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原发性进行性多发性硬化症:一种独特的综合征?

Primary progressive multiple sclerosis: a distinct syndrome?

作者信息

McDonnell G V, Hawkins S A

机构信息

Royal Victoria Hospital, Belfast, Northern Ireland.

出版信息

Mult Scler. 1996 Oct;2(3):137-41. doi: 10.1177/135245859600200304.

DOI:10.1177/135245859600200304
PMID:9345378
Abstract

Multiple sclerosis (MS) has long been recognised to have both a relapsing-remitting and progressive course. More recently patients with progressive disease have been further sub-divided into those with a progressive course from onset (primary progressive MS) and those with progressive decline following an initially relapsing-remitting period (secondary progressive MS). Diversity in MS may not however be restricted to clinical course. There is growing evidence that the subgroups of MS also differ with respect to clinical features, epidemiology, pathogenesis, genetics and neuroimaging appearances. In this review we outline the criteria variously applied in the classification of MS patients, addressing the need for a clear nomenclature. We evaluate the proposition that primary progressive MS has a profile distinct from other MS categories, contrasting the separate differential diagnoses and examining the implications for future therapeutic trials.

摘要

长期以来,人们一直认识到多发性硬化症(MS)有复发缓解型和进行性病程。最近,患有进行性疾病的患者被进一步细分为起病即呈进行性病程的患者(原发性进行性MS)和在最初的复发缓解期后出现进行性衰退的患者(继发性进行性MS)。然而,MS的多样性可能不限于临床病程。越来越多的证据表明,MS的亚组在临床特征、流行病学、发病机制、遗传学和神经影像学表现方面也存在差异。在本综述中,我们概述了在MS患者分类中各种应用的标准,讨论了明确命名法的必要性。我们评估原发性进行性MS具有与其他MS类别不同特征的观点,对比不同的鉴别诊断,并研究其对未来治疗试验的影响。

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