Spontaneous electrical muscle fiber activity in polymyositis and dermatomyositis.

abstract：:Abnormalities of GM2 ganglioside metabolism owing to hexosaminidase A (Hex A) deficiency have been associated with ALS phenotypes. The clinical features described in these ALS patients with Hex A deficiency include early onset, positive family history, and/or long disease duration. In an attempt to determine prospecti...

journal_title：Muscle & nerve

authors： Gudesblatt M,Ludman MD,Cohen JA,Desnick RJ,Chester S,Grabowski GA,Caroscio JT

更新日期：1988-03-01 00:00:00

abstract：INTRODUCTION:Despite the existence of guidelines, painful neuropathy is often inappropriately treated. We sought to determine the effectiveness of a clinical decision support system on guideline-recommended medication use. METHODS:We randomized neurology providers, stratified by subspecialty, to a best practice alert ...

journal_title：Muscle & nerve

authors： Reynolds EL,Burke JF,Banerjee M,Callaghan BC

更新日期：2020-05-01 00:00:00

abstract：:Cardiotrophin-1 (CT-1) has potent survival-promoting effects on motor neurons in vitro and in vivo and may be effective in treating motor neuron diseases (MND). We investigated the effects of CT-1 treatment in wobbler mouse MND. Wobbler mice were randomly assigned to receive subcutaneously injected CT-1 (1 mg/kg, n = ...

journal_title：Muscle & nerve

authors： Mitsumoto H,Klinkosz B,Pioro EP,Tsuzaka K,Ishiyama T,O'Leary RM,Pennica D

更新日期：2001-06-01 00:00:00

abstract：:Case reports and open-label studies suggest that coenzyme Q(10) (CoQ(10)) treatment may have beneficial effects in mitochondrial disease patients; however, controlled trials are warranted to clinically prove its effectiveness. Thirty patients with mitochondrial cytopathy received 1200 mg/day CoQ(10) for 60 days in a r...

journal_title：Muscle & nerve

authors： Glover EI,Martin J,Maher A,Thornhill RE,Moran GR,Tarnopolsky MA

更新日期：2010-11-01 00:00:00

abstract：:We have examined the clinical features of patients with femoral neuropathy and the factors that influence the prognosis. Of 80 consecutive patients referred for neurophysiological evaluations of proximal lower limb weakness, 32 fulfilled strict inclusion criteria and had adequate information, including estimates of ax...

journal_title：Muscle & nerve

authors： Kuntzer T,van Melle G,Regli F

更新日期：1997-02-01 00:00:00

abstract：:Neuromyotonia or Isaacs' syndrome is a rare peripheral nerve hyperexcitability disorder caused by antibodies against potassium channels of myelinated axons. We present the high-density surface electromyographic (EMG) recordings of a patient with fasciculations and cramps due to neuromyotonia. To characterize the time ...

journal_title：Muscle & nerve

authors： Kleine BU,Stegeman DF,Drost G,Zwarts MJ

更新日期：2008-02-01 00:00:00

abstract：:Patients with chronic inflammatory demyelinating polyneuropathy (CIDP) received immune globulin intravenous, 10% caprylate/chromatography purified (IGIV-C, Gamunex; n=59) or placebo (n=58) every 3 weeks for up to 24 weeks (first period) in a randomized, double-blind, parallel-group, response-conditional, crossover stu...

journal_title：Muscle & nerve

authors： Bril V,Katzberg H,Donofrio P,Banach M,Dalakas MC,Deng C,Hanna K,Hartung HP,Hughes RA,Latov N,Merkies IS,van Doorn PA,ICE Study Group.

更新日期：2009-04-01 00:00:00

abstract：INTRODUCTION:Eosinophilic fasciitis (EF) is a rare disorder that can present with muscle symptoms that mimic other neuromuscular diseases. METHODS:We report the case of a 43-year-old woman with chronic muscle aches, tightness, and stiffness with hypertrophied, well-defined muscles despite physical inactivity, and thic...

journal_title：Muscle & nerve

authors： Whitlock JB,Dimberg EL,Selcen D,Rubin DI

更新日期：2017-09-01 00:00:00

abstract：INTRODUCTION:Hand-held dynamometry (HHD) is commonly used to measure ankle plantarflexion strength but has variable reliability measuring higher forces. Fixed HHD is suggested to improve reliability. We, therefore, compared the reliability, consistency, and accuracy of measuring plantarflexion strength. METHODS:Planta...

journal_title：Muscle & nerve

authors： Davis PR,McKay MJ,Baldwin JN,Burns J,Pareyson D,Rose KJ

更新日期：2017-11-01 00:00:00

abstract：INTRODUCTION:Peripheral nerve injuries (PNI) are among the leading causes of physical disability in the United States. The majority of injuries occur in the upper extremities, and functional recovery is often limited. Robust animal models are critical first steps for developing effective therapies to restore function a...

journal_title：Muscle & nerve

authors： Meyers EC,Granja R,Solorzano BR,Romero-Ortega M,Kilgard MP,Rennaker RL 2nd,Hays S

更新日期：2017-12-01 00:00:00

abstract：:The intramembrane particle (IMP) profile of control and dystrophic (Bio 14.6) hamster cardiac muscle plasma membrane was assessed in freeze-fracture replicas to determine whether this animal model of muscular dystrophy exhibits the same membrane characteristics found in skeletal muscle from other more thoroughly studi...

journal_title：Muscle & nerve

authors： Graham KA,Shivers RR,Atkinson BG

更新日期：1984-09-01 00:00:00

abstract：:Single fiber electromyography ( SFEMG ) of the extensor digitorum communis (EDC) muscle and nerve conduction studies were performed on healthy, active elderly men (66-77 years old) to assess age-related changes in neuromuscular physiology and the effect of long-term increased muscular activity on these changes. The fo...

journal_title：Muscle & nerve

authors： Shields RW Jr,Robbins N,Verrilli AA 3rd

更新日期：1984-05-01 00:00:00

abstract：:This study analyses maximum voluntary isometric contraction (MVIC) and its measurement by recording the force profile during maximal-effort, 7-s hand-grip contractions. Six healthy subjects each performed three trials repeated at short intervals to study variation from fatigue. These three trials were performed during...

journal_title：Muscle & nerve

authors： Househam E,McAuley J,Charles T,Lightfoot T,Swash M

更新日期：2004-03-01 00:00:00

abstract：:Cultured human skin fibroblasts from 9 patients with Duchenne muscular dystrophy (DMD) and 8 normal age- and sex-matched controls were examined for creatine kinase (CK) activity. Both the normal and the DMD fibroblasts were found to have significant levels of CK activity (approximately 10 x 10(-3) IU per milligram of ...

journal_title：Muscle & nerve

authors： Davis MH,Cappel R,Vester JW,Samaha FJ,Gruenstein E

更新日期：1982-01-01 00:00:00

abstract：:Studies using quantitative sensory testing (QST) often present incongruent results due to intra- and intersubject as well as interobserver variability which limit widespread use of the technique. Eliminating or reducing the factors responsible for this variability is of great interest, as it increases reliability and ...

journal_title：Muscle & nerve

authors： Pavlaković G,Klinke I,Pavlaković H,Züchner K,Zapf A,Bachmann CG,Graf BM,Crozier TA

更新日期：2008-11-01 00:00:00

abstract：:Mitochondrial preparations derived from denervated rat skeletal muscle and paired control muscle are characterized with respect to their oxidative and phosphorylative capacities. Our data indicate that there is an impairment within the first 2 energy coupling regions of the respiratory chain and within the ATPase comp...

journal_title：Muscle & nerve

authors： Joffe M,Savage N,Isaacs H

更新日期：1981-11-01 00:00:00

abstract：:In order to assess the clinical utility of trigemino-facial reflexes in lower facial muscles, we studied perioral reflexes to mechanical and electrical stimulation in 13 patients with spasmodic dysphonia and orofacial dyskinesia and in 7 healthy subjects. Mechanical stimulation of the upper lip of all patients and ele...

journal_title：Muscle & nerve

authors： Topka H,Hallett M

更新日期：1992-09-01 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：:Skeletal muscle cells are hypertrophied by mechanical stresses, but the underlying molecular mechanisms are not fully understood. Two signaling pathways, phosphatidylinositol 3-kinase (PI3K)/Akt to target of rapamycin (TOR) and extracellular signal-regulated kinase kinase (MEK) to extracellular signal-regulated kinase...

journal_title：Muscle & nerve

authors： Sasai N,Agata N,Inoue-Miyazu M,Kawakami K,Kobayashi K,Sokabe M,Hayakawa K

更新日期：2010-01-01 00:00:00

abstract：:KANDID is an advanced EMG decision support system dedicated to the support of the clinical neurophysiologist during EMG examinations. It has facilities for test planning, automatized and structured data interpretation, EMG diagnosis, explanation, and reporting. In a prospective European multicenter field trial, the ag...

journal_title：Muscle & nerve

authors： Vingtoft S,Fuglsang-Frederiksen A,Rønager J,Petrera J,Stigsby B,Willison RG,Jarratt JA,Fawcett PR,Schofield IS,Otte G

更新日期：1993-05-01 00:00:00

abstract：:The lack of effective therapy for many of the chronic neuromuscular diseases such as amyotrophic lateral sclerosis, hereditary motor sensory neuropathy (Charcot-Marie-Tooth disease), spinocerebellar degenerations and idiopathic polyneuropathy has led to a search for substances that may stimulate peripheral nerve regen...

journal_title：Muscle & nerve

authors： Bradley WG

更新日期：1990-09-01 00:00:00

abstract：INTRODUCTION:Cranial muscle fasciculations may be difficult to detect in amyotrophic lateral sclerosis (ALS). Ultrasound (US) detection of fasciculations in these muscles may have clinical utility. METHODS:Patients with suspected ALS were prospectively enrolled. Nerve conduction studies, needle electromyography (EMG),...

journal_title：Muscle & nerve

authors： O'gorman CM,Weikamp JG,Baria M,Van Den Engel-Hoek L,Kassardjian C,Van Alfen N,Boon AJ

更新日期：2017-12-01 00:00:00

abstract：:Compound muscle action potentials (CMAPs) evoked by magnetic brain stimuli are larger if the subject provides a steady background voluntary contraction of the target muscle. This facilitation could be due either to cortical or spinal mechanisms, or both. Both magnetic and electrical stimuli given immediately after the...

journal_title：Muscle & nerve

authors： Mills KR,Kimiskidis V

更新日期：1996-08-01 00:00:00

abstract：INTRODUCTION:We quantified intraneural blood flow (INBF) in 18 patients with end-stage kidney disease (ESKD) and examined its relationship with nerve size, neuropathy severity, and nerve excitability parameters. METHODS:Sonographic measurements of the median nerve were performed at the same site before and after hemod...

journal_title：Muscle & nerve

authors： Borire AA,Arnold R,Pussell BA,Kwai NC,Visser LH,Simon NG,Kiernan MC,Krishnan AV

更新日期：2018-02-01 00:00:00

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：: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: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 model of chronic low-frequency stimulation for the study of muscle plasticity was developed over 30 years ago. This protocol leads to a transformation of fast, fatigable muscles toward slower, fatigue-resistant ones. It involves qualitative and quantitative changes of all elements of the muscle fiber studied so fa...

journal_title：Muscle & nerve

authors： Pette D,Vrbová G

更新日期：1999-06-01 00:00:00

abstract：INTRODUCTION:The 6-minute walk test (6MWT) is a well-established clinical assessment of functional endurance, validated as a measure of walking ability in spinal muscular atrophy (SMA). The current availability of disease-modifying therapies for SMA indicates a growing need for normative reference data to compare SMA p...

journal_title：Muscle & nerve

authors： Goodwin AM,Cornett KMD,McKay MJ,Burns J,Garber CE,De Vivo DC,Montes J

更新日期：2020-03-01 00:00:00

abstract：:An electrophysiologic study of peripheral nerve excitability was performed in patients with hyperkalemic periodic paralysis (HPP) and in normal controls. It was found that a marked degree of neural hyperexcitability existed in all patients with HPP who showed cold sensitivity. The results suggest involvement of the ne...

journal_title：Muscle & nerve

authors： Segura RP,Petajan JH

更新日期：1979-07-01 00:00:00