Clinical, physiological, and histological features in a kindred with the T3271C melas mutation.

abstract：:Microdrop samples of endoneurial fluid and serum from galactosemic rats were analyzed with energy dispersive spectrometry (EDS). The sodium concentration of endoneurial fluid from galactose-fed rats (295 meq/liter) was nearly twice that of age-matched controls (152 meq/liter) and indicates that sodium is present in an...

journal_title：Muscle & nerve

authors： Mizisin AP,Myers RR,Powell HC

更新日期：1986-06-01 00:00:00

abstract：:Limb-girdle muscular dystrophy 2D (LGMD2D) is caused by mutations in the alpha-sarcoglycan gene (SGCA). The most frequently reported mutation, 229CGC>TGC (R77C) in exon 3 of SGCA, results in the substitution of arginine by cysteine. We present here the clinical, immunohistochemical, and genetic data of 11 Finnish pati...

journal_title：Muscle & nerve

authors： Hackman P,Juvonen V,Sarparanta J,Penttinen M,Aärimaa T,Uusitalo M,Auranen M,Pihko H,Alén R,Junes M,Lönnqvist T,Kalimo H,Udd B

更新日期：2005-02-01 00:00:00

abstract：INTRODUCTION:The aim of this study was to examine the coping strategies of 49 patients with amyotrophic lateral sclerosis (ALS) and the relationships of these strategies to their perceived health-related quality of life (HRQoL). METHODS:Forty-nine subjects were assessed for collection of demographic and medical data. ...

journal_title：Muscle & nerve

authors： Montel S,Albertini L,Spitz E

更新日期：2012-01-01 00:00:00

abstract：:Clinicopathologic findings from two golden retriever dogs with an inherited, progressive, degenerative muscle disease that were studied until 27 and 40 months of age are described. Initial clinical signs included stilted gait and simultaneous advancement of their pelvic limbs. Further gait restriction and muscle hyper...

journal_title：Muscle & nerve

authors： Kornegay JN,Tuler SM,Miller DM,Levesque DC

更新日期：1988-10-01 00:00:00

abstract：:To investigate whether various myelin markers could detect pathological changes in myelination, the activity of 2'-3'-cyclic nucleotide 3'-phosphodiesterase (CNP) and the level of PO protein were compared in the peripheral nervous system (PNS) of the dystrophic mouse and chicken and their phenotypically normal sibling...

journal_title：Muscle & nerve

authors： Lewis LN,Mezei C

更新日期：1985-02-01 00:00:00

abstract：:This study attempts to gain a better understanding of the primary cause of doxorubicin myotoxicity to aid in the development of a protocol to increase its effectiveness in treating muscle spasm diseases. The time course of acute injury to the orbicularis oculi muscle after injection of doxorubicin into the eyelids of ...

journal_title：Muscle & nerve

authors： McLoon LK,Luo XX,Wirtschafter J

更新日期：1993-07-01 00:00:00

abstract：:The dystrophin protein complex (DPC), composed of at least 10 proteins that associate with dystrophin, is critical for the maintenance of normal muscle fiber structure and physiology. In this study, we used immunohistochemistry and confocal microscopy to examine the relative abundance and distribution of several of th...

journal_title：Muscle & nerve

authors： Draviam R,Billington L,Senchak A,Hoffman EP,Watkins SC

更新日期：2001-02-01 00:00:00

abstract：:Mitochondrial trifunctional protein (TFP) deficiency is a rare disorder of the fatty acid beta-oxidation cycle with heterogeneous phenotypes and occurs secondary to either alpha- or beta-subunit mutations. We characterized the neuromyopathic phenotype of TFP deficiency through adolescence or adulthood in 11 patients, ...

journal_title：Muscle & nerve

authors： Spiekerkoetter U,Bennett MJ,Ben-Zeev B,Strauss AW,Tein I

更新日期：2004-01-01 00:00:00

abstract：:Electromyographic (EMG) recordings may serve an important role in predicting torque during repetitive activation of paralyzed muscle. We compared the initial M-wave to the subsequent M-waves of the same train under fatigued and recovered conditions in the paralyzed human soleus muscle. Sixteen individuals with chronic...

journal_title：Muscle & nerve

authors： Chang YJ,Shields RK

更新日期：2002-11-01 00:00:00

abstract：INTRODUCTION:Slowly progressive, genetic neuromuscular diseases (gNMDs) often lead to important motor deficiencies and functional limitations. The Quality of Life in Genetic Neuromuscular Disease Questionnaire (QoL-gNMD) is a new health-related quality-of-life questionnaire developed for these patients. The purpose of ...

journal_title：Muscle & nerve

authors： Dany A,Rapin A,Lavrard B,Saoût V,Réveillère C,Bassez G,Tiffreau V,Péréon Y,Sacconi S,Eymard B,Dramé M,Jolly D,Novella JL,Hardouin JB,Boyer FC

更新日期：2017-12-01 00:00:00

abstract：:We report electrophysiological findings of conduction along peripheral sensory fibers in 64 patients with amyotrophic lateral sclerosis. Distribution of the values of action potential amplitudes and conduction velocities of peripheral afferent fibers were significantly lower than in normal age-matched controls. Sensor...

journal_title：Muscle & nerve

authors： Mondelli M,Rossi A,Passero S,Guazzi GC

更新日期：1993-02-01 00:00:00

abstract：:Ischemia-reperfusion injury is implicated in the failure of free flap and replant surgeries and is associated with the pathogenesis of a wide variety of clinical diseases including stroke, myocardial infarction, spinal injury, and compartment syndromes. We used a skeletal muscle flap model to test if the induction of ...

journal_title：Muscle & nerve

authors： Lille S,Su CY,Schoeller T,Suchy H,Lyons S,Russell RC,Neumeister M,Lai CC

更新日期：1999-03-01 00:00:00

abstract：:Deletions of muscle mitochondrial DNA are known in mitochondrial myopathy patients who have chronic progressive external ophthalmoplegia (CPEO). A 41-year-old patient with no apparent family history of this condition suffers from hypertrophic cardiomyopathy, slight muscle atrophy, and weakness of the extremities, but ...

journal_title：Muscle & nerve

authors： Takei Y,Ikeda S,Yanagisawa N,Takahashi W,Sekiguchi M,Hayashi T

更新日期：1995-11-01 00:00:00

abstract：INTRODUCTION:The ability to distinguish between normal thymus, thymic hyperplasia, and thymoma should aid clinical management and decision making in patients with myasthenia gravis (MG). We sought to determine the accuracy of routine imaging in predicting thymic pathology. METHODS:We retrospectively analyzed records o...

journal_title：Muscle & nerve

authors： Klimiec E,Quirke M,Leite MI,Hilton-Jones D

更新日期：2018-02-09 00:00:00

abstract：:Clinicians who use electromyographic (EMG) signals to help determine the presence or absence of abnormality in a muscle often, with varying degrees of success, evaluate sets of motor unit potentials (MUPs) qualitatively and/or quantitatively to characterize the muscle in a clinically meaningful way. The resulting musc...

journal_title：Muscle & nerve

authors： Pino LJ,Stashuk DW,Boe SG,Doherty TJ

更新日期：2010-01-01 00:00:00

abstract：INTRODUCTION:Pes cavus often signals the presence of Charcot-Marie-Tooth (CMT) in adult patients, although its prevalence in the general population makes it a finding of unclear significance. METHODS:We undertook a pilot double cohort study to investigate the feasibility of comparing preselected bedside and radiograph...

journal_title：Muscle & nerve

authors： Stino AM,Atway S,Anthony M,Kline D,Kissel JT

更新日期：2019-01-01 00:00:00

abstract：:Phospholipid methylation is thought to modulate such vital cellular processes as calcium transport, receptor function, and membrane microviscosity. As these processes are fundamental to the function of muscle cells and are thought to be altered in disease states, we have characterized several features of phospholipid ...

journal_title：Muscle & nerve

authors： Kuncl RW,Drachman DB,Kishimoto Y

更新日期：1985-06-01 00:00:00

abstract：:The objective of this study was to determine the self-reported prevalence of needlestick injuries among practicing electromyographers. In January 2008, an anonymous electronic survey was sent to all active members of the American Association for Neuromuscular and Electrodiagnostic Medicine (AANEM) who provided e-mail ...

journal_title：Muscle & nerve

authors： Mateen FJ,Grant IA,Sorenson EJ

更新日期：2008-12-01 00:00:00

abstract：INTRODUCTION:In this study we estimated the costs paid by U.S. health plans for treating myasthenia gravis (MG) in 2009 and determined the major cost drivers. METHODS:One hundred thirteen MG patients were matched by propensity scores with 339 non-MG patients from a comprehensive health-care insurance database. The mea...

journal_title：Muscle & nerve

authors： Guptill JT,Sharma BK,Marano A,Soucy A,Krueger A,Sanders DB

更新日期：2012-03-01 00:00:00

abstract：:In order to investigate whether Schwann cell or myelin was the principal antigen responsible for nerve graft rejection, fresh nerve grafts and those in which myelin had been previously allowed to degenerate (predegenerate grafts) from both isogeneic BALB/c and allogeneic C57/B1 mice were inserted into trembler BALB/c ...

journal_title：Muscle & nerve

authors： Pollard JD,McLeod JG

更新日期：1981-07-01 00:00:00

abstract：:A review of our current knowledge of the etiology and pathogenesis of myasthenia gravis is presented, with particular emphasis on the immunological aspects of the disease. Part 1, published in this issue, deals with the clinical and genetic features of myasthenia gravis which led to the autoimmune theory of the etiolo...

journal_title：Muscle & nerve

authors： Simpson JA

更新日期：1978-01-01 00:00:00

abstract：:The muscle-specific helix-loop-helix (HLH) transcription factors myoD, myogenin, MRF4, and myf-5 are called the muscle regulatory factor family (MRF). Levels of MRFs are strongly regulated by muscle electrical activity and are thought to control downstream genes that are important for muscle phenotype such as the acet...

journal_title：Muscle & nerve

authors： Carlsen H,Gundersen K

更新日期：2000-09-01 00:00:00

abstract：:Nerve guides seeded with Schwann cells (SCs) promote axonal regeneration in peripheral nerve lesions. We examined the applicability of bioluminescent imaging (BLI) for monitoring the fate of SCs in nerve guides after implantation. Rat SCs were transfected with the firefly luciferase (Fluc) gene and subsequently seeded...

journal_title：Muscle & nerve

authors： Ma MS,Van Dam G,Meek M,Boddeke E,Copray S

更新日期：2009-11-01 00:00:00

abstract：:Muscle-fiber loss is a characteristic of many progressive neuromuscular disorders. Over the past decade, identification of a growing number of apoptosis-associated factors and events in pathological skeletal muscle provided increasing evidence that apoptotic cell-death mechanisms account significantly for muscle-fiber...

journal_title：Muscle & nerve

authors： Tews DS

更新日期：2005-10-01 00:00:00

abstract：BACKGROUND:Despite use of qualitative laryngeal electromyography (LEMG) guided botulinum toxin A (BoNT-A) injection for treatment of adductor spasmodic dysphonia (AdSD), unsatisfactory injections and complete "misses" remain problematic. We aimed to determine if the quantitative LEMG measure of number of small segments...

journal_title：Muscle & nerve

authors： Dwyer CD,Leclerc AA,Nandedkar SD,Young VN,Rosen CA

更新日期：2020-12-31 00:00:00

abstract：INTRODUCTION:Psoriasis is a T-cell-mediated skin disorder with uncommon extracutaneous manifestations. Rare patients with psoriasis and myopathy have been reported. METHODS:We conducted a retrospective review of medical records of psoriasis patients seen at the Mayo Clinic during the period from January 1, 1996 to May...

journal_title：Muscle & nerve

authors： Liewluck T,Ernste FC,Tracy JA

更新日期：2013-11-01 00:00:00

abstract：:Many post-polio patients develop new muscle weakness decades after the initial illness. However, its mechanism and treatment are controversial. The purpose of this study was to test the hypotheses that: (1) after strength training, post-polio patients show strength improvement comparable to that seen in the healthy el...

journal_title：Muscle & nerve

authors： Chan KM,Amirjani N,Sumrain M,Clarke A,Strohschein FJ

更新日期：2003-03-01 00:00:00

abstract：:Although the molecular defect causing Becker muscular dystrophy (BMD) has been identified, the biochemical mechanisms that lead to muscle necrosis remain unclear. Exercise-related muscle metabolism in 9 mildly affected BMD patients was assessed by muscle 31-phosphorus magnetic resonance spectroscopy ((31)P MRS) during...

journal_title：Muscle & nerve

authors： Tosetti M,Linsalata S,Battini R,Volpi L,Cini C,Presciutti O,Muntoni F,Cioni G,Siciliano G

更新日期：2011-11-01 00:00:00

abstract：:The purpose of this long-term, open parallel-group, double-consent study of alternate-day, low-dose prednisone in 2-4-year-old patients with Duchenne muscular dystrophy (DMD) was to determine whether prednisone produces a beneficial effect when given earlier than usual. Muscle function was evaluated by timed tests, an...

journal_title：Muscle & nerve

authors： Merlini L,Cicognani A,Malaspina E,Gennari M,Gnudi S,Talim B,Franzoni E

更新日期：2003-02-01 00:00:00

abstract：:A mathematical model is presented that predicts the electric field induced in the arm during magnetic stimulation of a peripheral nerve. The arm is represented as a homogeneous, cylindrical volume conductor. The electric field arises from two sources: the time-varying magnetic field and the accumulation of charge on t...

journal_title：Muscle & nerve

authors： Roth BJ,Cohen LG,Hallett M,Friauf W,Basser PJ

更新日期：1990-08-01 00:00:00