Atypical phenotype of charcot-marie-tooth disease type 1A.

abstract：:Although the blood level of creatine kinase (CK) is the most commonly used marker of muscle injury, there is marked interindividual variability in this measure. Part of this variability may be attributed to variability in the rate of CK clearance from the circulation. In this study, we assessed the possibility that CK...

journal_title：Muscle & nerve

authors： Warren GL,O'farrell L,Rogers KR,Billings KM,Sayers SP,Clarkson PM

更新日期：2006-09-01 00:00:00

abstract：BACKGROUND:The goals of this study were to determine whether serum concentrations of endocannabinoids (eCB) and related lipids predict disease status in patients with amyotrophic lateral sclerosis (ALS) relative to healthy controls, and whether concentrations correlate with disease duration and severity. METHODS:Serum...

journal_title：Muscle & nerve

authors： Carter GT,McLaughlin RJ,Cuttler C,Sauber GJ,Weeks DL,Hillard CJ,Weiss MD

更新日期：2021-01-01 00:00:00

abstract：INTRODUCTION:Balance impairment contributes to gait dysfunction, falls, and reduced quality of life in adults with Charcot-Marie-Tooth disease (CMT) but has been minimally examined in pediatric CMT. METHODS:The CMT Pediatric Scale (CMTPedS) was administered to 520 children with CMT. Associations between balance functi...

journal_title：Muscle & nerve

authors： Estilow T,Glanzman AM,Burns J,Harrington A,Cornett K,Menezes MP,Shy R,Moroni I,Pagliano E,Pareyson D,Bhandari T,Muntoni F,Laurá M,Reilly MM,Finkel RS,Eichinger KJ,Herrmann DN,Troutman G,Bray P,Halaki M,Shy ME,Yu

更新日期：2019-09-01 00:00:00

abstract：:The role of motor innervation and muscle tension in the posthatching maturation of the slow-tonic anterior latissimus dorsi (ALD) muscle of the chicken has been investigated. Modification of the muscle tension was obtained either by maintaining ALD in a shortened state or by stretching, after or without denervation. I...

journal_title：Muscle & nerve

authors： Gardahaut MF,Rouaud TP,Renaud D,Khaskiye A,Le Douarin GH

更新日期：1989-11-01 00:00:00

abstract：INTRODUCTION:In this study we sought to determine the cross-sectional area (CSA) of peripheral nerves in patients with distinct subtypes of amyotrophic lateral sclerosis (ALS). METHODS:Ulnar and median nerve ultrasound was performed in 78 ALS patients [classic, n = 21; upper motor neuron dominant (UMND), n = 14; lower...

journal_title：Muscle & nerve

authors： Schreiber S,Abdulla S,Debska-Vielhaber G,Machts J,Dannhardt-Stieger V,Feistner H,Oldag A,Goertler M,Petri S,Kollewe K,Kropf S,Schreiber F,Heinze HJ,Dengler R,Nestor PJ,Vielhaber S

更新日期：2015-05-01 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：:We report a patient with clinical vermiform motor activity, muscle cramps, delayed relaxation of grip, and continuous motor unit discharges who developed mild symmetric symptoms in the upper extremities following a viral infection. Treatment with carbamazepine produced considerable symptomatic improvement. Needle elec...

journal_title：Muscle & nerve

authors： Tandan R,Fries TJ

更新日期：1988-03-01 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：:Thyrotropin-releasing hormone (TRH), present in high concentrations in the mammalian spinal cord, exerts excitatory effects on the alpha-motorneuron (AMN) via axodendritic contacts. We used the neurotoxin 5,7-dihydroxytryptamine (5,7-DHT) to deplete TRH from the ventral horn of the spinal cord of adult rats to determi...

journal_title：Muscle & nerve

authors： Van den Bergh P,Kelly JJ Jr,Adelman L,Munsat TL,Jackson IM,Lechan RM

更新日期：1987-06-01 00:00:00

abstract：:Action potentials of rabbit masseter motor units (n = 42) were registered at different jaw angles to examine whether the shape of the action potential is related to length of muscle fibers in motor units and depends on the intramuscular location of the motor unit. Twitches were elicited by stimulating motoneurons in t...

journal_title：Muscle & nerve

authors： Turkawski SJ,van Eijden TM

更新日期：2001-11-01 00:00:00

abstract：:To elucidate whether the frontal components of scalp somatosensory evoked potentials (SEPs) depend on the type of peripheral input, we compared scalp SEPs in response to electrical stimuli applied to: (i) the proximal phalanx of the thumb, involving both deep and cutaneous afferents; and (ii) the distal phalanx of the...

journal_title：Muscle & nerve

authors： Restuccia D,Valeriani M,Barba C,Le Pera D,Tonali P,Mauguière F

更新日期：1999-07-01 00:00:00

abstract：:Acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE) were studied in the diaphragm of early postnatal rats, using radiometric determination of enzyme activities, velocity sedimentation for separation of molecular forms, and electron microscopic cytochemistry to localize enzymes. AChE activity did not change s...

journal_title：Muscle & nerve

authors： Brzin M,Sketelj J,Tennyson VM,Kiauta T,Budininkas-Schoenebeck M

更新日期：1981-11-01 00:00:00

abstract：:Intraoperative electrophysiological monitoring of the spinal cord has traditionally been done by recording somatosensory evoked potentials (SEP). There is a risk that SEPs can be unaltered when significant injury to the anterior spinal cord has occurred. The purpose of this report is to describe a simple technique for...

journal_title：Muscle & nerve

authors： Phillips LH 2nd,Blanco JS,Sussman MD

更新日期：1995-03-01 00:00:00

abstract：:This investigation analyzes the temporal characteristics of maximal depolarization times for three waveforms: end-plate spikes, fibrillation potentials, and positive sharp waves (PSWs) to provide support for the electrode initiation hypothesis of PSW induction. The maximal depolarization times for PSWs are documented ...

journal_title：Muscle & nerve

authors： Dumitru D,Santa Maria DL

更新日期：2007-09-01 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：INTRODUCTION:In preparation for clinical trials we examine the validity, reliability, and patient understanding of the Myotonic Dystrophy Health Index (MDHI). METHODS:Initially we partnered with 278 myotonic dystrophy type-1 (DM1) patients and identified the most relevant questions for the MDHI. Next, we used factor a...

journal_title：Muscle & nerve

authors： Heatwole C,Bode R,Johnson N,Dekdebrun J,Dilek N,Heatwole M,Hilbert JE,Luebbe E,Martens W,Mcdermott MP,Rothrock N,Thornton C,Vickrey BG,Victorson D,Moxley R 3rd

更新日期：2014-06-01 00:00:00

abstract：:The absolute number of satellite cells (SC) in young and adult rat skeletal muscle was estimated by correlating data from light and electron microscopic quantitation of myofiber nuclei (myonuclei and satellite cell nuclei) with biochemical assays for determining total muscle DNA content. Expressed both as a proportion...

journal_title：Muscle & nerve

authors： Gibson MC,Schultz E

更新日期：1983-10-01 00:00:00

abstract：:Because of their central role in muscle development and maintenance, MEF2 family members represent excellent candidate effectors of the muscle pathology in myotonic dystrophy (DM). We investigated the expression and alternative splicing of all four MEF2 genes in muscle from neuromuscular disorder (NMD) patients, inclu...

journal_title：Muscle & nerve

authors： Bachinski LL,Sirito M,Böhme M,Baggerly KA,Udd B,Krahe R

更新日期：2010-12-01 00:00:00

abstract：:Five members of the same family, along three generations, presented with hypertrophic cardiomyopathy. Neurological examination, muscle strength, electromyography, and serum creatine kinase were normal. Skeletal muscle biopsy showed abnormal lipid accumulation and carnitine deficiency. In three patients the cardiac sym...

journal_title：Muscle & nerve

authors： Bautista J,Rafel E,Martinez A,Sainz I,Herrera J,Segura L,Chinchon I

更新日期：1990-03-01 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：:The clinical, pathologic, and genetic findings of a boy with intranuclear nemaline rod myopathy are described. Serial muscle biopsies revealed myocyte nuclei containing inclusions that were immunoreactive for alpha-actinin and increased with age. Genetic analysis revealed a Val163Leu ACTA1 mutation previously associat...

journal_title：Muscle & nerve

authors： Kaimaktchiev V,Goebel H,Laing N,Narus M,Weeks D,Nixon R

更新日期：2006-09-01 00:00:00

abstract：INTRODUCTION:Are there electrophysiological findings that predict response to intravenous immunoglobulin (IVIg) in patients with lower motor neuron (LMN) syndromes without multifocal conduction block (MCB)? METHODS:We enrolled 9 patients with LMN syndromes without MCB to receive 18 weeks of IVIg therapy. Response was ...

journal_title：Muscle & nerve

authors： Lange DJ,Nijjar R,Voustianiouk A,Seidel G,Panchal J,Wang AK

更新日期：2011-04-01 00:00:00

abstract：:Metabolic costs of force generation were compared for constant-frequency and catchlike-inducing electrical stimulation. Repetitive catchlike-inducing trains consisted of 2 interpulse intervals (IPIs) at 12.5 ms, 1 IPI at 25 ms, and 5 IPIs at 50 ms. Constant-frequency trains consisted of 8 IPIs at 37.5 ms. One train wa...

journal_title：Muscle & nerve

authors： Ratkevicius A,Quistorff B

更新日期：2002-03-01 00:00:00

abstract：INTRODUCTION:Symptoms often return in patients with carpal tunnel syndrome (CTS) who are treated with a local corticosteroid injection. We aimed to determine prognostic factors for a successful long-term outcome. METHODS:We prospectively followed 113 patients who received an injection for CTS. Clinical, electrophysiol...

journal_title：Muscle & nerve

authors： Meys V,Thissen S,Rozeman S,Beekman R

更新日期：2011-11-01 00:00:00

abstract：:Twenty-four adult albino rats were injected with tetanus toxin into the right gastrocnemius muscle and then subjected to sustained dorsiflexion of the right ankle joint for 2 to 14 days. Histologic examinations of the soleus after this procedure showed myopathic changes, characterized by variations in fiber diameters,...

journal_title：Muscle & nerve

authors： Mizuno Y,Chou SM

更新日期：1990-10-01 00:00:00

abstract：INTRODUCTION:Muscle weakness and functional disability have not been evaluated in a population-based study of patients with myasthenia gravis (MG). METHODS:All patients with MG in a well-defined catchment area were identified in the Danish National Patient Registry. Of the 175 eligible patients, 90 participated and we...

journal_title：Muscle & nerve

authors： Vinge L,Jakobsen J,Andersen H

更新日期：2019-02-01 00:00:00

abstract：:Loss of cells from the motor system occurs during the normal aging process, leading to reduction in the complement of motor neurons and muscle fibers. The latter age-related decrease in muscle mass has been termed "sarcopenia" and is often combined with the detrimental effects of a sedentary lifestyle in older adults,...

journal_title：Muscle & nerve

authors： Vandervoort AA

更新日期：2002-01-01 00:00:00

abstract：:Factors that affect the shape of the so-called "normal cloud" of the turns and amplitude measurements of the electromyographic interference pattern are investigated. As the force of voluntary contraction increases from low to moderate levels, the number of turns in the signal increase faster than does the mean amplitu...

journal_title：Muscle & nerve

authors： Nandedkar SD,Sanders DB,Stalberg EV

更新日期：1991-01-01 00:00:00

abstract：:Epidemiological studies of the carpal tunnel syndrome have generally overlooked the possibility of a familial occurrence. A prospective study was undertaken to determine the prevalence and significance of a positive family history of carpal tunnel syndrome. Seventy-five of 253 women and 40 of 168 men with a confirmed ...

journal_title：Muscle & nerve

authors： Radecki P

更新日期：1994-03-01 00:00:00

abstract：INTRODUCTION:Myelinating Schwann cells compartmentalize their outermost layer to form actin-rich channels known as Cajal bands. Herein we investigate changes in Schwann cell architecture and cytoplasmic morphology in a novel mouse model of carpal tunnel syndrome. METHODS:Chronic nerve compression (CNC) injury was crea...

journal_title：Muscle & nerve

authors： Gupta R,Nassiri N,Hazel A,Bathen M,Mozaffar T

更新日期：2012-02-01 00:00:00