Addressing heterogeneity in amyotrophic lateral sclerosis CLINICAL TRIALS.

abstract：INTRODUCTION:Skeletal muscle oxidative capacity decreases and fatigability increases after spinal cord injury. Transcription factor peroxisome proliferator-activated receptor δ (PPARδ) promotes a more oxidative phenotype. METHODS:We asked whether PPARδ overexpression could ameliorate these deficits in the medial gastr...

journal_title：Muscle & nerve

authors： Kim JA,Roy RR,Zhong H,Alaynick WA,Embler E,Jang C,Gomez G,Sonoda T,Evans RM,Edgerton VR

更新日期：2016-02-01 00:00:00

abstract：:Mutations in the sarcoglycan (SG) genes cause a subset of limb-girdle muscular dystrophies (LGMD). We report a Spanish patient with progressive LGMD exhibiting an almost isolated loss of gamma-SG and a homozygous Delta521-T mutation in the gamma-SG gene. These results suggest that isolated loss of gamma-SG might remai...

journal_title：Muscle & nerve

authors： Vorgerd M,Gencik M,Mortier J,Epplen JT,Malin JP,Mortier W

更新日期：2001-03-01 00:00:00

abstract：INTRODUCTION:The aim of this study was to compare ultrasound echo intensity (EI) with high-resolution T1 -weighted MRI and to establish calibration equations to estimate percent intramuscular fat from EI. METHODS:Thirty-one participants underwent both ultrasound and MRI testing of 4 muscles: rectus femoris (RF); bicep...

journal_title：Muscle & nerve

authors： Young HJ,Jenkins NT,Zhao Q,Mccully KK

更新日期：2015-12-01 00:00:00

abstract：:Guillain-Barré syndrome (GBS) generally presents sporadically. Epidemics of GBS are unusual. We reviewed the medical records of 26 patients hospitalized for GBS during the 2007 GBS epidemic in northeast China. The objective was to determine whether there were clinical and electrophysiological characteristics. All pati...

journal_title：Muscle & nerve

authors： Ye Y,Zhu D,Wang K,Wu J,Feng J,Ma D,Xing Y,Jiang X

更新日期：2010-09-01 00:00:00

abstract：INTRODUCTION:Chorea-acanthocytosis (ChAc) is a rare autosomal recessive disease characterized by involuntary movements, seizures, cognitive changes, myopathy, and axonal neuropathy. METHODS:We report a patient who presented with gait impairment and dysarthria. Clinical and neurophysiological assessment disclosed upper...

journal_title：Muscle & nerve

authors： Neutel D,Miltenberger-Miltenyi G,Silva I,de Carvalho M

更新日期：2012-02-01 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：:Mutations in CLCN1, the gene encoding the ClC-1 chloride channel in skeletal muscle, lead to myotonia congenita. The effects on the intramembranous channel forming domains have been investigated more than that at the intracellular C-terminus. We have performed a mutation screen involving the whole CLCN1 gene of patien...

journal_title：Muscle & nerve

authors： Chen L,Schaerer M,Lu ZH,Lang D,Joncourt F,Weis J,Fritschi J,Kappeler L,Gallati S,Sigel E,Burgunder JM

更新日期：2004-05-01 00:00:00

abstract：INTRODUCTION:Detection of denervation in muscles in the craniobulbar area is important to assure widespread lower motor neuron involvement in the diagnosis of amyotrophic lateral sclerosis (ALS). The value of spontaneous activity analysis in needle electromyography (EMG) of the tongue has been questioned in the recent ...

journal_title：Muscle & nerve

authors： Tankisi H,Otto M,Pugdahl K,Fuglsang-Frederiksen A

更新日期：2013-08-01 00:00:00

abstract：:Electrophysiologic findings in thallium intoxication are usually untimely, limited in extent, and often uninformative. This report documents serial conduction and electromyographic findings in a case of thallium poisoning, beginning 10 days after symptom onset and ending 24 months later. Initially, the plantar nerves ...

journal_title：Muscle & nerve

authors： Dumitru D,Kalantri A

更新日期：1990-05-01 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：INTRODUCTION:There have been few reports on subtypes of Guillain-Barré syndrome (GBS) in children. We compared clinical and laboratory findings of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). METHODS:One hundred forty children with GBS were included. Based on...

journal_title：Muscle & nerve

authors： Kalita J,Kumar M,Misra UK

更新日期：2018-05-01 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：INTRODUCTION:Stroke-related changes in maximal dynamic hip flexor muscle fatigability may be more relevant functionally than isometric hip flexor fatigability. METHODS:Ten chronic stroke survivors performed 5 sets of 30 hip flexion maximal dynamic voluntary contractions (MDVC). A maximal isometric voluntary contractio...

journal_title：Muscle & nerve

authors： Kuhnen HR,Rybar MM,Onushko T,Doyel RE,Hunter SK,Schmit BD,Hyngstrom AS

更新日期：2015-03-01 00:00:00

abstract：:Acetylcholine receptor (AChR) metabolism was studied in muscle from juvenile and adult dogs with congenital myasthenia gravis (CMG) and their unaffected littermates. Although the amount of AChR in the junctional region of innervated CMG muscle fibers was 25% of normal, or less, denervation of CMG fibers resulted in th...

journal_title：Muscle & nerve

authors： Oda K,Lennon VA,Lambert EH,Palmer AC

更新日期：1984-11-01 00:00:00

abstract：INTRODUCTION:Spinal and bulbar muscular atrophy (SBMA) is an adult-onset motor neuron disease caused by a CAG repeat expansion in the androgen receptor gene. The aim of this study was to verify whether urinary 8-hydroxydeoxyguanosine (8-OHdG), an oxidative stress marker, is a biomarker for SBMA. METHODS:We measured th...

journal_title：Muscle & nerve

authors： Mano T,Katsuno M,Banno H,Suzuki K,Suga N,Hashizume A,Tanaka F,Sobue G

更新日期：2012-11-01 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：:EDL muscles from normal and dystrophic (dy2j) mice of various ages were examined. Muscles were divided into three groups according to age: 7 to 14 days postnatal, 16 to 21 days postnatal, and 6 months old, to assess age and/or phenotype related differences in the muscle response to caffeine or high K+. The response of...

journal_title：Muscle & nerve

authors： Dangain J,Neering IR

更新日期：1993-01-01 00:00:00

abstract：INTRODUCTION:Accuracy of needle electromyography is typically ensured by use of anatomical landmarks and auditory feedback related to voluntary activation of the targeted muscle; however, in certain clinical situations, landmarks may not be palpable, auditory feedback may be limited or not present, and targeting a spec...

journal_title：Muscle & nerve

authors： Boon AJ,Oney-Marlow TM,Murthy NS,Harper CM,McNamara TR,Smith J

更新日期：2011-07-01 00:00:00

abstract：:Disappointing functional recovery following peripheral nerve repair can be improved by neurotrophic growth factors. Leukemia inhibitory factor (LIF) is unique in that it has independent neurotrophic and myotrophic actions. The aim of this study was to explain this finding by establishing the existence of anterograde a...

journal_title：Muscle & nerve

authors： Bennett TM,Dowsing BJ,Austin L,Messina A,Nicola NA,Morrison WA

更新日期：1999-01-01 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：:The compound muscle action potential (M(MAX)) during a maximal voluntary contraction (MVC) may be measured to determine if the motor nerve has been supramaximally stimulated during the interpolated twitch technique (ITT). Ten males performed isometric knee extension MVCs. M(MAX) for the vastus medialis was recorded du...

journal_title：Muscle & nerve

authors： Berger MJ,Watson BV,Doherty TJ

更新日期：2010-10-01 00:00:00

abstract：:Weakness is common in both hyper- and hypothyroidism, and skeletal muscle L-carnitine may play a role in this regard, as suggested by studies indicating abnormal levels of carnitine in serum and urine of patients with thyroid dysfunction. Skeletal muscle samples were obtained for carnitine analysis from control subjec...

journal_title：Muscle & nerve

authors： Sinclair C,Gilchrist JM,Hennessey JV,Kandula M

更新日期：2005-09-01 00:00:00

abstract：:When an active muscle is stretched, the force increases due to strain of contractile and noncontractile proteins. We examined this force enhancement in rat extensor digitorum longus (EDL) and soleus muscles, which differ in their composition of these proteins, and their susceptibility to damage. Small stretches were a...

journal_title：Muscle & nerve

authors： Ramsey KA,Bakker AJ,Pinniger GJ

更新日期：2010-11-01 00:00:00

abstract：INTRODUCTION:We investigated the possible role of intercostal surgical neurolysis in relieving chronic neuropathic pain refractory to other nonsurgical treatments in patients with postsurgical thoracic pain. METHODS:We retrospectively collected clinical data on patients referred to the Neurosurgery Unit of Policlinic ...

journal_title：Muscle & nerve

authors： Cappellari AM,Tiberio F,Alicandro G,Spagnoli D,Grimoldi N

更新日期：2018-11-01 00:00:00

abstract：:Many patients with the limb-girdle variant of congenital myasthenic syndrome (CMS) possess mutations in the human Dok-7 gene (DOK7). We identified six unrelated CMS patients with DOK7 mutations. Two patients, one mildly and the other moderately affected, were homozygous for the previously described 1263insC mutation. ...

journal_title：Muscle & nerve

authors： Anderson JA,Ng JJ,Bowe C,McDonald C,Richman DP,Wollmann RL,Maselli RA

更新日期：2008-04-01 00:00:00

abstract：:A link between the cardioprotective benefits of pharmacological preconditioning and natural mammalian hibernation is considered to involve the cellular activation of opioid receptors and subsequent opening of K(ATP) channels. In previous studies, we have demonstrated the protective effects of specific delta-opioid ago...

journal_title：Muscle & nerve

authors： Hong J,Sigg DC,Coles JA Jr,Oeltgen PR,Harlow HJ,Soule CL,Iaizzo PA

更新日期：2005-08-01 00:00:00

abstract：:We have devised a method of sensory nerve conduction in the interdigital nerves of the foot using the near-nerve needle and signal averaging techniques. In 40 normal controls between the ages of 20 and 59, there was no difficulty in recording sensory compound nerve action potential (CNAP) in the various interdigital n...

journal_title：Muscle & nerve

authors： Oh SJ,Kim HS,Ahmad BK

更新日期：1984-03-01 00:00:00

abstract：:The possibility has been raised that in familial periodic paralysis, the relatively large depolarization of the muscle fiber membrane seen during periods of paralysis might be attributable to a reduction in activity of the electrogenic Na(+)-K+ pump. The theoretical analysis presented here shows that although such hyp...

journal_title：Muscle & nerve

authors： Martin AR,Levinson SR

更新日期：1985-06-01 00:00:00

abstract：INTRODUCTION:In this study, we analyzed the crosstalk in mechanomyographic (MMG) signals generated by the extensor digitorum (ED), extensor carpi ulnaris (ECU), and flexor carpi ulnaris (FCU) muscles of the forearm during wrist flexion (WF) and extension (WE) and radial (RD) and ulnar (UD) deviations. METHODS:Twenty r...

journal_title：Muscle & nerve

authors： Islam A,Sundaraj K,Ahmad RB,Sundaraj S,Ahamed NU,Ali MA

更新日期：2015-06-01 00:00:00

abstract：:Several approaches to treat laminin alpha2 chain-deficient congenital muscular dystrophy (MDC1A) in mouse models have been undertaken. Most have shown promising results in young animals. However, older animals have only been characterized to some extent. Herein we analyze the lifespan of laminin alpha2 chain-deficient...

journal_title：Muscle & nerve

authors： Gawlik KI,Durbeej M

更新日期：2010-07-01 00:00:00