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[视神经脊髓炎/德维克综合征:新视角]

[Neuromyelitis optica/Devic's syndrome: new perspectives].

作者信息

Lalive P H, Perrin L, Chofflon M

机构信息

Service de neurologie, Laboratoire de neuroimmunologie, HUG, 1211 Genève 14.

出版信息

Rev Med Suisse. 2007 Apr 11;3(106):950-5.

Abstract

Neuromyelitis optica (NMO) or Devic's syndrome is a severe demyelinating disease of the central nervous system involving preferentially the optic nerves and the spinal cord. Until recently, NMO was described as an atypical multiple sclerosis IMS), characterized by an unusual clinical presentation, a severe relapsing progression, and a poor response to usual MS treatments. The recent discovery of the so-called NMO-IgG, a highly NMO-specific antibody directed against the blood brain barrier and the aquaporine-4, allowed to refine diagnostic criteria and to classify this disease as an autoimmune channelopathy. According to this recent advance, early diagnostic and specific treatment targeting the humoral response should be considered.

摘要

视神经脊髓炎(NMO)或德维克综合征是一种严重的中枢神经系统脱髓鞘疾病,主要累及视神经和脊髓。直到最近,NMO还被描述为一种非典型的多发性硬化症(MS),其特点是临床表现异常、严重的复发进展以及对常规MS治疗反应不佳。最近发现的所谓NMO-IgG,一种高度特异性针对血脑屏障和水通道蛋白4的抗体,使得诊断标准得以完善,并将这种疾病归类为自身免疫性通道病。基于这一最新进展,应考虑针对体液反应的早期诊断和特异性治疗。

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