Effect of gender and obesity on electrical current thresholds.

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：: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：:The effect of endurance training on muscle electrical activity during general exercise testing was investigated in physically active patients with chronic obstructive pulmonary disease (COPD). Before and after rehabilitation, patients performed identical incremental exercise tests. Pulmonary gas exchange, venous lacta...

journal_title：Muscle & nerve

authors： Gosselin N,Lambert K,Poulain M,Martin A,Préfaut C,Varray A

更新日期：2003-12-01 00:00:00

abstract：INTRODUCTION:We studied neurodevelopmental and behavioral/emotional symptoms in patients with Duchenne muscular dystrophy (DMD). METHODS:Retrospective case series of neurodevelopmental and behavioral/emotional symptoms obtained through review of systems of 700 DMD patients in relation to dystrophin gene mutations. RE...

journal_title：Muscle & nerve

authors： Darmahkasih AJ,Rybalsky I,Tian C,Shellenbarger KC,Horn PS,Lambert JT,Wong BL

更新日期：2020-04-01 00:00:00

abstract：INTRODUCTION:Intensive care unit (ICU) patients often develop weakness. Rehabilitation is initiated early to prevent physical deterioration, but knowledge of optimal training schedules is lacking. A reliable method to assess muscle activity during exercise is needed. In this study we explored the feasibility of electri...

journal_title：Muscle & nerve

authors： Sommers J,Van Den Boorn M,Engelbert RHH,Nollet F,Van Der Schaaf M,Horn J

更新日期：2018-11-01 00:00:00

abstract：INTRODUCTION:The aim of this study was to determine factors affecting health-related quality of life (HRQOL) and to propose appropriate treatment targets for patients with myasthenia gravis (MG). METHODS:We evaluated 640 consecutive patients with MG seen at 11 neurological centers. Two-year follow-up data were obtaine...

journal_title：Muscle & nerve

authors： Utsugisawa K,Suzuki S,Nagane Y,Masuda M,Murai H,Imai T,Tsuda E,Konno S,Nakane S,Suzuki Y,Fujihara K,Suzuki N

更新日期：2014-10-01 00:00:00

abstract：INTRODUCTION:The aim of this study was to assess age- and gender-specific incidence rates (IRs) of acetylcholine receptor (AChR)-antibody-positive myasthenia gravis (MG) in South Africa, and geographical variations in incidence. METHODS:IRs were calculated from positive AChR-antibody laboratory data between 2011 and 2...

journal_title：Muscle & nerve

authors： Mombaur B,Lesosky MR,Liebenberg L,Vreede H,Heckmann JM

更新日期：2015-04-01 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：:Chronic inflammatory demyelinating polyneuropathy (CIDP) is usually characterized by prominent motor deficits. A pure sensory presentation, labeled chronic sensory demyelinating neuropathy (CSDN), has been reported, but it is unclear if this neuropathy is a distinct clinical and immunologic entity or merely the sensor...

journal_title：Muscle & nerve

authors： Berger AR,Herskovitz S,Kaplan J

更新日期：1995-04-01 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：:Chronic inflammatory demyelinating polyneuropathy (CIDP) presents in rare instances with focal or multifocal upper limb involvement. We reviewed the clinical and electromyographic (EMG) characteristics of 10 such patients (UL-CIDP) and compared them with patients with typical generalized CIDP (G-CIDP) and multifocal m...

journal_title：Muscle & nerve

authors： Gorson KC,Ropper AH,Weinberg DH

更新日期：1999-06-01 00:00:00

abstract：:The distinction between chronic demyelinating polyneuropathies associated with IgM paraproteinemia and anti-myelin-associated glycoprotein (MAG) antibodies (MAG-PN) and chronic inflammatory demyelinating polyneuropathies (CIDPs) relies on the anti-MAG antibodies assay. The aim of the study was to identify clinical and...

journal_title：Muscle & nerve

authors： Isoardo G,Migliaretti G,Ciaramitaro P,Rota E,Poglio F,Tavella A,Paolasso I,Cavallo F,Bergamasco B,Cocito D

更新日期：2005-01-01 00:00:00

abstract：:The mdx mouse, an animal model used to study Duchenne muscular dystrophy, has a nonsense mutation in exon 23 of the dystrophin gene which should result in a truncated protein that cannot be correctly localized at the sarcolemma of the muscle fibers. Immunohistochemical staining with antidystrophin antibodies has shown...

journal_title：Muscle & nerve

authors： Wilton SD,Dye DE,Laing NG

更新日期：1997-06-01 00:00:00

abstract：:The role of motor innervation and muscle tension in the posthatching maturation of the slow-tonic anterior latissimus dorsi (ALD) muscle of the chicken has been investigated. Modification of the muscle tension was obtained either by maintaining ALD in a shortened state or by stretching, after or without denervation. I...

journal_title：Muscle & nerve

authors： Gardahaut MF,Rouaud TP,Renaud D,Khaskiye A,Le Douarin GH

更新日期：1989-11-01 00:00:00

abstract：:We compared the diaphragmatic electromyographic (EMG) recordings from 32 patients with known neuromuscular disease and respiratory symptoms (23 neuropathies, 9 myopathies) to recordings from 23 normal subjects. Turns analysis of 219-ms sections, or epochs, of the EMG demonstrated a significant overlap between diagnost...

journal_title：Muscle & nerve

authors： McKeown MJ,Bolton CF

更新日期：1998-07-01 00:00:00

abstract：:The purpose of the present study was to establish the relationship between muscle architecture and H-reflex recordings in quadriceps femoris muscle. H-reflexes were elicited in human quadriceps femoris muscle over a broad area of skin to document the shape and amplitude of the H-potentials. This, in combination with r...

journal_title：Muscle & nerve

authors： Garland SJ,Gerilovsky L,Enoka RM

更新日期：1994-06-01 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：:To define numerically the clinical severity of facioscapulohumeral muscular dystrophy (FSHD), we developed a protocol that quantifies muscle weakness by combining the functional evaluation of six muscle groups affected in this disease. To validate reproducibility of the protocol, 69 patients were recruited. Each patie...

journal_title：Muscle & nerve

authors： Lamperti C,Fabbri G,Vercelli L,D'Amico R,Frusciante R,Bonifazi E,Fiorillo C,Borsato C,Cao M,Servida M,Greco F,Di Leo R,Volpi L,Manzoli C,Cudia P,Pastorello E,Ricciardi L,Siciliano G,Galluzzi G,Rodolico C,Santoro L

更新日期：2010-08-01 00:00:00

abstract：BACKGROUND:This was a multicenter, double-blind, randomized clinical trial to investigate the efficacy of electrical stimulation of denervated muscle (ESDM) on recovery of patients with peripheral nerve injuries. METHODS:We enrolled 38 patients with traumatic peripheral nerve injuries with axonal damage and clinical i...

journal_title：Muscle & nerve

authors： Piccinini G,Cuccagna C,Caliandro P,Coraci D,Germanotta M,Pecchioli C,Padua L

更新日期：2020-06-01 00:00:00

abstract：:L-carnitine stimulated CO2 production from 1-14C pyruvate in mitochondria from human skeletal muscle nearly twofold. A comparable increase in the pyruvate dehydrogenase complex (PDHC) activity was seen. Moreover, in the presence of L-carnitine and at pyruvate concentration greater than 0.25 mM, this effect was associa...

journal_title：Muscle & nerve

authors： Uziel G,Garavaglia B,Di Donato S

更新日期：1988-07-01 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：:Platelet-derived microparticle (PDMP) levels were measured using an enzyme-linked immunosorbent assay (ELISA) to elucidate the role of platelet activation in patients with polymyositis or dermatomyositis (PM/DM). PDMP levels in active PM/DM patients (median 13.3 U/ml, interquartile range 9.9-20.7 U/ml, n = 16) and tho...

journal_title：Muscle & nerve

authors： Shirafuji T,Hamaguchi H,Higuchi M,Kanda F

更新日期：2009-05-01 00:00:00

abstract：INTRODUCTION:Cachexia increases morbidity and mortality of cancer patients. The progressive loss of muscle mass negatively affects physical function and quality of life. We previously showed reduced muscle insulin-like growth factor-1 (IGF-1) expression and enhanced myostatin signaling in tumor-bearing animals. This st...

journal_title：Muscle & nerve

authors： Bonetto A,Penna F,Aversa Z,Mercantini P,Baccino FM,Costelli P,Ziparo V,Lucia S,Rossi Fanelli F,Muscaritoli M

更新日期：2013-09-01 00:00:00

abstract：:Recent studies on the distribution of labeled endogenous proteins in the experimental neuropathies induced by streoptozotocin diabetes, galactose feeding, zinc pyridinethione, 2,5-hexanedione, acrylamide, and p-bromophenylacetylurea (BPAU) have demonstrated an impaired build up of retrogradely transported material der...

journal_title：Muscle & nerve

authors： Jakobsen J,Brimijoin S,Sidenius P

更新日期：1983-02-01 00:00:00

abstract：INTRODUCTION:Polyneuropathy signs (Neuropathy Impairment Score, NIS), neurophysiologic tests (m+7Ionis ), disability, and health scores were assessed in baseline evaluations of 100 patients entered into an oligonucleotide familial amyloidotic polyneuropathy (FAP) trial. METHODS:We assessed: (1) Proficiency of grading ...

journal_title：Muscle & nerve

authors： Dyck PJ,Kincaid JC,Dyck PJB,Chaudhry V,Goyal NA,Alves C,Salhi H,Wiesman JF,Labeyrie C,Robinson-Papp J,Cardoso M,Laura M,Ruzhansky K,Cortese A,Brannagan TH 3rd,Khoury J,Khella S,Waddington-Cruz M,Ferreira J,Wang AK,

更新日期：2017-11-01 00:00:00

abstract：:At least 50 disease-causing mutations in the skeletal muscle voltage-gated chloride channel gene (CLCN1), almost all of which originate from Caucasian families, have been identified. We investigated a Japanese family with Thomsen's myotonia congenita that included 16 affected individuals (8 men and 8 women) through fi...

journal_title：Muscle & nerve

authors： Sasaki R,Ito N,Shimamura M,Murakami T,Kuzuhara S,Uchino M,Uyama E

更新日期：2001-03-01 00:00:00

abstract：:Ion channels are complex proteins that span the lipid bilayer of the cell membrane, where they orchestrate the electrical signals necessary for normal function of the central nervous system, peripheral nerve, and both skeletal and cardiac muscle. The role of ion channel defects in the pathogenesis of numerous disorder...

journal_title：Muscle & nerve

authors： Kleopa KA,Barchi RL

更新日期：2002-09-01 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：INTRODUCTION:Pes cavus often signals the presence of Charcot-Marie-Tooth (CMT) in adult patients, although its prevalence in the general population makes it a finding of unclear significance. METHODS:We undertook a pilot double cohort study to investigate the feasibility of comparing preselected bedside and radiograph...

journal_title：Muscle & nerve

authors： Stino AM,Atway S,Anthony M,Kline D,Kissel JT

更新日期：2019-01-01 00:00:00

abstract：:There were 31 Duchenne patients placed on overnight mouth intermittent positive pressure ventilation for severe respiratory insufficiency at the average age of 19.9 years. Most patients had vital capacities less than 200 cc at their last evaluations. Of these, 23 patients are alive at the average age of 27 years and l...

journal_title：Muscle & nerve

authors： Bach JR,O'Brien J,Krotenberg R,Alba AS

更新日期：1987-02-01 00:00:00