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[多发性硬化症的自然病史]

[The natural history of multiple sclerosis].

作者信息

Confavreux Christian, Vukusic Sandra

机构信息

Service de neurologie A, centre de coordination Edmus et Inserm U433, hôpital neurologique Pierre-Wertheimer, 69677 Lyon, Bron.

出版信息

Rev Prat. 2006 Jun 30;56(12):1313-20.

Abstract

The course of multiple sclerosis (MS) results from the interplay of two clinical processes, relapses and progression. Three main clinical forms may be identified as relapsing-remitting, secondary progressive and primary progressive. The disease appears on average at the age of 30 and follows a remitting or a progressive course in 85% and 15% of the cases, respectively. Initial symptoms are related to an isolated or combined disturbance(s) of the long tracts of the central nervous system, of the brainstem, or of the optic nerve in 70%, 20% and 25% of the cases, respectively. The relapse rate is one relapse on average every other year. After an exacerbating-remitting onset of MS, secondary progression appears after 19 years on average. The median time to reach the landmarks of irreversible disability are 8 years regarding limitation of ambulation, 20 years for walking with a stick, and 30 years for wheel-chair dependency. In fact, MS prognosis is highly variable depending on individuals. All the intermediate types do exist between malignant forms, possibly lethal, and benign forms (circa 30% of the cases) that allow normal daily life. The clinical and paraclinical predictive factors identified so far have been acknowledged statistically. They provide little help however, if any, when an individual is concerned. Recent research has showed that relapses have only a marginal effect, relatively to progression, on the accumulation of irreversible neurological disability. Furthermore, the age when reaching the landmarks of irreversible disability is essentially similar whatever the mode of onset of MS, be it exacerbating-remitting or progressive. In spite of the outstanding clinical polymorphism of MS, a unifying concept of the disease ("complexity vs heterogeneity") can be put forward.

摘要

多发性硬化症(MS)的病程源于复发和进展这两个临床过程的相互作用。可识别出三种主要临床类型,即复发缓解型、继发进展型和原发进展型。该疾病平均发病年龄为30岁,分别有85%和15%的病例呈缓解型或进展型病程。初始症状分别在70%、20%和25%的病例中与中枢神经系统长束、脑干或视神经的孤立或合并功能障碍有关。复发率平均为每隔一年复发一次。MS在经历发作缓解期后,平均19年后会出现继发进展。达到不可逆残疾标志的中位时间,行走受限为8年,需拄拐杖行走为20年,依赖轮椅为30年。事实上,MS的预后因个体差异而有很大不同。在可能致命的恶性形式和允许正常日常生活的良性形式(约占病例的30%)之间,确实存在所有中间类型。目前已确定的临床和辅助临床预测因素在统计学上得到了认可。然而,就个体而言,这些因素即便有帮助,也微乎其微。最近的研究表明,相对于进展而言,复发对不可逆神经功能残疾的累积只有很小的影响。此外,无论MS的发病方式是发作缓解型还是进展型,达到不可逆残疾标志的年龄基本相似。尽管MS具有显著的临床多态性,但仍可提出一个关于该疾病的统一概念(“复杂性与异质性”)。

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