicine for Duchenne and Becker muscular dystrophies: characteristics of users and caregivers.

PubMed

Zhu, Yong; Romitti, Paul A; Conway, Kristin M; Andrews, Jennifer; Liu, Ke; Meaney, F John; Street, Natalie; Puzhankara, Soman; Druschel, Charlotte M; Matthews, Dennis J

2014-07-01

Complementary and alternative medicine is frequently used in the management of chronic pediatric diseases, but little is known about its use by those with Duchenne or Becker muscular dystrophy. Complementary and alternative medicine use by male patients with Duchenne or Becker muscular dystrophy and associations with characteristics of male patients and their caregivers were examined through interviews with 362 primary caregivers identified from the Muscular Dystrophy Surveillance, Tracking, and Research Network. Overall, 272 of the 362 (75.1%) primary caregivers reported that they had used any complementary and alternative medicine for the oldest Muscular Dystrophy Surveillance, Tracking, and Research Network male in their family. The most commonly reported therapies were from the mind-body medicine domain (61.0%) followed by those from the biologically based practice (39.2%), manipulative and body-based practice (29.3%), and whole medical system (6.9%) domains. Aquatherapy, prayer and/or blessing, special diet, and massage were the most frequently used therapies. Compared with nonusers, male patients who used any therapy were more likely to have an early onset of symptoms and use a wheel chair; their caregivers were more likely to be non-Hispanic white. Among domains, associations were observed with caregiver education and family income (mind-body medicines [excluding prayer and/or blessing only] and whole medical systems) and Muscular Dystrophy Surveillance, Tracking, and Research Network site (biologically based practices and mind-body medicines [excluding prayer and/or blessing only]). Complementary and alternative medicine use was common in the management of Duchenne and Becker muscular dystrophies among Muscular Dystrophy Surveillance, Tracking, and Research Network males. This widespread use suggests further study to evaluate the efficacy of integrating complementary and alternative medicine into treatment regimens for Duchenne and Becker muscular

Genetics Home Reference: limb-girdle muscular dystrophy

MedlinePlus

... age of onset, and features of limb-girdle muscle dystrophy vary among the many subtypes of this condition ... occurs in some people with limb-girdle muscular dystrophy . Weakening of the heart muscle (cardiomyopathy) occurs in some forms of limb-girdle ...

Estimating Reservoir Inflow Using RADAR Forecasted Precipitation and Adaptive Neuro Fuzzy Inference System

NASA Astrophysics Data System (ADS)

Yi, J.; Choi, C.

2014-12-01

Rainfall observation and forecasting using remote sensing such as RADAR(Radio Detection and Ranging) and satellite images are widely used to delineate the increased damage by rapid weather changeslike regional storm and flash flood. The flood runoff was calculated by using adaptive neuro-fuzzy inference system, the data driven models and MAPLE(McGill Algorithm for Precipitation Nowcasting by Lagrangian Extrapolation) forecasted precipitation data as the input variables.The result of flood estimation method using neuro-fuzzy technique and RADAR forecasted precipitation data was evaluated by comparing it with the actual data.The Adaptive Neuro Fuzzy method was applied to the Chungju Reservoir basin in Korea. The six rainfall events during the flood seasons in 2010 and 2011 were used for the input data.The reservoir inflow estimation results were comparedaccording to the rainfall data used for training, checking and testing data in the model setup process. The results of the 15 models with the combination of the input variables were compared and analyzed. Using the relatively larger clustering radius and the biggest flood ever happened for training data showed the better flood estimation in this study.The model using the MAPLE forecasted precipitation data showed better result for inflow estimation in the Chungju Reservoir.

Preliminary Thermal Characterization of a Fully-Passive Wireless Backscattering Neuro-Recording Microsystem

NASA Technical Reports Server (NTRS)

Schwerdt, H. N.; Xu, W.; Shekhar, S.; Chae, J.; Miranda, F. A.

2011-01-01

We present analytical and experimental thermal characteristics of a battery-less, fully-passive wireless backscattering microsystem for recording of neuropotentials. A major challenge for cortically implantable microsystems involves minimizing the heat dissipated by on-chip circuitry, which can lead to permanent brain damage. Therefore, knowledge of temperature changes induced by implantable microsystems while in operation is of utmost importance. In this work, a discrete diode appended to the neuro-recording microsystem has been used to indirectly monitor the aforesaid temperature changes. Using this technique, the maximum temperature rise measured for the microsystem while in operation was 0.15 +/- 0.1 C, which is significantly less than current safety guidelines. Specific absorption ratio (SAR) due to the microsystem was also computed to further demonstrate fully-passive functionality of the neuro-recording microsystem.

The role of morphine on rat neural stem cells viability, neuro-angiogenesis and neuro-steroidgenesis properties.

PubMed

Abdyazdani, Nima; Nourazarian, Alireza; Nozad Charoudeh, Hojjatollah; Kazemi, Masoumeh; Feizy, Navid; Akbarzade, Maryam; Mehdizadeh, Amir; Rezaie, Jafar; Rahbarghazi, Reza

2017-01-01

A lack of comprehensive data exists on the effect of morphine on neural stem cell neuro-steroidogenesis and neuro-angiogenesis properties. We, herein, investigated the effects of morphine (100μM), naloxone (100μM) and their combination on rat neural stem cells viability, clonogenicity and Ki-67 expression over a period of 72h. Any alterations in the total fatty acids profile under treatment protocols were elucidated by direct transesterification method. We also monitored the expression of p53, aromatase and 5-alpha reductase by real-time PCR assay. To examine angiogenic capacity, in vitro tubulogenesis and the level of VE-cadherin transcript were investigated during neural to endothelial differentiation under the experimental procedure. Cells supplemented with morphine displayed reduced survival (p<0.01) and clonogenicity (p<0.001). Flow cytometric analysis showed a decrease in Ki-67 during 72h. Naloxone potentially blunted morphine-induced all effects. The normal levels of fatty acids, including saturated and unsaturated were altered by naloxone and morphine supplements. Following 48h, the up-regulation of p53, aromatase and 5-alpha reductase genes occurred in morphine-primed cells. Using three-dimensional culture models of angiogenesis and real time PCR assay, we showed morphine impaired the tubulogenesis properties of neural stem cells (p<0.001) by the inhibition of trans-differentiation into vascular cells and led to decrease of in VE-cadherin expression. Collectively, morphine strongly impaired the healthy status of neural stem cells by inducing p53 and concurrent elevation of aromatase and 5-alpha reductase activities especially during early 48h. Also, neural stem cells-being exposed to morphine lost their potency to elicit angiogenesis. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

NeuroMind: Past, present, and future

PubMed Central

Kubben, Pieter L.

2017-01-01

This narrative report describes the underlying rationale and technical developments of NeuroMind, a mobile clinical decision support system for neurosurgery. From the perspective of a neurosurgeon – (app) developer it explains how technical progress has shaped the world's “most rated and highest rated” neurosurgical mobile application, with particular attention for operating system diversity on mobile hardware, cookbook medicine, regulatory affairs (in particular regarding software as a medical device), and new developments in the field of clinical data science, machine learning, and predictive analytics. Finally, the concept of “computational neurosurgery” is introduced as a vehicle to reach new horizons in neurosurgery. PMID:28966822

Neuro-Linguistic Programming as an Innovation in Education and Teaching

ERIC Educational Resources Information Center

Tosey, Paul; Mathison, Jane

2010-01-01

Neuro-linguistic programming (NLP)--an emergent, contested approach to communication and personal development created in the 1970s--has become increasingly familiar in education and teaching. There is little academic work on NLP to date. This article offers an informed introduction to, and appraisal of, the field for educators. We review the…

Drive for muscularity and drive for thinness: the impact of pro-anorexia websites.

PubMed

Juarez, Lilia; Soto, Ernesto; Pritchard, Mary E

2012-01-01

In recent years, websites that stress the message of thinness as the ideal and only choice have surfaced on the internet. The possibility that pro-anorexia websites may reinforce restrictive eating and exercise behaviors is an area of concern. In addition, friends may be influencing one another to view these websites, further contributing to drive for thinness in women and drive for muscularity in men. Three hundred male and female undergraduate psychology students responded to questionnaires assessing: internalization of pro-anorexia website content, internalization of general media content, influence of friends to view pro-anorexia websites, peer influence, drive for muscularity, and drive for thinness. Results showed internalization of pro-anorexia website content was positively correlated with drive for thinness in women, and negatively correlated with drive for muscularity in men. Internalization of pro-anorexia website content was found to be related to both drive for thinness in women and drive for muscularity in men.

The effects of exposure to slender and muscular images on male body dissatisfaction.

PubMed

Galioto, Rachel; Crowther, Janis H

2013-09-01

This research examined the effects of appearance-based comparisons to muscular and slender idealized male bodies and the contribution of internalization and social comparison to change in body dissatisfaction. Participants were 111 male undergraduates who completed measures of body dissatisfaction, internalization, and social comparison and viewed images of either muscular or slender men in advertisements or product-only advertisements. Results indicated that exposure to both muscular and slender images was associated with an increase in body dissatisfaction, with no significant differences in the change in body dissatisfaction between the two image conditions. Internalization and trait social comparison were each associated with an increase in body dissatisfaction; however, upward social comparison was only a significant predictor of a change in body dissatisfaction for the males who viewed muscular images. These results highlight the impact of slender models on young men's body dissatisfaction and support the examination of media literacy interventions with this population. Copyright © 2013 Elsevier Ltd. All rights reserved.

Sparks, signals and shock absorbers: how dystrophin loss causes muscular dystrophy.

PubMed

Batchelor, Clare L; Winder, Steve J

2006-04-01

The dystrophin-glycoprotein complex (DGC) can be considered as a specialized adhesion complex, linking the extracellular matrix to the actin cytoskeleton, primarily in muscle cells. Mutations in several components of the DGC lead to its partial or total loss, resulting in various forms of muscular dystrophy. These typically manifest as progressive wasting diseases with loss of muscle integrity. Debate is ongoing about the precise function of the DGC: initially a strictly mechanical role was proposed but it has been suggested that there is aberrant calcium handling in muscular dystrophy and, more recently, changes in MAP kinase and GTPase signalling have been implicated in the aetiology of the disease. Here, we discuss new and interesting developments in these aspects of DGC function and attempt to rationalize the mechanical, calcium and signalling hypotheses to provide a unifying hypothesis of the underlying process of muscular dystrophy.

Late-onset Becker-type muscular dystrophy in a Border terrier dog.

PubMed

Jeandel, A; Garosi, L S; Davies, L; Guo, L T; Salgüero, R; Shelton, G D

2018-01-29

A 9-year-old Border terrier was presented to a referral hospital after a 1-year history of progressive stiffness and exercise intolerance. Neurological examination was consistent with a neuromuscular disorder. Serum creatine kinase activity was mildly elevated. A myopathy was suspected based on MRI findings and electrophysiological examination. Muscle histopathology was consistent with a severe non-inflammatory myopathy of a dystrophic type. Immunofluorescence and western blotting confirmed a dystrophinopathy with an 80-kDa truncated dystrophin fragment similar to Becker muscular dystrophy in people. To our knowledge, this is the first description of a late-onset Becker-type muscular dystrophy in a dog, and the first description of a dystrophinopathy in a Border terrier. Muscular dystrophy in dogs should not be ruled out based on late onset clinical signs and only mildly elevated creatine kinase. © 2018 British Small Animal Veterinary Association.

Introduction to the Therapeutic Drawing Series

ERIC Educational Resources Information Center

Del Giacco, Maureen

2011-01-01

The primary purpose of Del Giacco's Neuro-Art Therapy is to help the client regenerate the sensory system at a decoding/encoding (for our purposes we use the two words interchangeably) levels in the brain while using developmental visual spatial exercises or the Therapeutic Drawing Series (TDS). The specialty of Del Giacco Neuro Art Therapy (DAT)…

Nose muscular dynamics: the tip trigonum.

PubMed

Figallo, E E; Acosta, J A

2001-10-01

In 1995, the senior author (E.E.F.) published an article in which he described the musculus digastricus septi nasi labialis. In the article presented here, work carried out by anatomists and other researchers who, over the last two centuries, studied nose muscular dynamics is described. The present study is based on Gray's Anatomy, which, in 1858, first described the nasal tip muscles, along with the other nasal muscles. Later works not only used different terminology for these muscles but also ignored some, creating tremendous confusion. The study presented here provides an update of the exact terms, location, insertions, and muscle functions of the muscles of the nose. Each nose muscle is described with regard to the two portions able to produce separate contractions. In this study, the term "dual function" is used and characterizes the nasal mimetic muscles that do not have well-defined fascia. Therefore, there is doubt about the existence of a real nasal superficial muscle aponeurotic system. The musculus myrtiformis seems to have a dual function, inserting in the canine fosse and in the periosteum of the central incisors, forming two portions-one to the septum and the other to the nostril-each of which has specific functions. This study has been based on research in physiognomy, the science of expression. With regard to the basis for nose expressions, common anatomical research is excluded because it provides a different view of the dynamics studied to date. The term trigonum musculare apicis nasi defines the interaction of the musculi compressor narium minor and dilator naris anterior, connecting with the columellar bundle of the musculus digastricus and levering the nasal spine. This muscular trigone creates circular concentric and eccentric movements of the nasal tip.

Physical Activity in Boys With Duchenne Muscular Dystrophy Is Lower and Less Demanding Compared to Healthy Boys.

PubMed

Heutinck, Lotte; Kampen, Nadine van; Jansen, Merel; Groot, Imelda J M de

2017-04-01

This study describes the amount of physical activity and perception of physical activity in boys with Duchenne muscular dystrophy (DMD) compared to healthy boys. A questionnaire described 6 domains of physical activity. Four Duchenne muscular dystrophy subgroups were made: early and late ambulatory, nonambulatory with relative good, or limited arm function. Eighty-four boys with Duchenne muscular dystrophy (15.0 ± 6.4 years) and 198 healthy boys (14.0 ± 4.3 years) participated. Daily activities were more passive for boys with Duchenne muscular dystrophy. Physical activity was less and low demanding compared to healthy boys. It decreased with disease severity ( P < .05), whereas screen time increased ( P < .05). Benefits of physical activity in boys with Duchenne muscular dystrophy were having fun and making friends. Barriers were lack of sport facilities and insufficient health. This study helps to quantify poor engagement in physical activity by boys with Duchenne muscular dystrophy, and demonstrates factors that contribute to it. Suggestions to stimulate physical activity are made.

Muscular pattern in three species of Macrostomum (platyhelminthes, macrostomorpha).

PubMed

Adami, Mariana L; Brusa, Francisco; Ronderos, Jorge R; Damborenea, Cristina

2017-02-01

Previous studies demonstrated complex architecture of the muscular system of Macrostomum species, especially in the rostrum area and the pharynx. However, little is known about the differences in muscular pattern between species of the genus. This study examines and compares the muscular systems of specimens belonging to three freshwater Macrostomum species (M. quiritium, M. tuba and M. velastylum), labeled with phalloidin-rhodamine and studied by confocal microscopy. Our results agree with the previous descriptions, confirming that the muscular patterns for the body wall, rostrum area, pharynx and caudal region differ among species. The muscles of the body wall follow the typical architecture, but the number of fibers in the species analyzed varies between dorsal and ventral surfaces, ranging from 80 to 100 fibers, this record being higher than previous observations. The arrangement of the fibers in the rostrum is complex, especially in the brain area. Macrostomum tuba and M. quiritium have a set of two muscles crossing at brain level and forming an "X," which is not evident in M. velastylum. We identified five different sets of fibers associated to the pharynx and mouth at ventral, medium and deep levels. These different sets are present in all three species studied. The caudal plate in M. tuba has an additional layer of diagonal fibers in the body wall, which is not evident in the other two species. The muscles of the reproductive system are independent of the body wall musculature in the species analyzed, but connected to the intestinal wall by specific fibers that may serve as an anchor. J. Morphol. 278:264-282, 2017. © 2016 Wiley Periodicals,Inc. © 2016 Wiley Periodicals, Inc.

Cardiomyopathy in becker muscular dystrophy: Overview

PubMed Central

Ho, Rady; Nguyen, My-Le; Mather, Paul

2016-01-01

Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. Cardiac involvement in BMD has been described and cardiomyopathy represents the number one cause of death in these patients. In this paper, the pathophysiology, clinical evaluations and management of cardiomyopathy in patients with BMD will be discussed. PMID:27354892

Translational Studies of GALGT2 Gene Therapy for Duchenne Muscular Dystrophy

DTIC Science & Technology

2013-10-01

muscles once the experimental problems related to expression are solved. Figure 2. Percentage change in specific force after GALGT2 treatment of wild...cytotoxic T cell GalNAc transferase in skeletal muscle inhibits muscular dystrophy in mdx mice. Proc Natl Acad Sci U S A 99, 5616-5621 (2002). 3. Xu, R...Camboni, M. & Martin, P.T. Postnatal overexpression of the CT GalNAc transferase inhibits muscular dystrophy in mdx mice without altering muscle growth

Immediate Effect of Alcohol on Voice Tremor Parameters and Speech Motor Control

ERIC Educational Resources Information Center

Krishnan, Gayathri; Ghosh, Vipin

2017-01-01

The complex neuro-muscular interplay of speech subsystems is susceptible to alcohol intoxication. Published reports have studied language formulation and fundamental frequency measures pre- and post-intoxication. This study aimed at tapping the speech motor control measure using rate, consistency, and accuracy measures of diadochokinesis and…

Assessment of a brain-tumour-specific Patient Concerns Inventory in the neuro-oncology clinic.

PubMed

Rooney, Alasdair G; Netten, Anouk; McNamara, Shanne; Erridge, Sara; Peoples, Sharon; Whittle, Ian; Hacking, Belinda; Grant, Robin

2014-04-01

Brain tumour patients may struggle to express their concerns in the outpatient clinic, creating a physician-focused rather than a shared agenda. We created a simple, practical brain-tumour-specific holistic needs assessment (HNA) tool for use in the neuro-oncology outpatient clinic. We posted the brain tumour Patient Concerns Inventory (PCI) to a consecutive sample of adult brain tumour attendees to a neuro-oncology outpatient clinic. Participants brought the completed PCI to their clinic consultation. Patients and staff provided feedback. Seventy seven patients were eligible and 53 participated (response rate = 68%). The PCI captured many problems absent from general cancer checklists. The five most frequent concerns were fatigue, fear of tumour coming back, memory, concentration, and low mood. Respondents used the PCI to formulate 105 specific questions, usually about the meaning of physical or psychological symptoms. Patients and staff found the PCI to be useful, and satisfaction with the instrument was high. This study demonstrates the clinical utility of the brain tumour PCI in a neuro-oncology clinic. The combination of a brain-tumour-specific concerns checklist and an intervention to focus patient agenda creates a simple and efficient HNA tool.

COUP-TFII regulates satellite cell function and muscular dystrophy.

PubMed

Xie, Xin; Tsai, Sophia Y; Tsai, Ming-Jer

2016-10-03

Duchenne muscular dystrophy (DMD) is a severe and progressive muscle-wasting disease caused by mutations in the dystrophin gene. Although dystrophin deficiency in myofiber triggers the disease's pathological changes, the degree of satellite cell (SC) dysfunction defines disease progression. Here, we have identified chicken ovalbumin upstream promoter-transcription factor II (COUP-TFII) hyperactivity as a contributing factor underlying muscular dystrophy in a dystrophin-deficient murine model of DMD. Ectopic expression of COUP-TFII in murine SCs led to Duchenne-like dystrophy in the muscles of control animals and exacerbated degenerative myopathies in dystrophin-deficient mice. COUP-TFII-overexpressing mice exhibited regenerative failure that was attributed to deficient SC proliferation and myoblast fusion. Mechanistically, we determined that COUP-TFII coordinated a regenerative program through combined regulation of multiple promyogenic factors. Furthermore, inhibition of COUP-TFII preserved SC function and counteracted the muscle weakness associated with Duchenne-like dystrophy in the murine model, suggesting that targeting COUP-TFII is a potential treatment for DMD. Together, our findings reveal a regulatory role of COUP-TFII in the development of muscular dystrophy and open up a potential therapeutic opportunity for managing disease progression in patients with DMD.

Association of physical activity with muscular strength and fat-free mass in adolescents: the HELENA study.

PubMed

Moliner-Urdiales, Diego; Ortega, Francisco B; Vicente-Rodriguez, Germán; Rey-Lopez, Juan P; Gracia-Marco, Luis; Widhalm, Kurt; Sjöström, Michael; Moreno, Luis A; Castillo, Manuel J; Ruiz, Jonatan R

2010-08-01

The objective of this study is to analyse the association of objectively assessed physical activity (PA) with muscular strength and fat-free mass in adolescents, and to determine whether meeting the current PA recommendations is associated with higher levels of muscular strength and fat-free mass. The present cross-sectional study comprised 363 Spanish adolescents (180 females) aged 12.5-17.5 years. PA was assessed by accelerometry and expressed as average PA (counts/min), and min/day of inactive, light, moderate, vigorous and moderate to vigorous PA (MVPA). MVPA was dichotomized into < 60 min/day and > or = 60. Upper body muscular strength was measured with the handgrip strength test, and lower body muscular strength was measured with the standing broad jump, squat jump, counter movement jump and Abalakov tests. Fat-free mass was measured by DXA. We observed positive associations between vigorous PA and all the lower body muscular strength tests except for the counter movement jump in males. PA was not associated with fat-free mass in both males and females. Male adolescents engaged in at least 60 min/day MVPA performed better in the standing broad jump test. In conclusion, the findings of the present study suggest that only vigorous PA is associated with muscular strength, particularly lower-body muscular strength in male adolescents.

[Pills for the Psyche. Neuro-enhancement among psychiatrists, trainees and other doctors in the Netherlands -- an explanatory study].

PubMed

Timmer, S J; Glas, G

2012-01-01

Psychotropic drugs are being used increasingly for the purpose of neuroenhancement. A survey conducted by Nature revealed that neuro-enhancing drugs were also being used quite widely by non-medical academics. To investigate the extent to which psychiatrists and other doctors working in psychiatry in the Netherlands actually take neuro-enhancing drugs themselves, and to record their views on the use of such drug. The findings form a basis for an ethical debate about the use of neuro-enhancing drugs in the Netherlands. We transmitted an online questionnaire, based on the survey conducted by Nature, to as many as possible psychiatrists, trainees and other doctors working in psychiatry in the Netherlands. 11% of the respondents reported that they had occasionally taken neuro-enhancing medication without any medical reason in order to improve their own mental functioning. 66% of respondents felt that no-one should be allowed to take psychotropic drugs unless these have been prescribed for a strictly medical reason. Compared to those who responded to the Nature survey, respondents to the Dutch survey seemed to have considerably more reservations about the private use of neuroenhancing drugs and seemed to be more critical about their use in general.

Oxidative stress and pathology in muscular dystrophies: focus on protein thiol oxidation and dysferlinopathies.

PubMed

Terrill, Jessica R; Radley-Crabb, Hannah G; Iwasaki, Tomohito; Lemckert, Frances A; Arthur, Peter G; Grounds, Miranda D

2013-09-01

The muscular dystrophies comprise more than 30 clinical disorders that are characterized by progressive skeletal muscle wasting and degeneration. Although the genetic basis for many of these disorders has been identified, the exact mechanism for pathogenesis generally remains unknown. It is considered that disturbed levels of reactive oxygen species (ROS) contribute to the pathology of many muscular dystrophies. Reactive oxygen species and oxidative stress may cause cellular damage by directly and irreversibly damaging macromolecules such as proteins, membrane lipids and DNA; another major cellular consequence of reactive oxygen species is the reversible modification of protein thiol side chains that may affect many aspects of molecular function. Irreversible oxidative damage of protein and lipids has been widely studied in Duchenne muscular dystrophy, and we have recently identified increased protein thiol oxidation in dystrophic muscles of the mdx mouse model for Duchenne muscular dystrophy. This review evaluates the role of elevated oxidative stress in Duchenne muscular dystrophy and other forms of muscular dystrophies, and presents new data that show significantly increased protein thiol oxidation and high levels of lipofuscin (a measure of cumulative oxidative damage) in dysferlin-deficient muscles of A/J mice at various ages. The significance of this elevated oxidative stress and high levels of reversible thiol oxidation, but minimal myofibre necrosis, is discussed in the context of the disease mechanism for dysferlinopathies, and compared with the situation for dystrophin-deficient mdx mice. © 2013 The Authors Journal compilation © 2013 FEBS.

Living with illness and self-transcendence: the lived experience of patients with spinal muscular atrophy.

PubMed

Ho, Hsin-Mei; Tseng, Ying-Hua; Hsin, Yu-Mei; Chou, Fan-Hao; Lin, Wei-Ting

2016-11-01

The aim of this study was to explore the lived experiences of patients afflicted with spinal muscular atrophy. Existing research studies on spinal muscular atrophy address the physical and psychological effects and complications of the disease; they also provide suggestions for how to improve the current management of this disease. However, information is limited on the disease process and the lived experience of spinal muscular atrophy patients. A phenomenological approach was conducted. Through 18 in-depth interviews recorded by a pen voice recorder, this study collected data obtained from a purposive sample of nine patients from the, 'Taiwan spinal muscular atrophy Families,' between November 2010-August 2011. The audio recordings were transcribed verbatim and data were analysed using Colaizzi's steps. Four themes and eight subthemes were identified: a loss of control (loss of muscular strength and independence), breaking limitations (assistive device use and mobility design), transcending limitations (independence/autonomy and social development) and living with hope (cherishing life and self-control). The results showed that the lived experiences of the spinal muscular atrophy patients involved living with illness, transcending the self and pursuing the meaning of life. Facing a life-threatening illness, these patients made self-adjustments in their lifestyles and exerted themselves to positively cope with hardships and maintain dignity and self-control. These findings could serve as evidence-based practice resources for healthcare professionals in helping individuals and their family members gain an in-depth understanding of spinal muscular atrophy's progression and life course and assist individuals in improving self-integrity to with hope. © 2016 John Wiley & Sons Ltd.

Congenital Bone Fractures in Spinal Muscular Atrophy: Functional Role for SMN Protein in Bone Remodeling

PubMed Central

Shanmugarajan, Srinivasan; Swoboda, Kathryn J.; Iannaccone, Susan T.; Ries, William L.; Maria, Bernard L.; Reddy, Sakamuri V.

2009-01-01

Spinal muscular atrophy is the second most common fatal childhood disorder. Core clinical features include muscle weakness caused by degenerating lower motor neurons and a high incidence of bone fractures and hypercalcemia. Fractures further compromise quality of life by progression of joint contractures or additional loss of motor function. Recent observations suggest that bone disease in spinal muscular atrophy may not be attributed entirely to lower motor neuron degeneration. The presence of the spinal muscular atrophy disease-determining survival motor neuron gene (SMN), SMN expression, and differential splicing in bone-resorbing osteoclasts was recently discovered. Its ubiquitous expression and the differential expression of splice variants suggest that SMN has specific roles in bone cell function. SMN protein also interacts with osteoclast stimulatory factor. Mouse models of human spinal muscular atrophy disease suggest a potential role of SMN protein in skeletal development. Dual energy x-ray absorptiometry analysis demonstrated a substantial decrease in total bone area and poorly developed caudal vertebra in the mouse model. These mice also had pelvic bone fractures. Studies delineating SMN signaling mechanisms and gene transcription in a cell-specific manner will provide important molecular insights into the pathogenesis of bone disease in children with spinal muscular atrophy. Moreover, understanding bone remodeling in spinal muscular atrophy may lead to novel therapeutic approaches to enhance skeletal health and quality of life. This article reviews the skeletal complications associated with spinal muscular atrophy and describes a functional role for SMN protein in osteoclast development and bone resorption activity. PMID:17761651

Cardiac manifestations of congenital LMNA-related muscular dystrophy in children: three case reports and recommendations for care.

PubMed

Heller, Felice; Dabaj, Ivana; Mah, Jean K; Bergounioux, Jean; Essid, Aben; Bönnemann, Carsten G; Rutkowski, Anne; Bonne, Gisèle; Quijano-Roy, Susana; Wahbi, Karim

2017-08-01

Skeletal and cardiac muscle laminopathies, caused by mutations in the lamin A/C gene, have a clinical spectrum from congenital LMNA-related muscular dystrophy to later-onset Emery-Dreifuss muscular dystrophy, limb girdle muscular dystrophy, and dilated cardiomyopathy. Although cardiac involvement is observed at all ages, it has only been well described in adults. We present the evolution of cardiac disease in three children with congenital muscular dystrophy presentation of LMNA-related muscular dystrophy. In this series, atrial arrhythmia was the presenting cardiac finding in all three patients. Heart failure developed up to 5 years later. Symptoms of right heart failure, including diarrhoea and peripheral oedema, preceded a rapid decline in left ventricular ejection fraction. Recommendations for cardiac surveillance and management in these patients are made.

Gray Matter and Intelligence Factors: Is There a Neuro-g?

ERIC Educational Resources Information Center

Haier, Richard J.; Colom, Roberto; Schroeder, David H.; Condon, Christopher A.; Tang, Cheuk; Eaves, Emily; Head, Kevin

2009-01-01

Heterogeneous results among neuro-imaging studies using psychometric intelligence measures may result from the variety of tests used. The g-factor may provide a common metric across studies. Here we derived a g-factor from a battery of eight cognitive tests completed by 6929 young adults, 40 of whom also completed structural MRI scans. Regional…

Perceptions of health and attractiveness: the effects of body fat, muscularity, gender, and ethnicity.

PubMed

Yanover, Tovah; Thompson, J Kevin

2010-10-01

Three hundred and thirty-three participants rated the health and attractiveness of schematic figures varying in muscularity, adiposity, gender, and race. In general, overweight and underweight figures were rated as less healthy and attractive. However, overweight figures with high levels of muscularity were rated more highly on these dimensions than figures with less muscularity. Females assigned higher attractiveness ratings than males; African Americans provided higher ratings than Caucasians and Hispanics for female figures and lower ratings than Caucasian and Hispanic raters for male figures. Theoretical explanations and applied implications are offered.

Masculinities and ethnicities: Ethnic differences in drive for muscularity in British men and the negotiation of masculinity hierarchies.

PubMed

Swami, Viren

2016-08-01

Although relatively little is known about ethnic differences in men's drive for muscularity, recent theoretical developments suggest that ethnic minority men may desire greater muscularity to contest their positions of relative subordinate masculinity. This study tested this hypothesis in a sample of 185 White, 180 Black British, and 182 South Asian British men. Participants completed self-report measures of drive for muscularity, need for power, adherence to traditional cultural values, and ethnic group affiliation. Taking into account between-group differences in body mass index, results indicated that White men had significantly lower drive for muscularity than Black and South Asian men, who were not significantly different from each other. In addition, greater need for power was significantly associated with higher drive for muscularity in ethnic minority, but not White, men. Greater adherence to traditional cultural values, but not ethnic group affiliation, was associated with lower drive for muscularity in all ethnic groups. These results suggest that ethnic minority men may desire greater muscularity as a means of negotiating masculinity and attendant ideals of appearance. © 2015 The British Psychological Society.

Reactive navigation for autonomous guided vehicle using neuro-fuzzy techniques

NASA Astrophysics Data System (ADS)

Cao, Jin; Liao, Xiaoqun; Hall, Ernest L.

1999-08-01

A Neuro-fuzzy control method for navigation of an Autonomous Guided Vehicle robot is described. Robot navigation is defined as the guiding of a mobile robot to a desired destination or along a desired path in an environment characterized by as terrain and a set of distinct objects, such as obstacles and landmarks. The autonomous navigate ability and road following precision are mainly influenced by its control strategy and real-time control performance. Neural network and fuzzy logic control techniques can improve real-time control performance for mobile robot due to its high robustness and error-tolerance ability. For a mobile robot to navigate automatically and rapidly, an important factor is to identify and classify mobile robots' currently perceptual environment. In this paper, a new approach of the current perceptual environment feature identification and classification, which are based on the analysis of the classifying neural network and the Neuro- fuzzy algorithm, is presented. The significance of this work lies in the development of a new method for mobile robot navigation.

NeuroSeek dual-color image processing infrared focal plane array

NASA Astrophysics Data System (ADS)

McCarley, Paul L.; Massie, Mark A.; Baxter, Christopher R.; Huynh, Buu L.

1998-09-01

Several technologies have been developed in recent years to advance the state of the art of IR sensor systems including dual color affordable focal planes, on-focal plane array biologically inspired image and signal processing techniques and spectral sensing techniques. Pacific Advanced Technology (PAT) and the Air Force Research Lab Munitions Directorate have developed a system which incorporates the best of these capabilities into a single device. The 'NeuroSeek' device integrates these technologies into an IR focal plane array (FPA) which combines multicolor Midwave IR/Longwave IR radiometric response with on-focal plane 'smart' neuromorphic analog image processing. The readout and processing integrated circuit very large scale integration chip which was developed under this effort will be hybridized to a dual color detector array to produce the NeuroSeek FPA, which will have the capability to fuse multiple pixel-based sensor inputs directly on the focal plane. Great advantages are afforded by application of massively parallel processing algorithms to image data in the analog domain; the high speed and low power consumption of this device mimic operations performed in the human retina.

Influence of exposure and geometric parameters on absorbed doses associated with common neuro-interventional procedures.

PubMed

Safari, Mohammad Javad; Wong, Jeannie Hsiu Ding; Jong, Wei Loong; Thorpe, Nathan; Cutajar, Dean; Rosenfeld, Anatoly; Ng, Kwan Hoong

2017-03-01

The purpose of this study was to investigate the effects of routine exposure parameters on patient's dose during neuro-interventional radiology procedures. We scrutinized the routine radiological exposure parameters during 58 clinical neuro-interventional procedures such as, exposure direction, magnification, frame rate, and distance between image receptor to patient's body and evaluate their effects on patient's dose using an anthropomorphic phantom. Radiation dose received by the occipital region, ears and eyes of the phantom were measured using MOSkin detectors. DSA imaging technique is a major contributor to patient's dose (80.9%) even though they are used sparingly (5.3% of total frame number). The occipital region of the brain received high dose largely from the frontal tube constantly placed under couch (73.7% of the total KAP). When rotating the frontal tube away from under the couch, the radiation dose to the occipital reduced by 40%. The use of magnification modes could increase radiation dose by 94%. Changing the image receptor to the phantom surface distance from 10 to 40cm doubled the radiation dose received by the patient's skin at the occipital region. Our findings provided important insights into the contribution of selected fluoroscopic exposure parameters and their impact on patient's dose during neuro-interventional radiology procedures. This study showed that the DSA imaging technique contributed to the highest patient's dose and judicial use of exposure parameters might assist interventional radiologists in effective skin and eye lens dose reduction for patients undergoing neuro-interventional procedures. Copyright © 2017 Associazione Italiana di Fisica Medica. All rights reserved.

Man Versus Machine Part 2: Comparison of Radiologists' Interpretations and NeuroQuant Measures of Brain Asymmetry and Progressive Atrophy in Patients With Traumatic Brain Injury.

PubMed

Ross, David E; Ochs, Alfred L; DeSmit, Megan E; Seabaugh, Jan M; Havranek, Michael D

2015-01-01

This study is an expanded version of an earlier study, which compared NeuroQuant measures of MRI brain volume with the radiologist's traditional approach in outpatients with mild or moderate traumatic brain injury. NeuroQuant volumetric analyses were compared with the radiologists' interpretations. NeuroQuant found significantly higher rates of atrophy (50.0%), abnormal asymmetry (83.3%), and progressive atrophy (70.0%) than the radiologists (12.5%, 0% and 0%, respectively). Overall, NeuroQuant was more sensitive for detecting at least one sign of atrophy, abnormal asymmetry, or progressive atrophy (95.8%) than the traditional radiologist's approach (12.5%).

The effects of progressive muscular relaxation as a nursing procedure used for those who suffer from stress due to multiple sclerosis.

PubMed

Novais, Paolla Gabrielle Nascimento; Batista, Karla de Melo; Grazziano, Eliane da Silva; Amorim, Maria Helena Costa

2016-09-01

íodo de oito semanas. O grupo experimental foi orientado a realizar diariamente o Relaxamento Muscular Progressivo. Após oito semanas de intervenção avaliou-se novamente os níveis de estresse. Para análise dos dados foi utilizado o pacote Estatístico para Ciências Sociais-versão 19.0. a aplicação do Teste t demonstrou uma diminuição significante dos escores da Escala de Stress Percebido no grupo experimental (p<0,001), evidenciando diminuição nos níveis de estresse após a prática do relaxamento. a intervenção Relaxamento Muscular Progressivo contribui para redução dos níveis de estresse em pessoas com Esclerose Múltipla, podendo ser incluída como prática na assistência de enfermagem prestada a esses pacientes. NCT 02673827. evaluar el efecto del Relajamiento Muscular Progresivo, como intervención de Enfermería en los niveles de estrés en personas con Esclerosis Múltiple. ensayo clínico aleatorio conducido en un ambulatorio de Neurología de un Hospital Universitario. La muestra estuvo constituida por 40 pacientes acompañados en ambulatorio (20 en el grupo control y 20 en el experimental). Se empleó la técnica de Relajamiento Muscular Progresivo. Las variables de control fueron recolectadas por la técnica de entrevista con registro en formulario y se aplicó la Escala de Estrés Percibido. Fueron realizados cinco encuentros quincenales en un período de ocho semanas. El grupo experimental fue orientado a realizar diariamente el Relajamiento Muscular Progresivo. Después de ocho semanas de intervención se evaluó nuevamente los niveles de estrés. Para analizar los datos fue utilizado el programa Estadístico para Ciencias Sociales versión 19.0. la aplicación de la Prueba t demostró una disminución significativa de los puntajes de la Escala de Estrés Percibido en el grupo experimental (p<0,001), evidenciando la disminución de los niveles de estrés después de la práctica del relajamiento. la intervención Relajamiento Muscular

A selected review of abstracts from the 4th Quadrennial Meeting of the World Federation of Neuro-Oncology.

PubMed

Chamberlain, Marc C

2014-03-01

The 4th Quadrennial Meeting of the World Federation of Neuro-Oncology (WFNO), San Francisco, CA, USA, 21-24 November 2013 The 4th Quadrennial Meeting of the World Federation of Neuro-Oncology (WFNO) was the largest neuro-oncology meeting that meets once every 4 years and brings together clinicians and scientists from all parts of the world whose focus is on new brain cancer clinical trials and research primarily pertaining to gliomas. The WFNO 2013 meeting included 1 education day, 2.5 days of presentation, 13 sunrise sessions, one town hall meeting, one mini-symposium, 130 oral presentations and 900 abstracts. This short meeting review highlights select adult clinical abstracts presented at WFNO 2013 that will only in part encompass the contents of a large and multifaceted meeting.

Monitoring the Performance of a Neuro-Adaptive Controller

NASA Technical Reports Server (NTRS)

Schumann, Johann; Gupta, Pramod

2004-01-01

Traditional control has proven to be ineffective to deal with catastrophic changes or slow degradation of complex, highly nonlinear systems like aircraft or spacecraft, robotics, or flexible manufacturing systems. Control systems which can adapt toward changes in the plant have been proposed as they offer many advantages (e.g., better performance, controllability of aircraft despite of a damaged wing). In the last few years, use of neural networks in adaptive controllers (neuro-adaptive control) has been studied actively. Neural networks of various architectures have been used successfully for online learning adaptive controllers. In such a typical control architecture, the neural network receives as an input the current deviation between desired and actual plant behavior and, by on-line training, tries to minimize this discrepancy (e.g.; by producing a control augmentation signal). Even though neuro-adaptive controllers offer many advantages, they have not been used in mission- or safety-critical applications, because performance and safety guarantees cannot b e provided at development time-a major prerequisite for safety certification (e.g., by the FAA or NASA). Verification and Validation (V&V) of an adaptive controller requires the development of new analysis techniques which can demonstrate that the control system behaves safely under all operating conditions. Because of the requirement to adapt toward unforeseen changes during operation, i.e., in real time, design-time V&V is not sufficient.

A method to accurately estimate the muscular torques of human wearing exoskeletons by torque sensors.

PubMed

Hwang, Beomsoo; Jeon, Doyoung

2015-04-09

In exoskeletal robots, the quantification of the user's muscular effort is important to recognize the user's motion intentions and evaluate motor abilities. In this paper, we attempt to estimate users' muscular efforts accurately using joint torque sensor which contains the measurements of dynamic effect of human body such as the inertial, Coriolis, and gravitational torques as well as torque by active muscular effort. It is important to extract the dynamic effects of the user's limb accurately from the measured torque. The user's limb dynamics are formulated and a convenient method of identifying user-specific parameters is suggested for estimating the user's muscular torque in robotic exoskeletons. Experiments were carried out on a wheelchair-integrated lower limb exoskeleton, EXOwheel, which was equipped with torque sensors in the hip and knee joints. The proposed methods were evaluated by 10 healthy participants during body weight-supported gait training. The experimental results show that the torque sensors are to estimate the muscular torque accurately in cases of relaxed and activated muscle conditions.

A Method to Accurately Estimate the Muscular Torques of Human Wearing Exoskeletons by Torque Sensors

PubMed Central

Hwang, Beomsoo; Jeon, Doyoung

2015-01-01

In exoskeletal robots, the quantification of the user’s muscular effort is important to recognize the user’s motion intentions and evaluate motor abilities. In this paper, we attempt to estimate users’ muscular efforts accurately using joint torque sensor which contains the measurements of dynamic effect of human body such as the inertial, Coriolis, and gravitational torques as well as torque by active muscular effort. It is important to extract the dynamic effects of the user’s limb accurately from the measured torque. The user’s limb dynamics are formulated and a convenient method of identifying user-specific parameters is suggested for estimating the user’s muscular torque in robotic exoskeletons. Experiments were carried out on a wheelchair-integrated lower limb exoskeleton, EXOwheel, which was equipped with torque sensors in the hip and knee joints. The proposed methods were evaluated by 10 healthy participants during body weight-supported gait training. The experimental results show that the torque sensors are to estimate the muscular torque accurately in cases of relaxed and activated muscle conditions. PMID:25860074

Improving neuro-oncological patients care: basic and practical concepts for nurse specialist in neuro-rehabilitation

PubMed Central

2012-01-01

Background Neuro-oncological population well expresses the complexity of neurological disability due to the multiple neurological deficits that affect these patients. Moreover, due to the therapeutical opportunities survival times for patients with brain tumor have increased and more of these patients require rehabilitation care. The figure of nurse in the interdisciplinary specialty of neurorehabilitation is not clearly defined, even if their role in this setting is recognized as being critical and is expanding. The purpose of the study is to identify the standard competencies for neurorehabilitation nurses that could be taught by means of a specialization course. Methods A literature review was conducted with preference given to works published between January 2000 and December 2008 in English. The search strategy identified 523 non-duplicated references of which 271 titles were considered relevant. After reviewing the abstracts, 147 papers were selected and made available to a group of healthcare professionals who were requested to classify them in few conceptual main areas defining the relative topics. Results The following five main areas were identified: clinical aspects of nursing; nursing techniques; nursing methodology; relational and organisational models; legal aspects of nursing. The relative topics were included within each area. As educational method a structured course based on lectures and practical sessions was designed. Also multi-choices questions were developed in order to evaluate the participants’ level of knowledge, while a semi-structured interview was prepared to investigate students’ satisfaction. Conclusions Literature shows that the development of rehabilitation depends on the improvement of scientific and practical knowledge of health care professionals. This structured training course could be incorporated into undergraduate nursing education programmes and also be inserted into continuing education programmes for graduate nurses

[Specific features of Becker Muscular Dystrophy patients and female carriers of Duchenne Muscular Dystrophy].

PubMed

Magot, A; Mercier, S; Péréon, Y

2015-12-01

Becker muscular dystrophy (BMD) was first described in 1955 and linked to the DMD gene in 1987. Compared to Duchenne muscular dystrophy (DMD), clinical onset of BMD usually occurs after the age of 12 and wheelchair is required after the age of 16. BMD is characterized by generalized weakness first affecting limb girdle muscles, hypertrophy of the calves and cardiomyopathy in males. Some patients have only mild symptoms such as cramps or elevated serum creatine kinases (SCK) throughout all their lives. SCK levels are usually elevated. Muscle biopsy (immunohistochemistry or immunoblotting) shows a dystrophic pattern with abnormal dystrophin staining. Diagnosis is confirmed by DMD gene sequencing. Deletions or duplications of one or several exons are identified in the majority of cases. A multidisciplinary approach is recommended for the care management of these patients with a particular attention to the cardiomyopathy, which is typically responsible for death but can be prevented by specific treatment. X-linked dilated cardiomyopathies linked to DMD gene are a phenotypic continuum of BMD. Some female carriers of DMD mutations exhibit clinical symptoms of variable severity, often milder and beginning later than in males. The cardiomyopathy is the most frequent feature that should be especially monitored in these patients. Genetic counselling should be systematically proposed. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Skeletal Muscle Metabolism in Duchenne and Becker Muscular Dystrophy-Implications for Therapies.

PubMed

Heydemann, Ahlke

2018-06-20

The interactions between nutrition and metabolism and skeletal muscle have long been known. Muscle is the major metabolic organ—it consumes more calories than other organs—and therefore, there is a clear need to discuss these interactions and provide some direction for future research areas regarding muscle pathologies. In addition, new experiments and manuscripts continually reveal additional highly intricate, reciprocal interactions between metabolism and muscle. These reciprocal interactions include exercise, age, sex, diet, and pathologies including atrophy, hypoxia, obesity, diabetes, and muscle myopathies. Central to this review are the metabolic changes that occur in the skeletal muscle cells of muscular dystrophy patients and mouse models. Many of these metabolic changes are pathogenic (inappropriate body mass changes, mitochondrial dysfunction, reduced adenosine triphosphate (ATP) levels, and increased Ca 2+ ) and others are compensatory (increased phosphorylated AMP activated protein kinase (pAMPK), increased slow fiber numbers, and increased utrophin). Therefore, reversing or enhancing these changes with therapies will aid the patients. The multiple therapeutic targets to reverse or enhance the metabolic pathways will be discussed. Among the therapeutic targets are increasing pAMPK, utrophin, mitochondrial number and slow fiber characteristics, and inhibiting reactive oxygen species. Because new data reveals many additional intricate levels of interactions, new questions are rapidly arising. How does muscular dystrophy alter metabolism, and are the changes compensatory or pathogenic? How does metabolism affect muscular dystrophy? Of course, the most profound question is whether clinicians can therapeutically target nutrition and metabolism for muscular dystrophy patient benefit? Obtaining the answers to these questions will greatly aid patients with muscular dystrophy.

Effects of systemic hypoxia on human muscular adaptations to resistance exercise training

PubMed Central

Kon, Michihiro; Ohiwa, Nao; Honda, Akiko; Matsubayashi, Takeo; Ikeda, Tatsuaki; Akimoto, Takayuki; Suzuki, Yasuhiro; Hirano, Yuichi; Russell, Aaron P.

2014-01-01

Abstract Hypoxia is an important modulator of endurance exercise‐induced oxidative adaptations in skeletal muscle. However, whether hypoxia affects resistance exercise‐induced muscle adaptations remains unknown. Here, we determined the effect of resistance exercise training under systemic hypoxia on muscular adaptations known to occur following both resistance and endurance exercise training, including muscle cross‐sectional area (CSA), one‐repetition maximum (1RM), muscular endurance, and makers of mitochondrial biogenesis and angiogenesis, such as peroxisome proliferator‐activated receptor‐γ coactivator‐1α (PGC‐1α), citrate synthase (CS) activity, nitric oxide synthase (NOS), vascular endothelial growth factor (VEGF), hypoxia‐inducible factor‐1 (HIF‐1), and capillary‐to‐fiber ratio. Sixteen healthy male subjects were randomly assigned to either a normoxic resistance training group (NRT, n =7) or a hypoxic (14.4% oxygen) resistance training group (HRT, n =9) and performed 8 weeks of resistance training. Blood and muscle biopsy samples were obtained before and after training. After training muscle CSA of the femoral region, 1RM for bench‐press and leg‐press, muscular endurance, and skeletal muscle VEGF protein levels significantly increased in both groups. The increase in muscular endurance was significantly higher in the HRT group. Plasma VEGF concentration and skeletal muscle capillary‐to‐fiber ratio were significantly higher in the HRT group than the NRT group following training. Our results suggest that, in addition to increases in muscle size and strength, HRT may also lead to increased muscular endurance and the promotion of angiogenesis in skeletal muscle. PMID:24907297

NeuroPigPen: A Scalable Toolkit for Processing Electrophysiological Signal Data in Neuroscience Applications Using Apache Pig.

PubMed

Sahoo, Satya S; Wei, Annan; Valdez, Joshua; Wang, Li; Zonjy, Bilal; Tatsuoka, Curtis; Loparo, Kenneth A; Lhatoo, Samden D

2016-01-01

The recent advances in neurological imaging and sensing technologies have led to rapid increase in the volume, rate of data generation, and variety of neuroscience data. This "neuroscience Big data" represents a significant opportunity for the biomedical research community to design experiments using data with greater timescale, large number of attributes, and statistically significant data size. The results from these new data-driven research techniques can advance our understanding of complex neurological disorders, help model long-term effects of brain injuries, and provide new insights into dynamics of brain networks. However, many existing neuroinformatics data processing and analysis tools were not built to manage large volume of data, which makes it difficult for researchers to effectively leverage this available data to advance their research. We introduce a new toolkit called NeuroPigPen that was developed using Apache Hadoop and Pig data flow language to address the challenges posed by large-scale electrophysiological signal data. NeuroPigPen is a modular toolkit that can process large volumes of electrophysiological signal data, such as Electroencephalogram (EEG), Electrocardiogram (ECG), and blood oxygen levels (SpO2), using a new distributed storage model called Cloudwave Signal Format (CSF) that supports easy partitioning and storage of signal data on commodity hardware. NeuroPigPen was developed with three design principles: (a) Scalability-the ability to efficiently process increasing volumes of data; (b) Adaptability-the toolkit can be deployed across different computing configurations; and (c) Ease of programming-the toolkit can be easily used to compose multi-step data processing pipelines using high-level programming constructs. The NeuroPigPen toolkit was evaluated using 750 GB of electrophysiological signal data over a variety of Hadoop cluster configurations ranging from 3 to 30 Data nodes. The evaluation results demonstrate that the toolkit

Biological insight, high-throughput datasets and the nature of neuro-degenerative disorders.

PubMed

Valente, André X C N; Oliveira, Paulo J; Khaiboullina, Svetlana F; Palotás, András; Rizvanov, Albert A

2013-09-01

Life sciences are experiencing a historical shift towards a quantitative, data-rich regime. This transition has been associated with the advent of bio-informatics: mathematicians, physicists, computer scientists and statisticians are now commonplace in the field, working on the analysis of ever larger data-sets. An open question regarding what should drive scientific progress in this new era remains: will biological insight become increasingly irrelevant in a world of hypothesis-free, unbiased data analysis? This piece offers a different perspective, pin-pointing that biological thought is more-than-ever relevant in a data-rich setting. Some of the novel highthroughput information being acquired in the field of neuro-degenerative disorders is highlighted here. As but one example of how theory and experiment can interact in this new reality, our efforts in developing an idiopathic neuro-degenerative disease hematopoietic stemcell ageing theory are described.

(Neuro)transmitter systems in circulating immune cells: a target of immunopharmacological interventions?

PubMed

Tayebati, Seyed Khosrow; Amenta, Francesco

2008-01-01

Increasing evidence indicates the existence of an association between nervous and immune systems. The two systems communicate with each-other to maintain immune homeostasis. Activated immune cells secrete cytokines that influence central nervous system activity. Nervous system, through its peripheral and/or autonomic divisions activates output regulating levels of immune cell activity and the subsequent magnitude of an immune response. On the other hand, neurotransmitters, which represent the main substances involved in nerve cell communications, can influence immune function. Immune organs and circulating immune cells express several (neuro)transmitter systems that can be involved in regulating their activity. The expression of neurotransmitter systems by different subsets of circulating immune cells was reviewed. The regulatory role of different families of (neuro)transmitters (catecholamines, 5-hydroxytryptamine, acetylcholine, histamine and neuropeptides) in modulating levels of immune mediators or specific immune responses is discussed.

Genetics Home Reference: spinal and bulbar muscular atrophy

MedlinePlus

... from a particular type of mutation in the AR gene. This gene provides instructions for making a ... as regulating hair growth and sex drive. The AR gene mutation that causes spinal and bulbar muscular ...

Spinal Cord Injury-Assessing Tolerability and Use of Combined Rehabilitation and NeuroAiD (SATURN Study): Protocol of An Exploratory Study In Assessing the Safety and Efficacy of NeuroAiD Amongst People Who Sustain Severe Spinal Cord Injury.

PubMed

Kumar, Ramesh; Htwe, Ohnmar; Baharudin, Azmi; Ariffin, Mohammad Hisam; Abdul Rhani, Shaharuddin; Ibrahim, Kamalnizat; Rustam, Aishah; Gan, Robert

2016-12-05

Spinal cord injury (SCI) is a devastating condition with limited therapeutic options despite decades of research. Current treatment options include use of steroids, surgery, and rehabilitation. Nevertheless, many patients with SCI remain disabled. MLC601 (NeuroAiD), a combination of natural products, has been shown to be safe and to aid neurological recovery after brain injuries and may have a potential role in improving recovery after SCI. The aim of this study is to evaluate the safety and efficacy of NeuroAiD amongst people who sustain SCI in the study setting. Spinal Cord Injury-Assessing Tolerability and Use of Combined Rehabilitation and NeuroAiD (SATURN) is a prospective cohort study of patients with moderately severe to severe SCI, defined as American Spinal Injury Association (ASIA) Impairment Scale (AIS) A and B. These patients will be treated with open-label NeuroAiD for 6 months in addition to standard care and followed for 24 months. Anonymized data will be prospectively collected at baseline and months 1, 3, 6, 12, 18, and 24 and will include information on demographics; main diagnostics; and neurological and functional state assessed by the Spinal Cord Independence Measure, ASIA-International Standard for Neurological Classification Spinal Cord Injury, and Short Form (SF-8) Health Survey. In addition, NeuroAiD treatment, compliance, concomitant therapies, and side effects, if any, will be collected. Investigators will use a secured online system for data entry. The study is approved by the ethics committee of Hospital University Kebangsaan Malaysia. The coprimary endpoints are safety, AIS grade, and improvement in ASIA motor score at 6 months. Secondary endpoints are AIS grade, ASIA motor scores and sensory scores, Spinal Cord Independence Measure (SCIM), SF-8 Health Survey, and compliance at other time points. SATURN investigates the promising role of NeuroAiD in SCI especially given its excellent safety profile. We described here the protocol and

Spinal Cord Injury—Assessing Tolerability and Use of Combined Rehabilitation and NeuroAiD (SATURN Study): Protocol of An Exploratory Study In Assessing the Safety and Efficacy of NeuroAiD Amongst People Who Sustain Severe Spinal Cord Injury

PubMed Central

Htwe, Ohnmar; Baharudin, Azmi; Ariffin, Mohammad Hisam; Abdul Rhani, Shaharuddin; Ibrahim, Kamalnizat; Rustam, Aishah; Gan, Robert

2016-01-01

Background Spinal cord injury (SCI) is a devastating condition with limited therapeutic options despite decades of research. Current treatment options include use of steroids, surgery, and rehabilitation. Nevertheless, many patients with SCI remain disabled. MLC601 (NeuroAiD), a combination of natural products, has been shown to be safe and to aid neurological recovery after brain injuries and may have a potential role in improving recovery after SCI. Objective The aim of this study is to evaluate the safety and efficacy of NeuroAiD amongst people who sustain SCI in the study setting. Methods Spinal Cord Injury—Assessing Tolerability and Use of Combined Rehabilitation and NeuroAiD (SATURN) is a prospective cohort study of patients with moderately severe to severe SCI, defined as American Spinal Injury Association (ASIA) Impairment Scale (AIS) A and B. These patients will be treated with open-label NeuroAiD for 6 months in addition to standard care and followed for 24 months. Anonymized data will be prospectively collected at baseline and months 1, 3, 6, 12, 18, and 24 and will include information on demographics; main diagnostics; and neurological and functional state assessed by the Spinal Cord Independence Measure, ASIA—International Standard for Neurological Classification Spinal Cord Injury, and Short Form (SF-8) Health Survey. In addition, NeuroAiD treatment, compliance, concomitant therapies, and side effects, if any, will be collected. Investigators will use a secured online system for data entry. The study is approved by the ethics committee of Hospital University Kebangsaan Malaysia. Results The coprimary endpoints are safety, AIS grade, and improvement in ASIA motor score at 6 months. Secondary endpoints are AIS grade, ASIA motor scores and sensory scores, Spinal Cord Independence Measure (SCIM), SF-8 Health Survey, and compliance at other time points. Conclusions SATURN investigates the promising role of NeuroAiD in SCI especially given its excellent

Gay male attraction toward muscular men: does mating context matter?

PubMed

Varangis, Eleanna; Lanzieri, Nicholas; Hildebrandt, Tom; Feldman, Matthew

2012-03-01

The purpose of this study was to examine gay men's perceived attractiveness of male figures based on short-term and long-term partner contexts. A sample of 190 gay adult men rated the attractiveness of line-drawings depicting male figures varying systematically in muscularity and body fat percentage in both short-term and long-term dating contexts. Mixed effects modeling was used to estimate the effects of figure (muscularity and body fat), dating context (short-term vs. long-term), and individual rater characteristics on attractiveness ratings. Results indicated that figure muscularity and body-fat had significant non-linear (i.e., quadratic) relationships with attractiveness ratings, and short-term dating context was associated with more discriminating ratings of attractiveness. Interactions between individual characteristics and figure characteristics indicated that the more available the individual and lower body fat, the more discriminating they were in ratings of attractiveness. The implications for future investigations considering both object and observer characteristics of attractiveness preferences are discussed. Copyright © 2012 Elsevier Ltd. All rights reserved.

Adaptability and Prediction of Anticipatory Muscular Activity Parameters to Different Movements in the Sitting Position.

PubMed

Chikh, Soufien; Watelain, Eric; Faupin, Arnaud; Pinti, Antonio; Jarraya, Mohamed; Garnier, Cyril

2016-08-01

Voluntary movement often causes postural perturbation that requires an anticipatory postural adjustment to minimize perturbation and increase the efficiency and coordination during execution. This systematic review focuses specifically on the relationship between the parameters of anticipatory muscular activities and movement finality in sitting position among adults, to study the adaptability and predictability of anticipatory muscular activities parameters to different movements and conditions in sitting position in adults. A systematic literature search was performed using PubMed, Science Direct, Web of Science, Springer-Link, Engineering Village, and EbscoHost. Inclusion and exclusion criteria were applied to retain the most rigorous and specific studies, yielding 76 articles, Seventeen articles were excluded at first reading, and after the application of inclusion and exclusion criteria, 23 were retained. In a sitting position, central nervous system activity precedes movement by diverse anticipatory muscular activities and shows the ability to adapt anticipatory muscular activity parameters to the movement direction, postural stability, or charge weight. In addition, these parameters could be adapted to the speed of execution, as found for the standing position. Parameters of anticipatory muscular activities (duration, order, and amplitude of muscle contractions constituting the anticipatory muscular activity) could be used as a predictive indicator of forthcoming movement. In addition, this systematic review may improve methodology in empirical studies and assistive technology for people with disabilities. © The Author(s) 2016.

Prediction of flood abnormalities for improved public safety using a modified adaptive neuro-fuzzy inference system.

PubMed

Aqil, M; Kita, I; Yano, A; Nishiyama, S

2006-01-01

It is widely accepted that an efficient flood alarm system may significantly improve public safety and mitigate economical damages caused by inundations. In this paper, a modified adaptive neuro-fuzzy system is proposed to modify the traditional neuro-fuzzy model. This new method employs a rule-correction based algorithm to replace the error back propagation algorithm that is employed by the traditional neuro-fuzzy method in backward pass calculation. The final value obtained during the backward pass calculation using the rule-correction algorithm is then considered as a mapping function of the learning mechanism of the modified neuro-fuzzy system. Effectiveness of the proposed identification technique is demonstrated through a simulation study on the flood series of the Citarum River in Indonesia. The first four-year data (1987 to 1990) was used for model training/calibration, while the other remaining data (1991 to 2002) was used for testing the model. The number of antecedent flows that should be included in the input variables was determined by two statistical methods, i.e. autocorrelation and partial autocorrelation between the variables. Performance accuracy of the model was evaluated in terms of two statistical indices, i.e. mean average percentage error and root mean square error. The algorithm was developed in a decision support system environment in order to enable users to process the data. The decision support system is found to be useful due to its interactive nature, flexibility in approach, and evolving graphical features, and can be adopted for any similar situation to predict the streamflow. The main data processing includes gauging station selection, input generation, lead-time selection/generation, and length of prediction. This program enables users to process the flood data, to train/test the model using various input options, and to visualize results. The program code consists of a set of files, which can be modified as well to match other

A groundwork for allostatic neuro-education

PubMed Central

Gerdes, Lee; Tegeler, Charles H.; Lee, Sung W.

2015-01-01

We propose to enliven educational practice by marrying a conception of education as guided human development, to an advanced scientific understanding of the brain known as allostasis (stability through change). The result is a groundwork for allostatic neuro-education (GANE). Education as development encompasses practices including the organic (homeschooling and related traditions), cognitive acquisition (emphasis on standards and testing), and the constructivist (aimed to support adaptive creativity for both learner and society). Allostasis views change to be the norm in biology, defines success in contexts of complex natural environments rather than controlled settings, and identifies the brain as the organ of central command. Allostatic neuro-education contrasts with education focused dominantly on testing, or neuroscience based on homeostasis (stability through constancy). The GANE perspective is to view learners in terms of their neurodevelopmental trajectories; its objective is to support authentic freedom, mediated by competent, integrated, and expansive executive functionality (concordant with the philosophy of freedom of Rudolf Steiner); and its strategy is to be attuned to rhythms in various forms (including those of autonomic arousal described in polyvagal theory) so as to enable experiential excitement for learning. The GANE presents a variety of testable hypotheses, and studies that explore prevention or mitigation of the effects of early life adversity or toxic stress on learning and development may be of particular importance. Case studies are presented illustrating use of allostatic neurotechnology by an adolescent male carrying diagnoses of Asperger’s syndrome and attention-deficit hyperactivity disorder, and a grade school girl with reading difficulties. The GANE is intended as a re-visioning of education that may serve both learners and society to be better prepared for the accelerating changes of the 21st century. PMID:26347688

A groundwork for allostatic neuro-education.

PubMed

Gerdes, Lee; Tegeler, Charles H; Lee, Sung W

2015-01-01

We propose to enliven educational practice by marrying a conception of education as guided human development, to an advanced scientific understanding of the brain known as allostasis (stability through change). The result is a groundwork for allostatic neuro-education (GANE). Education as development encompasses practices including the organic (homeschooling and related traditions), cognitive acquisition (emphasis on standards and testing), and the constructivist (aimed to support adaptive creativity for both learner and society). Allostasis views change to be the norm in biology, defines success in contexts of complex natural environments rather than controlled settings, and identifies the brain as the organ of central command. Allostatic neuro-education contrasts with education focused dominantly on testing, or neuroscience based on homeostasis (stability through constancy). The GANE perspective is to view learners in terms of their neurodevelopmental trajectories; its objective is to support authentic freedom, mediated by competent, integrated, and expansive executive functionality (concordant with the philosophy of freedom of Rudolf Steiner); and its strategy is to be attuned to rhythms in various forms (including those of autonomic arousal described in polyvagal theory) so as to enable experiential excitement for learning. The GANE presents a variety of testable hypotheses, and studies that explore prevention or mitigation of the effects of early life adversity or toxic stress on learning and development may be of particular importance. Case studies are presented illustrating use of allostatic neurotechnology by an adolescent male carrying diagnoses of Asperger's syndrome and attention-deficit hyperactivity disorder, and a grade school girl with reading difficulties. The GANE is intended as a re-visioning of education that may serve both learners and society to be better prepared for the accelerating changes of the 21st century.

Proximal muscular atrophy and weakness: An unusual adverse effect of deferasirox iron chelation therapy.

PubMed

Vill, K; Müller-Felber, W; Teusch, V; Blaschek, A; Gerstl, L; Huetker, S; Albert, M H

2016-01-01

Deferasirox is a standard treatment for chronic transfusional iron overload. Adverse effects of deferasirox have been reported in large prospective studies. We report two cases of monozygotic twins manifesting with proximal muscular atrophy and weakness under deferasirox. Discontinuation of deferasirox resulted in symptom improvement and ultimately in complete remission five months after successful haematopoietic stem cell transplantation. Broad diagnostic work-up could not bring evidence of another aetiology of muscular weakness. Iron overload or beta thalassemia itself as a cause is considered unlikely in our patients because the chronological coincidence of muscular symptoms was contra-directional to serum ferritin levels and significant clinical improvement was observed promptly after cessation of deferasirox even before transplantation. These observations suggest that the development of muscular weakness in patients on deferasirox should be recognised as a possible adverse effect of the drug. Copyright © 2016 Elsevier B.V. All rights reserved.

Screening for the presence of lipophilic marine biotoxins in shellfish samples using the neuro-2a bioassay.

PubMed

Bodero, Marcia; Bovee, Toine F H; Wang, Si; Hoogenboom, Ron L A P; Klijnstra, Mirjam D; Portier, Liza; Hendriksen, Peter J M; Gerssen, Arjen

2018-02-01

The neuro-2a bioassay is considered as one of the most promising cell-based in vitro bioassays for the broad screening of seafood products for the presence of marine biotoxins. The neuro-2a assay has been shown to detect a wide array of toxins like paralytic shellfish poisons (PSPs), ciguatoxins, and also lipophilic marine biotoxins (LMBs). However, the neuro-2a assay is rarely used for routine testing of samples due to matrix effects that, for example, lead to false positives when testing for LMBs. As a result there are only limited data on validation and evaluation of its performance on real samples. In the present study, the standard extraction procedure for LMBs was adjusted by introducing an additional clean-up step with n-hexane. Recovery losses due to this extra step were less than 10%. This wash step was a crucial addition in order to eliminate false-positive outcomes due to matrix effects. Next, the applicability of this assay was assessed by testing a broad range of shellfish samples contaminated with various LMBs, including diarrhetic shellfish toxins/poisons (DSPs). For comparison, the samples were also analysed by LC-MS/MS. Standards of all regulated LMBs were tested, including analogues of some of these toxins. The neuro-2a cells showed good sensitivity towards all compounds. Extracts of 87 samples, both blank and contaminated with various toxins, were tested. The neuro-2a outcomes were in line with those of LC-MS/MS analysis and support the applicability of this assay for the screening of samples for LMBs. However, for use in a daily routine setting, the test might be further improved and we discuss several recommended modifications which should be considered before a full validation is carried out.

An Intranasal Formulation of Erythropoietin (Neuro-EPO) Prevents Memory Deficits and Amyloid Toxicity in the APPSwe Transgenic Mouse Model of Alzheimer's Disease.

PubMed

Rodríguez Cruz, Yamila; Strehaiano, Manon; Rodríguez Obaya, Teresita; García Rodríguez, Julío César; Maurice, Tangui

2017-01-01

Erythropoietin (EPO) is a cytokine known to have effective cytoprotective action in the brain, particularly in ischemic, traumatic, inflammatory, and neurodegenerative conditions. We previously reported the neuroprotective effect of a low sialic form of EPO, Neuro-EPO, applied intranasally in rodent models of stroke or cerebellar ataxia and in a non-transgenic mouse model of Alzheimer's disease (AD). Here we analyzed the protective effect of Neuro-EPO in APPSwe mice, a reference transgenic mouse model of AD. Mice were administered 3 times a day, 3 days in the week with Neuro-EPO (125, 250 μg/kg) intranasally, between 12 and 14 months of age. Motor responses, general activity, and memory responses were analyzed during and after treatment. The deficits in spontaneous alternation, place learning in the water-maze, and novel object recognition observed in APPSwe mice were alleviated by the low dose of Neuro-EPO. Oxidative stress, neuroinflammation, trophic factor levels, and a synaptic marker were analyzed in the hippocampus or cortex of the animals. The increases in lipid peroxidation or in GFAP and Iba-1 contents in APPSwe mice were significantly reduced after Neuro-EPO. Activation of intrinsic and extrinsic apoptotic pathways was analyzed. The increases in Bax/Bcl-2 ratio, TNFα, or Fas ligand levels observed in APPSwe mice were reduced by Neuro-EPO. Finally, immunohistochemical and ELISA analyses of Aβ1-42 levels in the APPSwe mouse cortex and hippocampus showed a marked reduction in Aβ deposits and in soluble and insoluble Aβ1-42 forms. This study therefore confirmed the neuroprotective activity of EPO, particularly for an intranasally deliverable formulation, devoid of erythropoietic side effects, in a transgenic mouse model of AD. Neuro-EPO alleviated memory alterations, oxidative stress, neuroinflammation, apoptosis induction, and amyloid load in 14-month-old APPSwe mice.

Symptoms and signs of neuro-otologic disorders.

PubMed

Newman-Toker, David E

2012-10-01

Symptoms and signs of neuro-otologic disorders are critical components in the diagnostic assessment of patients with vestibular symptoms such as vertigo, dizziness, unsteadiness, and oscillopsia. Most diagnoses can be accomplished at the bedside. An understanding of key diagnostic principles is essential for all practicing neurologists, who are often faced with determining whether such patients warrant urgent diagnostic testing or hospital admission. This article introduces readers to core concepts and recent advances in the understanding of directed history taking and physical examination in patients with vestibular symptoms or suspected neuro-otologic disorders. International consensus definitions for vestibular symptoms have recently been published. During the past 5 years, a growing body of scientific evidence has demonstrated that the traditional approach to bedside diagnosis of patients with vertigo and dizziness is inadequate. Former teaching that history taking should first rely on categorizing symptoms by type (eg, vertigo, presyncope, disequilibrium, nonspecific dizziness) has been replaced by an emphasis on categorizing timing and triggers for vestibular symptoms, which focuses the clinician's attention on four key syndromic patterns: (1) acute, spontaneous, prolonged vestibular symptoms; (2) episodic, positional vestibular symptoms; (3) episodic, spontaneous vestibular symptoms; and (4) chronic unsteadiness (with or without oscillopsia). Each of these categories delineates a relatively narrow differential diagnosis within which a focused examination distinguishes between benign common causes and dangerous uncommon ones. A focused approach to bedside assessment of patients with vestibular symptoms is essential for accurate and efficient diagnosis. All neurologists should be aware of major recent advances.

Muscular dystrophy in the Japanese Spitz: an inversion disrupts the DMD and RPGR genes.

PubMed

Atencia-Fernandez, Sabela; Shiel, Robert E; Mooney, Carmel T; Nolan, Catherine M

2015-04-01

An X-linked muscular dystrophy, with deficiency of full-length dystrophin and expression of a low molecular weight dystrophin-related protein, has been described in Japanese Spitz dogs. The aim of this study was to identify the causative mutation and develop a specific test to identify affected cases and carrier animals. Gene expression studies in skeletal muscle of an affected animal indicated aberrant expression of the Duchenne muscular dystrophy (dystrophin) gene and an anomaly in intron 19 of the gene. Genome-walking experiments revealed an inversion that interrupts two genes on the X chromosome, the Duchenne muscular dystrophy gene and the retinitis pigmentosa GTPase regulator gene. All clinically affected dogs and obligate carriers that were tested had the mutant chromosome, and it is concluded that the inversion is the causative mutation for X-linked muscular dystrophy in the Japanese Spitz breed. A PCR assay that amplifies mutant and wild-type alleles was developed and proved capable of identifying affected and carrier individuals. Unexpectedly, a 7-year-old male animal, which had not previously come to clinical attention, was shown to possess the mutant allele and to have a relatively mild form of the disease. This observation indicates phenotypic heterogeneity in Japanese Spitz muscular dystrophy, a feature described previously in humans and Golden Retrievers. With the availability of a simple, fast and accurate test for Japanese Spitz muscular dystrophy, detection of carrier animals and selected breeding should help eliminate the mutation from the breed. © 2015 Stichting International Foundation for Animal Genetics.

Aerobic fitness, muscular strength and obesity in relation to risk of heart failure.

PubMed

Crump, Casey; Sundquist, Jan; Winkleby, Marilyn A; Sundquist, Kristina

2017-11-01

Low physical fitness and obesity have been associated with higher risk of developing heart failure (HF), but their interactive effects are unknown. Elucidation of interactions among these common modifiable factors may help facilitate more effective primary prevention. We conducted a national cohort study to examine the interactive effects of aerobic fitness, muscular strength and body mass index (BMI) among 1 330 610 military conscripts in Sweden during 1969-1997 (97%-98% of all 18-year-old men) on risk of HF identified from inpatient and outpatient diagnoses through 2012 (maximum age 62 years). There were 11 711 men diagnosed with HF in 37.8 million person-years of follow-up. Low aerobic fitness, low muscular strength and obesity were independently associated with higher risk of HF, after adjusting for each other, socioeconomic factors, other chronic diseases and family history of HF. The combination of low aerobic fitness and low muscular strength (lowest vs highest tertiles) was associated with a 1.7-fold risk of HF (95% CI 1.6 to 1.9; p<0.001; incidence rates per 100 000 person-years, 43.2 vs 10.8). These factors had positive additive and multiplicative interactions (p<0.001) and were associated with increased risk of HF even among men with normal BMI. Low aerobic fitness, low muscular strength and obesity at the age of 18 years were independently associated with higher risk of HF in adulthood, with interactive effects between aerobic fitness and muscular strength. These findings suggest that early-life interventions may help reduce the long-term risk of HF and should include both aerobic fitness and muscular strength, even among persons with normal BMI. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

NeuroMorpho.Org implementation of digital neuroscience: dense coverage and integration with the NIF

PubMed Central

Halavi, Maryam; Polavaram, Sridevi; Donohue, Duncan E.; Hamilton, Gail; Hoyt, Jeffrey; Smith, Kenneth P.; Ascoli, Giorgio A.

2009-01-01

Neuronal morphology affects network connectivity, plasticity, and information processing. Uncovering the design principles and functional consequences of dendritic and axonal shape necessitates quantitative analysis and computational modeling of detailed experimental data. Digital reconstructions provide the required neuromorphological descriptions in a parsimonious, comprehensive, and reliable numerical format. NeuroMorpho.Org is the largest web-accessible repository service for digitally reconstructed neurons and one of the integrated resources in the Neuroscience Information Framework (NIF). Here we describe the NeuroMorpho.Org approach as an exemplary experience in designing, creating, populating, and curating a neuroscience digital resource. The simple three-tier architecture of NeuroMorpho.Org (web client, web server, and relational database) encompasses all necessary elements to support a large-scale, integrate-able repository. The data content, while heterogeneous in scientific scope and experimental origin, is unified in format and presentation by an in house standardization protocol. The server application (MRALD) is secure, customizable, and developer-friendly. Centralized processing and expert annotation yields a comprehensive set of metadata that enriches and complements the raw data. The thoroughly tested interface design allows for optimal and effective data search and retrieval. Availability of data in both original and standardized formats ensures compatibility with existing resources and fosters further tool development. Other key functions enable extensive exploration and discovery, including 3D and interactive visualization of branching, frequently measured morphometrics, and reciprocal links to the original PubMed publications. The integration of NeuroMorpho.Org with version-1 of the NIF (NIFv1) provides the opportunity to access morphological data in the context of other relevant resources and diverse subdomains of neuroscience, opening

NeuroMorpho.Org implementation of digital neuroscience: dense coverage and integration with the NIF.

PubMed

Halavi, Maryam; Polavaram, Sridevi; Donohue, Duncan E; Hamilton, Gail; Hoyt, Jeffrey; Smith, Kenneth P; Ascoli, Giorgio A

2008-09-01

Neuronal morphology affects network connectivity, plasticity, and information processing. Uncovering the design principles and functional consequences of dendritic and axonal shape necessitates quantitative analysis and computational modeling of detailed experimental data. Digital reconstructions provide the required neuromorphological descriptions in a parsimonious, comprehensive, and reliable numerical format. NeuroMorpho.Org is the largest web-accessible repository service for digitally reconstructed neurons and one of the integrated resources in the Neuroscience Information Framework (NIF). Here we describe the NeuroMorpho.Org approach as an exemplary experience in designing, creating, populating, and curating a neuroscience digital resource. The simple three-tier architecture of NeuroMorpho.Org (web client, web server, and relational database) encompasses all necessary elements to support a large-scale, integrate-able repository. The data content, while heterogeneous in scientific scope and experimental origin, is unified in format and presentation by an in house standardization protocol. The server application (MRALD) is secure, customizable, and developer-friendly. Centralized processing and expert annotation yields a comprehensive set of metadata that enriches and complements the raw data. The thoroughly tested interface design allows for optimal and effective data search and retrieval. Availability of data in both original and standardized formats ensures compatibility with existing resources and fosters further tool development. Other key functions enable extensive exploration and discovery, including 3D and interactive visualization of branching, frequently measured morphometrics, and reciprocal links to the original PubMed publications. The integration of NeuroMorpho.Org with version-1 of the NIF (NIFv1) provides the opportunity to access morphological data in the context of other relevant resources and diverse subdomains of neuroscience, opening

Muscular strength and incident hypertension in normotensive and prehypertensive men.

PubMed

Maslow, Andréa L; Sui, Xuemei; Colabianchi, Natalie; Hussey, Jim; Blair, Steven N

2010-02-01

The protective effects of cardiorespiratory fitness (CRF) on hypertension (HTN) are well known; however, the association between muscular strength and incidence of HTN has yet to be examined. This study evaluated the strength-HTN association with and without accounting for CRF. Participants were 4147 men (age = 20-82 yr) in the Aerobics Center Longitudinal Study for whom an age-specific composite muscular strength score was computed from measures of a one-repetition maximal leg and a one-repetition maximal bench press. CRF was quantified by maximal treadmill exercise test time in minutes. Cox proportional hazards regression analysis was used to estimate hazard ratios (HR) and 95% confidence intervals of incident HTN events according to exposure categories. During a mean follow-up of 19 yr, there were 503 incident HTN cases. Multivariable-adjusted (excluding CRF) HR of HTN in normotensive men comparing middle- and high-strength thirds to the lowest third were not significant at 1.17 and 0.84, respectively. Multivariable-adjusted (excluding CRF) HR of HTN in baseline prehypertensive men comparing middle- and high-strength thirds to the lowest third were significant at 0.73 and 0.72 (P = 0.01 each), respectively. The association between muscular strength and incidence of HTN in baseline prehypertensive men was no longer significant after control for CRF (P = 0.26). The study indicated that middle and high levels of muscular strength were associated with a reduced risk of HTN in prehypertensive men only. However, this relationship was no longer significant after controlling for CRF.

Muscular Strength and Incident Hypertension in Normotensive and Prehypertensive Men

PubMed Central

Maslow, Andréa L.; Sui, Xuemei; Colabianchi, Natalie; Hussey, Jim; Blair, Steven N.

2009-01-01

The protective effects of cardiorespiratory fitness (CRF) on hypertension (HTN) are well known; however, the association between muscular strength and incidence of HTN has yet to be examined. Purpose This study evaluated the strength-HTN association with and without accounting for CRF. Methods Participants were 4147 men (20–82 years) in the Aerobics Center Longitudinal Study for whom an age-specific composite muscular strength score was computed from measures of a 1-repetition maximal leg and a 1-repetition maximal bench press. CRF was quantified by maximal treadmill exercise test time in minutes. Cox proportional hazards regression analysis was used to estimate hazard ratios (HRs) and 95% confidence intervals of incident HTN events according to exposure categories. Results During a mean follow-up of 19 years, there were 503 incident HTN cases. Multivariable-adjusted (excluding CRF) HRs of hypertension in normotensive men comparing middle and high strength thirds to the lowest third were not significant at 1.17 and 0.84, respectively. Multivariable-adjusted (excluding CRF) HRs of hypertension in baseline prehypertensive men comparing middle and high strength thirds to the lowest third were significant at 0.73 and 0.72 (p=.01 each), respectively. The association between muscular strength and incidence of HTN in baseline prehypertensive men was no longer significant after control for CRF (p=.26). Conclusions The study indicated that middle and high levels of muscular strength were associated with a reduced risk of HTN in prehypertensive men only. However, this relationship was no longer significant after controlling for CRF. PMID:19927030

Abnormal carbohydrate metabolism in a canine model for muscular dystrophy.

PubMed

Amaral, Andressa R; Brunetto, Márcio A; Brólio, Marina P; Cima, Daniela S; Miglino, Maria A; Santos, João Paulo F; Ambrósio, Carlos E

2017-01-01

The canine golden retriever muscular dystrophy (GRMD) model is the best animal model for studying Duchenne muscular dystrophy in humans. Considering the importance of glucose metabolism in the muscles, the existence of metabolic and endocrine alterations in a wide range of muscular dystrophies, and the pre-existing relationship between blood insulin concentration and muscular atrophy, the present study aimed to evaluate the postprandial glucose and insulin response in GRMD dogs. A total of eighteen golden retriever dogs were randomly distributed into three experimental groups: healthy/control (G1), female GRMD carriers (G2), and male dogs affected by GRMD (G3). Higher plasma resting glucose levels ( P = 0·0047) were seen in G2 and G3 compared with G1, as was the case for minimum ( P = <0·0001), mean ( P = 0·0002) and maximum ( P = 0·0359) glucose values for G3 compared with G1. Fructosamine concentrations were in accordance with reference values found in the literature for dogs. Insulin levels were lower in G3 compared with G1 ( P = 0·0065); however, there was no evidence of insulin resistance according to the homeostasis model assessment index values obtained. As for the evaluation of postprandial responses, fluctuations of glucose ( P = 0·0007) and insulin ( P = 0·0149) were observed in G1 and G2, while in G3 the values remained constant. The results allowed us to identify metabolic changes related to carbohydrate metabolism in GRMD dogs, highlighting the importance of adequate food management for these animals.

Effect of intermittent hypoxia on neuro-functional recovery post brain ischemia in mice.

PubMed

Qiao, Yanxiang; Liu, Zhenfang; Yan, Xianliang; Luo, Chuanming

2015-04-01

Intermittent hypoxia was a simulation of a high-altitude environment. Neuro-inflammation post brain ischemia was considered as a vital impact which contributed to cognitive-functional deficit. The isoform of nitric oxide synthase (iNOS) was an inflammation factor secreted by microglias in neuro-inflammation. In this study, we established a high-altitude environment as the hypoxic condition. Twenty mice were selected and randomized into a hypoxia group (n = 10) or a normoxia group (n = 10) post three vessel occlusion-induced brain ischemia. An enhancement of cognitive-functional recovery was presented in the hypoxia group by survival neuron counting and revealed by the Morris water maze test. Meanwhile, a high level of hypoxia-inducable factor 1 (HIF-1) expression associated with a lower expression of iNOS was observed in the border between infarcts and normal tissue of the hippocampus in the hypoxia group. However, these phenomenons were blocked by HIF-1 inhibition. This suggested that the acceleration of cognitive-functional recovery induced by intermittent hypoxia may depend on HIF-1 activating. An imitation of the hypoxic condition with or without HIF-1 inhibition was operated on the BV-2 cell. A high level of HIF-1 expression associated with a lower-level expression of iNOS was performed in the hypoxic condition. These data suggested that intermittent hypoxia can accelerate cognitive function recovery through attenuating neuro-inflammation.

[Muscular disorders associated with ankylosing spondylitis and their correction with the help of whole body cryotherapy].

PubMed

Kulikov, A G; Tabiev, V I; Rassulova, M A

2015-01-01

The objective of the present study was to evaluate the possibilities for the correction of muscular disorders associated with ankylosing spondylitis and their correction with the help of whole body cryotherapy. The study included 55 patients randomly allocated to two groups. Group 1 was comprised of the patients treated with the use of the common mineral baths, physiotherapy, therapeutic physical exercises, spinal massage, and whole body air-cryotherapy. Group 2 contained the patients who were treated in a similar way with the exception of whole body cryotherapy; they served as controls. Muscular disorders were diagnosed by means of functional muscular testing. The study has demonstrated the high prevalence of muscular disorders in the patients suffering from ankylosing spondylitis. Moreover, it revealed the profile of such disorders associated with ankylosing spondylitis and showed significant correlation between the results of functional muscular testing, BASMI and BASFI indices as well as characteristics of chest excursions (p<0.01). The analysis of the results of the treatment gave evidence of the higher effectiveness of the combined treatment including whole body cryotherapy in comparison with the alternative therapeutic modalities employed in the present study. This therapeutic modality ensured the statistically more pronounced improvement of functional muscular testing parameters (p<0.05), muscle strength and extensibility, as well as certain other clinical and functional characteristics. The groups of muscles most susceptible to cryogenic therapy have been identified. The data obtained in the present study shed light on some specific features of the action of whole body cryotherapy accounting for its corrective influence on the muscular disorders in the patients presenting with ankylosing spondylitis. It is concluded that the proposed approach can be recommended for the introduction in the combined therapeutic and rehabilitative treatment of muscular

The importance of genetic diagnosis for Duchenne muscular dystrophy

PubMed Central

Aartsma-Rus, Annemieke; Ginjaar, Ieke B; Bushby, Kate

2016-01-01

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy are caused by mutations in the dystrophin-encoding DMD gene. Large deletions and duplications are most common, but small mutations have been found as well. Having a correct diagnosis is important for family planning and providing proper care to patients according to published guidelines. With mutation-specific therapies under development for DMD, a correct diagnosis is now also important for assessing whether patients are eligible for treatments. This review discusses different mutations causing DMD, diagnostic techniques available for making a genetic diagnosis for children suspected of DMD and the importance of having a specific genetic diagnosis in the context of emerging genetic therapies for DMD. PMID:26754139

Temporalis muscle hypertrophy and reduced skull eccentricity in Duchenne muscular dystrophy.

PubMed

Straathof, C S M; Doorenweerd, N; Wokke, B H A; Dumas, E M; van den Bergen, J C; van Buchem, M A; Hendriksen, J G M; Verschuuren, J J G M; Kan, H E

2014-10-01

Muscle hypertrophy and muscle weakness are well known in Duchenne muscular dystrophy. Decreased muscle force can have secondary effects on skeletal growth and development such as facial and dental morphology changes. In this study, we quantified temporal muscle thickness, circumference, and eccentricity of the skull and the head on T1-weighted magnetic resonance imaging (MRI) scans of the head of 15 Duchenne muscular dystrophy patients and 15 controls. Average temporal muscle thickness was significantly increased in patients (12.9 ± 5.2 mm) compared to controls (6.8 ± 1.4 mm) (P < .0001), whereas the shape of the skull was significantly rounder compared to controls. Temporal muscle thickness and skull eccentricity were significantly negatively correlated in patients, and positively in controls. Hypertrophy of the temporal muscles and changes in skull eccentricity appear to occur early in the course of Duchenne muscular dystrophy. Further studies in younger patients are needed to confirm a causal relationship. © The Author(s) 2014.

The muscular force transmission system: role of the intramuscular connective tissue.

PubMed

Turrina, Andrea; Martínez-González, Miguel Antonio; Stecco, Carla

2013-01-01

The objective of this review is to analyze in detail the microscopic structure and relations among muscular fibers, endomysium, perimysium, epimysium and deep fasciae. In particular, the multilayer organization and the collagen fiber orientation of these elements are reported. The endomysium, perimysium, epimysium and deep fasciae have not just a role of containment, limiting the expansion of the muscle with the disposition in concentric layers of the collagen tissue, but are fundamental elements for the transmission of muscular force, each one with a specific role. From this review it appears that the muscular fibers should not be studied as isolated elements, but as a complex inseparable from their fibrous components. The force expressed by a muscle depends not only on its anatomical structure, but also the angle at which its fibers are attached to the intramuscular connective tissue and the relation with the epimysium and deep fasciae. Copyright © 2012 Elsevier Ltd. All rights reserved.

Management of cardiac involvement in muscular dystrophies: paediatric versus adult forms.

PubMed

Palladino, Alberto; D'Ambrosio, Paola; Papa, Andrea Antonio; Petillo, Roberta; Orsini, Chiara; Scutifero, Marianna; Nigro, Gerardo; Politano, Luisa

2016-12-01

Muscular dystrophies are a group of genetic disorders characterized by muscle degeneration and consequent substitution by fat and fibrous tissue. Cardiac involvement is an almost constant feature in a great part of these diseases, as both primary myocardial involvement and secondary involvement due to respiratory insufficiency, pulmonary hypertension or reduced mobility. Primary myocardial involvement usually begins more precociously compared to the secondary involvement. In fact the first signs of cardiomyopathy can be observed in the first decade of life in muscular dystrophies with childhood onset and later in adult form of muscular dystrophies as myotonic dystrophy type 1. At least an annual cardiac follow-up is recommended in these patients including clinical and instrumental examination (ECG, 24h Holter monitoring, ECHO), to detect cardiac involvement. A more frequent monitoring may be required according to the type of cardiomyopathy and the patient's needs. In this short review practical guide-lines are shown for physicians routinely involved in the management of these patients.

Neuro-Behcet disease presenting as a solitary cerebellar hemorrhagic lesion: a case report and review of the literature.

PubMed

Yeo, Minju; Lee, Hye-Lim; Cha, Minju; Kim, Ji Seon; Han, Ho-Seong; Lee, Sung-Hyun; Lee, Sang-Soo; Shin, Dong-Ick

2016-12-20

Behcet's disease is a heterogeneous, multisystem, inflammatory disorder of unknown etiology. The classic triad of oral and genital ulcerations in conjunction with uveitis was originally described by the Turkish dermatologist Hulusi Behcet in 1937, but associated symptoms of the cardiovascular, central nervous, pulmonary, and gastrointestinal systems were later identified. In fact, Behcet's disease with neurological involvement (neuro-Behcet's disease) is not uncommon. Patients with neuro-Behcet's disease typically exhibit a diverse array of symptoms, most commonly in the brainstem and diencephalic regions. Herein, we report an unusual case of neuro-Behcet's disease in a patient who presented with a solitary cerebellar hemorrhage. A 39-year-old Asian woman was admitted to our hospital with complaints of a sudden speech difficulty that had manifested the same morning, and dizziness and mild vomiting experienced over the previous 3 days. Magnetic resonance images revealed target-like hemorrhagic lesions in the right hemisphere of the cerebellum. Risk factors that may result in cerebellar hemorrhage, such as high blood pressure or bleeding diathesis, were ruled out, and subsequent brain angiograms were normal. These findings suggest that the patient's cerebellar hemorrhage could have been due to intracranial vasculitis in a rare, if not unique, complication of neuro-Behcet's disease.

Prenatal molecular diagnosis of inherited neuromuscular diseases: Duchenne/Becker muscular dystrophy, myotonic dystrophy type 1 and spinal muscular atrophy.

PubMed

Esposito, Gabriella; Ruggiero, Raffaella; Savarese, Maria; Savarese, Giovanni; Tremolaterra, Maria Roberta; Salvatore, Francesco; Carsana, Antonella

2013-12-01

Neuromuscular disease is a broad term that encompasses many diseases that either directly, via an intrinsic muscle disorder, or indirectly, via a nerve disorder, impairs muscle function. Here we report the experience of our group in the counselling and molecular prenatal diagnosis of three inherited neuromuscular diseases, i.e., Duchenne/Becker muscular dystrophy (DMD/BMD), myotonic dystrophy type 1 (DM1), spinal muscular atrophy (SMA). We performed a total of 83 DMD/BMD, 15 DM1 and 54 SMA prenatal diagnoses using a combination of technologies for either direct or linkage diagnosis. We identified 16, 5 and 10 affected foetuses, respectively. The improvement of analytical procedures in recent years has increased the mutation detection rate and reduced the analytical time. Due to the complexity of the experimental procedures and the high, specific professional expertise required for both laboratory activities and the related counselling, these types of analyses should be preferentially performed in reference molecular diagnostic centres.

Molecular Signatures of Membrane Protein Complexes Underlying Muscular Dystrophy*

PubMed Central

Turk, Rolf; Hsiao, Jordy J.; Smits, Melinda M.; Ng, Brandon H.; Pospisil, Tyler C.; Jones, Kayla S.; Campbell, Kevin P.; Wright, Michael E.

2016-01-01

Mutations in genes encoding components of the sarcolemmal dystrophin-glycoprotein complex (DGC) are responsible for a large number of muscular dystrophies. As such, molecular dissection of the DGC is expected to both reveal pathological mechanisms, and provides a biological framework for validating new DGC components. Establishment of the molecular composition of plasma-membrane protein complexes has been hampered by a lack of suitable biochemical approaches. Here we present an analytical workflow based upon the principles of protein correlation profiling that has enabled us to model the molecular composition of the DGC in mouse skeletal muscle. We also report our analysis of protein complexes in mice harboring mutations in DGC components. Bioinformatic analyses suggested that cell-adhesion pathways were under the transcriptional control of NFκB in DGC mutant mice, which is a finding that is supported by previous studies that showed NFκB-regulated pathways underlie the pathophysiology of DGC-related muscular dystrophies. Moreover, the bioinformatic analyses suggested that inflammatory and compensatory mechanisms were activated in skeletal muscle of DGC mutant mice. Additionally, this proteomic study provides a molecular framework to refine our understanding of the DGC, identification of protein biomarkers of neuromuscular disease, and pharmacological interrogation of the DGC in adult skeletal muscle https://www.mda.org/disease/congenital-muscular-dystrophy/research. PMID:27099343

Discontinuation of statin therapy due to muscular side effects: a survey in real life.

PubMed

Rosenbaum, D; Dallongeville, J; Sabouret, P; Bruckert, E

2013-09-01

To assess the burden of statin related muscular symptom in real life. We conducted a wide survey on 10,409 French subjects. Among these, 2850 (27%) had hypercholesterolemia and 1074 were treated with statins. Muscular symptoms were reported by 104 (10%) statin treated patients and led to discontinuation in 30% of the symptomatic patients. The main prescribed statins were low doses rosuvastatin, atorvastatin and simvastatin. Pains were the most commonly described symptoms (87%) but many patients also reported stiffness (62%), cramps (67%), weakness or a loss of strength during exertion (55%). Pain was localized in 70% but mostly described as affecting several muscular groups. Approximately 38% of patients reported that their symptoms prevented even moderate exertion during everyday activities, while 42% of patients suffered major disruption to their everyday life. Muscular symptoms associated with average dosage statin therapy are more frequent than in clinical trials and have a greater impact on patients' life than usually thought. Copyright © 2012 Elsevier B.V. All rights reserved.

Muscular imbalance and shoulder pain in volleyball attackers.

PubMed Central

Kugler, A; Krüger-Franke, M; Reininger, S; Trouillier, H H; Rosemeyer, B

1996-01-01

OBJECTIVE: In overhead sports such as volleyball, baseball, or tennis shoulder problems are very common. The aim of this study was to identify features which may correlate with shoulder problems in volleyball attackers. METHODS: 30 competitive volleyball attackers (mean age 25 years) were included in the study; 15 were suffering from shoulder pain and 15 had no history of shoulder pain. The results were compared with those of a control group of 15 recreational athletes without any overhead sports activities. RESULTS: Volleyball attackers have a different muscular and capsular pattern at the playing shoulder compared to the opposite shoulder. Their playing shoulder is depressed, the scapula lateralised, and the dorsal muscles and the posterior and inferior part of the shoulder capsule shortened. These differences were of more significance in volleyball attackers with shoulder pain than in volleyball players without shoulder pain. In contrast to recreational athletes without any overhead sports activity, there were no significant difference in the comparison of the two shoulders. The histories, clinical and sonographic findings did not reveal further typical features for volleyball attackers with shoulder pain. CONCLUSIONS: Muscular balance of the shoulder girdle is very important in this sport. It is therefore imperative to include adequate stretching and muscular training programme for the prevention, as well as for therapy, of shoulder pain in volleyball attackers. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 PMID:8889124

Enhanced Expression of WD Repeat-Containing Protein 35 via Nuclear Factor-Kappa B Activation in Bupivacaine-Treated Neuro2a Cells

PubMed Central

Huang, Lei; Kondo, Fumio; Harato, Misako; Feng, Guo-Gang; Ishikawa, Naoshisa; Fujiwara, Yoshihiro; Okada, Shoshiro

2014-01-01

The family of WD repeat proteins comprises a large number of proteins and is involved in a wide variety of cellular processes such as signal transduction, cell growth, proliferation, and apoptosis. Bupivacaine is a sodium channel blocker administered for local infiltration, nerve block, epidural, and intrathecal anesthesia. Recently, we reported that bupivacaine induces reactive oxygen species (ROS) generation and p38 mitogen-activated protein kinase (MAPK) activation, resulting in an increase in the expression of WD repeat-containing protein 35 (WDR35) in mouse neuroblastoma Neuro2a cells. It has been shown that ROS activate MAPK through phosphorylation, followed by activation of nuclear factor-kappa B (NF-κB) and activator protein 1 (AP-1). The present study was undertaken to test whether NF-κB and c-Jun/AP-1 are involved in bupivacaine-induced WDR35 expression in Neuro2a cells. Bupivacaine activated both NF-κB and c-Jun in Neuro2a cells. APDC, an NF-κB inhibitor, attenuated the increase in NF-κB activity and WDR35 protein expression in bupivacaine-treated Neuro2a cells. GW9662, a selective peroxisome proliferator-activated receptor-γ antagonist, enhanced the increase in NF-κB activity and WDR35 protein expression in bupivacaine-treated Neuro2a cells. In contrast, c-Jun siRNA did not inhibit the bupivacaine-induced increase in WDR35 mRNA expression. These results indicate that bupivacaine induces the activation of transcription factors NF-κB and c-Jun/AP-1 in Neuro2a cells, while activation of NF-κB is involved in bupivacaine-induced increases in WDR35 expression. PMID:24466034

Reduction of low back muscular discomfort through an applied ergonomics intervention program.

PubMed

Poosanthanasarn, Nitaya; Sriboorapa, Sooth; Fungladda, Wijitr; Lohachit, Chantima

2005-01-01

An applied ergonomics intervention program (AEIP) was conducted with male employees who work in the pressing and storage sections of a metal auto parts factory in eastern Thailand. The objective of this study was to reduce worker muscular discomfort at the low back. The study design was a participatory research approach, with quasi-experimental pretest-posttest, and with a non-equivalent control group. Thirty-five persons participated in the AEIP (AEIP group) and 17 persons did not (non-AEIP group). The AEIP was composed of three major categories: (1) top management support; (2) equipment designed for workstations and manual material handling; and (3) administrative intervention, training, and health education. Muscle activity was measured by surface electromyography of the left and right erector spinae, and multifidus muscles; and evaluated by multivariate test for dependent samples (paired observation) and for independent samples. After the AEIP, the low back muscular loads of the AEIP group was significantly reduced, while those of the non-AEIP group were not. Comparison of the means of percentage maximum voluntary contractions (% MVC) of low back muscular activity between the AEIP group and non-AEIP group indicated that the AEIP group had significantly reduced low back muscular load, with a 95% confidence level (p-value < 0.05).

A novel recessive mutation in the gene ELOVL4 causes a neuro-ichthyotic disorder with variable expressivity

PubMed Central

2014-01-01

Background A rare neuro-ichthyotic disorder characterized by ichthyosis, spastic quadriplegia and intellectual disability and caused by recessive mutations in ELOVL4, encoding elongase-4 protein has recently been described. The objective of the study was to search for sequence variants in the gene ELOVL4 in three affected individuals of a consanguineous Pakistani family exhibiting features of neuro-ichthyotic disorder. Methods Linkage in the family was searched by genotyping microsatellite markers linked to the gene ELOVL4, mapped at chromosome 6p14.1. Exons and splice junction sites of the gene ELOVL4 were polymerase chain reaction amplified and sequenced in an automated DNA sequencer. Results DNA sequence analysis revealed a novel homozygous nonsense mutation (c.78C > G; p.Tyr26*). Conclusions Our report further confirms the recently described ELOVL4-related neuro-ichthyosis and shows that the neurological phenotype can be absent in some individuals. PMID:24571530

Development of a neuro early mobilisation protocol for use in a neuroscience intensive care unit.

PubMed

Brissie, Megan A; Zomorodi, Meg; Soares-Sardinha, Sharmila; Jordan, J Dedrick

2017-10-01

Through evaluation of the literature and working with a team of multidisciplinary healthcare providers, our objective was to refine an interprofessional Neuro Early Mobilisation Protocol for complex patients in the Neuroscience Intensive Care Unit. Using the literature as a guide, key stakeholders, from multiple professions, designed and refined a Neuro Early Mobilisation Protocol. This project took place at a large academic medical center in the southeast United States classified as both a Level I Trauma Center and Comprehensive Stroke Center. Goals for protocol development were to: (1) simplify the protocol to allow for ease of use, (2) make the protocol more generalizable to the patient population cared for in the Neuroscience Intensive Care Unit, (3) receive feedback from those using the original protocol on ways to improve the protocol and (4) ensure patients were properly screened for inclusion and exclusion in the protocol. Using expert feedback and the evidence, an evidence-based Neuro Early Mobilisation Protocol was created for use with all patients in the Neuroscience Intensive Care Unit. Future work will consist of protocol implementation and evaluation in order to increase patient mobilisation in the Neuroscience Intensive Care Unit. Copyright © 2017 Elsevier Ltd. All rights reserved.

Sexuality and the drive for muscularity: evidence of associations among British men.

PubMed

Swami, Viren; Diwell, Rachel; McCreary, Donald R

2014-09-01

Previous studies have documented associations between sexuality and body image, but the directionality of this association is unclear among men. This study examined whether men's drive for muscularity can be considered a correlate of their sexuality. A community-based sample of 292 heterosexual men from London, UK, completed a survey consisting of measures of drive for muscularity, sociosexuality, sexual assertiveness, sexual esteem, and sexual sensation seeking. A multiple regression analysis showed that greater drive for muscularity was predicted by more unrestricted sociosexuality (i.e., a greater proclivity for short-term, transient relationships), greater sexual sensation seeking, and greater sexual assertiveness, once the effects of participant age and body mass index had been accounted for. Possible avenues for intervention based on a sex-positive approach are discussed in conclusion. Copyright © 2014 Elsevier Ltd. All rights reserved.

Developmental Defects in a Zebrafish Model for Muscular Dystrophies Associated with the Loss of Fukutin-Related Protein (FKRP)

ERIC Educational Resources Information Center

Thornhill, Paul; Bassett, David; Lochmuller, Hanns; Bushby, Kate; Straub, Volker

2008-01-01

A number of muscular dystrophies are associated with the defective glycosylation of [alpha]-dystroglycan and many are now known to result from mutations in a number of genes encoding putative or known glycosyltransferases. These diseases include severe forms of congenital muscular dystrophy (CMD) such as Fukuyama type congenital muscular dystrophy…

Ectopic Expression of Retrotransposon-Derived PEG11/RTL1 Contributes to the Callipyge Muscular Hypertrophy

PubMed Central

Xu, Xuewen; Ectors, Fabien; Davis, Erica E.; Pirottin, Dimitri; Cheng, Huijun; Farnir, Frédéric; Hadfield, Tracy; Cockett, Noelle; Charlier, Carole; Georges, Michel; Takeda, Haruko

2015-01-01

The callipyge phenotype is an ovine muscular hypertrophy characterized by polar overdominance: only heterozygous + Mat /CLPG Pat animals receiving the CLPG mutation from their father express the phenotype. + Mat /CLPG Pat animals are characterized by postnatal, ectopic expression of Delta-like 1 homologue (DLK1) and Paternally expressed gene 11/Retrotransposon-like 1 (PEG11/RTL1) proteins in skeletal muscle. We showed previously in transgenic mice that ectopic expression of DLK1 alone induces a muscular hypertrophy, hence demonstrating a role for DLK1 in determining the callipyge hypertrophy. We herein describe newly generated transgenic mice that ectopically express PEG11 in skeletal muscle, and show that they also exhibit a muscular hypertrophy phenotype. Our data suggest that both DLK1 and PEG11 act together in causing the muscular hypertrophy of callipyge sheep. PMID:26474044

A comprehensive approach in high-grade glioma management: position statement from the Neuro-Oncology Scientific Club (NOSC), Shiraz, Iran

PubMed Central

Ansari, Mansour; Mosalaei, Ahmad; Ahmadloo, Niloufar; Rasekhi, Alireza; Geramizadeh, Bita; Razmkon, Ali; Anvari, Kazem; Afarid, Mohammad; Dadras, Ali; Nafarieh, Leila; Mohammadianpanah, Mohammad; Nasrolahi, Hamid; Hamedi, Seyed Hasan; Omidvari, Shapour; Nami, Mohammad

2017-01-01

Establishing a robust teamwork model in the practice of neuro-oncology requires continued interdisciplinary efforts. The Neuro-Oncology Scientific Club (NOSC) initiative is an interdisciplinary clinical forum promoting the comprehensive approach across involved disciplines in the management of central nervous system (CNS) malignancies. With its provincial founding panels and national steering board, NOSC has been operational in Iran since 2011. This initiative has pursued its mission through interval strategic meetings, tumor boards, case discussions as well as publishing neuro-oncology updates, case study periodicals, and newsletters. A provincial meeting of NOSC in Shiraz put together insights from international practice guidelines, emerging evidence, and expert opinions to draw a position statement on high-grade glioma management in adults. The present report summarizes key highlights from the above clinical forum. PMID:28325997

A comprehensive approach in high-grade glioma management: position statement from the Neuro-Oncology Scientific Club (NOSC), Shiraz, Iran.

PubMed

Ansari, Mansour; Mosalaei, Ahmad; Ahmadloo, Niloufar; Rasekhi, Alireza; Geramizadeh, Bita; Razmkon, Ali; Anvari, Kazem; Afarid, Mohammad; Dadras, Ali; Nafarieh, Leila; Mohammadianpanah, Mohammad; Nasrolahi, Hamid; Hamedi, Seyed Hasan; Omidvari, Shapour; Nami, Mohammad

2017-01-01

Establishing a robust teamwork model in the practice of neuro-oncology requires continued interdisciplinary efforts. The Neuro-Oncology Scientific Club (NOSC) initiative is an interdisciplinary clinical forum promoting the comprehensive approach across involved disciplines in the management of central nervous system (CNS) malignancies. With its provincial founding panels and national steering board, NOSC has been operational in Iran since 2011. This initiative has pursued its mission through interval strategic meetings, tumor boards, case discussions as well as publishing neuro-oncology updates, case study periodicals, and newsletters. A provincial meeting of NOSC in Shiraz put together insights from international practice guidelines, emerging evidence, and expert opinions to draw a position statement on high-grade glioma management in adults. The present report summarizes key highlights from the above clinical forum.

NeuroVR 1.5 - a free virtual reality platform for the assessment and treatment in clinical psychology and neuroscience.

PubMed

Riva, Giuseppe; Carelli, Laura; Gaggioli, Andrea; Gorini, Alessandra; Vigna, Cinzia; Corsi, Riccardo; Faletti, Gianluca; Vezzadini, Luca

2009-01-01

At MMVR 2007 we presented NeuroVR (http://www.neurovr.org) a free virtual reality platform based on open-source software. The software allows non-expert users to adapt the content of 14 pre-designed virtual environments to the specific needs of the clinical or experimental setting. Following the feedbacks of the 700 users who downloaded the first version, we developed a new version - NeuroVR 1.5 - that improves the possibility for the therapist to enhance the patient's feeling of familiarity and intimacy with the virtual scene, by using external sounds, photos or videos. Specifically, the new version now includes full sound support and the ability of triggering external sounds and videos using the keyboard. The outcomes of different trials made using NeuroVR will be presented and discussed.

A Rare Case Report of Neurodegenerative Disease: Duchenne Muscular Dystrophy in Two Male Siblings

PubMed Central

Suneja, B; Suneja, ES; Chandna, P

2015-01-01

ABSTRACT Duchenne muscular dystrophy (DMD) is an recessive X-linked mediated, musculoskeletal disorder that affects only males. It is the most common and severe form of muscular dystrophy where there is failure to manufacture dystrophin. Clinically, it is characterized by progressive muscle wasting eventually leading to premature death. This case report describes the genetic, oral and systemic findings in two cases of DMD in male siblings. How to cite this article: Suneja B, Suneja ES, Adlakha VK, Chandna P. A Rare Case Report of Neurodegenerative Disease: Duchenne Muscular Dystrophy in Two Male Siblings. Int J Clin Pediatr Dent 2015;8(2):163-165. PMID:26379389

Muscular hypertrophy of the left diaphragmatic crus: an unusual cause of a paraspinal "mass".

PubMed

Woodring, J H; Bognar, B

1998-04-01

We present a case of marked muscular hypertrophy of the muscular portion of the diaphragm and of the diaphragmatic crura in a professional opera singer. In this case the right and left crus each measured 20 mm in maximum thickness. The left crus, by nature of its vertical orientation in the sagittal plane, produced marked deviation of the inferior left paraspinal line near the diaphragm mimicking a retrocrural or paraspinal mass on the posteroanterior chest radiograph. The correct diagnosis was made by computed tomography. Muscular hypertrophy of the diaphragmatic crura should be included in the differential diagnosis of retrocrural or paraspinal masses at the level of the diaphragm.

Clinical effectiveness of dramatherapy in the recovery from neuro-trauma.

PubMed

McKenna, P; Haste, E

1999-04-01

To investigate the clinical effectiveness of a short course of dramatherapy (an eclectic term encompassing all the arts therapies), delivered in a one-to-one interaction, in a sample of 10 patients in a neuro-rehabilitation unit. Each participant received five individual one-to-one sessions of therapy over a 5 week period. A semi-structured interview was carried out with each participant following the course. Qualitative analysis of the taped interviews elicited how the therapy contrasted and complemented the rest of the rehabilitation setting and therapies and how it helped psychological adjustment to severe disabilities resulting from neurotrauma. There were four ways in which it appeared to empower the participants and nurture their self esteem. It provided them with a sense of personal space in an otherwise institutional setting; it allowed escapism and enjoyment; it awakened creativity and a sense of potency; and it provided a metaphor to explore personal issues. Dramatherapy made an important contribution to the healthy adjustment of some patients both to hospital life and to acquired disability. The reports from the patients indicated that this approach to rehabilitation should be further incorporated and developed in neuro-rehabilitation.

Neural stem cells and neuro/gliogenesis in the central nervous system: understanding the structural and functional plasticity of the developing, mature, and diseased brain.

PubMed

Yamaguchi, Masahiro; Seki, Tatsunori; Imayoshi, Itaru; Tamamaki, Nobuaki; Hayashi, Yoshitaka; Tatebayashi, Yoshitaka; Hitoshi, Seiji

2016-05-01

Neurons and glia in the central nervous system (CNS) originate from neural stem cells (NSCs). Knowledge of the mechanisms of neuro/gliogenesis from NSCs is fundamental to our understanding of how complex brain architecture and function develop. NSCs are present not only in the developing brain but also in the mature brain in adults. Adult neurogenesis likely provides remarkable plasticity to the mature brain. In addition, recent progress in basic research in mental disorders suggests an etiological link with impaired neuro/gliogenesis in particular brain regions. Here, we review the recent progress and discuss future directions in stem cell and neuro/gliogenesis biology by introducing several topics presented at a joint meeting of the Japanese Association of Anatomists and the Physiological Society of Japan in 2015. Collectively, these topics indicated that neuro/gliogenesis from NSCs is a common event occurring in many brain regions at various ages in animals. Given that significant structural and functional changes in cells and neural networks are accompanied by neuro/gliogenesis from NSCs and the integration of newly generated cells into the network, stem cell and neuro/gliogenesis biology provides a good platform from which to develop an integrated understanding of the structural and functional plasticity that underlies the development of the CNS, its remodeling in adulthood, and the recovery from diseases that affect it.

RESPIRATORY DYSFUNCTION IN UNSEDATED DOGS WITH GOLDEN RETRIEVER MUSCULAR DYSTROPHY

PubMed Central

DeVanna, Justin C.; Kornegay, Joe N.; Bogan, Daniel J.; Bogan, Janet R.; Dow, Jennifer L.; Hawkins, Eleanor C.

2013-01-01

Golden retriever muscular dystrophy (GRMD) is a well-established model of Duchenne muscular dystrophy. The value of this model would be greatly enhanced with practical tools to monitor progression of respiratory dysfunction during treatment trials. Arterial blood gas analysis, tidal breathing spirometry, and respiratory inductance plethysmography (RIP) were performed to determine if quantifiable abnormalities could be identified in unsedated, untrained, GRMD dogs. Results from 11 dogs with a mild phenotype of GRMD and 11 age-matched carriers were compared. Arterial blood gas analysis was successfully performed in all dogs, spirometry in 21 of 22 (95%) dogs, and RIP in 18 of 20 (90%) dogs. Partial pressure of carbon dioxide and bicarbonate concentration were higher in GRMD dogs. Tidal breathing peak expiratory flows were markedly higher in GRMD dogs. Abnormal abdominal motion was present in 7 of 10 (70%) GRMD dogs. Each technique provided objective, quantifiable measures that will be useful for monitoring respiratory function in GRMD dogs during clinical trials while avoiding the influence of sedation on results. Increased expiratory flows and the pattern of abdominal breathing are novel findings, not reported in people with Duchenne muscular dystrophy, and might be a consequence of hyperinflation. PMID:24295812

Visuospatial Attention Disturbance in Duchenne Muscular Dystrophy

ERIC Educational Resources Information Center

De Moura, Maria Clara Drummond Soares; do Valle, Luiz Eduardo Ribeiro; Resende, Maria Bernadete Dutra; Pinto, Katia Osternack

2010-01-01

Aim: The cognitive deficits present in the Duchenne muscular dystrophy (DMD) are not yet well characterized. Attention, considered to be the brain mechanism responsible for the selection of sensory stimuli, could be disturbed in DMD, contributing, at least partially, to the observed global cognitive deficit. The aim of this study was to…

NeuroPigPen: A Scalable Toolkit for Processing Electrophysiological Signal Data in Neuroscience Applications Using Apache Pig

PubMed Central

Sahoo, Satya S.; Wei, Annan; Valdez, Joshua; Wang, Li; Zonjy, Bilal; Tatsuoka, Curtis; Loparo, Kenneth A.; Lhatoo, Samden D.

2016-01-01

The recent advances in neurological imaging and sensing technologies have led to rapid increase in the volume, rate of data generation, and variety of neuroscience data. This “neuroscience Big data” represents a significant opportunity for the biomedical research community to design experiments using data with greater timescale, large number of attributes, and statistically significant data size. The results from these new data-driven research techniques can advance our understanding of complex neurological disorders, help model long-term effects of brain injuries, and provide new insights into dynamics of brain networks. However, many existing neuroinformatics data processing and analysis tools were not built to manage large volume of data, which makes it difficult for researchers to effectively leverage this available data to advance their research. We introduce a new toolkit called NeuroPigPen that was developed using Apache Hadoop and Pig data flow language to address the challenges posed by large-scale electrophysiological signal data. NeuroPigPen is a modular toolkit that can process large volumes of electrophysiological signal data, such as Electroencephalogram (EEG), Electrocardiogram (ECG), and blood oxygen levels (SpO2), using a new distributed storage model called Cloudwave Signal Format (CSF) that supports easy partitioning and storage of signal data on commodity hardware. NeuroPigPen was developed with three design principles: (a) Scalability—the ability to efficiently process increasing volumes of data; (b) Adaptability—the toolkit can be deployed across different computing configurations; and (c) Ease of programming—the toolkit can be easily used to compose multi-step data processing pipelines using high-level programming constructs. The NeuroPigPen toolkit was evaluated using 750 GB of electrophysiological signal data over a variety of Hadoop cluster configurations ranging from 3 to 30 Data nodes. The evaluation results demonstrate that

Neuro-endoscopic management of intraventricular neurocysticercosis (NCC).

PubMed

Husain, M; Jha, D K; Rastogi, M; Husain, N; Gupta, R K

2007-01-01

Various approaches including endoscopy have been used for the treatment of intraventricular and cisternal NCC. We present our technique of Neuro-endoscopic management of intraventricular NCC. Twenty-one cases, 13 females and 8 males (age range 12-50 years; mean, 25.7 years), of intraventricular NCC [lateral (n = 6), third (n = 6), fourth (n = 10) ventricles including a patient with both lateral and third ventricular cysts] producing obstructive hydrocephalus formed the group of study. Gaab Universal Endoscope System along with 4 mm 0 degrees and 30 degrees rigid telescopes were used through a frontal burr-hole for removal of intraventricular including intra-fourth ventricular (n = 10) NCC. Endoscopic third ventriculostomy (ETV) was done for internal cerebrospinal fluid (CSF) diversion. Average follow up was 18 months. Complete (n = 18) or partial (n = 2) removal of NCC was done in 20 patients, while a cyst located at foramen of Monro slipped and migrated to occipital or temporal horn in 1 patient. Thirty-degree 4-mm rigid telescope provided excellent image quality with ability to address even intra-fourth ventricular NCC through the dilated aqueduct using a curved tip catheter. No patient required further surgery for their hydrocephalus. There was no operative complication and post-operative ventriculitis was not seen in any case despite partial removal of NCC. Neuro-endoscopic surgery is an effective treatment modality for patients with intraventricular NCC. It effectively restores CSF flow and is capable of removing cysts completely or partially from accessible locations causing mass effect. Partial removal or rupture of the cyst does not affect the clinical outcome of the patients.

Purα Repaired Expanded Hexanucleotide GGGGCC Repeat Noncoding RNA-Caused Neuronal Toxicity in Neuro-2a Cells.

PubMed

Shen, Jianying; Zhang, Yu; Zhao, Shi; Mao, Hong; Wang, Zhongjing; Li, Honglian; Xu, Zihui

2018-05-01

Expanded hexanucleotide GGGGCC repeat in a noncoding region of C9ORF72 is the most common cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). However, its molecular pathogenesis remains unclear. In our previous study, the expanded GGGGCC repeats have been shown to be sufficient to cause neurodegeneration. In order to investigate the further role of expanded GGGGCC repeats in the neuron, the normal r(GGGGCC) 3 and mutant-type expanded r(GGGGCC) 30 expression vectors were transfected into Neuro-2a cells. Cell proliferation, dendrite development, and the proteins' levels of microtubule-associated protein-2 (MAP2) and cyclin-dependent kinase-5 (CDK5) were used to evaluate the cell toxicity of GGGGCC repeats on Neuro-2a cells. The results were shown that expression of expanded GGGGCC repeats caused neuronal cell toxicity in Neuro-2a cells, enhanced the expression of pMAP2 and pCDK5. Moreover, overexpression of Purα repaired expanded GGGGCC repeat-inducing neuronal toxicity in Neuro-2a cells and reduced the expression of pMAP2 and pCDK5. In all, our findings suggested that the expanded GGGGCC repeats might cause neurodegeneration through destroyed neuron cells. And the GGGGCC repeat-induced neuronal cell toxicity was inhibited by upregulation of Purα. We inferred that Purα inhibits expanded GGGGCC repeat-inducing neurodegeneration, which might reveal a novel mechanism of neurodegenerative diseases ALS and FTD.

Effect of a core conditioning intervention on tests of trunk muscular endurance in school-aged children.

PubMed

Allen, Brett A; Hannon, James C; Burns, Ryan D; Williams, Skip M

2014-07-01

Trunk and core muscular development has been advocated to increase athletic performance and for maintenance of musculoskeletal health, especially related to the prevention of low back pain (LBP). The purpose of this study was to examine the effects of a simple core conditioning routine on tests of trunk and core muscular endurance in school-aged children. Participants included 164 students (86 girls, 78 boys; mean age, 11.5 ± 2.5 years) recruited from a grade school in a metropolitan area located in the southwestern United States. Students performed an equipment-free, moderate-to-high intensity, dynamic core conditioning warm-up routine once a week for a period of 6 weeks during the start of their physical education classes. The intervention consisted of 10 different dynamic core conditioning exercises performed at a 30-second duration per exercise totaling 5 minutes per session. Pre- and post-assessments of muscular endurance consisted of 5 different trunk and core muscular endurance tests: Parallel Roman Chair Dynamic Back Extension, Prone Plank, Lateral Plank, Dynamic Curl-Up, and Static Curl-up. A generalized estimation equation was used to analyze differences in pre- and post-intervention muscular fitness assessments controlling for gender and grade level. Analysis of the data revealed significant increases in muscular fitness test performance for each of the 5 measured outcomes (p < 0.001). Because risk factors of LBP are thought to commence during childhood, results of this study suggest that it may be desirable for children and adolescents to perform moderate-to-high intensity dynamic core exercises during physical education warm-up to improve trunk and core muscular endurance.

β-Hydroxy-β-Methylbutyrate (HMB) Promotes Neurite Outgrowth in Neuro2a Cells.

PubMed

Salto, Rafael; Vílchez, Jose D; Girón, María D; Cabrera, Elena; Campos, Nefertiti; Manzano, Manuel; Rueda, Ricardo; López-Pedrosa, Jose M

2015-01-01

β-Hydroxy-β-methylbutyrate (HMB) has been shown to enhance cell survival, differentiation and protein turnover in muscle, mainly activating phosphoinositide-3-kinase/protein kinase B (PI3K/Akt) and mitogen-activated protein kinases/ extracellular-signal-regulated kinases (MAPK/ERK) signaling pathways. Since these two pathways are related to neuronal survival and differentiation, in this study, we have investigated the neurotrophic effects of HMB in mouse neuroblastoma Neuro2a cells. In Neuro2a cells, HMB promotes differentiation to neurites independent from any effects on proliferation. These effects are mediated by activation of both the PI3K/Akt and the extracellular-signal-regulated kinases (ERK1/2) signaling as demonstrated by the use of specific inhibitors of these two pathways. As myocyte-enhancer factor 2 (MEF2) family of transcription factors are involved in neuronal survival and plasticity, the transcriptional activity and protein levels of MEF2 were also evaluated. HMB promoted MEF2-dependent transcriptional activity mediated by the activation of Akt and ERK1/2 pathways. Furthermore, HMB increases the expression of brain glucose transporters 1 (GLUT1) and 3 (GLUT3), and mTOR phosphorylation, which translates in a higher protein synthesis in Neuro2a cells. Furthermore, Torin1 and rapamycin effects on MEF2 transcriptional activity and HMB-dependent neurite outgrowth support that HMB acts through mTORC2. Together, these findings provide clear evidence to support an important role of HMB in neurite outgrowth.

β-Hydroxy-β-Methylbutyrate (HMB) Promotes Neurite Outgrowth in Neuro2a Cells

PubMed Central

Girón, María D.; Cabrera, Elena; Campos, Nefertiti; Manzano, Manuel; Rueda, Ricardo; López-Pedrosa, Jose M.

2015-01-01

β-Hydroxy-β-methylbutyrate (HMB) has been shown to enhance cell survival, differentiation and protein turnover in muscle, mainly activating phosphoinositide-3-kinase/protein kinase B (PI3K/Akt) and mitogen-activated protein kinases/ extracellular-signal-regulated kinases (MAPK/ERK) signaling pathways. Since these two pathways are related to neuronal survival and differentiation, in this study, we have investigated the neurotrophic effects of HMB in mouse neuroblastoma Neuro2a cells. In Neuro2a cells, HMB promotes differentiation to neurites independent from any effects on proliferation. These effects are mediated by activation of both the PI3K/Akt and the extracellular-signal-regulated kinases (ERK1/2) signaling as demonstrated by the use of specific inhibitors of these two pathways. As myocyte-enhancer factor 2 (MEF2) family of transcription factors are involved in neuronal survival and plasticity, the transcriptional activity and protein levels of MEF2 were also evaluated. HMB promoted MEF2-dependent transcriptional activity mediated by the activation of Akt and ERK1/2 pathways. Furthermore, HMB increases the expression of brain glucose transporters 1 (GLUT1) and 3 (GLUT3), and mTOR phosphorylation, which translates in a higher protein synthesis in Neuro2a cells. Furthermore, Torin1 and rapamycin effects on MEF2 transcriptional activity and HMB-dependent neurite outgrowth support that HMB acts through mTORC2. Together, these findings provide clear evidence to support an important role of HMB in neurite outgrowth. PMID:26267903

Indian Society of Neuro-Oncology consensus guidelines for the contemporary management of medulloblastoma.

PubMed

Gupta, Tejpal; Sarkar, Chitra; Rajshekhar, Vedantam; Chatterjee, Sandip; Shirsat, Neelam; Muzumdar, Dattatreya; Pungavkar, Sona; Chinnaswamy, Girish; Jalali, Rakesh

2017-01-01

The high success rate in the management medulloblastoma achieved in the western world is not exactly mirrored in developing countries including India. Socio-demographic differences, health-care disparity, and lack in uniformity of care with resultant widespread variations in the clinical practice are some of the reasons that may partly explain this difference in outcomes. Patients with medulloblastoma require a multi-disciplinary team approach involving but not limited to neuro-radiology, neurosurgery; neuropathology, molecular biology, radiation oncology, pediatric medical oncology and rehabilitative services for optimizing outcomes. The Indian Society of Neuro-Oncology (ISNO) constituted an expert multi-disciplinary panel with adequate representation from all stakeholders to prepare national consensus guidelines for the contemporary management of medulloblastoma. Minimum desirable, as well as preferable though optional recommendations (as appropriate), were developed and adopted for the pre-surgical work-up including neuroimaging; neurosurgical management including surgical principles, techniques, and complications; neuropathology reporting and molecular testing; contemporary risk-stratification in the molecular era; appropriate adjuvant therapy (radiotherapy and chemotherapy); and follow-up schedule in medulloblastoma. The current document represents a broad consensus reached amongst various stakeholders within the neuro-oncology community involved in the contemporary curative-intent management of children with medulloblastoma. It provides both general as well as specific guidelines and recommendations to be adopted by physicians and health care providers across India to achieve uniformity of care, improve disease-related outcomes, and compare results between institutions within the country.

Society for Neuro-Oncology 2014 annual meeting updates on central nervous system metastases.

PubMed

Lukas, Rimas V; Mehta, Minesh P; Lesniak, Maciej S

2015-06-01

The 19th Annual Meeting of the Society for Neuro-Oncology (SNO) took place in November of 2014. The focus of many abstracts, as well as the Education Day, was on recent advances in the study of central nervous system (CNS) metastases. Key studies evaluating the factors in tumors and their microenvironment associated with the development and growth of brain metastases are reviewed. Studies investigating the factors that independently influence survival in participants with brain metastases are presented. The Response Assessment for Neuro-Oncology criteria for brain metastases (RANO-BM) and the Neurological Assessment in Neuro-Oncology (NANO) criteria, which were both presented, are recapped. Studies are reviewed evaluating factors that influence survival outcomes in participants with brain metastases who were treated with radiotherapy. Studies investigating the potential risk of radiation necrosis with the combination of radiotherapy and immunotherapies are presented. Brain metastases-focused subset analyses from the ASCEND-1 trial for ALK-translocated non-small cell lung cancer are presented. Preclinical and clinical work on solid tumor leptomeningeal carcinomatosis is also covered. An overview is provided of treatment- related toxicities as well as important concepts that may influence strategies to protect against these toxicities. Key concepts regarding tumor biology, prognostication, response assessment, therapeutic management, and sequelae of treatment for CNS metastases are summarized. Advances in our understanding of the basic and clinical science of CNS metastases have the potential to improve outcomes for patients.

A Human Pluripotent Stem Cell Model of Facioscapulohumeral Muscular Dystrophy-Affected Skeletal Muscles.

PubMed

Caron, Leslie; Kher, Devaki; Lee, Kian Leong; McKernan, Robert; Dumevska, Biljana; Hidalgo, Alejandro; Li, Jia; Yang, Henry; Main, Heather; Ferri, Giulia; Petek, Lisa M; Poellinger, Lorenz; Miller, Daniel G; Gabellini, Davide; Schmidt, Uli

2016-09-01

: Facioscapulohumeral muscular dystrophy (FSHD) represents a major unmet clinical need arising from the progressive weakness and atrophy of skeletal muscles. The dearth of adequate experimental models has severely hampered our understanding of the disease. To date, no treatment is available for FSHD. Human embryonic stem cells (hESCs) potentially represent a renewable source of skeletal muscle cells (SkMCs) and provide an alternative to invasive patient biopsies. We developed a scalable monolayer system to differentiate hESCs into mature SkMCs within 26 days, without cell sorting or genetic manipulation. Here we show that SkMCs derived from FSHD1-affected hESC lines exclusively express the FSHD pathogenic marker double homeobox 4 and exhibit some of the defects reported in FSHD. FSHD1 myotubes are thinner when compared with unaffected and Becker muscular dystrophy myotubes, and differentially regulate genes involved in cell cycle control, oxidative stress response, and cell adhesion. This cellular model will be a powerful tool for studying FSHD and will ultimately assist in the development of effective treatments for muscular dystrophies. This work describes an efficient and highly scalable monolayer system to differentiate human pluripotent stem cells (hPSCs) into skeletal muscle cells (SkMCs) and demonstrates disease-specific phenotypes in SkMCs derived from both embryonic and induced hPSCs affected with facioscapulohumeral muscular dystrophy. This study represents the first human stem cell-based cellular model for a muscular dystrophy that is suitable for high-throughput screening and drug development. ©AlphaMed Press.

Neuro-immune interactions in inflammation and host defense: Implications for transplantation.

PubMed

Chavan, Sangeeta S; Ma, Pingchuan; Chiu, Isaac M

2018-03-01

Sensory and autonomic neurons of the peripheral nervous system (PNS) play a critical role in regulating the immune system during tissue inflammation and host defense. Recent studies have identified the molecular mechanisms underlying the bidirectional communication between the nervous system and the immune system. Here, we highlight the studies that demonstrate the importance of the neuro-immune interactions in health and disease. Nociceptor sensory neurons detect immune mediators to produce pain, and release neuropeptides that act on the immune system to regulate inflammation. In parallel, neural reflex circuits including the vagus nerve-based inflammatory reflex are physiological regulators of inflammatory responses and cytokine production. In transplantation, neuro-immune communication could significantly impact the processes of host-pathogen defense, organ rejection, and wound healing. Emerging approaches to target the PNS such as bioelectronics could be useful in improving the outcome of transplantation. Therefore, understanding how the nervous system shapes the immune response could have important therapeutic ramifications for transplantation medicine. © 2017 The American Society of Transplantation and the American Society of Transplant Surgeons.

Effect of Movement Velocity During Resistance Training on Dynamic Muscular Strength: A Systematic Review and Meta-Analysis.

PubMed

Davies, Timothy B; Kuang, Kenny; Orr, Rhonda; Halaki, Mark; Hackett, Daniel

2017-08-01

Movement velocity is an acute resistance-training variable that can be manipulated to potentially optimize dynamic muscular strength development. However, it is unclear whether performing faster or slower repetitions actually influences dynamic muscular strength gains. We conducted a systematic review and meta-analysis to examine the effect of movement velocity during resistance training on dynamic muscular strength. Five electronic databases were searched using terms related to movement velocity and resistance training. Studies were deemed eligible for inclusion if they met the following criteria: randomized and non-randomized comparative studies; published in English; included healthy adults; used isotonic resistance-exercise interventions directly comparing fast or explosive training to slower movement velocity training; matched in prescribed intensity and volume; duration ≥4 weeks; and measured dynamic muscular strength changes. A total of 15 studies were identified that investigated movement velocity in accordance with the criteria outlined. Fast and moderate-slow resistance training were found to produce similar increases in dynamic muscular strength when all studies were included. However, when intensity was accounted for, there was a trend for a small effect favoring fast compared with moderate-slow training when moderate intensities, defined as 60-79% one repetition maximum, were used (effect size 0.31; p = 0.06). Strength gains between conditions were not influenced by training status and age. Overall, the results suggest that fast and moderate-slow resistance training improve dynamic muscular strength similarly in individuals within a wide range of training statuses and ages. Resistance training performed at fast movement velocities using moderate intensities showed a trend for superior muscular strength gains as compared to moderate-slow resistance training. Both training practices should be considered for novice to advanced, young and older

Changes in muscular fitness and its association with blood pressure in adolescents.

PubMed

Agostinis-Sobrinho, César; Ruiz, Jonatan R; Moreira, Carla; Lopes, Luís; Ramírez-Vélez, Robinson; García-Hermoso, Antonio; Mota, Jorge; Santos, Rute

2018-05-08

The aims of this study were to examine the longitudinal association between muscular fitness (MF) and blood pressure (BP) 2 years later, and to determine whether changes in MF over a 2-year period were associated with BP at follow-up, in adolescents. The sample comprised 734 youths (349 girls) aged from 12 to 18 years. MF was assessed with the standing long jump and handgrip tests. Socioeconomic status, pubertal stage, waist circumference, resting BP, and cardiorespiratory fitness were measured according to standard procedures. Regression analyses showed a significant inverse association between MF at baseline and systolic BP (β = - 0.072; p = 0.032) and rate pressure product (β = - 0.124; p < 0.001) at follow-up, after adjustments for age, sex, height, pubertal stage, and socioeconomic status. However, when analyses were further adjusted for waist circumference and cardiorespiratory fitness, these associations did not remain significant. Adolescents with persistently high and increasing MF exhibited the lowest levels of diastolic BP (F (3, 721)  = 3.814, p = 0.018) and systolic BP (F (3, 721)  = 3.908, p = 0.014) when compared to those with persistent low MF after adjustment for age, sex, height, socioeconomic status, cardiorespiratory fitness, and waist circumference. This study suggests that persistent greater and increasing MF in youth are associated with lower levels of BP across the adolescence. What is Known: • Currently, there is a growing interest on the health benefits of muscular fitness. • Cross-sectional studies have identified an association between muscular fitness and blood pressure in adolescents. What is New: • Changes in muscular fitness during adolescence were associated with systolic and diastolic BP over a 2-year period. • Adolescents with persistently low muscular fitness exhibited the highest levels of diastolic and systolic BP.

The Child with Muscular Dystrophy in School. Revised.

ERIC Educational Resources Information Center

Schock, Nancy C.

Practical information on children with muscular dystrophy is intended to help parents and teachers facilitate their inclusion in mainstreamed classrooms. Major topics addressed include the following: transportation arrangements; providing full information to the teacher regarding the child's specific abilities and physical limitations;…

Satellite Cells in Muscular Dystrophy - Lost in Polarity

PubMed Central

Chang, Natasha C.; Chevalier, Fabien P.; Rudnicki, Michael A.

2016-01-01

Recent findings employing the mdx mouse model for Duchenne muscular dystrophy (DMD) have revealed that muscle satellite stem cells play a direct role in contributing to disease etiology and progression of DMD, the most common and severe form of muscular dystrophy. Lack of dystrophin expression in DMD has critical consequences in satellite cells including an inability to establish cell polarity, abrogation of asymmetric satellite stem cell divisions and failure to enter the myogenic program. Thus, muscle wasting in dystrophic mice is not only caused by myofiber fragility, but is exacerbated by intrinsic satellite cell dysfunction leading to impaired regeneration. Despite intense research and clinical efforts, there is still no effective cure for DMD. In this review, we highlight recent research advances in DMD and discuss the current state of treatment and importantly, how we can incorporate satellite cell-targeted therapeutic strategies to correct satellite cell dysfunction in DMD. PMID:27161598

Integrating molecular markers into the World Health Organization classification of CNS tumors: a survey of the neuro-oncology community.

PubMed

Aldape, Kenneth; Nejad, Romina; Louis, David N; Zadeh, Gelareh

2017-03-01

Molecular markers provide important biological and clinical information related to the classification of brain tumors, and the integration of relevant molecular parameters into brain tumor classification systems has been a widely discussed topic in neuro-oncology over the past decade. With recent advances in the development of clinically relevant molecular signatures and the 2016 World Health Organization (WHO) update, the views of the neuro-oncology community on such changes would be informative for implementing this process. A survey with 8 questions regarding molecular markers in tumor classification was sent to an email list of Society for Neuro-Oncology members and attendees of prior meetings (n=5065). There were 403 respondents. Analysis was performed using whole group response, based on self-reported subspecialty. The survey results show overall strong support for incorporating molecular knowledge into the classification and clinical management of brain tumors. Across all 7 subspecialty groups, ≥70% of respondents agreed to this integration. Interestingly, some variability is seen among subspecialties, notably with lowest support from neuropathologists, which may reflect their roles in implementing such diagnostic technologies. Based on a survey provided to the neuro-oncology community, we report strong support for the integration of molecular markers into the WHO classification of brain tumors, as well as for using an integrated "layered" diagnostic format. While membership from each specialty showed support, there was variation by specialty in enthusiasm regarding proposed changes. The initial results of this survey influenced the deliberations underlying the 2016 WHO classification of tumors of the central nervous system. © The Author(s) 2017. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.

Translational Immuno- and Neuro-imaging Demonstrate Corneal Neuro-immune Crosstalk

PubMed Central

Hamrah, Pedram; Seyed-Razavi, Yashar; Yamaguchi, Takefumi

2017-01-01

Corneal immuno- and neuro-imaging approaches facilitate in vivo analyses of the cornea, including high-resolution imaging of corneal immune cells and nerves. This approach facilitates the analyses of underlying immune and nerve alterations not detected by clinical slit-lamp examination alone. In this review, we describe recent work performed in our translational ocular immunology center with a focus on ‘bench-to-bedside’ and ‘bedside-to-bench’ research. The ability to visualize dendritiform immune cells (DCs) in patients with laser in vivo confocal microscopy (IVCM), recently discovered in the central murine cornea, has allowed us to demonstrated their utility as a potential surrogate biomarker for inflammatory ocular surface diseases. This biomarker for inflammation allows the measurement of therapeutic efficacy of anti-inflammatory drugs and its utility as an endpoint in clinical trials with high inter-observer agreement. IVCM image analyses from our studies demonstrated a significant increase in DC density and size in ocular disease, a positive correlation between DC density and clinical signs and symptoms of disease and pro-inflammatory tear cytokines, and a strong negative correlation between DC density and subbasal nerve density. In conjunction with pre-clinical research investigating the inflammatory state in a partial or fully denervated cornea, our results indicated that corneal nerves are directly involved in the regulation of homeostasis and immune privilege in the cornea. PMID:27631352

Media exposure, mediated social comparison to idealized images of muscularity, and anabolic steroid use.

PubMed

Melki, Jad P; Hitti, Eveline A; Oghia, Michael J; Mufarrij, Afif A

2015-01-01

This study examined the association between anabolic-androgenic steroid (AAS) use and dominant sociocultural factors, specifically media exposure to idealized images of male muscularity, and mediated social comparison trends among a sample of young Arab adults. The study found evidence that participants more exposed to content that promotes muscularity and those who idealize images of muscularity and perceive them as motivators for achieving muscularity are more likely to be AAS users. It also found that a significant percentage of participants used at least one kind of dietary supplement and that the level of AAS use among health club participants indicates it is a significant public health problem in Lebanon. The study suggests that dealing with this problem requires a unique approach, beyond the typical awareness of risks strategy, since some users were well aware of the risks yet continue to use AAS, and their motivations pertain more to body image and sexuality. A stronger approach that utilizes critical media literacy teaching that ingrains these issues into school and university curricula will have a more lasting impact.

Muscular system in the pacific bluefin tuna Thunnus orientalis (Teleostei: Scombridae).

PubMed

Nakae, Masanori; Sasaki, Kunio; Shinohara, Gento; Okada, Tokihiko; Matsuura, Keiichi

2014-02-01

The muscular system in the Pacific bluefin tuna Thunnus orientalis is studied in detail. For the first time, a complete description of the muscular anatomy of a thunnid is provided here. Eighty-two elements including subdivisions of components of the muscular system are identified. This is less than found in a basal perciform and two other investigated scombrid species, owing mainly to the absence or fusion of pectoral, pelvic and caudal fin muscles. The absence of elements of the basal perciform pattern was most prominent in the caudal fin, which includes only the flexor dorsalis, flexor ventralis, hypochordal longitudinalis, and interradialis. In the caudal fin, the medial fan-shaped ray was identified as the first dorsal ray, judging from myological and neuroanatomical characters. The highly developed gill filament muscles in Thunnus orientalis and sheet-like rectus communis control gill ventilation. Long body muscle tendons reduce the metabolic energy needed during rapid and continuous swimming. These characters are interpreted as adaptations in the context of the oceanic life style of the species. Copyright © 2013 Wiley Periodicals, Inc.

Regulatory T cells suppress muscle inflammation and injury in muscular dystrophy

PubMed Central

Villalta, S. Armando; Rosenthal, Wendy; Martinez, Leonel; Kaur, Amanjot; Sparwasser, Tim; Tidball, James G.; Margeta, Marta; Spencer, Melissa J.; Bluestone, Jeffrey A.

2016-01-01

We examined the hypothesis that regulatory T cells (Tregs) modulate muscle injury and inflammation in the mdx mouse model of Duchenne muscular dystrophy (DMD). Although Tregs were largely absent in the muscle of wildtype mice and normal human muscle, they were present in necrotic lesions, displayed an activated phenotype and showed increased expression of interleukin (IL)-10 in dystrophic muscle from mdx mice. Depletion of Tregs exacerbated muscle injury and the severity of muscle inflammation, which was characterized by an enhanced interferon-gamma (IFNγ) response and activation of M1 macrophages. To test the therapeutic value of targeting Tregs in muscular dystrophy, we treated mdx mice with IL-2/anti-IL-2 complexes (IL-2c), and found that Tregs and IL-10 concentrations were increased in muscle, resulting in reduced expression of cyclooygenase-2 and decreased myofiber injury. These findings suggest that Tregs modulate the progression of muscular dystrophy by suppressing type 1 inflammation in muscle associated with muscle fiber injury, and highlight the potential of Treg-modulating agents as therapeutics for DMD. PMID:25320234

Muscular dystrophies due to defective glycosylation of dystroglycan

PubMed Central

Muntoni, F; Brockington, M; Godfrey, C; Ackroyd, M; Robb, S.; Manzur, A; Kinali, M; Mercuri, E; Kaluarachchi, M; Feng, L; Jimenez-Mallebrera, C.; Clement, E; Torelli, S; Sewry, CA; Brown, SC

2007-01-01

Summary Muscular dystrophies are a clinically and genetically heterogeneous group of disorders. Until recently most of the proteins associated with muscular dystrophies were believed to be proteins of the sarcolemma associated with reinforcing the plasma membrane or in facilitating its re-sealing following injury. In the last few years a novel and frequent pathogenic mechanism has been identified that involves the abnormal glycosylation of alpha-dystroglycan (ADG). This peripheral membrane protein undergoes complex and crucial glycosylation steps that enable it to interact with LG domain containing extracellular matrix proteins such as laminins, agrin and perlecan. Mutations in six genes (POMT1, POMT2, POMGnT1, fukutin, FKRP and LARGE) have been identified in patients with reduced glycosylation of ADG. While initially a clear correlation between gene defect and phenotype was observed for each of these 6 genes (for example, Walker Warburg syndrome was associated with mutations in POMT1 and POMT2, Fukuyama congenital muscular dystrophy associated with fukutin mutations, and Muscle Eye Brain disease associated with POMGnT1 mutations), we have recently demonstrated that allelic mutations in each of these 6 genes can result in a much wider spectrum of clinical conditions. Thus, the crucial aspect in determining the phenotypic severity is not which gene is primarily mutated, but how severely the mutation affects the glycosylation of ADG. Systematic mutation analysis of these 6 glycosyltransferases in patients with a dystroglycan glycosylation disorder identifies mutations in approximately 65% suggesting that more genes have yet to be identified. PMID:18646561

Authenticity Anyone? The Enhancement of Emotions via Neuro-Psychopharmacology.

PubMed

Kraemer, Felicitas

2011-04-01

This article will examine how the notion of emotional authenticity is intertwined with the notions of naturalness and artificiality in the context of the recent debates about 'neuro-enhancement' and 'neuro-psychopharmacology.' In the philosophy of mind, the concept of authenticity plays a key role in the discussion of the emotions. There is a widely held intuition that an artificial means will always lead to an inauthentic result. This article, however, proposes that artificial substances do not necessarily result in inauthentic emotions. The literature provided by the philosophy of mind on this subject usually resorts to thought experiments. On the other hand, the recent literature in applied ethics on 'enhancement' provides good reasons to include real world examples. Such case studies reveal that some psychotropic drugs such as antidepressants actually cause people to undergo experiences of authenticity, making them feel 'like themselves' for the first time in their lives. Beginning with these accounts, this article suggests three non-naturalist standards for emotions: the authenticity standard, the rationality standard, and the coherence standard. It argues that the authenticity standard is not always the only valid one, but that the other two ways of assessing emotions are also valid, and that they can even have repercussions on the felt authenticity of emotions. In conclusion, it sketches some of the normative implications if not ethical intricacies that accompany the enhancement of emotions.

Electricity Consumption in the Industrial Sector of Jordan: Application of Multivariate Linear Regression and Adaptive Neuro-Fuzzy Techniques

NASA Astrophysics Data System (ADS)

Samhouri, M.; Al-Ghandoor, A.; Fouad, R. H.

2009-08-01

In this study two techniques, for modeling electricity consumption of the Jordanian industrial sector, are presented: (i) multivariate linear regression and (ii) neuro-fuzzy models. Electricity consumption is modeled as function of different variables such as number of establishments, number of employees, electricity tariff, prevailing fuel prices, production outputs, capacity utilizations, and structural effects. It was found that industrial production and capacity utilization are the most important variables that have significant effect on future electrical power demand. The results showed that both the multivariate linear regression and neuro-fuzzy models are generally comparable and can be used adequately to simulate industrial electricity consumption. However, comparison that is based on the square root average squared error of data suggests that the neuro-fuzzy model performs slightly better for future prediction of electricity consumption than the multivariate linear regression model. Such results are in full agreement with similar work, using different methods, for other countries.

Effects of oral ATP supplementation on anaerobic power and muscular strength.

PubMed

Jordan, Alexander N; Jurca, Radim; Abraham, Edward H; Salikhova, Anna; Mann, Julia K; Morss, Gina M; Church, Timothy S; Lucia, Alejandro; Earnest, Conrad P

2004-06-01

We examined 14 d of oral adenosine 5'-triphosphate (ATP) supplementation on indices of anaerobic capacity and muscular strength. Twenty-seven healthy males successfully completed the trial, after randomly receiving in a double-blind manner an oral dose of low dose (150 mg) or high dose (225 mg) ATP, or matched placebo. To improve absorption characteristics, the ATP was enterically coated. Total blood ATP (whole blood and plasma ATP) concentrations, two Wingate anaerobic power tests (30 s), and muscular strength (1RM and three sets of repetitions to fatigue at 70% of 1RM) were measured under three conditions: (i) baseline; (ii) acutely (7d later, no prior supplementation and 75 min after ATP ingestion); and (iii) after 14 d of daily ingestion (post). Statistical analyses showed no significant between or within group treatment effects for whole blood ATP or plasma ATP concentrations for any treatment condition. We also did not observe any treatment effects for any Wingate testing parameter including peak PO, total work, average PO for 30 s, or post-Wingate lactate accumulation. Overall, we observed no significant between group treatment effects for any muscular strength parameter. We did observe several within group differences for the group ingesting the high ATP dosage including 1RM (6.6%; P < 0.04) and repetitions to fatigue during set 1 of posttesting (18.5%; P < 0.007) and total lifting volume at post (22%; P < 0.003). We conclude that enterically coated oral ATP supplementation may provide small ergogenic effects on muscular strength under some treatment conditions.

Cathepsin S Contributes to the Pathogenesis of Muscular Dystrophy in Mice.

PubMed

Tjondrokoesoemo, Andoria; Schips, Tobias G; Sargent, Michelle A; Vanhoutte, Davy; Kanisicak, Onur; Prasad, Vikram; Lin, Suh-Chin J; Maillet, Marjorie; Molkentin, Jeffery D

2016-05-06

Duchenne muscular dystrophy (DMD) is an X-linked recessive disease caused by mutations in the gene encoding dystrophin. Loss of dystrophin protein compromises the stability of the sarcolemma membrane surrounding each muscle cell fiber, leading to membrane ruptures and leakiness that induces myofiber necrosis, a subsequent inflammatory response, and progressive tissue fibrosis with loss of functional capacity. Cathepsin S (Ctss) is a cysteine protease that is actively secreted in areas of tissue injury and ongoing inflammation, where it participates in extracellular matrix remodeling and healing. Here we show significant induction of Ctss expression and proteolytic activity following acute muscle injury or in muscle from mdx mice, a model of DMD. To examine the functional ramifications associated with greater Ctss expression, the Ctss gene was deleted in the mdx genetic background, resulting in protection from muscular dystrophy pathogenesis that included reduced myofiber turnover and histopathology, reduced fibrosis, and improved running capacity. Mechanistically, deletion of the Ctss gene in the mdx background significantly increased myofiber sarcolemmal membrane stability with greater expression and membrane localization of utrophin, integrins, and β-dystroglycan, which anchor the membrane to the basal lamina and underlying cytoskeletal proteins. Consistent with these results, skeletal muscle-specific transgenic mice overexpressing Ctss showed increased myofiber necrosis, muscle histopathology, and a functional deficit reminiscent of muscular dystrophy. Hence, Ctss induction during muscular dystrophy is a pathologic event that partially underlies disease pathogenesis, and its inhibition might serve as a new therapeutic strategy in DMD. © 2016 by The American Society for Biochemistry and Molecular Biology, Inc.

Atmospheric correction of SeaWiFS ocean color imagery in the presence of absorbing aerosols off the Indian coast using a neuro-variational method

NASA Astrophysics Data System (ADS)

Brajard, J.; Moulin, C.; Thiria, S.

2008-10-01

This paper presents a comparison of the atmospheric correction accuracy between the standard sea-viewing wide field-of-view sensor (SeaWiFS) algorithm and the NeuroVaria algorithm for the ocean off the Indian coast in March 1999. NeuroVaria is a general method developed to retrieve aerosol optical properties and water-leaving reflectances for all types of aerosols, including absorbing ones. It has been applied to SeaWiFS images of March 1999, during an episode of transport of absorbing aerosols coming from pollutant sources in India. Water-leaving reflectances and aerosol optical thickness estimated by the two methods were extracted along a transect across the aerosol plume for three days. The comparison showed that NeuroVaria allows the retrieval of oceanic properties in the presence of absorbing aerosols with a better spatial and temporal stability than the standard SeaWiFS algorithm. NeuroVaria was then applied to the available SeaWiFS images over a two-week period. NeuroVaria algorithm retrieves ocean products for a larger number of pixels than the standard one and eliminates most of the discontinuities and artifacts associated with the standard algorithm in presence of absorbing aerosols.

Stem cell transplantation for treating Duchenne muscular dystrophy: A Web of Science-based literature analysis.

PubMed

Yang, Xiaofeng

2012-08-05

To identify global research trends in stem cell transplantation for treating Duchenne muscular dystrophy using a bibliometric analysis of Web of Science. We performed a bibliometric analysis of studies on stem cell transplantation for treating Duchenne muscular dystrophy from 2002 to 2011 retrieved from Web of Science. (a) peer-reviewed published articles on stem cell transplantation for treating Duchenne muscular dystrophy indexed in Web of Science; (b) original research articles, reviews, meeting abstracts, proceedings papers, book chapters, editorial material, and news items; and (c) publication between 2002 and 2011. (a) articles that required manual searching or telephone access; (b) documents that were not published in the public domain; and (c) corrected papers. (1) Annual publication output; (2) distribution according to subject areas; (3) distribution according to journals; (4) distribution according to country; (5) distribution according to institution; (6) distribution according to institution in China; (7) distribution according to institution that cooperated with Chinese institutions; (8) top-cited articles from 2002 to 2006; (9) top-cited articles from 2007 to 2011. A total of 318 publications on stem cell transplantation for treating Duchenne muscular dystrophy were retrieved from Web of Science from 2002 to 2011, of which almost half derived from American authors and institutes. The number of publications has gradually increased over the past 10 years. Most papers appeared in journals with a focus on gene and molecular research, such as Molecular Therapy, Neuromuscular Disorders, and PLoS One. The 10 most-cited papers from 2002 to 2006 were mostly about different kinds of stem cell transplantation for muscle regeneration, while the 10 most-cited papers from 2007 to 2011 were mostly about new techniques of stem cell transplantation for treating Duchenne muscular dystrophy. The publications on stem cell transplantation for treating Duchenne muscular

Endoscopic trans-nasal approach for biopsy of orbital tumours using image-guided neuro-navigation system.

PubMed

Sieskiewicz, A; Lyson, T; Mariak, Z; Rogowski, M

2008-05-01

Histopathological diagnosis of intraorbital tumours is of crucial value for planning further therapy. The aim of the study was to explore clinical utility of image-guided endoscopy for biopsy of orbital tumours. Trans-nasal endoscopic biopsy of intraorbital mass lesions was performed in 6 patients using a neuro-navigation system (Medtronic Stealth Station Treon plus). The CT and MRI 1 mm slice images were fused by the system in order to visualise both bony and soft tissue structures. The anatomic fiducial registration protocol was used during the procedure. All lesions were precisely localised and the biopsies could be taken from the representative part of the pathological mass. None of the patients developed aggravation of ocular symptoms after the procedure. The operative corridor as well as the size of orbital wall fenestration could be limited to a minimum. The accuracy of neuro-navigation remained high and stable during the entire procedure. The image-guided neuro-navigation system facilitated endoscopic localisation and biopsy of intraorbital tumours and contributed to the reduction of surgical trauma during the procedure. The technique was particularly useful in small, medially located, retrobulbar tumours and in unclear situations when the structure of the lesion resembled surrounding intraorbital tissue.

H2S Regulates Hypobaric Hypoxia-Induced Early Glio-Vascular Dysfunction and Neuro-Pathophysiological Effects

PubMed Central

Kumar, Gaurav; Chhabra, Aastha; Mishra, Shalini; Kalam, Haroon; Kumar, Dhiraj; Meena, Ramniwas; Ahmad, Yasmin; Bhargava, Kalpana; Prasad, Dipti N.; Sharma, Manish

2016-01-01

Hypobaric Hypoxia (HH) is an established risk factor for various neuro-physiological perturbations including cognitive impairment. The origin and mechanistic basis of such responses however remain elusive. We here combined systems level analysis with classical neuro-physiological approaches, in a rat model system, to understand pathological responses of brain to HH. Unbiased ‘statistical co-expression networks’ generated utilizing temporal, differential transcriptome signatures of hippocampus—centrally involved in regulating cognition—implicated perturbation of Glio-Vascular homeostasis during early responses to HH, with concurrent modulation of vasomodulatory, hemostatic and proteolytic processes. Further, multiple lines of experimental evidence from ultra-structural, immuno-histological, substrate-zymography and barrier function studies unambiguously supported this proposition. Interestingly, we show a significant lowering of H2S levels in the brain, under chronic HH conditions. This phenomenon functionally impacted hypoxia-induced modulation of cerebral blood flow (hypoxic autoregulation) besides perturbing the strength of functional hyperemia responses. The augmentation of H2S levels, during HH conditions, remarkably preserved Glio-Vascular homeostasis and key neuro-physiological functions (cerebral blood flow, functional hyperemia and spatial memory) besides curtailing HH-induced neuronal apoptosis in hippocampus. Our data thus revealed causal role of H2S during HH-induced early Glio-Vascular dysfunction and consequent cognitive impairment. PMID:27211559

A novel multi-model neuro-fuzzy-based MPPT for three-phase grid-connected photovoltaic system

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

Chaouachi, Aymen; Kamel, Rashad M.; Nagasaka, Ken

This paper presents a novel methodology for Maximum Power Point Tracking (MPPT) of a grid-connected 20 kW photovoltaic (PV) system using neuro-fuzzy network. The proposed method predicts the reference PV voltage guarantying optimal power transfer between the PV generator and the main utility grid. The neuro-fuzzy network is composed of a fuzzy rule-based classifier and three multi-layered feed forwarded Artificial Neural Networks (ANN). Inputs of the network (irradiance and temperature) are classified before they are fed into the appropriated ANN for either training or estimation process while the output is the reference voltage. The main advantage of the proposed methodology,more » comparing to a conventional single neural network-based approach, is the distinct generalization ability regarding to the nonlinear and dynamic behavior of a PV generator. In fact, the neuro-fuzzy network is a neural network based multi-model machine learning that defines a set of local models emulating the complex and nonlinear behavior of a PV generator under a wide range of operating conditions. Simulation results under several rapid irradiance variations proved that the proposed MPPT method fulfilled the highest efficiency comparing to a conventional single neural network and the Perturb and Observe (P and O) algorithm dispositive. (author)« less

BMI as a mediator of the relationship between muscular fitness and cardiometabolic risk in children: a mediation analysis.

PubMed

Díez-Fernández, Ana; Sánchez-López, Mairena; Gulías-González, Roberto; Notario-Pacheco, Blanca; Cañete García-Prieto, Jorge; Arias-Palencia, Natalia; Martínez-Vizcaíno, Vicente

2015-01-01

Muscular fitness levels have been associated with cardiometabolic risk in children, although whether body weight acts as a confounder or as an intermediate variable in this relationship remains controversial. The aim of this study was to examine whether the association between muscular fitness and cardiometabolic risk factors is mediated by body mass index (BMI). Cross-sectional study using a sample of 1158 schoolchildren aged 8-11 years from the province of Cuenca, Spain. We measured anthropometrics and biochemical variables and we calculated a muscular fitness index as the sum of z-scores of handgrip dynamometry/weight and standing long jump, and we estimated a previously validated cardiometabolic risk index (CMRI). Linear regression models were fitted for mediation analysis to assess whether the association between muscular fitness and cardiometabolic risk was mediated by BMI. Children with normal weight (NW) had a better cardiometabolic risk profile than their overweight (OW) or obese (OB) peers after controlling for muscular fitness. Marginal estimated mean ± SE values for NW, OW and OB categories of CMRI were -0.75 ± 0.06 < 0.84 ± 0.10 < 2.18 ± 0.16 in boys and -0.73 ± 0.06 < 0.96 ± 0.10 < 2.71 ± 0.17 in girls, both p < 0.001. Children with higher levels of muscular fitness had a better cardiometabolic risk profile (CMRI marginal estimated mean ± SE 1.04 ± 0.13 > 0.05 ± 0.09 >-1.16 ± 0.13 for lower, middle and upper quartiles of muscular fitness in boys and 1.01 ± 0.16 > 0.10 ± 0.09 > -1.02 ± 0.15 in girls, both p < 0.001), but differences disappeared when controlling for BMI. BMI acted as a full mediator between muscular fitness and most cardiometabolic risk factors (Sobel test z = -11.44 for boys; z = -11.83 for girls; p < 0.001 in CMRI mediation model) and as a partial mediator in the case of waist circumference (Sobel test z=-14.86 for boys; z=-14.51 for girls; p<0.001). BMI mediates the association between muscular fitness and

[Percutaneous closure of ductus arteriosus and muscular ventricular defect with amplatzer occluder in a patient with severe pulmonary hypertension].

PubMed

García-Montes, José Antonio; Zabal Cerdeira, Carlos; Calderón-Colmenero, Juan; Espínola, Nilda; Fernández de la Reguera, Guillermo; Buendía Hernández, Alfonso

2005-01-01

Surgical treatment of multiple muscular ventricular septal defects with associated lesions and severe pulmonary hypertension has a high morbility and mortality. Closure of these defects by the Amplatzer muscular VSD occluder is an alternative to surgery, avoiding the need of cardiopulmonary bypass. We present the case of a 38 year-old woman with signs of heart failure in NYHA functional class IV, with two muscular ventricular septal defects, patent ductus arteriosus and severe pulmonary hypertension, that were treated with three Amplatzer muscular VSD occluders, with significant reduction of pulmonary pressure and functional class improvement.

The effect of pentoxifylline on spontaneous and experimental metastasis of the mouse Neuro2a neuroblastoma.

PubMed

Amirkhosravi, A; Warnes, G; Biggerstaff, J; Malik, Z; May, K; Francis, J L

1997-07-01

Pentoxifylline (PTX) has been reported to have both direct and indirect anti-tumor effects in experimental tumor models. We studied the effect of PTX on (1) the proliferation of Neuro2a mouse neuroblastoma cells in vitro and in vivo, (2) spontaneous and experimental metastasis, (3) tumor cell membrane fluidity and (4) adhesion to a fibronectin-coated surface. PTX significantly reduced the proliferation of Neuro2a cells in vitro as determined by DNA measurement (P < 0.01) and total cell count (P < 0.02). In vivo, PTX reduced the growth of subcutaneously transplanted primary tumors in syngeneic A/J mice (P < 0.01; n = 15). All seven animals (100%) receiving intravenous tumor cells developed extensive liver metastasis. In contrast, only 1/11 (9%) of animals pre-treated with oral PTX and injected with PTX-treated cells developed liver metastases. Of five mice receiving PTX-treated cells without oral pretreatment of PTX, two out of five (40%) developed liver metastases. There was a slight, but not significant (P = 0.08) increase in both experimental and spontaneous lung metastases formation in PTX-treated animals. However, tumor nodule formation on the lung surface was inefficient. PTX also increased membrane fluidity of the Neuro2a cells and significantly decreased tumor cell adhesion to fibronectin-coated microtiter wells (P < 0.01). We conclude that PTX has a cytostatic effect on the Neuro2a mouse neuroblastoma and exerts an anti-tumor effect on liver metastases following intravenous administration of neuroblastoma cells. Whether these results are directly related to the changes in membrane properties caused by pentoxifylline remains to be established.

Prevalence and Characteristics of Chinese Patients With Duchenne and Becker Muscular Dystrophy

PubMed Central

Lo, Ivan F. M.; Cherk, Sharon W. W.; Cheng, Wai Wai; Fung, Eva L. W.; Yeung, Wai Lan; Ngan, Mary; Lee, Wing Cheong; Kwong, Ling; Wong, Suet Na; Ma, Che Kwan; Tai, Shuk Mui; Ng, Grace S. F.; Wu, Shun Ping; Wong, Virginia C. N.

2015-01-01

The aim of this collaborative study on Duchenne muscular dystrophy and Becker muscular dystrophy is to determine the prevalence and to develop data on such patients as a prelude to the development of registry in Hong Kong. Information on clinical and molecular findings, and patient care, was systematically collected in 2011 and 2012 from all Pediatric Neurology Units in Hong Kong. Ninety patients with dystrophinopathy were identified, and 83% has Duchenne muscular dystrophy. The overall prevalence of dystrophinopathy in Hong Kong in 2010 is 1.03 per 10 000 males aged 0 to 24 years. Among the Duchenne group, we observed a higher percentage (40.6%) of point mutations with a lower percentage (45.3%) of exon deletions in our patients when compared with overseas studies. Although we observed similar percentage of Duchenne group received scoliosis surgery, ventilation support, and cardiac treatment when compared with other countries, the percentage (25%) of steroid use is lower. PMID:28503591

Whole-body vibration training in children with Duchenne muscular dystrophy and spinal muscular atrophy.

PubMed

Vry, Julia; Schubert, Isabel J; Semler, Oliver; Haug, Verena; Schönau, Eckhard; Kirschner, Janbernd

2014-03-01

Whole-body-vibration training is used to improve muscle strength and function and might therefore constitute a potential supportive therapy for neuromuscular diseases. To evaluate safety of whole-body vibration training in ambulatory children with Duchenne muscular dystrophy (DMD) and spinal muscular atrophy (SMA). 14 children with DMD and 8 with SMA underwent an 8-week vibration training programme on a Galileo MedM at home (3 × 3 min twice a day, 5 days a week). Primary outcome was safety of the training, assessed clinically and by measuring serum creatine kinase levels. Secondary outcome was efficacy as measured by changes in time function tests, muscle strength and angular degree of dorsiflexion of the ankles. All children showed good clinical tolerance. In boys with DMD, creatine kinase increased by 56% after the first day of training and returned to baseline after 8 weeks of continuous whole-body vibration training. No changes in laboratory parameters were observed in children with SMA. Secondary outcomes showed mild, but not significant, improvements with the exception of the distance walked in the 6-min walking test in children with SMA, which rose from 371.3 m to 402.8 m (p < 0.01). Whole-body vibration training is clinically well tolerated in children with DMD and SMA. The relevance of the temporary increase in creatine kinase in DMD during the first days of training is unclear, but it is not related to clinical symptoms or deterioration. Copyright © 2013 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

A new case of limb girdle muscular dystrophy 2G in a Greek patient, founder effect and review of the literature.

PubMed

Brusa, Roberta; Magri, Francesca; Papadimitriou, Dimitra; Govoni, Alessandra; Del Bo, Roberto; Ciscato, Patrizia; Savarese, Marco; Cinnante, Claudia; Walter, Maggie C; Abicht, Angela; Bulst, Stefanie; Corti, Stefania; Moggio, Maurizio; Bresolin, Nereo; Nigro, Vincenzo; Comi, Giacomo Pietro

2018-04-13

Limb girdle muscular dystrophy (LGMD) type 2G is a rare form of muscle disease, described only in a few patients worldwide, caused by mutations in TCAP gene, encoding the protein telethonin. It is characterised by proximal limb muscle weakness associated with distal involvement of lower limbs, starting in the first or second decade of life. We describe the case of a 37-year-old woman of Greek origin, affected by disto-proximal lower limb weakness. No cardiac or respiratory involvement was detected. Muscle biopsy showed myopathic changes with type I fibre hypotrophy, cytoplasmic vacuoles, lipid overload, multiple central nuclei and fibre splittings; ultrastructural examination showed metabolic abnormalities. Next generation sequencing analysis detected a homozygous frameshift mutation in the TCAP gene (c.90_91del), previously described in one Turkish family. Immunostaining and Western blot analysis showed complete absence of telethonin. Interestingly, Single Nucleotide Polymorphism analysis of the 10 Mb genomic region containing the TCAP gene showed a shared homozygous haplotype of both the Greek and the Turkish patients, thus suggesting a possible founder effect of TCAP gene c.90_91del mutation in this part of the Mediterranean area. Copyright © 2018 Elsevier B.V. All rights reserved.

Swallow Characteristics in Patients with Oculopharyngeal Muscular Dystrophy

ERIC Educational Resources Information Center

Palmer, Phyllis M.; Neel, Amy T.; Sprouls, Gwyneth; Morrison, Leslie

2010-01-01

Purpose: This prospective investigation evaluates oral weakness and its impact on swallow function, weight, and quality of life in patients with oculopharyngeal muscular dystrophy (OPMD). Method: Intraoral pressure, swallow pressure, and endurance were measured using an Iowa Oral Performance Instrument in participants with OPMD and matched…

Genetic modifiers of muscular dystrophy act on sarcolemmal resealing and recovery from injury

PubMed Central

Quattrocelli, Mattia; Capote, Joanna; Ohiri, Joyce C.; Warner, James L.; Vo, Andy H.; Hadhazy, Michele; Demonbreun, Alexis R.; Spencer, Melissa J.; McNally, Elizabeth M.

2017-01-01

Genetic disruption of the dystrophin complex produces muscular dystrophy characterized by a fragile muscle plasma membrane leading to excessive muscle degeneration. Two genetic modifiers of Duchenne Muscular Dystrophy implicate the transforming growth factor β (TGFβ) pathway, osteopontin encoded by the SPP1 gene and latent TGFβ binding protein 4 (LTBP4). We now evaluated the functional effect of these modifiers in the context of muscle injury and repair to elucidate their mechanisms of action. We found that excess osteopontin exacerbated sarcolemmal injury, and correspondingly, that loss of osteopontin reduced injury extent both in isolated myofibers and in muscle in vivo. We found that ablation of osteopontin was associated with reduced expression of TGFβ and TGFβ-associated pathways. We identified that increased TGFβ resulted in reduced expression of Anxa1 and Anxa6, genes encoding key components of the muscle sarcolemma resealing process. Genetic manipulation of Ltbp4 in dystrophic muscle also directly modulated sarcolemmal resealing, and Ltbp4 alleles acted in concert with Anxa6, a distinct modifier of muscular dystrophy. These data provide a model in which a feed forward loop of TGFβ and osteopontin directly impacts the capacity of muscle to recover from injury, and identifies an intersection of genetic modifiers on muscular dystrophy. PMID:29065150

Genetic modifiers of muscular dystrophy act on sarcolemmal resealing and recovery from injury.

PubMed

Quattrocelli, Mattia; Capote, Joanna; Ohiri, Joyce C; Warner, James L; Vo, Andy H; Earley, Judy U; Hadhazy, Michele; Demonbreun, Alexis R; Spencer, Melissa J; McNally, Elizabeth M

2017-10-01

Genetic disruption of the dystrophin complex produces muscular dystrophy characterized by a fragile muscle plasma membrane leading to excessive muscle degeneration. Two genetic modifiers of Duchenne Muscular Dystrophy implicate the transforming growth factor β (TGFβ) pathway, osteopontin encoded by the SPP1 gene and latent TGFβ binding protein 4 (LTBP4). We now evaluated the functional effect of these modifiers in the context of muscle injury and repair to elucidate their mechanisms of action. We found that excess osteopontin exacerbated sarcolemmal injury, and correspondingly, that loss of osteopontin reduced injury extent both in isolated myofibers and in muscle in vivo. We found that ablation of osteopontin was associated with reduced expression of TGFβ and TGFβ-associated pathways. We identified that increased TGFβ resulted in reduced expression of Anxa1 and Anxa6, genes encoding key components of the muscle sarcolemma resealing process. Genetic manipulation of Ltbp4 in dystrophic muscle also directly modulated sarcolemmal resealing, and Ltbp4 alleles acted in concert with Anxa6, a distinct modifier of muscular dystrophy. These data provide a model in which a feed forward loop of TGFβ and osteopontin directly impacts the capacity of muscle to recover from injury, and identifies an intersection of genetic modifiers on muscular dystrophy.

Muscular and Cardiorespiratory Fitness in Homeschool versus Public School Children.

PubMed

Kabiri, Laura S; Mitchell, Katy; Brewer, Wayne; Ortiz, Alexis

2017-08-01

The growth and unregulated structure of homeschooling creates an unknown population in regard to muscular and cardiorespiratory fitness. The purpose of this research was to compare muscular and cardiorespiratory fitness between elementary school aged homeschool and public school children. Homeschool children ages 8-11 years old (n = 75) completed the curl-up, 90° push-up, and Progressive Aerobic Capacity Endurance Run (PACER) portions of the FitnessGram to assess abdominal and upper body strength and endurance as well as cardiorespiratory fitness. Comparisons to public school children (n = 75) were made using t tests and chi-square tests. Homeschool children showed significantly lower abdominal (t(148) = -11.441, p < .001; χ 2 (1) = 35.503, p < .001) and upper body (t(148) = -3.610, p < .001; χ 2 (1) = 4.881, p = .027) strength and endurance. There were no significant differences in cardiorespiratory fitness by total PACER laps (t(108) = 0.879, p = .381) or estimated VO 2max (t(70) = 1.187, p = .239; χ 2 (1) = 1.444, p = .486). Homeschool children showed significantly lower levels of both abdominal and upper body muscular fitness compared with their age and gender matched public school peers but no difference in cardiorespiratory fitness.

Treatment of Glioma Using neuroArm Surgical System

PubMed Central

2016-01-01

The use of robotic technology in the surgical treatment of brain tumour promises increased precision and accuracy in the performance of surgery. Robotic manipulators may allow superior access to narrow surgical corridors compared to freehand or conventional neurosurgery. This paper reports values and ranges of tool-tissue interaction forces during the performance of glioma surgery using an MR compatible, image-guided neurosurgical robot called neuroArm. The system, capable of microsurgery and stereotaxy, was used in the surgical resection of glioma in seven cases. neuroArm is equipped with force sensors at the end-effector allowing quantification of tool-tissue interaction forces and transmits force of dissection to the surgeon sited at a remote workstation that includes a haptic interface. Interaction forces between the tool tips and the brain tissue were measured for each procedure, and the peak forces were quantified. Results showed maximum and minimum peak force values of 2.89 N (anaplastic astrocytoma, WHO grade III) and 0.50 N (anaplastic oligodendroglioma, WHO grade III), respectively, with the mean of peak forces varying from case to case, depending on type of the glioma. Mean values of the peak forces varied in range of 1.27 N (anaplastic astrocytoma, WHO grade III) to 1.89 N (glioblastoma with oligodendroglial component, WHO grade IV). In some cases, ANOVA test failed to reject the null hypothesis of equality in means of the peak forces measured. However, we could not find a relationship between forces exerted to the pathological tissue and its size, type, or location. PMID:27314044

Neuro-adaptive backstepping control of SISO non-affine systems with unknown gain sign.

PubMed

Ramezani, Zahra; Arefi, Mohammad Mehdi; Zargarzadeh, Hassan; Jahed-Motlagh, Mohammad Reza

2016-11-01

This paper presents two neuro-adaptive controllers for a class of uncertain single-input, single-output (SISO) nonlinear non-affine systems with unknown gain sign. The first approach is state feedback controller, so that a neuro-adaptive state-feedback controller is constructed based on the backstepping technique. The second approach is an observer-based controller and K-filters are designed to estimate the system states. The proposed method relaxes a priori knowledge of control gain sign and therefore by utilizing the Nussbaum-type functions this problem is addressed. In these methods, neural networks are employed to approximate the unknown nonlinear functions. The proposed adaptive control schemes guarantee that all the closed-loop signals are semi-globally uniformly ultimately bounded (SGUUB). Finally, the theoretical results are numerically verified through simulation examples. Simulation results show the effectiveness of the proposed methods. Copyright © 2016 ISA. All rights reserved.

Rapidly progressive heart failure requiring transplantation in muscular dystrophy: a need for frequent screening.

PubMed

Pick, Justin M; Ellis, Zachary D; Alejos, Juan C; Chang, Anthony C

2017-11-01

Fukuyama congenital muscular dystrophy weakens both skeletal and cardiac muscles, but the rate of cardiomyopathic progression can accelerate faster than that of skeletal muscles. A 14-year-old boy with Fukuyama congenital muscular dystrophy presented with mild skeletal myopathy but severe cardiomyopathy requiring heart transplantation within 1 year of declining heart function. These patients need frequent screening regardless of musculoskeletal symptoms.

A Cross-Sectional Study of School Experiences of Boys with Duchenne and Becker Muscular Dystrophy

ERIC Educational Resources Information Center