摘要:

ObjectivesTo better characterize the clinical, electrodiagnostic, and pathologic aspects of amiodarone-induced neuromyopathy.MethodsWe reviewed medical records, performed electrodiagnostic studies, and examined histopathologic specimens of muscle and nerve in three patients with amiodarone-induced neuromuscular toxicity, as well as summarized the relevant literature.ResultsTwo patients had a slightly asymmetric, mixed, but primarily demyelinating sensorimotor polyneuropathy. One also had a substantial myopathy. The third had an acute neuropathy resembling Guillain-Barré syndrome. Seemingly, creatine kinase levels did not correlate with clinical or electromyographic evidence of myopathy. Histologic evaluations of peripheral nerves revealed demyelination, some axon loss, and a variable number of characteristic lysosomal inclusions. Muscle specimens from two patients showed evidence of a vacuolar myopathy. After discontinuation of amiodarone, two patients improved and one died of cardiac arrhythmia.ConclusionsAlthough the clinical features and onset times vary, amiodarone neuromuscular toxicity manifests as a mixed polyneuropathy, vacuolar myopathy, or both. Creatine kinase levels may not correlate with the degree of myopathy. Variable numbers of lysosomal inclusions in peripheral nerve, endothelial cells, and muscle are characteristic.

ISSN:1522-0443    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

ObjectiveTo determine the range of phenotypic expression in individuals with hereditary neuropathy with liability to pressure palsy (HNPP) with the chromosome 17 deletion.MethodsTwenty-one patients from 10 families were studied. Genetic testing was performed in at least one member of each family. Every patient was examined clinically, electrophysiological data was available in 18 patients, and a sural nerve biopsy was performed on 4 patients. In addition, a patient symptom questionnaire was administered over the telephone to identify symptomatic individuals from the at-risk population.ResultsThe identified phenotypes were those of compressive neuropathy, symmetric peripheral neuropathy (often misdiagnosed as Charcot-Marie-Tooth neuropathy), acute brachial paralysis, and confluent mononeuropathy multiplex. Many individuals were oligosymptomatic and these formed the majority of undiagnosed patients.ConclusionsThe presence of mild symptoms and the marked phenotypic variability of the disease result in underdiagnosis of HNPP.

ISSN:1522-0443    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

A 72-year-old woman on immunosuppressive therapy for renal transplantation was admitted to the hospital for diffuse, progressive weakness that developed over several weeks. Serum creatine kinase steadily increased and she developed myoglobinuria. Blood cultures were positive for group AStreptococcus pyogenes. The muscle biopsy demonstrated a necrotizing myopathy without cellular infiltrate. Despite treatment, she evolved to multiorgan system failure and cardiovascular collapse. This is a novel presentation of group A streptococcal muscle infection with diffuse, generalized muscle weakness. It stands in striking contrast to the usual infection with this organism confined to one extremity and associated with a prolific cellular response in the muscle.

ISSN:1522-0443    

出版商:OVID    

年代:2002

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Sporadic inclusion body myositis (s-IBM) is a common but under-recognized myopathy in individuals over 50 years of age. An awareness of the clinical phenotype and of the electrodiagnostic and histopathologic features should lead to improved recognition, and should minimize confusion with polymyositis, motor neuron disease, and other neuromuscular disorders. Treatment efficacy has been difficult to judge because of the insidious progression of the disease over many years, but immunomodulating therapy is generally less effective than in polymyositis and dermatomyositis, and may not be effective at all in many patients. The hereditary inclusion body myopathies (h-IBM) are a heterogeneous group of recessively and dominantly inherited vacuolar myopathies that share some histologic features with s-IBM. Oxidative stress may play a role in the pathogenesis of both s-IBM and h-IBM.

ISSN:1522-0443    

出版商:OVID    

年代:2002

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:2002

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:2002

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:2002

数据来源: OVID

ISSN:1522-0443    

出版商:OVID    

年代:2002

数据来源: OVID