Autophagy markers LC3 and p62 accumulate in immune-mediated necrotizing myopathy.

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：INTRODUCTION:Myasthenia gravis (MG) is a B-cell-mediated autoimmune disease. B-cell-activating factor (BAFF) is a major factor in B-cell development and activation. In this study we investigated serum BAFF levels in MG patients. METHODS:We compared the serum BAFF levels of 20 MG patients with gender-matched healthy co...

journal_title：Muscle & nerve

authors： Kang SY,Kang CH,Lee KH

更新日期：2016-12-01 00:00:00

abstract：INTRODUCTION:Spinal and bulbar muscular atrophy (SBMA) and amyotrophic lateral sclerosis (ALS) are motor neuron diseases. Sensory impairment is sometimes observed, and electrophysiological involvement has been described in the sensory nerves of SBMA patients. We hypothesized that a sensory nerve conduction study (NCS) ...

journal_title：Muscle & nerve

authors： Hama T,Hirayama M,Hara T,Nakamura T,Atsuta N,Banno H,Suzuki K,Katsuno M,Tanaka F,Sobue G

更新日期：2012-02-01 00:00:00

abstract：:A patient with neuromyotonia, peripheral neuropathy and myasthenia gravis (MG) is described. Neurophysiological studies, at rest, showed continuous muscle discharges of motor unit action potentials (MUAPs) in duplets and triplets. Motor (MNCV) and sensory (SNCV) nerve conduction studies revealed mild axonal and demyel...

journal_title：Muscle & nerve

authors： Martinelli P,Patuelli A,Minardi C,Cau A,Riviera AM,Dal Pozzo F

更新日期：1996-04-01 00:00:00

abstract：:The authors reported a large study of 93 children presenting a severe form of progressive muscular dystrophy. The first clinical symptoms were noticed between 3 to 12 years. The atrophy affects, predominantly, the girdle and truncal muscles. The hypertrophy of the calves is almost consistent. The progression of the di...

journal_title：Muscle & nerve

authors： Ben Hamida M,Fardeau M,Attia N

更新日期：1983-09-01 00:00:00

abstract：INTRODUCTION:We investigated the viscoelastic stress relaxation response before and after a 10-week hamstrings static stretch program. METHODS:The maximal range of motion (ROMmax ) and maximal torque (Torquemax ) were assessed before and after a 10-week stretching program. Six 30-s static stretches at a predetermined ...

journal_title：Muscle & nerve

authors： Peixoto GH,Andrade AG,Menzel HJ,Araújo SR,Pertence AE,Chagas MH

更新日期：2015-05-01 00:00:00

abstract：:Focal myositis is a rare inflammatory pseudotumor of skeletal muscle which usually has a benign course. We report a 56-year-old woman with a painful mass in the left arm with a radial nerve palsy. Magnetic resonance imaging (MRI) of the left arm showed a mass in the triceps muscle that was suggestive of a soft-tissue ...

journal_title：Muscle & nerve

authors： Alzagatiti BI,Bertorini TE,Horner LH,Maccarino VS,O'Brien T

更新日期：1999-07-01 00:00:00

abstract：:Electron microscopic studies of long-term denervated rat muscles have identified very small, immature myofibers that are believed to arise from detached satellite cells that have fused to form new fibers within the interstitial space. At present, it is unknown whether and to what extent equivalent fibers exist in dene...

journal_title：Muscle & nerve

authors： Doppler K,Mittelbronn M,Bornemann A

更新日期：2008-01-01 00:00:00

abstract：:A method for quantifying shape variability, the jiggle, or motor unit potentials (MUPs) recorded with conventional EMG electrodes is presented. Amplitude variability at each point of time of the MUP was analyzed. Two new parameters are proposed: the normalized value of the consecutive amplitude differences (CAD), and ...

journal_title：Muscle & nerve

authors： Stålberg EV,Sonoo M

更新日期：1994-10-01 00:00:00

abstract：:We report a patient who experienced progressive diplopia and distal weakness of the upper limbs. Magnetic resonance imaging of the brain showed extensive white matter lesions and analysis of cerebrospinal fluid revealed acute human T-lymphotropic virus type I (HTLV-I) infection. Myasthenia gravis (MG) was evidenced by...

journal_title：Muscle & nerve

authors： Lalive PH,Allali G,Truffert A

更新日期：2007-04-01 00:00:00

abstract：:When an active muscle is stretched, the force increases due to strain of contractile and noncontractile proteins. We examined this force enhancement in rat extensor digitorum longus (EDL) and soleus muscles, which differ in their composition of these proteins, and their susceptibility to damage. Small stretches were a...

journal_title：Muscle & nerve

authors： Ramsey KA,Bakker AJ,Pinniger GJ

更新日期：2010-11-01 00:00:00

abstract：INTRODUCTION:Nemaline myopathy is a rare disorder characterized by skeletal muscle weakness of varying severity and onset, with the presence of nemaline rods on muscle biopsy. Congenital nemaline body myopathy due to mutations in TNNT1 has hitherto only been described as a result of a single founder mutation in patient...

journal_title：Muscle & nerve

authors： Abdulhaq UN,Daana M,Dor T,Fellig Y,Eylon S,Schuelke M,Shaag A,Elpeleg O,Edvardson S

更新日期：2016-04-01 00:00:00

abstract：:Phospholipid methylation is thought to modulate such vital cellular processes as calcium transport, receptor function, and membrane microviscosity. As these processes are fundamental to the function of muscle cells and are thought to be altered in disease states, we have characterized several features of phospholipid ...

journal_title：Muscle & nerve

authors： Kuncl RW,Drachman DB,Kishimoto Y

更新日期：1985-06-01 00:00:00

abstract：:Disappointing functional recovery following peripheral nerve repair can be improved by neurotrophic growth factors. Leukemia inhibitory factor (LIF) is unique in that it has independent neurotrophic and myotrophic actions. The aim of this study was to explain this finding by establishing the existence of anterograde a...

journal_title：Muscle & nerve

authors： Bennett TM,Dowsing BJ,Austin L,Messina A,Nicola NA,Morrison WA

更新日期：1999-01-01 00:00:00

abstract：:This study was designed to compare electromyogram (EMG) and acoustic myogram (AMG) recordings of biceps brachii muscles in patients with spastic cerebral palsy (CP). The maximal voluntary contraction (MVC) in the CP group was approximately one half of that of the normal group even after being normalized by the muscle ...

journal_title：Muscle & nerve

authors： Akataki K,Mita K,Itoh K,Suzuki N,Watakabe M

更新日期：1996-10-01 00:00:00

abstract：:Many post-polio patients develop new muscle weakness decades after the initial illness. However, its mechanism and treatment are controversial. The purpose of this study was to test the hypotheses that: (1) after strength training, post-polio patients show strength improvement comparable to that seen in the healthy el...

journal_title：Muscle & nerve

authors： Chan KM,Amirjani N,Sumrain M,Clarke A,Strohschein FJ

更新日期：2003-03-01 00:00:00

abstract：:In spite of extensive studies it is unclear whether impaired fasting glucose (IFG) or impaired glucose tolerance (IGT), i.e., impaired glucose metabolism (IGM), causes diabetic sensorimotor polyneuropathy (DSPN) or chronic idiopathic axonal polyneuropathy (CIAP); the results and conclusions vary considerably in differ...

journal_title：Muscle & nerve

authors： Dyck PJ,Dyck PJ,Klein CJ,Weigand SD

更新日期：2007-10-01 00:00:00

abstract：:Electromyographic (EMG) recordings may serve an important role in predicting torque during repetitive activation of paralyzed muscle. We compared the initial M-wave to the subsequent M-waves of the same train under fatigued and recovered conditions in the paralyzed human soleus muscle. Sixteen individuals with chronic...

journal_title：Muscle & nerve

authors： Chang YJ,Shields RK

更新日期：2002-11-01 00:00:00

abstract：INTRODUCTION:In this study, we determined normal levels of dysferlin expression in CD14+ monocytes by flow cytometry (FC) as a screening tool for dysferlinopathies. METHODS:Monocytes from 183 healthy individuals and 29 patients were immunolabeled, run on an FACScalibur flow cytometer, and analyzed by FlowJo software. ...

journal_title：Muscle & nerve

authors： Sánchez-Chapul L,Ángel-Muñoz MD,Ruano-Calderón L,Luna-Angulo A,Coral-Vázquez R,Hernández-Hernández Ó,Magaña JJ,León-Hernández SR,Escobar-Cedillo RE,Vargas S

更新日期：2016-12-01 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：:A randomized double-blind therapeutic trial of isaxonine was completed over a 2-year period for 20 ambulant boys with Duchenne muscular dystrophy aged 5 1/2-10 years. The effect of the drug was monitored by measurement of walking times over 28 and 150 ft, motor ability score, MRC score based on 32 muscle groups, and m...

journal_title：Muscle & nerve

authors： Heckmatt JZ,Hyde SA,Gabain A,Dubowitz V

更新日期：1988-08-01 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：:We report three patients with inflammatory myopathy who presented clinically with weakness and wasting of only one limb. The myopathy progressed over 6 months and 5 years, respectively, in two patients and was stable after 8 years in the third patient. One patient had a skin rash. Serum CK was elevated in the two pati...

journal_title：Muscle & nerve

authors： Lederman RJ,Salanga VD,Wilbourn AJ,Hanson MR,Dudley AW Jr

更新日期：1984-02-01 00:00:00

abstract：:To evaluate the effect of SMN2 copy number on disease severity in spinal muscular atrophy (SMA), we stratified 45 adult SMA patients based on SMN2 copy number (3 vs. 4 copies). Patients with 3 copies had an earlier age of onset and lower spinal muscular atrophy functional rating scale (SMAFRS) scores and were more lik...

journal_title：Muscle & nerve

authors： Elsheikh B,Prior T,Zhang X,Miller R,Kolb SJ,Moore D,Bradley W,Barohn R,Bryan W,Gelinas D,Iannaccone S,Leshner R,Mendell JR,Mendoza M,Russman B,Smith S,King W,Kissel JT

更新日期：2009-10-01 00:00:00

abstract：BACKGROUND:Our pilot study tested the feasibility and performance of an eye-controlled power wheelchair for amyotrophic lateral sclerosis (ALS) patients. METHODS:In this prospective pilot study, participants drove the wheelchair three times around an indoor course. We assessed the time to complete the course; starting...

journal_title：Muscle & nerve

authors： Elliott MA,Malvar H,Maassel LL,Campbell J,Kulkarni H,Spiridonova I,Sophy N,Beavers J,Paradiso A,Needham C,Rifley J,Duffield M,Crawford J,Wood B,Cox EJ,Scanlan JM

更新日期：2019-11-01 00:00:00

abstract：:Genetic testing is rapidly becoming an increasingly significant part of the diagnostic armamentarium of neuromuscular clinicians. Although technically easy to order, the results of such testing, whether positive or negative, have potentially enormous consequences for the individual tested and for family members. As a ...

journal_title：Muscle & nerve

authors： Su X,Kang PB,Russell JA,Simmons Z

更新日期：2016-12-01 00:00:00

abstract：:Force fluctuations in leg muscles increase after bedrest, perhaps due to modulation of the neural strategy that is specific to a muscle or common to agonist muscles. The purpose of this study was to examine the modulation of muscle activity and force fluctuations during steady contractions with variable involvement of...

journal_title：Muscle & nerve

authors： Yoshitake Y,Kouzaki M,Fukuoka H,Fukunaga T,Shinohara M

更新日期：2007-06-01 00:00:00

abstract：:Dystrophic changes of Schwann cells and demyelination occurred in rats with chronic nerve edema induced by feeding a galactose-rich diet for two years. The mechanism for edema is the sorbitol pathway which generates osmotically active polyols from galactose or glucose. The blood-nerve barrier impedes diffusion of macr...

journal_title：Muscle & nerve

authors： Powell HC,Myers RR

更新日期：1983-03-01 00:00:00

abstract：:Muscle weakness in myasthenia gravis is due to autoantibody-induced loss of functional acetylcholine receptors (AChR). About 15% of myasthenia gravis patients, however, do not have detectable anti-AChR antibodies. To investigate the effect of their plasma immunoglobulins on neuromuscular transmission, mice were inject...

journal_title：Muscle & nerve

authors： Burges J,Vincent A,Molenaar PC,Newsom-Davis J,Peers C,Wray D

更新日期：1994-12-01 00:00:00

abstract：:The inflammatory myopathies include dermatomyositis (DM), polymyositis (PM), and sporadic inclusion-body myositis (s-IBM). In DM, the main immune effector response appears to be humoral and directed against the microvasculature, whereas in both PM and s-IBM, cytotoxic CD8+ T cells and macrophages invade and eventually...

journal_title：Muscle & nerve

authors： Figarella-Branger D,Civatte M,Bartoli C,Pellissier JF

更新日期：2003-12-01 00:00:00