Peripheral neuropathy in glycogen storage disease type III: Fact or myth?

abstract：INTRODUCTION:We adopted a proteomics-based approach to gain insights into phenotypic differences between A/J and B10.SJL murine dysferlinopathy models. METHODS:We optimized immunoblotting of dysferlin by preparing homogenates of the tibialis anterior (TA) muscle under several different conditions. We compared TA muscl...

journal_title：Muscle & nerve

authors： Mueller AL,Desmond PF,Hsia RC,Roche JA

更新日期：2014-08-01 00:00:00

abstract：INTRODUCTION:To address the need for greater standardization within the field of electrodiagnostic medicine, the Normative Data Task Force (NDTF) was formed to identify nerve conduction studies (NCS) in the literature, evaluate them using consensus-based methodological criteria derived by the NDTF, and identify those s...

journal_title：Muscle & nerve

authors： Chen S,Andary M,Buschbacher R,Del Toro D,Smith B,So Y,Zimmermann K,Dillingham TR

更新日期：2016-09-01 00:00:00

abstract：:Toxoplasma gondii encysts in skeletal muscle. Although only rarely found at muscle biopsy, this parasite has previously been regarded as a possible cause of polymyositis. We report a case of biopsy-proven toxoplasmic myositis in a non-HIV-infected patient that led to recognition of idiopathic CD4 lymphocytopenia (ICL)...

journal_title：Muscle & nerve

authors： Plonquet A,Bassez G,Authier FJ,Dray JM,Farcet JP,Gherardi RK

更新日期：2003-06-01 00:00:00

abstract：:Seventy-five consecutive patients with clinical symptoms and signs of meralgia paresthetica underwent bilateral somatosensory evoked potential (SEP) studies involving stimulation of skin areas innervated by the lateral and anterior femoral cutaneous nerves of the thighs. The most common abnormality was an absolute lat...

journal_title：Muscle & nerve

authors： Cordato DJ,Yiannikas C,Stroud J,Halpern JP,Schwartz RS,Akbunar M,Cook M

更新日期：2004-01-01 00:00:00

abstract：:The utilization of viable biological nerve graft substitutes and nerve allografts raises the problem of nerve storage. To clarify this, rat sciatic nerve segments were harvested and stored in Dulbecco's modified eagle medium. The segments were divided into three groups. In the first group, no cryoprotectant was added,...

journal_title：Muscle & nerve

authors： Fansa H,Lassner F,Kook PH,Keilhoff G,Schneider W

更新日期：2000-08-01 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：:Muscle of donor origin was formed after implantation of H-2Kb-tsA58 muscle precursor cells (mpc) into irradiated mdx nu/nu mouse muscles. A series of injections of the myotoxin, notexin, which destroys mature muscle fibers but spares muscle precursor cells and other tissues, was made into the mpc-injected muscles, lea...

journal_title：Muscle & nerve

authors： Gross JG,Morgan JE

更新日期：1999-02-01 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：: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 effects of altering (cross-reinnervation, CR) and/or eliminating (spinal isolation, SI) the activation-dependent neural input to the cat soleus for 8 months on its mechanical and biochemical properties were determined. The percent fast fibers was 0, 27, 38, and 54% for normal control (NC), NC-CR, SI, and SI-CR cat...

journal_title：Muscle & nerve

authors： Roy RR,Eldridge L,Baldwin KM,Edgerton VR

更新日期：1996-06-01 00:00:00

abstract：:Repetitive nerve stimulation (RNS) of the median nerve is rarely studied in myasthenia gravis (MG). We performed a retrospective analysis of RNS studies performed on 448 patients at our center between 2010 and 2016. Among 95 patients with MG, an abnormal decrement of the compound muscle action potential amplitude was ...

journal_title：Muscle & nerve

authors： Lee TH,Li Y

更新日期：2019-12-01 00:00:00

abstract：:Myophosphorylase deficiency (McArdle's disease) may present with episodic renal failure following exertion. We present a case of adult-onset myophosphorylase deficiency in which recurrent bouts of renal failure could not be associated with any exertional events until a tonic seizure was witnessed after the fourth epis...

journal_title：Muscle & nerve

authors： Walker AR,Tschetter K,Matsuo F,Flanigan KM

更新日期：2003-11-01 00:00:00

abstract：INTRODUCTION:We evaluated anatomic variations of distal branches of the superficial fibular sensory nerve electrophysiologically. METHODS:Orthodromic nerve conduction studies (NCS) of the first and third branches (M-I, M-III) of the medial dorsal cutaneous nerve and the fourth and fifth branches (I-IV, I-V) of the int...

journal_title：Muscle & nerve

authors： Hemmi S,Kurokawa K,Nagai T,Kushida R,Okamoto T,Murakami T,Sunada Y

更新日期：2017-01-01 00:00:00

abstract：:The pathophysiology of hepatic neuropathy is poorly understood, but membrane depolarization due to a toxic inhibition of oxidative metabolism has been proposed. We investigated the relationship between nerve excitability properties, nerve dysfunction, and liver function in 11 pretransplant patients, the majority of wh...

journal_title：Muscle & nerve

authors： Ng K,Lin CS,Murray NM,Burroughs AK,Bostock H

更新日期：2007-06-01 00:00:00

abstract：INTRODUCTION:Improving axonal outgrowth and remyelination is crucial for peripheral nerve regeneration. Miconazole appears to enhance remyelination in the central nervous system. In this study we assess the effect of miconazole on axonal regeneration using a sciatic nerve crush injury model in rats. METHODS:Fifty Spra...

journal_title：Muscle & nerve

authors： Lin T,Qiu S,Yan L,Zhu S,Zheng C,Zhu Q,Liu X

更新日期：2018-05-01 00:00:00

abstract：:The motor end-plate fine structure and distribution of junctional acetylcholine receptors (AChRs) in patients with amyotrophic lateral sclerosis (ALS) were studied. Morphometric analysis was carried out in 74 end-plates (92 innervated and 47 denuded postsynaptic regions) from 10 patients with ALS. AChR was studied by ...

journal_title：Muscle & nerve

authors： Tsujihata M,Hazama R,Yoshimura T,Satoh A,Mori M,Nagataki S

更新日期：1984-03-01 00:00:00

abstract：:Ocular movements and coordination require complex and integrated functions of somatic and autonomic nervous systems. Neurological disorders affecting these nervous systems may cause ocular dysfunction involving extraocular muscles and pupils. In this article, the prevalence, clinical presentations, and management of o...

journal_title：Muscle & nerve

authors： Evliyaoglu F,Karadag R,Burakgazi AZ

更新日期：2012-11-01 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：:The goal of this study was to examine the effects of altered thyroid state and denervation (Den) on skeletal myosin heavy chain (MHC) expression in the plantaris and soleus muscles. Rats were subjected to unilateral denervation (Den) and randomly assigned to one of three groups: (1) euthyroid; (2) hyperthyroid; (3) an...

journal_title：Muscle & nerve

authors： Haddad F,Arnold C,Zeng M,Baldwin K

更新日期：1997-12-01 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：:We applied the singular value decomposition (SVD) method to study single motor unit firing patterns. Two projects were carried out: (1) a computer simulation study to confirm the meanings of two SVD parameters, the eigenvalue corresponding to the positive-slope eigenvector (PEV) and that corresponding to the negative-...

journal_title：Muscle & nerve

authors： Sun TY,Chen JJ,Lin TS

更新日期：2000-07-01 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:In this study we noninvasively evaluated skeletal muscle mitochondrial capacity, muscle-specific endurance, and energy/fatigue feelings in persons with Friedreich ataxia (FRDA) and able-bodied controls (AB). METHODS:Forearm mitochondrial capacity was measured in FRDA (n = 16) and AB (n = 10) study partici...

journal_title：Muscle & nerve

authors： Bossie HM,Willingham TB,Schoick RAV,O'Connor PJ,McCully KK

更新日期：2017-10-01 00:00:00

abstract：INTRODUCTION:We sought to define the whole-body MRI (WB-MRI) fingerprint of muscle involvement in pediatric LMNA-related dystrophy (LMNA-RD) and to compare it with SEPN1-related myopathy (SEPN1-RM). METHODS:Signal abnormality and atrophy in 109 muscles were scored by semiquantitative scales in 8 children with LMNA-RD ...

journal_title：Muscle & nerve

authors： Gómez-Andrés D,Dabaj I,Mompoint D,Hankiewicz K,Azzi V,Ioos C,Romero NB,Ben Yaou R,Bergounioux J,Bonne G,Richard P,Estournet B,Yves-Carlier R,Quijano-Roy S

更新日期：2016-08-01 00:00:00

abstract：:Electrophysiologic detection of the first signs of gastrocnemius muscle reinnervation shows that after a single localized freezing of the rat sciatic nerve, isaxonine does not significantly increase the rate of axonal regeneration. However, this drug does significantly enhance axonal sprouting, as it induces an increa...

journal_title：Muscle & nerve

authors： Pecot-Dechavassine M,Mira JC

更新日期：1985-02-01 00:00:00

abstract：:Statins can induce necrotizing or inflammatory myopathies in some patients. Increased major histocompatibility complex class I (MHC I) expression has been shown in muscle biopsies of statin-induced myopathy. Therefore, we investigated the effect of statins on the expression of MHC I in muscle cells. Using flow cytomet...

journal_title：Muscle & nerve

authors： Singh P,Kohr D,Kaps M,Blaes F

更新日期：2010-02-01 00:00:00

abstract：INTRODUCTION:The purpose of this study was to investigate in vivo median nerve longitudinal mobility in different segments of the carpal tunnel associated with active finger motion in carpal tunnel syndrome (CTS) patients in a comparison with healthy controls. METHODS:Eleven healthy volunteers and 11 CTS patients part...

journal_title：Muscle & nerve

authors： Yao Y,Grandy E,Evans PJ,Seitz WH Jr,Li ZM

更新日期：2020-10-01 00:00:00

abstract：:Immersion of rat hemidiaphragms in Ca2+-free Krebs solution (KS) containing Ca2+ chelator in vitro leads to separation of basal lamina from the plasma membrane, as well as transient contracture and rapid loss of twitch response [calcium paradox (CP) phase 1]. Subsequent immersion in regular KS results in necrosis of m...

journal_title：Muscle & nerve

authors： Soza M,Karpati G,Carpenter S

更新日期：1986-03-01 00:00:00

abstract：INTRODUCTION:Herein we present an exploratory study of orofacial function in children with congenital myotonic dystrophy (CDM) vs. healthy controls. METHODS:We evaluated 41 children with CDM and 29 healthy controls for speech and swallow function and for lingual and labial strength. RESULTS:The Iowa Oral Performance ...

journal_title：Muscle & nerve

authors： Berggren KN,Hung M,Dixon MM,Bounsanga J,Crockett B,Foye MD,Gu Y,Campbell C,Butterfield RJ,Johnson NE

更新日期：2018-09-01 00:00:00