A freeze-fracture analysis of intramembrane particle densities on dystrophic hamster heart sarcolemma.

abstract：:Exercise intolerance with myalgia, muscle stiffness, and recurrent rhabdomyolysis due to mutations in the DMD gene can mimic metabolic myopathies leading to delayed or inaccurate diagnoses. In this retrospective chart review, we report 3 unrelated boys with exertional myalgia, muscle stiffness, myoglobinuria, and norm...

journal_title：Muscle & nerve

authors： Veerapandiyan A,Shashi V,Jiang YH,Gallentine WB,Schoch K,Smith EC

更新日期：2010-12-01 00:00:00

abstract：:Of 185 patients with myopathy, 22 showed abnormal muscle mitochondria. In 12 of the 22 patients, all of whom had ocular myopathy or the ophthalmoplegia-plus syndrome, muscle biopsies contained 5%-25% "ragged red" fibers. In 4 patients with a facioscapulohumeral distribution of weakness, ragged red fibers were less num...

journal_title：Muscle & nerve

authors： Kamieniecka Z,Schmalbruch H

更新日期：1978-09-01 00:00:00

abstract：INTRODUCTION:The objective of this study was to identify the pattern of neurological deficits and document electrophysiological changes in women with macromastia. METHODS:Patients with macromastia and neurological complaints underwent clinical evaluation and electrodiagnostic (EDx) studies of the upper limbs. RESULTS...

journal_title：Muscle & nerve

authors： Ansell JR,Buchholz MC

更新日期：2013-02-01 00:00:00

abstract：INTRODUCTION:We sought to translate, culturally adapt, and assess the Arabic version of the 15-item myasthenia gravis quality-of-life revised scale (MGQOL15R). METHODS:We assessed reliability with Cronbach α; reproducibility with intraclass correlation coefficient (ICC); validity with Spearman correlations for myasthe...

journal_title：Muscle & nerve

authors： Alanazy MH,Abuzinadah AR,Muayqil T

更新日期：2018-04-01 00:00:00

abstract：:McArdle disease is an autosomal recessive muscle glycogenosis. In the typical clinical presentation, only exercise-related symptoms are noted. Nevertheless, permanent weakness may occur, usually late in life. In this study we report on the clinical and genetic features of fixed muscle weakness in McArdle disease. Amon...

journal_title：Muscle & nerve

authors： Nadaj-Pakleza AA,Vincitorio CM,Laforêt P,Eymard B,Dion E,Teijeira S,Vietez I,Jeanpierre M,Navarro C,Stojkovic T

更新日期：2009-09-01 00:00:00

abstract：:Denervation induces muscle fiber atrophy and changes in the gene expression rates of skeletal muscle. Electrical stimulation (ES) is a procedure generally used to treat denervated muscles in humans. This study evaluated the effect of ES based on chronaxie and rheobase on the expression of the myoD and atrogin-1 genes ...

journal_title：Muscle & nerve

authors： Russo TL,Peviani SM,Freria CM,Gigo-Benato D,Geuna S,Salvini TF

更新日期：2007-01-01 00:00:00

abstract：:Recurrent idiopathic lumbosacral plexopathy has rarely been reported in the literature. The present report describes a 59-year-old man presenting with recurrent episodes of acute leg pain, followed by weakness. After each episode, symptoms progressed for several months before peaking. Thereafter, gradual recovery ensu...

journal_title：Muscle & nerve

authors： Yee T

更新日期：2000-09-01 00:00:00

abstract：:A stand-alone ultrasound shear wave imaging technology has been developed to quantify and visualize Young's modulus distribution by remotely applying ultrasound radiation force and tracking the resulting microvibrations in soft tissues with ultrafast ultrasound imaging. We report the first preliminary data that detect...

journal_title：Muscle & nerve

authors： Shinohara M,Sabra K,Gennisson JL,Fink M,Tanter M

更新日期：2010-09-01 00:00:00

abstract：:Two sisters presented with distal weakness and their muscle biopsy was dystrophic. This distal muscular dystrophy has an autosomal recessive inheritance and its features are somewhat different from the more common autosomal dominant distal muscular dystrophy and include: a) onset in early adult life; b) involvement of...

journal_title：Muscle & nerve

authors： Scoppetta C,Vaccario ML,Casali C,Di Trapani G,Mennuni G

更新日期：1984-07-01 00:00:00

abstract：:Muscle fatigue or neuromuscular disease may result in central activation failure during maximal voluntary contractions (MVCs). Superimposition of an electrically stimulated contraction during an MVC has been used to detect central activation failure. To determine the most sensitive means of quantitating central activa...

journal_title：Muscle & nerve

authors： Kent-Braun JA,Le Blanc R

更新日期：1996-07-01 00:00:00

abstract：INTRODUCTION:The aim of this study was to assess age- and gender-specific incidence rates (IRs) of acetylcholine receptor (AChR)-antibody-positive myasthenia gravis (MG) in South Africa, and geographical variations in incidence. METHODS:IRs were calculated from positive AChR-antibody laboratory data between 2011 and 2...

journal_title：Muscle & nerve

authors： Mombaur B,Lesosky MR,Liebenberg L,Vreede H,Heckmann JM

更新日期：2015-04-01 00:00:00

abstract：:Spinal muscular atrophy (SMA) is generally associated with proximal weakness and muscle wasting. An X-linked variant with calf hypertrophy has been reported. We describe a young man with SMA type 4 with prominent calf hypertrophy in whom DNA analysis showed a homozygous deletion of exons 7 and 8 in the telomeric copy ...

journal_title：Muscle & nerve

authors： Yiu EM,Ravat S,Ryan MM,Shield LK,Smith LJ,Kornberg AJ

更新日期：2008-07-01 00:00:00

abstract：INTRODUCTION:This study sought to estimate the global prevalence of transthyretin familial amyloid polyneuropathy (ATTR-FAP). METHODS:Prevalence estimates and information supporting prevalence calculations was extracted from records yielded by reference-database searches (2005-2016), conference proceedings, and nonpee...

journal_title：Muscle & nerve

authors： Schmidt HH,Waddington-Cruz M,Botteman MF,Carter JA,Chopra AS,Hopps M,Stewart M,Fallet S,Amass L

更新日期：2018-05-01 00:00:00

abstract：:We used peristimulus time histograms (PSTHs) to estimate characteristics of the composite excitatory postsynaptic potentials (EPSPs) generated at the anterior horn cell by a descending cortical volley induced by subthreshold transcranial magnetic stimuli in 11 normal subjects, 9 patients with DNA-confirmed Kennedy's d...

journal_title：Muscle & nerve

authors： Weber M,Eisen A

更新日期：1999-03-01 00:00:00

abstract：INTRODUCTION:We sought to compare median nerve elasticity between leprosy patients (LPs) and healthy volunteers (HVs) using ultrasound elastography (UE). METHODS:Two radiologists independently measured the strain ratio of the median nerve/flexor digitorum superficialis muscle (MN/FDSM) of 18 LP and 18 HV using real-ti...

journal_title：Muscle & nerve

authors： Nogueira-Barbosa MH,Lugão HB,Gregio-Júnior E,Crema MD,Kobayashi MTT,Frade MAC,Pavan TZ,Carneiro AAO

更新日期：2017-09-01 00:00:00

abstract：:The extensor of the second digit of the chicken wing (EDII) is a small, fast-twitch muscle susceptible to chicken muscular dystrophy and well suited for correlated studies of the morphology and physiology of identified nerve and muscle fibers. In cross section, the dystrophic EDII shows morphological abnormalities com...

journal_title：Muscle & nerve

authors： Gunther JS,Letinsky MS

更新日期：1982-01-01 00:00:00

abstract：:There is evidence that growth hormone may be related to the progression of weakness in Duchenne dystrophy. We conducted a 12-month controlled trial of mazindol, a putative growth hormone secretion inhibitor, in 83 boys with Duchenne dystrophy. Muscle strength, contractures, functional ability and pulmonary function we...

journal_title：Muscle & nerve

authors： Griggs RC,Moxley RT 3rd,Mendell JR,Fenichel GM,Brooke MH,Miller PJ,Mandel S,Florence J,Schierbecker J,Kaiser KK

更新日期：1990-12-01 00:00:00

abstract：:Local cooling of the muscle resulted in significant increases in M wave surface areas in patients with ALS, myasthenia gravis, the Lambert-Eaton myasthenic syndrome, and also in controls. The most striking increases were seen in patients with early ALS who had minimal lower motor neuron involvement and/or little defec...

journal_title：Muscle & nerve

authors： Denys EH

更新日期：1990-07-01 00:00:00

abstract：INTRODUCTION:The Accurate Test of Limb Isometric Strength (ATLIS) device can reliably measure the strength of 12 muscle groups using a fixed load cell. The purpose of this study was to analyze ATLIS data from healthy adults to calculate an individual's predicted strength scores. METHODS:ATLIS data were collected from ...

journal_title：Muscle & nerve

authors： Andres PL,English R,Mendoza M,Florence J,Malkus E,Schierbecker J,Siener C,Malspeis S,Schoenfeld DA,Munsat TL,Cudkowicz ME

更新日期：2013-02-01 00:00:00

abstract：:In median nerve lesions of the hand, electrophysiologic evaluation of the second lumbrical muscle can help to differentiate between lesions of the common digital nerves and the proper digital nerves, as illustrated by this case report. Median nerve conduction studies to the second lumbrical muscle can be performed by ...

journal_title：Muscle & nerve

authors： Busis NA,Logigian EL,Shahani BT

更新日期：1986-03-01 00:00:00

abstract：:The superficial peroneal nerve subserves sensation on the entire surface of the dorsum of the foot, except in small areas. All previously reported techniques for evaluating nerve conduction along this nerve tested a proximal portion of the nerve. We report a new method for evaluating sensory nerve conduction of the fo...

journal_title：Muscle & nerve

authors： Oh SJ,Demirci M,Dajani B,Melo AC,Claussen GC

更新日期：2001-05-01 00:00:00

abstract：:The acronym CANOMAD encompasses chronic ataxic neuropathy combined with ophthalmoplegia, M protein, cold agglutinins, and anti-disialosyl antibodies.Herein we describe 2 patients presenting with progressive ataxic neuropathy who only developed ophthalmoplegia after a significant delay post-presentation, which in 1 cas...

journal_title：Muscle & nerve

authors： Kam C,Balaratnam MS,Purves A,Mills KR,Riordan-Eva P,Pollock S,Bodi I,Munro NA,Bennett DL

更新日期：2011-11-01 00:00:00

abstract：:A careful monitoring of the accuracy of diagnosis in six cases of Guillain-Barre syndrome has shown that a substantial proportion of these patients initially diagnosed as having Guillain-Barre syndrome on the basis of characteristic clinical findings and an elevated level of protein in the spinal fluid had a neuropath...

journal_title：Muscle & nerve

authors： Feit H,Tindall RS,Glasberg M

更新日期：1982-02-01 00:00:00

abstract：:The mdx mouse, an animal model used to study Duchenne muscular dystrophy, has a nonsense mutation in exon 23 of the dystrophin gene which should result in a truncated protein that cannot be correctly localized at the sarcolemma of the muscle fibers. Immunohistochemical staining with antidystrophin antibodies has shown...

journal_title：Muscle & nerve

authors： Wilton SD,Dye DE,Laing NG

更新日期：1997-06-01 00:00:00

abstract：:Congenital myasthenic syndromes (CMS) stem from defects in presynaptic, synaptic basal lamina, and postsynaptic proteins. The presynaptic CMS are associated with defects that curtail the evoked release of acetylcholine (ACh) quanta or ACh resynthesis. Defects in ACh resynthesis have now been traced to mutations in cho...

journal_title：Muscle & nerve

authors： Engel AG,Ohno K,Sine SM

更新日期：2003-01-01 00:00:00

abstract：:We compared the diaphragmatic electromyographic (EMG) recordings from 32 patients with known neuromuscular disease and respiratory symptoms (23 neuropathies, 9 myopathies) to recordings from 23 normal subjects. Turns analysis of 219-ms sections, or epochs, of the EMG demonstrated a significant overlap between diagnost...

journal_title：Muscle & nerve

authors： McKeown MJ,Bolton CF

更新日期：1998-07-01 00:00:00

abstract：:We report two female patients with a history of alcohol abuse presenting with proximal painful muscle weakness following aversion therapy with emetine hydrochloride. Muscle biopsy of Case 1 showed a reversible floccular-shaped loss of myosin ATPase and dehydrogenase, an accumulation of PAS positive material, and a nor...

journal_title：Muscle & nerve

authors： Sugie H,Russin R,Verity MA

更新日期：1984-01-01 00:00:00

abstract：:A randomized double-blind therapeutic trial of isaxonine was completed over a 2-year period for 20 ambulant boys with Duchenne muscular dystrophy aged 5 1/2-10 years. The effect of the drug was monitored by measurement of walking times over 28 and 150 ft, motor ability score, MRC score based on 32 muscle groups, and m...

journal_title：Muscle & nerve

authors： Heckmatt JZ,Hyde SA,Gabain A,Dubowitz V

更新日期：1988-08-01 00:00:00

abstract：:Several studies have suggested the presence of central nervous system involvement manifesting as cognitive impairment in diseases traditionally confined to the peripheral nervous system. The aim of this review is to highlight the character of clinical, genetic, neurofunctional, cognitive, and psychiatric deficits in n...

journal_title：Muscle & nerve

authors： D'Angelo MG,Bresolin N

更新日期：2006-07-01 00:00:00

abstract：:The clinical efficacy of functional electrical stimulation (FES) is limited by the rapid onset of fatigue. Functional electrical stimulation applications typically stimulate skeletal muscles with constant-frequency trains (CFTs). Our laboratory has identified trains that we call doublet-frequency trains (DFTs) and tha...

journal_title：Muscle & nerve

authors： Scott WB,Lee SC,Johnston TE,Binder-Macleod SA

更新日期：2005-05-01 00:00:00