Inflammatory demyelinating neuropathy heralding accelerated chediak-higashi syndrome.

abstract：:Based on the known trophic effects of growth hormone (GH) on nerve and muscle 75 patients with ALS were treated for up to 18 months with synthetic human growth hormone (Protropin) or a placebo. The course of ALS was assessed serially using a quantitative (TQNE) neuromuscular and manual exam (MRC) and laboratory chemis...

journal_title：Muscle & nerve

authors： Smith RA,Melmed S,Sherman B,Frane J,Munsat TL,Festoff BW

更新日期：1993-06-01 00:00:00

abstract：:Motor evoked potentials (MEPs) obtained by electrical root stimulation and F waves were used to examine the proximal nerve conduction velocity (CV) to tibialis anterior (TA), extensor digitorum brevis (EDB), flexor carpi radialis (FCR), and abductor pollicis brevis (APB) muscles in 40 humans. By subtracting motor late...

journal_title：Muscle & nerve

authors： Zwarts MJ,Guechev A

更新日期：1995-11-01 00:00:00

abstract：:Two sisters with a Charcot-Marie-Tooth disease type 1A (CMT1A) duplication, who had an unusual CMT1A clinical phenotype, are described. The 63-year-old proband presented with dysesthesia on the inner side of the right leg. Neurological examination revealed a localized sensory disturbance in the lower extremities and m...

journal_title：Muscle & nerve

authors： Murakami T,Oomori H,Hara A,Uyama E,Mita S,Uchino M

更新日期：1999-11-01 00:00:00

abstract：INTRODUCTION:Although formal spirometry is the gold standard for monitoring respiratory function in patients with myasthenia gravis (MG), such testing is often delayed or unavailable. There is a need for a simple bedside test that can accurately measure respiratory function. METHODS:We conducted a prospective, cross-s...

journal_title：Muscle & nerve

authors： Elsheikh B,Arnold WD,Gharibshahi S,Reynolds J,Freimer M,Kissel JT

更新日期：2016-01-01 00:00:00

abstract：:The purpose of this study was to investigate the influence of an 8-week resistance training program on force steadiness and common drive for the vastus lateralis muscle. Eight untrained men performed a resistance training program. Before the program and at the end of each week, the subjects performed a trapezoid isome...

journal_title：Muscle & nerve

authors： Beck TW,Defreitas JM,Stock MS,Dillon MA

更新日期：2011-02-01 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：INTRODUCTION:Pretreatment with pyridostigmine bromide (PB) of human intercostal muscle fibers exposed to the irreversible acetylcholinesterase (AChE) inhibitor soman was investigated. METHODS:Muscles were pretreated with 3 × 10(-6) M PB or saline for 20 minutes, then exposed to 10(-7) M soman for 10 minutes. RESULTS:...

journal_title：Muscle & nerve

authors： Maselli RA,Henderson JD,Ng J,Follette D,Graves G,Wilson BW

更新日期：2011-04-01 00:00:00

abstract：:The axonal distribution of voltage-dependent Na+ channels was determined during inflammatory demyelinating disease of the peripheral nervous system. Experimental allergic neuritis was induced in Lewis rats by active immunization. In diseased spinal roots Na+ channel immunofluorescence at many nodes of Ranvier changed ...

journal_title：Muscle & nerve

authors： Novakovic SD,Levinson SR,Schachner M,Shrager P

更新日期：1998-08-01 00:00:00

abstract：:The consequences of general hypoxemia (PaO2 = 51 mmHg) on two muscle groups (adductor pollicis and diaphragm) sustaining 80% maximal isometric voluntary contraction were studied in healthy individuals. For adductor pollicis, contractions were also executed after 10-s or 3-min rest ischemia. Compared to control, i.e., ...

journal_title：Muscle & nerve

authors： Badier M,Guillot C,Lagier-Tessonnier F,Jammes Y

更新日期：1994-05-01 00:00:00

abstract：INTRODUCTION:Anecdotal reports from families and care providers suggest a wide variation in services received by individuals with Duchenne/Becker muscular dystrophy (DBMD). METHODS:We documented the type and frequency of health services received by individuals with DBMD using the Muscular Dystrophy Surveillance Tracki...

journal_title：Muscle & nerve

authors： Pandya SK,Campbell KA,Andrews JG,Meaney FJ,Ciafaloni E

更新日期：2016-02-01 00:00:00

abstract：:The degree of upper extremity active range of motion provided by an admittance control robot compared with a commercially available passive arm support for individuals with DMD who have limited arm function was investigated in this study. The reachable workspace evaluation was used to assess active range of motion pro...

journal_title：Muscle & nerve

authors： Corrigan MC,Foulds RA

更新日期：2020-06-01 00:00:00

abstract：:A careful monitoring of the accuracy of diagnosis in six cases of Guillain-Barre syndrome has shown that a substantial proportion of these patients initially diagnosed as having Guillain-Barre syndrome on the basis of characteristic clinical findings and an elevated level of protein in the spinal fluid had a neuropath...

journal_title：Muscle & nerve

authors： Feit H,Tindall RS,Glasberg M

更新日期：1982-02-01 00:00:00

abstract：:Immunologic syndromes are associated with hepatitis B viral (HBV) infection. However, mononeuropathy multiplex (MM), a syndrome in which immune factors may play a role, is rare in acute HBV infection. Few cases of MM associated with HBV infection have been reported. We report a case of acute HBV infection in which MM ...

journal_title：Muscle & nerve

authors： Cohen JA,Wilborn SL,Rector WG Jr,Golitz LE

更新日期：1990-03-01 00:00:00

abstract：:The absolute number of satellite cells (SC) in young and adult rat skeletal muscle was estimated by correlating data from light and electron microscopic quantitation of myofiber nuclei (myonuclei and satellite cell nuclei) with biochemical assays for determining total muscle DNA content. Expressed both as a proportion...

journal_title：Muscle & nerve

authors： Gibson MC,Schultz E

更新日期：1983-10-01 00:00:00

abstract：:In anterior interosseous nerve syndrome and ulnar neuropathy, paralysis or weakness of the flexor digitorum profundus (FDP) muscles has been shown to vary according to the degree of involvement of the median and ulnar nerves, respectively. We traced these nerves in 50 cadaveric specimens in which each FDP was complete...

journal_title：Muscle & nerve

authors： Oh CS,Won HS,Lee KS,Chung IH,Kim SM

更新日期：2009-04-01 00:00:00

abstract：:Being the smallest functional units under neural control, motor units play an integral role in muscle physiology. However, at the present time, there does not exist any widely accepted technique for quantifying or estimating the number of motor units in a muscle. Specifically, the existing techniques are the increment...

journal_title：Muscle & nerve

authors： Slawnych MP,Laszlo CA,Hershler C

更新日期：1990-11-01 00:00:00

abstract：INTRODUCTION:Mutations in the guanosine diphosphate-mannose pyrophosphorylase-B gene (GMPPB) have been identified in congenital muscular dystrophies, limb-girdle muscular dystrophy (LGMD2T), and congenital myasthenic syndromes (CMSs); overall, 41 patients have been described. METHODS:Two patients presented with a myas...

journal_title：Muscle & nerve

authors： Montagnese F,Klupp E,Karampinos DC,Biskup S,Gläser D,Kirschke JS,Schoser B

更新日期：2017-08-01 00:00:00

abstract：:We present three new and 14 retrospective cases of polyradiculopathy in sarcoidosis. Of these, 71% had weakness and 59% areflexia of the lower extremities, and 35% had sphincter dysfunction. Cases often were associated with central nervous system sarcoidosis. All cases involved thoracolumbar or lumbosacral roots, exce...

journal_title：Muscle & nerve

authors： Koffman B,Junck L,Elias SB,Feit HW,Levine SR

更新日期：1999-05-01 00:00:00

abstract：:The extensor of the second digit of the chicken wing (EDII) is a small, fast-twitch muscle susceptible to chicken muscular dystrophy and well suited for correlated studies of the morphology and physiology of identified nerve and muscle fibers. In cross section, the dystrophic EDII shows morphological abnormalities com...

journal_title：Muscle & nerve

authors： Gunther JS,Letinsky MS

更新日期：1982-01-01 00:00:00

abstract：INTRODUCTION:In Duchenne muscular dystrophy (DMD), fat replacement of muscle may be a useful endpoint in trials of therapy, although progression in different muscle groups is uneven. In this study we assessed the progression of fat replacement with T(1) -weighted imaging over 2 9-month periods. METHODS:Eight ambulant,...

journal_title：Muscle & nerve

authors： Hollingsworth KG,Garrood P,Eagle M,Bushby K,Straub V

更新日期：2013-10-01 00:00:00

abstract：INTRODUCTION:Duchenne muscular dystrophy (DMD) subjects ≥5 years with nonsense mutations were followed for 48 weeks in a multicenter, randomized, double-blind, placebo-controlled trial of ataluren. Placebo arm data (N = 57) provided insight into the natural history of the 6-minute walk test (6MWT) and other endpoints. ...

journal_title：Muscle & nerve

authors： McDonald CM,Henricson EK,Abresch RT,Florence JM,Eagle M,Gappmaier E,Glanzman AM,PTC124-GD-007-DMD Study Group.,Spiegel R,Barth J,Elfring G,Reha A,Peltz S

更新日期：2013-09-01 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

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 measured the peak voltage induced in a sensing loop by a Magstim 200 magnetic stimulator. Coil output varied little for repeated stimulation at the same intensity over a wide range of coil output. In contrast, the first stimulus immediately after a change in intensity was of larger amplitude and showed greater vari...

journal_title：Muscle & nerve

authors： Reutens DC,MacDonell RA,Berkovic SF

更新日期：1993-12-01 00:00:00

abstract：:Large muscle genes are often sequenced using complementary DNA (cDNA) made from muscle messenger RNA (mRNA) to reduce the cost and workload associated with sequencing from genomic DNA. Two potential barriers are the availability of a frozen muscle biopsy, and difficulties in detecting nonsense mutations due to nonsens...

journal_title：Muscle & nerve

authors： Waddell LB,Monnier N,Cooper ST,North KN,Clarke NF

更新日期：2011-08-01 00:00:00

abstract：:A new approach to the quantification of fiber type grouping is presented, in which the distribution of histochemical type in a muscle cross section is regarded as a realization of a binary Markov random field (BMRF). Methods for the estimation of the parameters of this model are discussed. The first order BMRF, which ...

journal_title：Muscle & nerve

authors： Venema HW

更新日期：1992-06-01 00:00:00

abstract：INTRODUCTION:We studied the functional effects of combined strength and aerobic anti-gravity training in severely affected patients with Becker and Limb-Girdle muscular dystrophies. METHODS:Eight patients performed 10-week progressive combined strength (squats, calf raises, lunges) and aerobic (walk/run, jogging in pl...

journal_title：Muscle & nerve

authors： Jensen BR,Berthelsen MP,Husu E,Christensen SB,Prahm KP,Vissing J

更新日期：2016-08-01 00:00:00

abstract：:The values obtained from two different macro EMG methods were compared, and found to be different. Normally, macro electromyography (EMG) is performed with a modified single fiber (SF) needle using the SF potential as a trigger; a new method recommends a modified concentric needle and triggers on the concentric EMG si...

journal_title：Muscle & nerve

authors： Nix WA,Scherer A

更新日期：1992-02-01 00:00:00

abstract：:Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disorder characterized by recurrent mononeuropathies or brachial plexopathies, commonly associated with a chromosome 17p11.2-12 deletion encompassing the peripheral myelin protein-22 (PMP22) gene. We tried to identify criteria dis...

journal_title：Muscle & nerve

authors： Pareyson D,Solari A,Taroni F,Botti S,Fallica E,Scaioli V,Ciano C,Sghirlanzoni A

更新日期：1998-12-01 00:00:00

abstract：INTRODUCTION:To evaluate the sensitivity of electrophysiologic assessments, we compared F-waves and motor and sensory nerve conduction studies (MNCS and SNCS) in patients with diabetes mellitus (DM). METHODS:We tested median, ulnar, tibial, and fibular nerves in 132 DM patients divided into those with and without clin...

journal_title：Muscle & nerve

authors： Pan H,Jian F,Lin J,Chen N,Zhang C,Zhang Z,Ding Z,Wang Y,Cui L,Kimura J

更新日期：2014-06-01 00:00:00