Myopathy with muscle spindle excess: A new congenital neuromuscular syndrome?

abstract：:Body components were measured noninvasively in patients with neuromuscular disease by using dual-energy X-ray absorptiometry (DXA), capable of separately analyzing fat, bone, and muscle content. In all patients with muscle atrophy of myogenic or neurogenic origin, muscle mass was markedly reduced. Although all three c...

journal_title：Muscle & nerve

authors： Kanda F,Fujii Y,Takahashi K,Fujita T

更新日期：1994-04-01 00:00:00

abstract：:To determine which sensory nerve conduction studies (S-NCS) are helpful in detecting supraclavicular axon loss brachial plexopathies, we selected 53 cases (of 417 reviewed) in whom complicating factors were absent and which, by needle electrode examination findings, involved only a single "truncal" element (upper, mid...

journal_title：Muscle & nerve

authors： Ferrante MA,Wilbourn AJ

更新日期：1995-08-01 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：INTRODUCTION:This investigation aimed to: (1) confirm whether women were more vulnerable to the negative neuromuscular adaptations elicited by muscle unloading and if so, (2) determine which physiological mechanism(s) explain those gender-related differences. METHODS:Healthy young men (20.7 ± 0.3 years) and women (20....

journal_title：Muscle & nerve

authors： Deschenes MR,McCoy RW,Mangis KA

更新日期：2012-08-01 00:00:00

abstract：:Muscle fatigue occurs in many neuromuscular diseases, including the muscular dystrophies, and it contributes to a loss of functional capacity and reduced quality of life for affected patients. An improvement in fatigue resistance has been observed in diaphragm muscles of mdx mice following insulin-like growth factor-I...

journal_title：Muscle & nerve

authors： Gregorevic P,Plant DR,Lynch GS

更新日期：2004-09-01 00:00:00

abstract：:We report the case of a 27-year-old man treated for bilateral optic neuritis 5 and 3 years before who within a few months developed sensorimotor disorders of the arms and legs characterized by asymmetric distribution and distal prominence. In addition to sensorimotor defects, which were particularly marked in the left...

journal_title：Muscle & nerve

authors： Péréon Y,Jardel J,Guillon B,Guihéneuc P

更新日期：1994-11-01 00:00:00

abstract：:Voluntary and stimulation single-fiber electromyography were performed in the extensor digitorum communis muscle of 15 myasthenic patients. The increase in mean and individual mean consecutive difference as well as the proportion of blocking in the volitional activation were greater than in the stimulation method. The...

journal_title：Muscle & nerve

authors： Murga L,Sánchez F,Menéndez C,Castilla JM

更新日期：1998-08-01 00:00:00

abstract：:An appreciation of the comparative functions of the corticospinal tract is of direct relevance to the understanding of how results from animal models can advance knowledge of the human motor system and its disorders. Two critical functions of the corticospinal tract are discussed: first, the role of descending project...

journal_title：Muscle & nerve

authors： Lemon RN,Griffiths J

更新日期：2005-09-01 00:00:00

abstract：:The clinical effect of carbamazepine (CBZ) on neuromuscular transmission is described in two children who presented in coma with diffuse hypotonia and areflexia following CBZ overdose. Repetitive nerve stimulation (RNS) showed a decremental response only at high-frequency stimulation. With supportive care, the patient...

journal_title：Muscle & nerve

authors： Zaidat OO,Kaminski HJ,Berenson F,Katirji B

更新日期：1999-09-01 00:00:00

abstract：:We wished to determine whether evoked force output from a human muscle could be inferred from the amplitude and the frequency of evoked muscular sounds. Sounds from adductor pollicis were recorded with a microphone and compared to evoked force changes produced by varying the intensity of nerve stimulation or of volunt...

journal_title：Muscle & nerve

authors： Chen D,Durand LG,Bellemare F

更新日期：1997-08-01 00:00:00

abstract：:Cytoplasmic body myopathy (CBM) is characterized by proteinaceous inclusion bodies in muscle tissue. A 43-year-old woman presented with rapidly progressive weakness and dysphagia. Electromyography (EMG) elsewhere demonstrated lower-limb chronic partial denervation. Muscle biopsy showed fiber size variation without dia...

journal_title：Muscle & nerve

authors： Krishnan AV,Pamphlett R,Burke D,Wills EJ,Kiernan MC

更新日期：2004-11-01 00:00:00

abstract：:Neurotrophic factors are growth factors or cytokines that are inducible polypeptides and permit intercellular communication. An explosion of information about neurotrophic factors is setting the stage for significant advances in neural disease therapy in the next century. The effects of these trophic factors are overl...

journal_title：Muscle & nerve

authors： Mitsumoto H,Tsuzaka K

更新日期：1999-08-01 00:00:00

abstract：INTRODUCTION:Duchenne muscular dystrophy (DMD) is characterized by progressive muscle degeneration leading to immobility, respiratory failure, and premature death. As chronic inflammation and stress are implicated in DMD pathology, the efficacy of an anti-inflammatory and anti-stress intervention strategy in ameliorati...

journal_title：Muscle & nerve

authors： Manning J,Buckley MM,O'Halloran KD,O'Malley D

更新日期：2017-12-01 00:00:00

abstract：:Morphological changes of the subneural apparatus (SNA) during denervation and reinnervation are demonstrated using the hamster peroneus longus muscle. One week after nerve section, synaptic grooves (SGs) were more shallow, with lowered sarcoplasmic ridges. After 4 weeks, the whole SNA area was elevated above the sarco...

journal_title：Muscle & nerve

authors： Matsuda Y,Oki S,Kitaoka K,Nagano Y,Nojima M,Desaki J

更新日期：1988-12-01 00:00:00

abstract：INTRODUCTION:Myasthenia gravis (MG) is a B-cell-mediated autoimmune disease. B-cell-activating factor (BAFF) is a major factor in B-cell development and activation. In this study we investigated serum BAFF levels in MG patients. METHODS:We compared the serum BAFF levels of 20 MG patients with gender-matched healthy co...

journal_title：Muscle & nerve

authors： Kang SY,Kang CH,Lee KH

更新日期：2016-12-01 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：:Muscle weakness, studied in 4 patients with multiple sclerosis (MS), was compared with values from normal subjects. Twitch occlusion showed that normal subjects could activate their muscles maximally, but patients rarely achieved greater than 60% activation. In both groups, motoneuron firing rates increased linearly w...

journal_title：Muscle & nerve

authors： Rice CL,Vollmer TL,Bigland-Ritchie B

更新日期：1992-10-01 00:00:00

abstract：INTRODUCTION:Amyloidosis derived from transthyretin (TTR) molecules is typically caused by mutations of the TTR gene. METHODS:We describe an elderly patient with a severe length-dependent polyneuropathy that unexpectedly proved to be caused by wild-type transthyretin amyloidosis. RESULTS:The diagnosis was made by mus...

journal_title：Muscle & nerve

authors： Lam L,Margeta M,Layzer R

更新日期：2015-07-01 00:00:00

abstract：:A middle-aged male developed a right posterior femoral cutaneous neuralgia that persisted for 10 years without any associated neurologic, electrophysiologic, or radiologic findings. Five years after onset, surgical exploration of the subgluteal area identified a venous malformation surrounding the nerve. The syndrome ...

journal_title：Muscle & nerve

authors： Chutkow JG

更新日期：1988-11-01 00:00:00

abstract：:Biochemical, morphologic, and biophysical studies support the concept that the red blood cell (RBC) membrane is altered in both myotonic muscular dystrophy (MyD) and Duchenne muscular dystrophy (DMD). These studies have not identified a primary metabolic defect that would explain the various alterations of membrane pr...

journal_title：Muscle & nerve

authors： Plishker GA,Appel SH

更新日期：1980-01-01 00:00:00

abstract：:Contractions of rat extensor digitorum longus (EDL, a fast muscle) and soleus (SOL, a slow muscle) muscles of different ages (1-4 weeks) were recorded in vitro with direct stimulation and at different temperatures (range 35-10 degrees C). Twitch tension in 4-week-old EDL muscle increased in cooling from 35 to 20 degre...

journal_title：Muscle & nerve

authors： Wylie SR,Ranatunga KW

更新日期：1987-11-01 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：INTRODUCTION:To evaluate the sensitivity of electrophysiologic assessments, we compared F-waves and motor and sensory nerve conduction studies (MNCS and SNCS) in patients with diabetes mellitus (DM). METHODS:We tested median, ulnar, tibial, and fibular nerves in 132 DM patients divided into those with and without clin...

journal_title：Muscle & nerve

authors： Pan H,Jian F,Lin J,Chen N,Zhang C,Zhang Z,Ding Z,Wang Y,Cui L,Kimura J

更新日期：2014-06-01 00:00:00

abstract：INTRODUCTION:We aimed to investigate whether sonographic peripheral cross-sectional nerve area (CSA) and progranulin (PGRN), a neuritic growth factor, are related to each other and whether they interact to predict clinical and paraclinical measures in amyotrophic lateral sclerosis (ALS). METHODS:We included 55 ALS pat...

journal_title：Muscle & nerve

authors： Schreiber S,Debska-Vielhaber G,Abdulla S,Machts J,Schreiber F,Kropf S,KÖrtvelyessy P,KÖrner S,Kollewe K,Petri S,Dengler R,Kunz WS,Nestor PJ,Vielhaber S

更新日期：2018-02-01 00:00:00

abstract：:Normal motor units (MUs) were simulated and their architecture altered to simulate the changes produced by myopathy. The concentric needle electromyographic recordings of motor unit action potentials (MUAPs) from the MUs were then also simulated. These simulated MUAPs showed features that are seen in myopathy: normal ...

journal_title：Muscle & nerve

authors： Nandedkar SD,Sanders DB

更新日期：1989-03-01 00:00:00

abstract：:The site of the recording electrode influences the amplitude of the compound muscle action potential (CMAP) and its variation over a length of nerve. The effects of large electrodes on this source of intraindividual variability were assessed. Right median nerves of 20 healthy subjects were studied, and recordings made...

journal_title：Muscle & nerve

authors： van Dijk JG,Tjon-a-Tsien A,van der Kamp W

更新日期：1995-01-01 00:00:00

abstract：:We studied membrane ultrastructural localization of anionic phospholipids (AP) and sialic acid (SA) calcium binding sites in muscle biopsies from Duchenne muscular dystrophy (DMD) and 3 Becker's muscular dystrophy (BMD) patients using polymyxin B (PXB) and limulus polyphemus (LP) as cytochemical markers. We found that...

journal_title：Muscle & nerve

authors： Moggio M,Fagiolari G,Prelle A,Gallanti A,Sciacco M,Scarlato G

更新日期：1992-03-01 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：:Little attention has been paid to small-fiber dysfunction in carpal tunnel syndrome (CTS) although its symptoms are common. This study investigates vasomotor dysfunction, which is controlled by small nerve fibers, in patients with CTS compared with control subjects. Vasomotor function was quantified by measuring, with...

journal_title：Muscle & nerve

authors： Wilder-Smith EP,Fook-Chong S,Chew SE,Chow A,Guo Y

更新日期：2003-11-01 00:00:00

abstract：INTRODUCTION:YouTube is an important resource for patients. No study has evaluated the information on peripheral neuropathy disseminated by YouTube videos. In this study, our aim was to perform a systematic review of information on YouTube regarding peripheral neuropathy. METHODS:The Web site (www.youtube.com) was sea...

journal_title：Muscle & nerve

authors： Gupta HV,Lee RW,Raina SK,Behrle BL,Hinduja A,Mittal MK

更新日期：2016-01-01 00:00:00