Update on immune-mediated therapies for myasthenia gravis.

abstract：INTRODUCTION:UDP N-acetylglucosamine2-epimerase/N-acetylmannosamine-kinase (GNE) gene mutations can cause mostly autosomal-recessive myopathy with juvenile-onset known as hereditary inclusion-body myopathy (HIBM). METHODS:We describe a family of a patient showing an unusual HIBM with both vacuolar myopathy and myositi...

journal_title：Muscle & nerve

authors： Chakravorty S,Berger K,Arafat D,Nallamilli BRR,Subramanian HP,Joseph S,Anderson ME,Campbell KP,Glass J,Gibson G,Hegde M

更新日期：2019-07-01 00:00:00

abstract：:We have devised a method of sensory nerve conduction in the interdigital nerves of the foot using the near-nerve needle and signal averaging techniques. In 40 normal controls between the ages of 20 and 59, there was no difficulty in recording sensory compound nerve action potential (CNAP) in the various interdigital n...

journal_title：Muscle & nerve

authors： Oh SJ,Kim HS,Ahmad BK

更新日期：1984-03-01 00:00:00

abstract：INTRODUCTION:Although there are numerous protocols to adjust the amplitude of the Hoffmann reflex (H-reflex) relative to the size of the direct motor response (M-response), the optimal stimulating location has not been described. We sought to determine the optimal positioning of the stimulating cathode when evoking the...

journal_title：Muscle & nerve

authors： Özyurt MG,Shabsog M,Dursun M,Türker KS

更新日期：2018-12-01 00:00:00

abstract：:New developments in the rapid diagnosis and treatment of boys with Duchenne muscular dystrophy (DMD) have led to growing enthusiasm for instituting DMD newborn screening (NBS) in the United States. Our group has been interested in developing clinical guidance to be implemented consistently in specialty care clinics ch...

journal_title：Muscle & nerve

authors： Kwon JM,Abdel-Hamid HZ,Al-Zaidy SA,Mendell JR,Kennedy A,Kinnett K,Cwik VA,Street N,Bolen J,Day JW,Connolly AM

更新日期：2016-08-01 00:00:00

abstract：:Single muscle fibers were prepared from biopsy specimens of Duchenne muscular dystrophy (DMD), normal, and neuromuscular disease controls. Single muscle cells were classified as type 1, type 2, or intermediate by the skinned fiber method. The intermediate fiber was most abundant in DMD, comprising 29% of fibers examin...

journal_title：Muscle & nerve

authors： Takagi A,Ishiura S,Nonaka I,Sugita H

更新日期：1982-05-01 00:00:00

abstract：:Schwartz-Jampel syndrome (SJS) is an autosomal-recessive condition characterized by muscle stiffness and chondrodysplasia. It is due to loss-of-function hypomorphic mutations in the HSPG2 gene that encodes for perlecan, a proteoglycan secreted into the basement membrane. The origin of muscle stiffness in SJS is debate...

journal_title：Muscle & nerve

authors： Echaniz-Laguna A,Rene F,Marcel C,Bangratz M,Fontaine B,Loeffler JP,Nicole S

更新日期：2009-07-01 00:00:00

abstract：:Several studies have suggested the presence of central nervous system involvement manifesting as cognitive impairment in diseases traditionally confined to the peripheral nervous system. The aim of this review is to highlight the character of clinical, genetic, neurofunctional, cognitive, and psychiatric deficits in n...

journal_title：Muscle & nerve

authors： D'Angelo MG,Bresolin N

更新日期：2006-07-01 00:00:00

abstract：:Although it has been shown that muscle magnetic resonance imaging (MRI) improves the phenotypic characterization of patients with neuromuscular disorders and allows accurate quantification of muscle and adipose tissue distribution, to date quantitative MRI has not been used to assess the therapeutic response in clinic...

journal_title：Muscle & nerve

authors： Pichiecchio A,Poloni GU,Ravaglia S,Ponzio M,Germani G,Maranzana D,Costa A,Repetto A,Tavazzi E,Danesino C,Moglia A,Bastianello S

更新日期：2009-07-01 00:00:00

abstract：:Epidemiological studies of the carpal tunnel syndrome have generally overlooked the possibility of a familial occurrence. A prospective study was undertaken to determine the prevalence and significance of a positive family history of carpal tunnel syndrome. Seventy-five of 253 women and 40 of 168 men with a confirmed ...

journal_title：Muscle & nerve

authors： Radecki P

更新日期：1994-03-01 00:00:00

abstract：:Multifocal motor neuropathy (MMN) is now a well-defined purely motor multineuropathy characterized by the presence of multifocal partial motor conduction blocks (CB), frequent association with anti-GM1 IgM antibodies, and usually a good response to high-dose intravenous immunoglobulin (IVIg) therapy. However, several ...

journal_title：Muscle & nerve

authors： Nobile-Orazio E,Cappellari A,Priori A

更新日期：2005-06-01 00:00:00

abstract：INTRODUCTION:In compressive neuropathies, large myelinated nerve fibers are generally thought to be more susceptible. In this study, we investigated small myelinated Aδ and unmyelinated C fiber function in patients with mild, moderate, and severe carpal tunnel syndrome. METHODS:Forty-four healthy controls and 81 carpa...

journal_title：Muscle & nerve

authors： Clarke C,Christensen C,Curran MWT,Chan KM

更新日期：2017-10-01 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：:Reliable unipedal balance is fundamental to safe ambulation. Accordingly, older persons with peripheral neuropathy (PN), who are at increased risk for falls, demonstrate impaired unipedal balance. To explore the relationship between afferent function and unipedal balance, frontal plane proprioceptive thresholds at the...

journal_title：Muscle & nerve

authors： Son J,Ashton-Miller JA,Richardson JK

更新日期：2009-02-01 00:00:00

abstract：:Cultured human skin fibroblasts from 9 patients with Duchenne muscular dystrophy (DMD) and 8 normal age- and sex-matched controls were examined for creatine kinase (CK) activity. Both the normal and the DMD fibroblasts were found to have significant levels of CK activity (approximately 10 x 10(-3) IU per milligram of ...

journal_title：Muscle & nerve

authors： Davis MH,Cappel R,Vester JW,Samaha FJ,Gruenstein E

更新日期：1982-01-01 00:00:00

abstract：:Myasthenia gravis (MG) and its animal model experimental autoimmune myasthenia gravis (EAMG) are caused by autoantibodies against nicotinic acetylcholine receptor (AChR) in skeletal muscle. The production of anti-AChR antibodies is mediated by cytokines produced by CD4+ and CD8+ T helper (Th) cells. Emerging investiga...

journal_title：Muscle & nerve

authors： Zhang GX,Navikas V,Link H

更新日期：1997-05-01 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：:Intraoperative electrophysiological monitoring of the spinal cord has traditionally been done by recording somatosensory evoked potentials (SEP). There is a risk that SEPs can be unaltered when significant injury to the anterior spinal cord has occurred. The purpose of this report is to describe a simple technique for...

journal_title：Muscle & nerve

authors： Phillips LH 2nd,Blanco JS,Sussman MD

更新日期：1995-03-01 00:00:00

abstract：:To better define electrophysiological abnormalities in myasthenia gravis (MG) patients with muscle-specific tyrosine kinase (MuSK) antibodies (Ab), we compared electrophysiological features of 14 MuSK Ab-positive, 73 acetylcholine receptor antibody (AChR Ab)-positive, and 22 MuSK and AChR Ab-negative (seronegative) pa...

journal_title：Muscle & nerve

authors： Oh SJ,Hatanaka Y,Hemmi S,Young AM,Scheufele ML,Nations SP,Lu L,Claussen GC,Wolfe GI

更新日期：2006-04-01 00:00:00

abstract：:We examined and compared a mechanosensitive human sural neuroma and a contralateral sural nerve collected simultaneously from a patient involved in a diabetic neuropathy research protocol. Using indirect immunofluorescence staining. we identified a striking difference in the content within axons of two neuropeptides, ...

journal_title：Muscle & nerve

authors： Zochodne DW,Theriault M,Sharkey KA,Cheng C,Sutherland G

更新日期：1997-07-01 00:00:00

abstract：:Weakness is common in both hyper- and hypothyroidism, and skeletal muscle L-carnitine may play a role in this regard, as suggested by studies indicating abnormal levels of carnitine in serum and urine of patients with thyroid dysfunction. Skeletal muscle samples were obtained for carnitine analysis from control subjec...

journal_title：Muscle & nerve

authors： Sinclair C,Gilchrist JM,Hennessey JV,Kandula M

更新日期：2005-09-01 00:00:00

abstract：:Morphological changes of the subneural apparatus (SNA) during denervation and reinnervation are demonstrated using the hamster peroneus longus muscle. One week after nerve section, synaptic grooves (SGs) were more shallow, with lowered sarcoplasmic ridges. After 4 weeks, the whole SNA area was elevated above the sarco...

journal_title：Muscle & nerve

authors： Matsuda Y,Oki S,Kitaoka K,Nagano Y,Nojima M,Desaki J

更新日期：1988-12-01 00:00:00

abstract：:The myogenic regulatory factor MyoD plays an important role in embryonic and adult skeletal muscle growth. Even though it is best known as a marker for activated satellite cells, it is also expressed in myonuclei, and its expression can be induced by a variety of different conditions. Several model systems have been u...

journal_title：Muscle & nerve

authors： Legerlotz K,Smith HK

更新日期：2008-09-01 00:00:00

abstract：:Familial amyotrophic lateral sclerosis (ALS) with the autosomal-recessively inherited D90A CuZn-superoxide dismutase (CuZn-SOD) mutation is characterized by a stereotypic slowly progressive, distinctive phenotype and very slow central motor conduction. To determine the basis of this slowing, we assessed corticomotoneu...

journal_title：Muscle & nerve

authors： Weber M,Eisen A,Stewart HG,Andersen PM,Hirota N

更新日期：2001-01-01 00:00:00

abstract：:To investigate the role of the thymus in the pathogenesis of myasthenia gravis (MG), immunohistochemical expression of CD44, CD45R0, B7-1, and IL-2 was studied in: (1) hyperplastic thymuses of patients with MG whose symptoms markedly improved after thymectomy, (2) remnant thymuses of patients with MG whose symptoms di...

journal_title：Muscle & nerve

authors： Utsugisawa K,Nagane Y,Tohgi H

更新日期：2000-04-01 00:00:00

abstract：INTRODUCTION:The radial nerve and posterior interosseous nerve (PIN) are prone to injury at multiple sites. Electrodiagnostic (EDx) studies may only identify the most proximal lesion. Nerve ultrasound could augment EDx by visualizing additional pathology. METHODS:This investigation was a retrospective examination of u...

journal_title：Muscle & nerve

authors： Dietz AR,Bucelli RC,Pestronk A,Zaidman CM

更新日期：2016-03-01 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：INTRODUCTION:We sought to translate, cross-culturally adapt, and evaluate the internal consistency and validity of the Chinese version of the 15-Item Myasthenia Gravis Quality of Life (MG-QOL15). METHODS:Translation and cross-cultural adaptation of the MG-QOL15 were performed. We used Cronbach's α to test internal con...

journal_title：Muscle & nerve

authors： Miao X,Lian Z,Liu J,Chen H,Shi Z,Li M,Zhou H,Hu X,Yang R

更新日期：2019-01-01 00:00:00

abstract：INTRODUCTION:Antimitochondrial antibodies are autoantibodies detected in 90% of primary biliary cirrhosis (PBC) patients. Some PBC cases are complicated by myositis, which is difficult to confirm due to minimal histological evidence of inflammation in limb muscles. METHODS:Our aim was to determine the extent of inflam...

journal_title：Muscle & nerve

authors： Uenaka T,Kowa H,Sekiguchi K,Nagata K,Ohtsuka Y,Kanda F,Toda T

更新日期：2013-05-01 00:00:00

abstract：:Cortical and spinal somatosensory evoked potentials (SEP) were recorded in healthy individuals following stimulation of digits I, III, and V with an intensity below discomfort level. Peak latency of the earliest cortical negativity (N1) was found to be the most consistent and easily measured parameter, whereas a spina...

journal_title：Muscle & nerve

authors： Kwast-Rabben O,Libelius R,Heikkilä H

更新日期：2002-10-01 00:00:00

abstract：:The loss of contractile protein in Duchenne muscular dystrophy could result from low rates of synthesis, abnormally high rates of protein degradation, or a combination of both. We measured overall protein degradation rates in cultured human muscle cells obtained at biopsy from patients with Duchenne dystrophy or vario...

journal_title：Muscle & nerve

authors： Neville HE,Harrold S

更新日期：1985-03-01 00:00:00