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Atypical double nerve lesion after humeral fracture: Diagnosis by ultrasoundEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
No abstract. (Source: Muscle and Nerve)
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Giovanna Liotta, Giuseppe Granata, Alessia Librante, Antonella Di Pasquale, Pietro Caliandro, Carlo Martinoli, Luca Padua Source Type: journals

Diabetic neuropathy: Electrophysiological and morphological study of peripheral nerve degeneration and regeneration in transgenic mice that express IFN[beta] in [beta] cellsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study was undertaken to characterize the degenerative and regenerative responses of peripheral nerves after induced sciatic nerve damage in transgenic rat insulin I promoter / human interferon beta (RIP/IFN[beta]) mice made diabetic with a low dose of streptozotocin (STZ) as an animal model of diabetic complications. In vivo, histological and immunohistological studies of cutaneous and sciatic nerves were performed after left sciatic crush. Functional tests, cutaneous innervation, and sciatic nerve evaluation showed pronounced neurological reduction in all groups 2 weeks after crush. All animals showed a gradual recov...
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Anna Serafín, Jessica Molín, Merce Márquez, Ester Blasco, Enric Vidal, Laia Foradada, Sonia Añor, Rosa M. Rabanal, Dolors Fondevila, Fàtima Bosch, Martí Pumarola Source Type: journals

Chemotherapy is successful in sporadic late onset nemaline myopathy (SLONM) with monoclonal gammopathyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
No abstract. (Source: Muscle and Nerve)
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Jan Novy, Anne Rosselet, Olivier Spertini, Johannes Alexander Lobrinus, Thomas Pabst, Thierry Kuntzer Source Type: journals

Multilevel ulnar neuropathy caused by multiple intraneural hemangiomasEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report the case of a 47-year-old man with multilevel ulnar neuropathy caused by multiple intraneural hemangiomas in an ipsilateral ulnar nerve. Multilevel ulnar neuropathy was detected by electrodiagnosis, and intraneural hemangiomas were suggested by ultrasonographic evaluation before the operation. Careful surgical excision under an operating microscope ameliorated the patient's symptoms without recurrence. Muscle Nerve, 2010 (Source: Muscle and Nerve)
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Dong Hwee Kim, Jong Woo Kang, Jong Woong Park Source Type: journals

Ocular myasthenia gravis in a senior population: Diagnosis, therapy, and prognosisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The objectives of this study were (I) to explore the prognosis of ocular myasthenia gravis (OMG) in patients with onset at age 70 years and above (i.e. senior persons); (2) to identify predictors of secondary generalization in this age group; and 3) to address the effects of immunotherapy on this population of patients. We performed a retrospective analysis of 39 patients with myasthenia gravis who presented with only ocular signs and symptoms after age 70 years. Generalized myasthenia gravis (GMG) developed in 12 OMG patients (31%). None of the GMG patients required ventilator assistance or a feeding tube, Of the 12 ocula...
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Jeffrey A. Allen, Stephanie Scala, H. Royden Jones Source Type: journals

Genetic variability in the myostatin gene does not explain the muscle hypertrophy and clinical penetrance in myotonia congenitaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
No abstract. (Source: Muscle and Nerve)
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Viviane P. Muniz, Adriano S. Senkevics, Dinorah Zilbersztajn, Juliana Gurgel-Giannetti, Helga C. Silva, Lydia U. Yamamoto, Rita C.M. Pavanello, Peter L. Pearson, Mayana Zatz, Mariz Vainzof Source Type: journals

Acute-onset chronic inflammatory demyelinating polyneuropathy with cranial nerve involvement, dysautonomia, respiratory failure, and autoantibodiesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We examined a 27-year-old woman who developed rapidly progressive quadriplegia and acute respiratory failure that required mechanical ventilation in the intensive care unit. It was unclear whether this was a presentation of Guillain-Barré syndrome (GBS) or acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP). Remarkable features included multiple cranial nerve involvement, respiratory failure, dysautonomia, and skin manifestations. Several autoantibodies were elevated, including antinuclear (ANA), anticardiolipin (aCL), thyroid, and calcium-sensing receptor (CaSR) autoantibodies. The patient was ...
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Philippe Hantson, Luc Kevers, Nicole Fabien, Peter Van Den Bergh Source Type: journals

The illusion of severe carpal tunnel syndrome (CTS)Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this report we present a young woman with mild bilateral CTS, based on electrophysiological studies, in whom marked thenar atrophy was on a congenital basis related to the VATER association (vertebral anomalies, anal atresia, tracheoesophageal fistula, and radial or renal abnormalities). Muscle Nerve, 2010 (Source: Muscle and Nerve)
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Ludwig Gutmann, Christopher Nance Source Type: journals

A Patient with neurofibromatosis type 1 and Charcot-Marie-Tooth disease type 1BEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We describe a patient with both neurofibromatosis type 1 and Charcot-Marie-Tooth disease type 1B. Although one might expect an overwhelming tumor burden due to the combination of these two disorders, the two mutations did not appear to interact. Muscle Nerve, 2010 (Source: Muscle and Nerve)
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Eric Lancaster, Lauren B. Elman, Steven S. Scherer Source Type: journals

Diffuse large B-cell lymphoma presenting as piriformis syndromeEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Piriformis syndrome (PS) is a rare condition characterized by pain and paresthesia of the buttock, often radiating to the posterior thigh. A patient with sciatica that was clinically suspicious for PS, underwent diagnostic work-up. A diagnosis of diffuse large B-cell lymphoma with neurolymphomatosis (NL) was made. To our knowledge, this is the first report of NL presenting as PS. NL is a possible cause of secondary PS. Muscle Nerve, 2010 (Source: Muscle and Nerve)
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Byoung Seok Ye, Il Nam Sunwoo, Bum Chun Suh, Jong-Pil Park, Dong-Suk Shim, Seung Min Kim Source Type: journals

Schwann cells as a source of insulin-like growth factor-1 for extraocular musclesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Precise force regulation is fundamentally important for extraocular muscle (EOM) function. Insulin-like growth factor-1 (IGF-1) plays a major role in EOM force regulation, but the source of endogenous IGF-1 is unclear. Multiple IGF-1 sources may supply EOMs, including: the EOM itself; the systemic circulation; innervating motoneurons; and Schwann cells within nerves. IGF-1 expression was measured in chicken during oculomotor system maturation by using real-time polymerase chain reaction (PCR). Accumulation of radiolabeled IGF-1 in EOMs was compared after either injection into the vascular circulation or into the trochlear ...
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Chengyuan Feng, Christopher S. Von Bartheld Source Type: journals

Redetermination of the optimal stimulation intensity modifies resting H-reflex recovery after a sustained moderate-intensity muscle contractionEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study aimed to determine whether the time-course of maximal resting H-reflex amplitude (Hmax) recovery after a prolonged moderate-intensity muscle contraction differs according to the optimal stimulation intensity used (predetermined vs. readjusted). Thirteen males performed a sustained isometric plantar flexion at 40% of their maximal voluntary contraction torque output until exhaustion. Hmax of the soleus muscle was recorded before and 2, 6, 10, and 14 min after the end of the contraction, then normalized by the respective maximal M-wave to form the Hmax/Mmax ratio. During recovery, pre- and redetermined optimal sti...
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Thomas Rupp, Olivier Girard, Stéphane Perrey Source Type: journals

Evidence of gender-specific motor templates to resist valgus loading at the kneeEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study, we sought to explore the effect of gender on volitional and reflex neuromuscular responses to a rapid valgus perturbation at the knee applied under "intervene" and "do not intervene" conditions. Multiple 7° ramp-and-hold valgus perturbations were applied at the neutrally extended knee of 12 male and 12 female healthy subjects, while surface electromyography over the quadriceps and hamstrings recorded the neuromuscular response. Volitional responses did not vary between groups, perhaps reflecting the relative novelty of the loading direction. However, reflex responses observed under the "do not intervene" pa...
Source: Muscle and Nerve - November 13, 2009 Category: Internal Medicine Authors: Martha L. Cammarata, Yasin Y. Dhaher Source Type: journals

Clinical and electrophysiological parameters distinguishing acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study suggests that patients presenting acutely with a demyelinating polyneuropathy and the aforementioned clinical features should be closely monitored as they may be more likely to have CIDP at follow-up. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Annie Dionne, Michael W. Nicolle, Angelika F. Hahn Source Type: journals

Martin-Gruber anastomosis with anomalous superficial radial innervation to ulnar dorsum of hand: A pitfall when common variants coexistEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The Martin-Gruber anastomosis (MGA) is the most common anatomic variation in the upper extremity. Anomalous superficial radial innervation to the ulnar dorsum of the hand is the most common cause of an absent dorsal ulnar cutaneous (DUC) response. The coexistence of these variants introduces a relatively common yet underrecognized potential pitfall in nerve conduction studies (NCS). We performed confirmatory NCS in two cases referred for ulnar neuropathy in the forearm (case 1) and at the elbow (UNE, case 2). Initial NCS in both cases suggested ulnar nerve injury at the forearm and elbow, respectively, based on an apparent...
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: A. Arturo Leis, Ivana Stetkarova, Keionna J. Wells Source Type: journals

Clinical and electrophysiological parameters that distinguishing acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study suggests that patients presenting acutely with a demyelinating polyneuropathy and the aforementioned clinical features should be closely monitored as they may be more likely to have CIDP at follow-up. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Annie Dionne, Michael W. Nicolle, Angelika F. Hahn Source Type: journals

Kinetics of neuromuscular changes during low-frequency electrical stimulationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The purpose of the study was to examine the time course of neuromuscular fatigue components during a low-frequency electrostimulation (ES) session. Three bouts of 17 trains of stimulation at 30 HZ (4 s on, 6 s off) were used to electrically induce fatigue in the plantar flexor muscles. Before and after every 17-train bout, torque, electromyographic activity [expressed as root mean square (RMS) and median frequency (MF) values], evoked potentials (M-wave and H-reflex), and the level of voluntary activation (LOA, using twitch interpolation technique) were assessed. Torque during maximal voluntary contraction decreased signif...
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Maria Papaiordanidou, David Guiraud, Alain Varray Source Type: journals

Magnetic resonance imaging diagnosis of panniculitis in dermatomyositisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
No abstract. (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Shoji Hemmi, Ryutaro Kushida, Hirotake Nishimura, Tatsufumi Murakami, Yoshihide Sunada Source Type: journals

Clinical findings in MuSK-antibody positive myasthenia gravis: A U.S. experienceEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We performed a retrospective chart review on 53 muscle-specific kinase antibody (MuSK-Ab)-positive myasthenia gravis (MG) patients at nine university-based centers in the U.S. Of these, 66% were Caucasian, 85% were women, and age of onset was 9-79 years. Twenty-seven patients were nonresponsive to anticholinesterase therapy. Myasthenia Gravis Foundation of America improvement status was achieved in 53% patients on corticosteroids, 51% with plasma exchange, and in 20% on intravenous immunoglobulin (IVIG). Thymectomy was beneficial in 7/18 patients at 3 years. Long-term ([ge]3 years) outcome was very favorable in 58% of pati...
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Mamatha Pasnoor, Gil I. Wolfe, Sharon Nations, Jaya Trivedi, Richard J. Barohn, Laura Herbelin, April McVey, Mazen Dimachkie, John Kissel, Ronan Walsh, Anthony Amato, Tahseen Mozaffar, Marcel Hungs, Luis Chui, Jonathan Goldstein, Steven Novella, Ted Burns Source Type: journals

Coactivation at the ankle joint is not sufficient to estimate agonist and antagonist mechanical contributionEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The aim of this study was to assess, via an electromyographic (EMG) biofeedback method, the mechanical contribution of both agonist and antagonist muscles during maximal voluntary contraction (MVC). We compared this original method with the MVC-EMGmax ratio and the torque/EMG relationship method, both of which are commonly used to estimate antagonist torque. The plantarflexion (PF) and dorsiflexion (DF) MVCs were measured simultaneously with EMG activity of triceps surae (TS) and tibialis anterior in 15 young adults (mean age 23 years). Antagonist torques obtained from the torque/EMG relationship and EMG biofeedback method...
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Maxime Billot, Emilie Simoneau, Jacques Van Hoecke, Alain Martin Source Type: journals

Joint angle dependence of intermuscle difference in postactivation potentiationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The purpose of this study was to examine the effect of ankle joint angle on the intermuscle difference in postactivation potentiation (PAP) between the medial gastrocnemius (MG) and soleus (SOL) muscles. At the neutral position of joint angle, dorsiflexion of 20°, and plantarflexion of 20°, twitch responses were evoked by stimulating the posterior tibial nerve with supramaximal intensity before and after a 10-s maximal voluntary plantarflexion at each joint angle. Mechanical properties of the MG and SOL muscles were assessed simultaneously and separately by using mechanomyography (MMG), and the extent of potentiation of ...
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Naokazu Miyamoto, Naotoshi Mitsukawa, Norihide Sugisaki, Tetsuo Fukunaga, Yasuo Kawakami Source Type: journals

Coexistent autoimmune autonomic ganglionopathy and myasthenia gravis associated with non-small-cell lung cancerEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report the case of a 55-year-old man with non-small-cell lung cancer who underwent radiation, chemotherapy with carbotaxol and paclitaxel, and left upper lobe removal 2 years prior to evaluation. He was referred for disabling orthostatic hypotension (113/69 mm Hg supine and 66/47 mm Hg standing after 10 minutes) without a compensatory heart rate increase (57 to 59 beats per minute), fatigue, and constipation with episodes of ileus. Clinical examination showed mild ptosis bilaterally, fatiguable neck flexor weakness, and hip flexor weakness. Blood pressure response to Valsalva maneuver was abnormal with an absence of pha...
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Amanda C. Peltier, Bonnie K. Black, Satish R. Raj, Peter Donofrio, David Robertson, Italo Biaggioni Source Type: journals

Unusual Charcot-Marie-Tooth phenotype due to a mutation within the intracellular domain of myelin protein zeroEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report a family with a missense mutation, c.700G>T p.Asp234Tyr (deviant nomenclature: c.670G>T, p.Asp224Tyr), within the intracellular domain of myelin protein zero, who has distal sensorimotor symptoms, cramps, restless legs syndrome, neuropathic pain, and carpal tunnel syndrome. The index patient responded to intravenous immunoglobulin and immunosuppression, so there may be a possible secondary autoimmune process, probably triggered by altered antigen presentation due to mutated MPZ protein. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Christiane Schneider-Gold, Judith Kötting, Jörg T. Epplen, Ralf Gold, Wanda M. Gerding Source Type: journals

Tibialis anterior branch involvement in fibular intraneural gangliaEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study characterizes the cystic involvement of this tibialis anterior branch and evaluates its significance. The magnetic resonance imaging (MRI) and clinical data of 23 patients with fibular intraneural ganglia were retrospectively reviewed. The tibialis anterior branch was consistently involved with the cyst, and this involvement, although variable, was more prominent than the cystic involvement of other terminal branches of the fibular nerve. The finding of cyst extension within a muscle end-organ branch seems likely to explain, in part, the characteristic clinical finding of preferential foot drop in patients with ...
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Marie-Noëlle Hébert-Blouin, Kimberly K. Amrami, Huan Wang, John A. Skinner, Robert J. Spinner Source Type: journals

Homozygosity for dominant mutations increases severity of muscle channelopathiesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report herein the first cases of homozygous patients for sodium channel mutations responsible for paramyotonia congenita (I1393T) or hypokalemic periodic paralysis (R1132Q). A parallel was drawn between this unprecedented situation and that of myotonia congenita by including patients homozygous or heterozygous for the CLCN1 I556N channel mutation, which is known for incomplete dominance and penetrance. Standardized electromyographic (EMG) protocols combining exercise and cold served as provocative tests to compare homozygotes with heterozygotes for each of the three mutations. Surface-recorded compound muscle action pot...
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Marianne Arzel-hézode, Damien Sternberg, Nacira Tabti, Savine Vicart, Cyril Goizet, Bruno Eymard, Bertrand Fontaine, Emmanuel Fournier Source Type: journals

Muscle imaging analogies in a cohort of patients with different clinical phenotypes caused by LMNA gene mutationsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
This study demonstrates that LMNA-gene-mutated patients devoid of any clinically detectable skeletal muscle involvement have the same pattern of leg muscle involvement as patients with overt skeletal muscle compromise. This finding suggests the presence of a continuum of skeletal muscle involvement among phenotypes of LMNA-gene-mutation-related skeletalmyopathy and cardiomyopathy. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Nicola Carboni, Marco Mura, Giovanni Marrosu, Eleonora Cocco, Stefano Marini, Elisabetta Solla, Anna Mateddu, Maria Antonietta Maioli, Rachele Piras, Giorgio Mallarini, Giuseppe Mercuro, Maurizio Porcu, Maria Giovanna Marrosu Source Type: journals

Early effects of carbachol on the morphology of motor endplates of mammalian skeletal muscle fibersEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Long-term disturbance of the calcium homeostasis of motor endplates (MEPs) causes necrosis of muscle fibers. The onset of morphological changes in response to this disturbance, particularly in relation to the fiber type, is presently unknown. Omohyoid muscles of mice were incubated for 1-30 minutes in 0.1 mM carbachol, an acetylcholine agonist that causes an inward calcium current. In these muscles, the structural changes of the sarcomeres and the MEP sarcoplasm were evaluated at the light- and electron-microscopic level. Predominantly in type I fibers, carbachol incubation resulted in strong contractures of the sarcomeres...
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Tilman Voigt Source Type: journals

HIV lumbosacral radiculoplexus neuropathy mimicking lymphoma: Diffuse infiltrative lymphocytosis syndrome (DILS) restricted to nerve?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Diffuse infiltrative lymphocytosis syndrome (DILS) is a hyperimmune reaction against HIV. It leads to MHC-restricted clonal expansion of CD8 T cells characterized by circulating CD8 hyperlymphocytosis and CD8 T-cell infiltration in organs. Our patient presented with painful lumbosacral radiculoplexus neuropathy and tested positive for HIV. Nerve biopsy showed large collections of CD8 lymphocytes suspicious for lymphoma. Symptoms, signs, and repeat biopsy improved with antiretroviral treatment. The presentation and treatment response suggest that this case is localized DILS. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: Nizar Chahin, Zelalem Temesgen, Paul J. Kurtin, Robert J. Spinner, P. James B. Dyck Source Type: journals

Driving with polyneuropathyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Polyneuropathy may result in pain, numbness, and weakness, which may in turn affect driving ability. Medications used to treat neuropathic pain may alter cognition, which may further affect driving. Although such impairments have engendered questions about the driving safety in this group of patients, the rate of motor vehicle accidents (MVAs) in patients with neuropathy has not been studied rigorously. We surveyed patients with neuropathy from three medical centers for reported accident rate, and we analyzed variables related to increased risk for accidents compared to National Highway Traffic Safety Administration data. ...
Source: Muscle and Nerve - October 30, 2009 Category: Internal Medicine Authors: S. Charles Cho, Hans D. Katzberg, Anil Rama, Byung-Jo Kim, Hakjae Roh, Jongsoo Park, Jonathan Katz, Yuen T. So Source Type: journals

Rituximab in the management of refractory myasthenia gravisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study, patients with refractory MG who were treated with rituximab were identified. A review of patients referred to the Yale Neuromuscular Clinic was performed. Patients with refractory MG who were treated with rituximab were reviewed for response to treatment. Patients who had muscle-specific kinase (MuSK+) or acetylcholine receptor (AChR+) antibodies were included. Six patients were identified who met the criteria described. All patients tolerated rituximab without side effects and had a reduced need for immunosuppressants and/or improvement in clinical function. Patients with refractory MG appeared to respond t...
Source: Muscle and Nerve - October 21, 2009 Category: Internal Medicine Authors: Nazlee Zebardast, Huned S. Patwa, Steven P. Novella, Jonathan M. Goldstein Source Type: journals

Sporadic late onset nemaline myopathy responsive to IVIg and immunotherapyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report two HIV-negative SLONM/monoclonal gammopathy patients who improved following intravenous immunoglobulin (IVIg) treatment alone or in combination with immunosuppressant agents. This favorable response to treatment suggests that a dysimmune mechanism is operative in some SLONM individuals. We suggest that IVIg deserves initial consideration for SLONM therapy. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 21, 2009 Category: Internal Medicine Authors: Margherita Milone, Amiram Katz, Anthony A. Amato, Carl A. Soderland, Miruna Segarceanu, Nathan P. Young, H. Royden Jones Source Type: journals

Reply to: Myotonic disorder without myotonia?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
No abstract. (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 21, 2009 Category: Internal Medicine Authors: Margherita Milone, Jasper R. Daube Source Type: journals

Does display sensitivity influence motor latency determination?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
It is known that display sensitivity can affect onset latency measurement of compound muscle action potentials (CMAPs). We compared two display sensitivities with respect to intrarater and interrater reliability and measured to what degree display sensitivity influences the motor latency. We asked seven raters to mark the onset points of median CMAPs from 24 patients printed with display sensitivities of 5 mV/division and 0.5 mV/division on paper. The onset latencies were measured, and intraclass correlation coefficients (ICCs) were calculated. Most of the raters had excellent intrarater repeatability within a single sensi...
Source: Muscle and Nerve - October 7, 2009 Category: Internal Medicine Authors: Nobushige Takahashi, Lawrence R. Robinson Source Type: journals

Factors associated with response to calcineurin inhibitors in myasthenia gravisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The objective was to assess which clinical factors of patients with myasthenia gravis (MG) are associated with responsiveness to calcineurin inhibitors (CNIs, cyclosporine and tacrolimus). We retrospectively analyzed the 6-month effects of CNIs in 62 MG patients. We excluded the influence of other immune treatments and determined factors associated with response to CNIs. The frequency of patients who achieved neither a [ge]3-point reduction in quantitative MG score nor a [ge]25% reduction in daily dose of prednisolone (poor responders) reached 35.5% (22/62) and 64.5% (40/62), respectively, compared with patients who achiev...
Source: Muscle and Nerve - October 7, 2009 Category: Internal Medicine Authors: Yuriko Nagane, Shigeaki Suzuki, Norihiro Suzuki, Kimiaki Utsugisawa Source Type: journals

Use of evans blue dye to compare limb muscles in exercised young and old mdx miceEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Evans blue dye (EBD) is used to mark damaged and permeable muscle fibers in mouse models of muscular dystrophy and as an endpoint in therapeutic trials. We counted EBD-positive muscle fibers and extracted EBD from muscles sampled throughout the hindlimbs in young adult and old mdx mice to determine if the natural variability in morphology would allow measurement of a functional improvement in one limb compared to the contralateral limb. Following one bout of rotarod or treadmill exercise that greatly increased serum creatine kinase levels, the number of EBD+ muscle fibers in 12-19-month-old mdx mice increased 3-fold, EBD i...
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Christine I. Wooddell, Guofeng Zhang, Jacob B. Griffin, Julia O. Hegge, Thierry Huss, Jon A. Wolff Source Type: journals

Ros-mediated activation of NF-[kappa]B and Foxo during muscle disuseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We examined reactive oxygen species as upstream activators of nuclear factor [kappa]B; (NF-[kappa]B) and forkhead box O (Foxo) in skeletal muscle during disuse atrophy. Catalase, an enzyme that degrades H2O2, was overexpressed in soleus muscles via plasmid injection prior to 7 days of hindlimb immobilization. The increased catalase activity abolished immobilization-induced transactivation of both NF-[kappa]B and Foxo and attenuated the loss of muscle mass. Thus, H2O2 may be an important initiator of these signaling pathways that lead to muscle atrophy. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Stephen L. Dodd, Brittany J. Gagnon, Sarah M. Senf, Brian A. Hain, Andrew R. Judge Source Type: journals

Central nervous system involvement in axonal Charcot-Marie-Tooth diseaseEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
No abstract. (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Xinli Du, G. Frederick Wooten, Julie A. Matsumoto, Lawrence H. Phillips II Source Type: journals

Facioscapulohumeral muscular dystrophy: Do neurotrophins play a role?Email this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Although the molecular defect of facioscapulohumeral muscular dystrophy (FSHD) is well established and involves the contraction of the polymorphic 3.3 kb D4Z4 repeat on the subtelomeric region of chromosome 4q35, the pathologic effects of this deletion remain largely unknown. As a consequence, no specific treatment for FSHD is at present available. Thus, there is the need to explore new areas in an attempt to better characterize pathophysiological alterations in FSHD that might be useful for managing the disease. Neurotrophins (nerve growth factor, brain-derived neurotrophic factor, neurotrophin-3, and neurotrophin-4/5) ar...
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Francesco Angelucci, Luca Colantoni Source Type: journals

Effects of exercise and muscle type on BDNF, NT-4/5, and TrKB expression in skeletal muscleEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Muscle-derived neurotrophins are thought to contribute to the adaptation of skeletal muscle to exercise, but the effects of brief exercise interventions on BDNF, NT-4/5, and trkB are not understood. RNA was extracted for RT-PCR from soleus and medial gastrocnemius of Sprague-Dawley rats exercised on a treadmill at speeds up to 20 m/min at 5% incline for 5 or 10 days. BDNF expression was elevated in soleus following 5 days (184%, P < 0.001) but not 10 days of exercise. NT-4/5 and trkB were not affected at either time-point. BDNF mRNA was significantly higher in soleus at rest when compared with medial gastrocnemius (193%, P...
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Daniel I. Ogborn, Phillip F. Gardiner Source Type: journals

Syrian hamster infected with Leishmania infantum: A new experimental model for inflammatory myopathiesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Idiopathic inflammatory myopathies (IIMs) are inflammatory disorders of unknown origin. On the basis of clinical, histopathological, and immunological features, they can be differentiated into three major and distinct subsets: dermatomyositis; polymyositis; and inclusion-body myositis. Although a few animal models for IIM are currently available, they lack several characteristic aspects of IIMs. The aim of our study was to examine skeletal muscle involvement in an experimental animal model of visceral leishmaniasis, a disseminated infection caused by the protozoan parasite Leishmania infantum, and to compare features of as...
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Orlando Paciello, Slawomir Wojcik, Luigi Gradoni, Gaetano Oliva, Francesca Trapani, Valentina Iovane, Luisa Politano, Serenella Papparella Source Type: journals

Sonographic measurements of longitudinal median nerve sliding in patients following nerve repairEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Nerve sliding may be restricted following nerve repair. This could result in increased tension across the repair site and lead to poor functional recovery of the nerve. Ultrasound was used to examine longitudinal median nerve sliding in 10 patients who had previously undergone nerve repair surgery following complete division of the median nerve. The median longitudinal movement in the forearm in response to metacarpophalangeal (MCP) joint movements was 2.15 mm on the injured side, compared with 2.54 mm on the uninjured side, a difference that was significant. There was a significant reduction in nerve sliding following rep...
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Ertan Erel, Andrew Dilley, Sarah Turner, Prem Kumar, Waqar A. Bhatti, Vivien C. Lees Source Type: journals

Added sampling improves reproducibility of multipoint motor unit estimatesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Motor unit number estimation (MUNE) has been used to track motor unit attrition. Studies have used the modified multiple-point stimulation (MPS) technique, collecting three surface motor unit action potentials (sMUAPs) from 3 sites to calculate MUNE. Factoring additional sMUAPs should theoretically improve reproducibility, but the optimal number has not been defined. We evaluated the effect of increased sMUAP sampling on test-retest reproducibility of the modified MPS MUNE technique and found that MUNE reproducibility increased with additional sampling. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Namita Goyal, Johnny S. Salameh, Laura E. Baldassari, William S. David Source Type: journals

Non-lethal neonatal neuromuscular variant of glycogenosis type IV with novel GBE1 mutationsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report a recent case of the severe congenital variant of glycogen storage disease type IV with prolonged survival. The patient was found to be a compound heterozygote for two novel mutations, a missense mutation in exon 5 (p.H188P, c.563A>C) and a severe mutation in intron 5 (c.691+2T>C). We propose that the genotype and the quality of medical care may account for the severe but non-lethal phenotype. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Carla Fernandez, Cécile Halbert, André Maues De Paula, Valerié Lacroze, Roseline Froissart, Dominique Figarella-Branger, Brigitte Chabrol, Jean-François Pellissier Source Type: journals

Conduction block of peripheral nerve using high-frequency alternating currents delivered through an intrafascicular electrodeEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
In this study we show that high-frequency conduction block is feasible using intrafascicular electrodes. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: D. Michael Ackermann Jr, Emily L. Foldes, Niloy Bhadra, Kevin L. Kilgore Source Type: journals

Sensory ataxic neuropathy dysarthria and ophthalmoparesis (SANDO) in a sibling pair with a homozygous p.A467T POLG mutationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Two siblings who developed fifth-decade-onset, concurrent progressive sensory ataxia, dysarthria, and ophthalmoparesis were found to be homozygous for the p.A467T mutation of the polymerase gamma (POLG) gene. The clinical course in both subjects was progression to severe disability. The enlarging spectrum of sensory ataxic neuropathies associated with mitochondrial DNA (mtDNA) instability and POLG mutations should be recognized and considered in the differential diagnosis of this unusual presentation. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: John C. McHugh, Roisin Lonergan, Rachel Howley, Killian O'Rourke, Robert W. Taylor, Michael Farrell, Michael Hutchinson, Sean Connolly Source Type: journals

Muscle strength and fatigue in patients with generalized myasthenia gravisEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The objective of this study was to quantify, by direct measurement of muscle force, the strength and fatigue of patients with MG. A maximal voluntary isometric contraction protocol of shoulder abductors was used in conjunction with conventional fatigue and disease-severity instruments. Results from patients with (D-MG) and without (ND-MG) decrement on repetitive nerve stimulation (RNS) of the spinal accessory and axillary nerves were compared with healthy controls. Patients with MG reported greater fatigue than controls. Muscle strength was lowest in the D-MG group, followed by the ND-MG group and controls. Normalized shou...
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Caitlin J. Symonette, Bradley V. Watson, Wilma J. Koopman, Michael W. Nicolle, Timothy J. Doherty Source Type: journals

Linearity and reliability of the mechanomyographic amplitude versus dynamic torque relationships for the superficial quadriceps femoris musclesEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
The purpose of this investigation was to examine the linearity and reliability of the mechanomyographic (MMG) amplitude versus dynamic torque relationships for the vastus lateralis (VL), rectus femoris (RF), and vastus medialis (VM) muscles. Nine healthy men and 11 healthy women performed submaximal to maximal, concentric, isokinetic muscle actions of the leg extensors at 30° s-1 on two occasions. Surface MMG signals were detected from the VL, RF, and VM of the dominant thigh during both trials. The ranges of the coefficients of determination for the MMG amplitude versus dynamic torque relationships were 0.01-0.94 for the...
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Matthew S. Stock, Travis W. Beck, Jason M. DeFreitas, Michael A. Dillon Source Type: journals

Immune-mediated necrotizing myopathy associated with statinsEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report patients from two neuromuscular centers who were evaluated between the years 2000 and 2008 and met the following criteria: (1) proximal muscle weakness occurring during or after treatment with statins; (2) elevated serum creatine kinase (CK); (3) persistence of weakness and elevated CK despite discontinuation of the statin; (4) improvement with immunosuppressive agents; and (5) muscle biopsy showing necrotizing myopathy without significant inflammation. Twenty-five patients fulfilled our inclusion criteria. Twenty-four patients required multiple immunosuppressive agents. Fifteen patients relapsed after being tape...
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Phyllis Grable-Esposito, Hans D. Katzberg, Steven A. Greenberg, Jayashri Srinivasan, Jonathan Katz, Anthony A. Amato Source Type: journals

Frequency and predictors of nonalcoholic fatty liver disease in myotonic dystrophyEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
Nonalcoholic fatty liver disease (NAFLD) is a common chronic liver disease that is strongly associated with insulin resistance. Myotonic dystrophy (DM1) is the most common form of adult-onset muscular dystrophy, and there is a high frequency of insulin resistance due to insulin receptor mRNA splicing defects in muscle tissue. The frequency and predictors of NAFLD in this population have not been described. Thirty-six patients with DM1 were prospectively assessed for the presence of NAFLD and insulin resistance. NAFLD was defined by abnormal liver chemistry tests with ultrasound or pathologic evidence of steatosis in the ab...
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Kenneth Shieh, James M. Gilchrist, Kittichai Promrat Source Type: journals

Primary AL amyloid polyneuropathy successfully treated with high-dose melphalan followed by autologous stem cell transplantationEmail this article to a colleague. Save this article to My Clippings. Discuss or comment on this article.
We report 2 patients with polyneuropathy associated with amyloid derived from light chains (AL) who were treated successfully with high-dose melphalan followed by autologous peripheral blood stem cell transplantation (HDM/SCT). Neuropathic symptoms improved in conjunction with normalization of serum-free light chains. In addition to amyloid deposits in tissues, an amyloidogenic light chain itself produced by abnormal plasma cells might be harmful to peripheral nerve function, and thus HDM/SCT seems to be a promising therapy for primary AL amyloid polyneuropathy. Muscle Nerve, 2009 (Source: Muscle and Nerve)
Source: Muscle and Nerve - October 6, 2009 Category: Internal Medicine Authors: Nagaaki Katoh, Masayuki Matsuda, Takuhiro Yoshida, Masahide Yazaki, Hiroshi Morita, Kazuo Sakashita, Shu-Ichi Ikeda Source Type: journals