VCP myopathy: A family with unusual clinical manifestations.

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：:Using poststimulus time histograms, it has been reported that stimulation of the median nerve at the elbow produces a monosynaptic EPSP in voluntarily active single motoneurons of the human biceps brachii. The present study was undertaken to: (i) determine whether such stimulation could evoke a reproducible reflex res...

journal_title：Muscle & nerve

authors： Miller TA,Mogyoros I,Burke D

更新日期：1995-06-01 00:00:00

abstract：:The dystrophin protein complex (DPC), composed of at least 10 proteins that associate with dystrophin, is critical for the maintenance of normal muscle fiber structure and physiology. In this study, we used immunohistochemistry and confocal microscopy to examine the relative abundance and distribution of several of th...

journal_title：Muscle & nerve

authors： Draviam R,Billington L,Senchak A,Hoffman EP,Watkins SC

更新日期：2001-02-01 00:00:00

abstract：:Motoneurons can be activated both reflexly and antidromically following electrical stimulation of peripheral nerves. These H reflexes and F waves are clinically useful responses which interface at the level of the peripheral nerves and the spinal cord. Because these responses are commonly employed in the electrodiagno...

journal_title：Muscle & nerve

authors： Fisher MA

更新日期：1992-11-01 00:00:00

abstract：INTRODUCTION:Bifacial weakness with paresthesias is a rare subtype of Guillain-Barré syndrome (GBS), characterized by facial diplegia in the absence of any other cranial neuropathies, limb weakness, or ataxia. Frequently, patients also complain of distal limb paresthesias before or at the time they develop facial weakn...

journal_title：Muscle & nerve

authors： Morgan C,Fuller G,Wakerley BR

更新日期：2016-05-01 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：INTRODUCTION:We determined whether instrumenting timed functional tasks with wireless inertial motion sensors were responsive to facioscapulohumeral muscular dystrophy (FSHD) progression and movement pattern changes. METHODS:Ten individuals who were clinically affected with genetically confirmed FSHD, mean age 54 year...

journal_title：Muscle & nerve

authors： Statland JM,Karanevich A,Bruetsch A,Huisinga J

更新日期：2019-11-01 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：: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：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：:In acquired polyneuropathies, symptoms and signs are typically distal and symmetrical, more prominent in the lower limbs than the upper limbs. This study was undertaken to measure the extent of the decrease in excitability produced by single impulses and by impulse trains in cutaneous afferents in the median and sural...

journal_title：Muscle & nerve

authors： Lin CS,Mogyoros I,Burke D

更新日期：2000-05-01 00:00:00

abstract：:Intravenous immunoglobulin (IVIg) is a useful tool in the treatment of a variety of neuromuscular disorders. Though IVIg therapy is generally safe, hemolytic anemia is a potentially serious complication that is often overlooked, and is currently not listed in product inserts. We analyzed 45 patients who received IVIg ...

journal_title：Muscle & nerve

authors： Wilson JR,Bhoopalam H,Fisher M

更新日期：1997-09-01 00:00:00

abstract：:Adult skeletal muscle fibers are among the few cell types that are truly multinucleated. Recently, evidence has accumulated supporting a role for the modulation of myonuclear number during muscle remodeling in response to injury, adaptation, and disease. These studies have demonstrated that muscle hypertrophy is assoc...

journal_title：Muscle & nerve

authors： Allen DL,Roy RR,Edgerton VR

更新日期：1999-10-01 00:00:00

abstract：:Masseter muscle biopsies were obtained from nine patients undergoing orthognatic surgery or surgery for parotid tumors. A detailed enzyme-histochemical and immunocytochemical study of these muscles was performed using antibodies specific to the various isozymes of the myosin heavy chain (MHC) in order to identify the ...

journal_title：Muscle & nerve

authors： Butler-Browne GS,Eriksson PO,Laurent C,Thornell LE

更新日期：1988-06-01 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：:The purpose of this study was to investigate whether the previously demonstrated heterogeneity of myosin heavy chain (MHC) in type 2B fibers of the superficial portion of the lateral gastrocnemius (SLG) muscle of the rat may be due to presence of type 2D/X fibers. Immunohistochemical identification of MHC heterogeneit...

journal_title：Muscle & nerve

authors： Sawchak JA,Leung B,Shafiq SA

更新日期：1992-12-01 00:00:00

abstract：:Clinical testing for neuromuscular dysfunction is supported by an extensive amount of excellent basic information about normal and abnormal subcellular physiology and ultrastructure. This information provides an essential frame of reference for describing the rationale of single-fiber electromyography (SFEMG). SFEMG i...

journal_title：Muscle & nerve

authors： Keesey JC

更新日期：1989-08-01 00:00:00

abstract：:Isometric contractile properties of human elbow flexor muscles were examined in 27 normal subjects (7 females and 20 males). Contractions of elbow flexors were elicited by application of electrical pulses to the biceps motor points. The tension development was recorded at the wrist with the elbow angle fixed at 90 deg...

journal_title：Muscle & nerve

authors： Ismail HM,Ranatunga KW

更新日期：1981-05-01 00:00:00

abstract：:The development of the personal computer has simplified the process of quantitating sensory thresholds using various testing algorithms. We reviewed the technical aspects and reproducibility of different methods to determine threshold for light touch-pressure, vibration, thermal, and pain stimuli. Clinical uses and li...

journal_title：Muscle & nerve

authors： Chong PS,Cros DP

更新日期：2004-05-01 00:00:00

abstract：INTRODUCTION:Far-field potentials (FFPs) from muscles other than the abductor digiti minimi (ADM) may interfere with motor unit number estimation (MUNE) from that muscle. METHODS:We identified the origin of each surface motor unit potential (SMUP) during hypothenar MUNE using the multiple point stimulation method in 2...

journal_title：Muscle & nerve

authors： Kawamura Y,Sonoo M,Higashihara M,Chiba T,Hatanaka Y

更新日期：2013-08-01 00:00:00

abstract：:The electrical characteristics of myotonia in skeletal muscle appear remarkably similar in a variety of natural disorders and animal models. Recent experimental work has made it clear, however, that the pathophysiologic mechanisms underlying these myotonic discharges can be quite different. A mechanistic classificatio...

journal_title：Muscle & nerve

authors： Barchi RL

更新日期：1982-01-01 00:00:00

abstract：:The F-wave was used to estimate motor nerve conduction velocity (MNCV) along the proximal segment of the fastest axons of the deep peroneal nerve (anterior-horn cells to knee) in 13 patients with amyotrophic lateral sclerosis (ALS) and in 21 age- and sex-matched control subjects. Measurements were based on the shortes...

journal_title：Muscle & nerve

authors： Argyropoulos CJ,Panayiotopoulos CP,Scarpalezos S

更新日期：1978-11-01 00:00:00

abstract：:If anti-GM1 antibody plays a role in the axonal damage in Guillain-Barré syndrome, the common entrapment sites may be preferentially involved with evidence of axonal dysfunction. To assess this hypothesis, we studied nerve conduction across the cubital tunnel in 44 patients. Abnormal amplitude reduction of compound mu...

journal_title：Muscle & nerve

authors： Kuwabara S,Mori M,Ogawara K,Mizobuchi K,Hattori T,Koga M,Yuki N

更新日期：1999-07-01 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：INTRODUCTION:POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) syndrome may be mistaken for chronic inflammatory demyelinating polyneuropathy (CIDP). Differentiating the 2 entities is crucial, as there are major treatment implications. METHODS:We compared platelet ...

journal_title：Muscle & nerve

authors： Naddaf E,Dispenzieri A,Mandrekar J,Mauermann ML

更新日期：2015-10-01 00:00:00

abstract：:Little attention has been paid to small-fiber dysfunction in carpal tunnel syndrome (CTS) although its symptoms are common. This study investigates vasomotor dysfunction, which is controlled by small nerve fibers, in patients with CTS compared with control subjects. Vasomotor function was quantified by measuring, with...

journal_title：Muscle & nerve

authors： Wilder-Smith EP,Fook-Chong S,Chew SE,Chow A,Guo Y

更新日期：2003-11-01 00:00:00

abstract：INTRODUCTION:This study was designed to assess the effect of extracorporeal shock-wave lithotripsy (ESWL) exposure of the parotid gland on oxidative stress and some trace element levels in the facial nerves of rats. METHODS:Twelve male Wistar albino rats were divided into two groups, each consisting of 6 animals. The ...

journal_title：Muscle & nerve

authors： Kavak S,Garca MF,Gecit I,Meral I,Cengiz N,Demir H

更新日期：2012-04-01 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：:The objective of this study was to examine the contribution of lower motor neuron (LMN) and upper motor neuron (UMN) dysfunction to weakness and impaired motor control in 27 patients with amyotrophic lateral sclerosis (ALS). Isometric strength was measured by dorsiflexor maximum voluntary contraction force (MVC). LMN ...

journal_title：Muscle & nerve

authors： Kent-Braun JA,Walker CH,Weiner MW,Miller RG

更新日期：1998-06-01 00:00:00

abstract：INTRODUCTION:Amyloidosis derived from transthyretin (TTR) molecules is typically caused by mutations of the TTR gene. METHODS:We describe an elderly patient with a severe length-dependent polyneuropathy that unexpectedly proved to be caused by wild-type transthyretin amyloidosis. RESULTS:The diagnosis was made by mus...

journal_title：Muscle & nerve

authors： Lam L,Margeta M,Layzer R

更新日期：2015-07-01 00:00:00