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慢性运动神经病和下运动神经元综合征

Chronic motor neuropathies and lower motor neurone syndromes.

作者信息

Kornberg A J, Pestronk A

机构信息

Department of Paediatrics, University of Melbourne, Parkville, Australia.

出版信息

Baillieres Clin Neurol. 1995 Nov;4(3):427-41.

PMID:8599719
Abstract

Immune-mediated motor neuropathies are most often disorders manifest clinically by slowly progressive, asymmetrical, distal weakness, starting in the hands more often than the legs. In some cases, electrophysiological findings show multifocal conduction block along the length of motor axons. Other patients have findings consistent with only axonal loss. Laboratory testing is unremarkable except for high-titre serum autoantibodies to GM1 and other neural antigens. Diagnosis of immune-mediated motor neuropathies provides an opportunity for effective immunomodulating therapy that can significantly improve quality of life in affected patients. The identification of antigenic targets of serum autoantibodies in patients with motor neuropathies lends hope that immunotherapies to specifically treat the autoimmune disorder can be developed.

摘要

免疫介导的运动神经病通常是临床上表现为缓慢进展、不对称、远端无力的疾病,更多始于手部而非腿部。在某些情况下,电生理检查结果显示运动轴突全长存在多灶性传导阻滞。其他患者的检查结果仅符合轴突丢失。实验室检查无异常,除非存在针对GM1和其他神经抗原的高滴度血清自身抗体。免疫介导的运动神经病的诊断为有效的免疫调节治疗提供了机会,这可以显著改善受影响患者的生活质量。运动神经病患者血清自身抗体抗原靶点的鉴定为开发特异性治疗自身免疫性疾病的免疫疗法带来了希望。

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