Myotonic Dystrophy Health Index: initial evaluation of a disease-specific outcome measure.

abstract：:The ideal electrodiagnostic procedure to assess possible plantar neuropathies continues to elude investigators. Motor studies are rarely abnormal, pure sensory studies may be difficult to obtain, needle electromyography can demonstrate membrane instability in normal feet. Mixed nerve plantar studies may be more diagno...

journal_title：Muscle & nerve

authors： Dumitru D,Kalantri A,Dierschke B

更新日期：1991-07-01 00:00:00

abstract：INTRODUCTION:Pneumothorax is a potentially serious complication of electromyography (EMG). Data on the frequency of pneumothorax after EMG are lacking. The purpose of this study was to determine the frequency, timing, and risk factors for iatrogenic pneumothorax after EMG. METHODS:Cases of pneumothorax after EMG were ...

journal_title：Muscle & nerve

authors： Kassardjian CD,O'gorman CM,Sorenson EJ

更新日期：2016-04-01 00:00:00

abstract：:Diseases involving chronic inflammation can lead to prolonged exposure of skeletal muscle to inflammatory cytokines such as tumor necrosis factor alpha (TNFalpha), which may contribute to the skeletal muscle weakness seen in these conditions. In this study we examined the effect of a prolonged exposure to TNFalpha on ...

journal_title：Muscle & nerve

authors： van Kann LN,Bakker AJ

更新日期：2007-02-01 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：:Congenital myasthenic syndromes (CMS) stem from defects in presynaptic, synaptic basal lamina, and postsynaptic proteins. The presynaptic CMS are associated with defects that curtail the evoked release of acetylcholine (ACh) quanta or ACh resynthesis. Defects in ACh resynthesis have now been traced to mutations in cho...

journal_title：Muscle & nerve

authors： Engel AG,Ohno K,Sine SM

更新日期：2003-01-01 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:Glucocorticosteroids (GC) are effective in slowing weakness in boys with Duchenne muscular dystrophy (DMD). METHODS:This is a multisite, 1-year, open-label trial of twice-weekly prednisolone (5 mg/kg/dose) in infants/young boys (0.4-2.4 years) with DMD. We compared changes in Bayley III Scales of Infant D...

journal_title：Muscle & nerve

authors： Connolly AM,Zaidman CM,Golumbek PT,Cradock MM,Flanigan KM,Kuntz NL,Finkel RS,McDonald CM,Iannaccone ST,Anand P,Siener CA,Florence JM,Lowes LP,Alfano LN,Johnson LB,Nicorici A,Nelson LL,Mendell JR,MDA DMD Clinical Resea

更新日期：2019-06-01 00:00:00

abstract：:Six members originating from two families with hereditary motor and sensory neuropathy (hypertrophic and neuronal types) were noted to have enlarged calf muscles. Muscle computed tomography revealed that muscle enlargement in the propositus of the family with the hypertrophic type of HMSN was due to an increase in mus...

journal_title：Muscle & nerve

authors： De Visser M,Hoogendijk JE,Ongerboer BW,Verbeeten BJ Jr

更新日期：1990-01-01 00:00:00

abstract：INTRODUCTION:Piriformis muscle syndrome (PS) is a disorder encompassing a constellation of symptoms, including buttock and hip pain. In this study we aimed to assess the value of ultrasound (US) in the diagnosis of PS. METHODS:Thirty-three clinically diagnosed PS patients and 26 healthy volunteers underwent a clinical...

journal_title：Muscle & nerve

authors： Zhang W,Luo F,Sun H,Ding H

更新日期：2019-04-01 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：:In this study we investigated the gene expression of proteins related to myostatin (MSTN) signaling during skeletal muscle longitudinal growth. To promote muscle growth, Wistar male rats were submitted to a stretching protocol for different durations (12, 24, 48, and 96 hours). Following this protocol, soleus weight a...

journal_title：Muscle & nerve

authors： Aoki MS,Soares AG,Miyabara EH,Baptista IL,Moriscot AS

更新日期：2009-12-01 00:00:00

abstract：:Lamins are the principal components of the nuclear lamina, a network constituting the major structural framework of the nuclear envelope. Alterations in lamin A/C have been associated with a heterogeneous series of human disorders known as laminopathies. We report the finding of a novel deletion in the central rod dom...

journal_title：Muscle & nerve

authors： Maioli MA,Marrosu G,Mateddu A,Solla E,Carboni N,Tacconi P,Lai C,Marrosu MG

更新日期：2007-12-01 00:00:00

abstract：INTRODUCTION:Mutations in gap junction protein beta 1 (GJB1) on the X chromosome represent one of the most common causes of hereditary neuropathy. We assessed manifestations associated with a rare 3' untranslated region mutation (UTR) of GJB1 in a large family with X-linked Charcot-Marie-Tooth disease (CMTX). METHODS:...

journal_title：Muscle & nerve

authors： Chen DH,Ma M,Scavina M,Blue E,Wolff J,Karna P,Dorschner MO,Raskind WH,Bird TD

更新日期：2018-05-01 00:00:00

abstract：:Genetically homozygous line 413 dystrophic chickens were given twice-daily intraperitoneal injections of solutions containing branched-chain amino acids (BCCA-leucine, valine, isoleucine) either alone or in combination; and their alpha-ketoacid analogs (alpha-ketoisocaproic and alpha-ketoisovaleric acids). Another tri...

journal_title：Muscle & nerve

authors： Hudecki MS,Pollina CM,Heffner RR

更新日期：1982-07-01 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：INTRODUCTION:Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are considered part of the same pathological spectrum. There is an increased risk of ALS in patients who have had melanoma. The risk of FTLD in melanoma (or cancer) patients is unknown. We aimed to study if C9ORF72 expansion i...

journal_title：Muscle & nerve

authors： Tábuas-Pereira M,Almendra L,Almeida MR,Durães J,Pinho A,Matos A,Negrão L,Geraldo A,Santana I

更新日期：2019-03-01 00:00:00

abstract：:The dropped head syndrome occurs in a variety of neuromuscular disorders. We present a woman with chronic inflammatory demyelinating polyneuropathy who developed this syndrome, likely reflecting severe demyelination of nerves to cervical paraspinal muscles. ...

journal_title：Muscle & nerve

authors： Hoffman D,Gutmann L

更新日期：1994-07-01 00:00:00

abstract：:The objective of this study was to investigate different methods of estimating muscle inactivation, derived from single and multiple voluntary contractions. Ten subjects performed maximal and submaximal leg extensor contractions to determine an interpolation (IT) or central activation ratio (CAR). A superimposed evoke...

journal_title：Muscle & nerve

authors： Behm D,Power K,Drinkwater E

更新日期：2001-07-01 00:00:00

abstract：:In normal skeletal muscle, prolonged stimulation results in some cellular adenosine triphosphate (ATP) being converted to adenosine monophosphate (AMP) and then deaminated to inosine monophosphate (IMP). Here, we investigate whether the build-up of IMP contributes to muscle fatigue and also determine what happens if A...

journal_title：Muscle & nerve

authors： Blazev R,Lamb GD

更新日期：1999-12-01 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：INTRODUCTION:The molecular mechanism of immune-mediated necrotizing myopathy (IMNM) remains unknown. Autophagy impairment, described in autoimmune diseases, is a key process in myofiber protein degradation flux and muscle integrity and has not been studied in IMNM. METHODS:Muscle biopsies from patients with IMNM (n = ...

journal_title：Muscle & nerve

authors： Girolamo F,Lia A,Annese T,Giannini M,Amati A,D'Abbicco D,Tampoia M,Virgintino D,Ribatti D,Serlenga L,Iannone F,Trojano M

更新日期：2019-09-01 00:00:00

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：:In recent years, the following ideas have been expressed: (a) that all cases of a discrete, inherited neuromuscular syndrome should prove to be due to a single biochemical defect, (b) that any single biochemical defect should give rise only to one syndrome, and (c) that an enzymatic defect cannot give rise to a diseas...

journal_title：Muscle & nerve

authors： Kark RA,Becker DM

更新日期：1981-01-01 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

abstract：INTRODUCTION:Information related to the long-term follow-up of neuropathy in patients with familial amyloid polyneuropathy after liver transplantation is still scarce. METHODS:We describe the neuropathic features of 3 patients with the transthyretin Val30Met mutation. Each patient underwent liver transplantation at an...

journal_title：Muscle & nerve

authors： Koike H,Hashimoto R,Tomita M,Kawagashira Y,Iijima M,Nakamura T,Watanabe H,Kamei H,Kiuchi T,Sobue G

更新日期：2012-12-01 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：:The distribution of collagen types I, III, and V and fibronectin was investigated by means of immunofluorescent techniques in denervated and normal rat skeletal muscle. During a period of 28 days, a distinct atrophy developed in the denervated gastrocnemius muscle and was accompanied by an increase in types I and III ...

journal_title：Muscle & nerve

authors： Salonen V,Lehto M,Kalimo M,Penttinen R,Aro H

更新日期：1985-02-01 00:00:00

abstract：:We studied the effects of a 12-week progressive resistance strength training program in weakened muscles of 5 patients with sporadic inclusion body myositis (IBM). Strength was evaluated with Medical Research Council (MRC) scale ratings and quantitative isometric and dynamic tests. Changes in serum creatine kinase (CK...

journal_title：Muscle & nerve

authors： Spector SA,Lemmer JT,Koffman BM,Fleisher TA,Feuerstein IM,Hurley BF,Dalakas MC

更新日期：1997-10-01 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：:The purpose of this study was to evaluate the effects of spinal cord injury (SCI) and functional electrical stimulation (FES) of paralyzed muscles on type IV collagen content and proteins involving its degradation, which is initiated by matrix metalloproteinase (MMP)-2 and -9 and regulated by their tissue inhibitors (...

journal_title：Muscle & nerve

authors： Koskinen SO,Kjaer M,Mohr T,Sørensen FB,Suuronen T,Takala TE

更新日期：2000-04-01 00:00:00