摘要:

AbstractNeuropathies are common in patients with known or suspected connective tissue disease. A vasculitic mononeuropathy multiplex is often seen in patients initially presenting with polyarteritis nodosa or developing arteritis as a complication of rheumatoid arthritis. However, vasulitic neuropathy may become confluent and present as as distal symmetrical polyneuropathy or occur without systemic necrotizing vasculitis. Distal symmetrical polyneuropathies without associated vasculitis are also common in many connective tissue diseases. Compression neuropathies, especially carpal tunnel syndrome, occur with increased frequency in rheumatoid arthritis. Finally, certain neurpathies may be the major presenting feature of particular connective tissue diseases. For example, trigeminal neuropathy often heralds the onset of systemic sclerosis or mixed connective tissue disease, and sensory neuronopathy may be the initial presenting feature of Sjögren's syndrome

ISSN:0148-639X    

DOI:10.1002/mus.880150502

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1992

数据来源: WILEY

摘要:

AbstractExamination of inbred and wild mice has shown that the gene coding for the acetylcholine receptor gamma subunit is polymorphic and the polymorphism correlates with differences in neuromuscular function tested by two different methods. Polymorphism of the AChR γ gene was demonstrated after digestion of genomic DNA with Pstl and Xbal. These two restriction enzymes demonstrated RFLP patterns specific for C57Bl/6J (PsXs) and C3H/HeJ (PRXR) mice, respectively. Examination of F1 (C3H/HeJ × C57Bl/6J) and F2 hybrid populations, and other murine inbred strains, showed the inheritance and strain specificity of the RFLPs. Testing wild mice demonstrated that the PSXSform of the AChR γ gene is the most common in the wild. The AChR γ and AChR δ subunits are closely linked and carried on the same chromosome as several contractile proteins. Because these genes are essential for correct neuromuscular development and activity, we investigated the possibility that the observed AChR γ polymorphisms mark a haplotype which correlates with variations in neuromuscular function. Analysis of exercise times showed a correlation between AChR γ polymorphism and neuromuscular function. To our knowledge, this is the first description of AChR polymorphism cosegregating with variations in neuromuscular fu

ISSN:0148-639X    

DOI:10.1002/mus.880150503

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1992

数据来源: WILEY

摘要:

AbstractMyoblasts from immunocompatible donors have been transplanted into the muscles (tibialis anterior, biceps brachii, and/or extensor carpi radialis longus) of 4 Duchenne patients in the advanced stages of the disease. Although no immunosuppressive treatment was used, none of the patients showed any clinical signs of rejection such as fever, redness, and inflammation. One patient transiently produced antibodies against the donor myoblasts as determined by cytofluorometric analysis. This patient and 2 others were shown to form antibodies against their donor's myotubes. Muscle biopsies of the injected tibialis anterior of 4 patients revealed that 80%, 75%, 25% and 0% of the muscle fibers, respectively, showed some degree of dystrophin immunostaining, The contralateral noninjectd muscles of the latter 3 patients did not contain any dystrophin positive fibers, while that of the first patient showed dystrophin expression in 16% of the fibers examined. Myoblasts were also injected into the extensor carpi radialis longus or the biceps brachii of these patients. A few months subsequent to injection, one patient was shown to have a 143% increase of strength during static wrist extension. This result must be interpreted with caution because a double‐blind strength‐measuring protocol was not used. Furthermore, we have noted that this change slowly decayed over time. The strength of 2 other patients was increased less remarkably (41% and 51%), while the strength of the fourth patient was unchan

ISSN:0148-639X    

DOI:10.1002/mus.880150504

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1992

数据来源: WILEY

摘要:

AbstractEosinophilia–myalgia syndrome (EMS) is disorder characterized by generalized muscle pain and eosinophilia. The etiology of this syndrome appears to be related to the ingestion ofL‐tryptophan. Most studies to date describe an associated peripheral neuropathy or combined myopathy and peripheral neuropathy. This report presents 2 EMS patients with myopathy, confirmed by muscle biopsy in 1 case and electrophysiology in both cases. No clinical evidence of neuropathy was found. Both routine and single fiber electromyography failed to demonstrate abnormalities, suggesting neuropathy. Electrodiagnostic abnormalities paralleled the clinical course. After 10 months, both patients continued to have symptoms of muscle cramping and reduced endurance, with mild electromyographic abnormalities, perhaps reflecting changes in their motor u

ISSN:0148-639X    

DOI:10.1002/mus.880150505

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1992

数据来源: WILEY

摘要:

AbstractT‐cell infiltration was detected by immunohistochemistry in only 2 of 10 sural nerve biopsies from patients with Guillain–Barré syndrome (GBS). The number of endoneurial macrophages, identified by the monoclonal antibody MAC 387, was increased, compared with the number in 10 cases of axonal neuropathy. Macrophage‐associated demyelination was identified in 7 and axonal degeneration in 8 cases. Cytomegalovirus (CMV) genome was not detected with the polymerase chain re

ISSN:0148-639X    

DOI:10.1002/mus.880150506

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1992

数据来源: WILEY

摘要:

AbstractOne hundred (100) left arms were examined for median‐to‐ulnar or ulnar‐to‐median nerve anastomoses using surface electrodes. Muscles innervated by the anastomosis were determined using a concentric needle electrode. We found a motor median‐to‐ulnar nerve anastomosis in the forearm in 32% of the cases (Martin–Gruber anastomosis, MGA). No case of motor ulnar‐to‐median nerve anastomosis in the forearm could be found. The MGA mainly innervated muscles, which are normally supplied by the ulnar nerve. The high incidence of MGA in this study shows that all clinically relevant motor median‐to‐ular nerve communications in the forearm can be detected using surface electrodes in routine examinations. Stimulus spread must be looked for and eliminated by

ISSN:0148-639X    

DOI:10.1002/mus.880150507

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1992

数据来源: WILEY

摘要:

AbstractThe X‐linked dystrophic animal model, the mdx mouse, shows an extraordinary capacity for sustained muscle regeneration compared with X‐linked dystrophic golden retriever dogs and humans with Duchenne muscular dystrophy. To test the hypothesis that muscles of mdx mice might have inherently superior muscle regeneration to that in nondystrophic animals, muscle regeneration in response to a crush injury was examined in mdx mice and in the nondystrophic, parental strain C57Bl/10Sn. Autoradiographic techniques were used specifically to investigate the timing of muscle precursor replication after crush injury. Little difference in the regenerative capacity, either histologically or with respect to the timing of muscle precursor replication, was found between mdx mice and C57Bl/10Sn or other nondystrophic strains of m

ISSN:0148-639X    

DOI:10.1002/mus.880150508

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1992

数据来源: WILEY

摘要:

AbstractWe compared the compound motor action potentials (CMAPs) evoked in the biceps, triceps, and abductor digiti minimi (ADM) muscles by conventional electrical stimulation at Erb's point (EP), and by magnetic coil stimulation of the supraclavicular region in 11 normal subjects. We found that magnetic coil stimulation was less effective than conventional stimulation in activating motor fibers in the brachial plexus in 45% of the recordings analyzed. CMAP amplitudes greater than those obtained with EP electrical stimulation were seen in 16% of recordings with supraclavicular magnetic stimulation, and in 33% of recordings with cervical magnetic stimulation, indicating that EP electrical stimulation is submaximal in a large proportion of cases.

ISSN:0148-639X    

DOI:10.1002/mus.880150509

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1992

数据来源: WILEY

摘要:

AbstractWe report a study, assessing involvement of the heart in 33 familial cases of Becker muscular dystrophy (BMD), 31 familiar cases of facioscapulohumeral (FSH) dystrophy, and 27 familial cases of Bethlem myopathy. In the patients with BMD, correlations of myocardial involvement with age and extent of musculoskeletal involvement were made. We performed physical examination, chest X‐ray, electrocardiographic (EKG), and echocardiographic extients with FSH dystrophy. Thirteen patients with BMD (45%) showed EKG changes similar to those found in Duchenne muscular dystrophy. Only 1 of the 13 individuals with cardiac invovement was wheelchairbound. We found no evidence of cardiac changes in the patients with FSH dystrophy. In Bethlem myopathy, only 1 patient had a form of hypertrophic cardiomyopathy (asymmetrical septal hypertrophy

ISSN:0148-639X    

DOI:10.1002/mus.880150510

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1992

数据来源: WILEY

摘要:

AbstractTo refine in the technique of phrenic nerve conduction, we first studied electrode positioning and sources of chest wall artifact. Diaphragmatic compound motor action potentials (DCMAPs) were mapped at close intervals over 4 hemithoraces of two subjects, finding optimum recording sites which were then used to quantitate artifacts due to EKG, chest wall EMG, and configurational thoracic changes of respiration. Based on these findings, 20 normal subjects were studied, showing ease of application and good side‐to‐side agreement for DCMAP laterncies; but, in contrast to prior reports, right‐to left correlation for amplitude and waveform was poor, making the unaffected side an unreliable standard in unilateral partial phrenic nerve le

ISSN:0148-639X    

DOI:10.1002/mus.880150511

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1992

数据来源: WILEY