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 injected with plasma (and in some cases purified immunoglobulin G (IgG)) from 7 "seronegative" myasthenia gravis (SMG) patients, and neuromuscular transmission parameters were examined. When injected for 15 days, all patients' plasma caused reductions in miniature endplate potential amplitudes, while endplate potential quantal content was significantly reduced by plasma from 4 of the 7 patients. There were no changes in ACh-induced depolarization or single channel properties, and 125I-alpha-bungarotoxin binding studies showed no effect on AChR number, except in 1 case. Purified IgG injected for 3 days had similar effects to plasma injected for 15 days. Our findings confirm that SMG is autoantibody mediated and that there are pathogenic IgG antibodies. SMG appears to be a heterogeneous disorder and the target(s) for the antibodies may be diverse.
journal_name
Muscle Nervejournal_title
Muscle & nerveauthors
Burges J,Vincent A,Molenaar PC,Newsom-Davis J,Peers C,Wray Ddoi
10.1002/mus.880171208subject
Has Abstractpub_date
1994-12-01 00:00:00pages
1393-400issue
12eissn
0148-639Xissn
1097-4598journal_volume
17pub_type
杂志文章相关文献
MUSCLE & NERVE文献大全abstract::We measured acetylcholinesterase (AChE) in the non-endplate region of rat muscle, documenting its intrinsic activity within muscle fibers, as well as the extrinsic level in the capillaries and endomysium. When each muscle was considered as a whole, intrinsic AChE activity detected within the fibers was stronger in the...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880130805
更新日期:1990-08-01 00:00:00
abstract:INTRODUCTION:The minimal clinically important difference (MCID) is the smallest outcome change that has clinical significance. Its use has not been established in the study of myasthenia gravis (MG). METHODS:Patients from a published intravenous immunoglobulin (IVIg) vs. placebo study were studied. One anchor-based an...
journal_title:Muscle & nerve
pub_type: 杂志文章,随机对照试验
doi:10.1002/mus.23988
更新日期:2014-05-01 00:00:00
abstract::Spastic paresis follows chronic disruption of the central execution of volitional command. Motor function in patients with spastic paresis is subjected over time to three fundamental insults, of which the last two are avoidable: (1) the neural insult itself, which causes paresis, i.e., reduced voluntary motor unit rec...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:10.1002/mus.20284
更新日期:2005-05-01 00:00:00
abstract:INTRODUCTION:We investigated the possible role of intercostal surgical neurolysis in relieving chronic neuropathic pain refractory to other nonsurgical treatments in patients with postsurgical thoracic pain. METHODS:We retrospectively collected clinical data on patients referred to the Neurosurgery Unit of Policlinic ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26298
更新日期:2018-11-01 00:00:00
abstract:INTRODUCTION:We assessed antibodies against low-density lipoprotein receptor-related protein 4 (LRP4-Ab) in a Chinese population with myasthenia gravis (MG). METHODS:Serum samples from 116 patients and 80 controls were collected. Acetylcholine receptor antibodies(AChR-Ab) and muscle-specific receptor tyrosine kinase a...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.25591
更新日期:2017-11-01 00:00:00
abstract:INTRODUCTION:Muscle weakness and functional disability have not been evaluated in a population-based study of patients with myasthenia gravis (MG). METHODS:All patients with MG in a well-defined catchment area were identified in the Danish National Patient Registry. Of the 175 eligible patients, 90 participated and we...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26356
更新日期:2019-02-01 00:00:00
abstract::Normal motor units (MUs) were simulated and their architecture altered to simulate the changes produced by myopathy. The concentric needle electromyographic recordings of motor unit action potentials (MUAPs) from the MUs were then also simulated. These simulated MUAPs showed features that are seen in myopathy: normal ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880120306
更新日期:1989-03-01 00:00:00
abstract::Impaired torque production is a major physical impairment following stroke, and has been studied extensively in isometric conditions. However, functional use of a limb requires torque production during movement, and the effects of velocity on maximal torque production may be abnormally enhanced in the paretic limb. Th...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20157
更新日期:2004-12-01 00:00:00
abstract::The majority of patients with MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes) have an A-->G mutation at nucleotide 3243 in mitochondrial transfer (t)RNA. To date there have only been 10 reported cases of MELAS syndrome in patients with a T-->C mutation at position 3271...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199801)21:1<25::aid-mus4>3
更新日期:1998-01-01 00:00:00
abstract::Nine patients with adult-onset acid maltase deficiency (Pompe's disease) were examined clinically and with computed tomography (CT). The CT scan showed early and severe involvement of the muscles of trunk and thighs, with selective sparing of the tensor fasciae latae, short head of biceps femoris, gracilis, and sartor...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(sici)1097-4598(199803)21:3<398::aid-mus15
更新日期:1998-03-01 00:00:00
abstract::Exertional muscle pain, contractures, recurrent rhabdomyolysis, and pigmenturia are common in certain muscle glycolytic disorders. However, the frequency, distribution, and long-term significance of these findings are poorly understood. First we performed magnetic resonance imaging (MRI) of the extremities as a screen...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880121011
更新日期:1989-10-01 00:00:00
abstract::Forty patients with polymyositis or dermatomyositis underwent detailed electromyographic evaluation. The paraspinal muscles of all patients were examined, as were several extremity muscles. The distribution of fibrillation potentials (FPs) in different muscles is discussed. FPs were most frequent in paraspinal muscles...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880020103
更新日期:1979-01-01 00:00:00
abstract::Danon disease is a rare X-linked dominant disorder caused by lysosomal-associated membrane protein 2 (LAMP2) deficiency and is characterized by hypertrophic cardiomyopathy, cardiac conduction abnormalities, skeletal vacuolar myopathy, variable degree of mental retardation, and peripheral pigmentary retinopathy. We des...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20930
更新日期:2008-03-01 00:00:00
abstract:INTRODUCTION:Patients with DMD have low bone mineral density (BMD) and are at high risk for fractures. We examined changes in BMD and the effects of alendronate in DMD patients treated at our institution in the last decade. METHODS:Retrospective cohort study of 39 DMD patients. RESULTS:Patients had screening dual ene...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23948
更新日期:2014-04-01 00:00:00
abstract:INTRODUCTION:We adopted a proteomics-based approach to gain insights into phenotypic differences between A/J and B10.SJL murine dysferlinopathy models. METHODS:We optimized immunoblotting of dysferlin by preparing homogenates of the tibialis anterior (TA) muscle under several different conditions. We compared TA muscl...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.24220
更新日期:2014-08-01 00:00:00
abstract::Twenty patients with neurogenic disorders, polymyositis, or muscular dystrophies were assessed clinically and by CT imaging of limb, limb girdle, and trunk muscles, using a standard protocol. On each side of these patients 26 movements were graded by the MRC scale, and 20 muscles were assessed by CT imaging. The clini...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880180706
更新日期:1995-07-01 00:00:00
abstract::Limb-girdle muscular dystrophy 2D (LGMD2D) is caused by mutations in the alpha-sarcoglycan gene (SGCA). The most frequently reported mutation, 229CGC>TGC (R77C) in exon 3 of SGCA, results in the substitution of arginine by cysteine. We present here the clinical, immunohistochemical, and genetic data of 11 Finnish pati...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.20267
更新日期:2005-02-01 00:00:00
abstract::EDL muscles from normal and dystrophic (dy2j) mice of various ages were examined. Muscles were divided into three groups according to age: 7 to 14 days postnatal, 16 to 21 days postnatal, and 6 months old, to assess age and/or phenotype related differences in the muscle response to caffeine or high K+. The response of...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880160108
更新日期:1993-01-01 00:00:00
abstract:INTRODUCTION:Rapid and accessible methods for diagnosing diabetic polyneuropathy (DPN) have been developed, but not validated, in large cohorts of people with diabetes. METHODS:The performance of a point-of-care device (POCD) was studied in 168 patients with type 2 diabetes, estimating the sensitivity, specificity, po...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26361
更新日期:2019-02-01 00:00:00
abstract:INTRODUCTION:Phospholipids are essential components of cellular membranes and are closely associated with cellular functions, but relationships involving skeletal muscle phospholipid profiles and their physiological phenotypes have remained unclear. METHODS:We carried out comprehensive phospholipid analyses using liqu...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26993
更新日期:2020-09-01 00:00:00
abstract::Ultrastructural, biochemical, and electrophysiological analyses were done on 12-14-month-old mice deficient for myelin-associated glycoprotein (MAG) to further characterize the neuropathy that develops as they age. Electron microscopy demonstrated normal myelin compaction and axonal degeneration in a large number of m...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.1159
更新日期:2001-10-01 00:00:00
abstract::Acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE) were studied in the diaphragm of early postnatal rats, using radiometric determination of enzyme activities, velocity sedimentation for separation of molecular forms, and electron microscopic cytochemistry to localize enzymes. AChE activity did not change s...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880040607
更新日期:1981-11-01 00:00:00
abstract:INTRODUCTION:Mutations in gap junction protein beta 1 (GJB1) on the X chromosome represent one of the most common causes of hereditary neuropathy. We assessed manifestations associated with a rare 3' untranslated region mutation (UTR) of GJB1 in a large family with X-linked Charcot-Marie-Tooth disease (CMTX). METHODS:...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.26037
更新日期:2018-05-01 00:00:00
abstract::False motor points (FMPs) can occur in intrinsic foot or hand muscles, causing spuriously prolonged distal motor latencies by misrepresenting the compound muscle action potential (CMAP) onset. We investigated the motor point (MP) and possible FMPs in abductor hallucis (AH) by three methods: (1) electrophysiologic mapp...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/(SICI)1097-4598(199609)19:9<1138::AID-MUS9
更新日期:1996-09-01 00:00:00
abstract::Intensive care unit-acquired weakness (ICU-AW) causes significant morbidity and impairment in critically ill patients. Recent advances in neuromuscular ultrasound (NMUS) allow evaluation of neuromuscular pathology early in critical illness. Here we review application of ultrasound in ICU-AW. MEDLINE-indexed articles w...
journal_title:Muscle & nerve
pub_type: 杂志文章,评审
doi:10.1002/mus.24728
更新日期:2015-11-01 00:00:00
abstract::In a woman with a slowly progressive adult onset proximal myopathy, muscle biopsy showed storage of PAS positive material in type 1 fibers. This material consisted of a branched chain polysaccharide associated with a mucoprotein. No abnormality of glycogen-pathway enzymes was detected. This suggested that this polysac...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.880110411
更新日期:1988-04-01 00:00:00
abstract::Sialorrhea is frequently a socially disabling symptom in patients with bulbar amyotrophic lateral sclerosis (ALS). In this open-label prospective study, we report the effect of botulinum toxin A (Botox) injection into the parotid glands in 10 patients with bulbar ALS and socially disabling sialorrhea. We applied three...
journal_title:Muscle & nerve
pub_type: 临床试验,杂志文章
doi:10.1002/mus.20545
更新日期:2006-08-01 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
pub_type: 杂志文章
doi:10.1002/mus.10245
更新日期:2002-11-01 00:00:00
abstract:INTRODUCTION:The Accurate Test of Limb Isometric Strength (ATLIS) device can reliably measure the strength of 12 muscle groups using a fixed load cell. The purpose of this study was to analyze ATLIS data from healthy adults to calculate an individual's predicted strength scores. METHODS:ATLIS data were collected from ...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.23516
更新日期:2013-02-01 00:00:00
abstract::Myasthenia gravis (MG) exacerbations may be triggered by infections.In the winter of 2009/2010, the Israeli Ministry of Health recommended that each individual with a chronic condition be vaccinated against both the seasonal influenza and the H1N1 virus. We examined vaccination practices in 74 MG patients. Only 38 pat...
journal_title:Muscle & nerve
pub_type: 杂志文章
doi:10.1002/mus.22077
更新日期:2011-06-01 00:00:00