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多发性硬化症的自然史:一项基于地域的研究。I. 临床病程与残疾情况

The natural history of multiple sclerosis: a geographically based study. I. Clinical course and disability.

作者信息

Weinshenker B G, Bass B, Rice G P, Noseworthy J, Carriere W, Baskerville J, Ebers G C

机构信息

Department of Clinical Neurological Sciences, University of Western Ontario, London, Canada.

出版信息

Brain. 1989 Feb;112 ( Pt 1):133-46. doi: 10.1093/brain/112.1.133.

Abstract

The outcome of multiple sclerosis (MS), assessed according to the Kurtzke Disability Status Scale (DSS), was reviewed in 1,099 consecutive patients followed in London, Canada, between 1972 and 1984. A geographically based subgroup of 196 patients representing 90% of Middlesex County MS patients as well as a group of 197 patients seen from onset of disease were separately analysed. The clinical course was progressive from onset in 33% of the total population and in 28% of the Middlesex County subgroup. Of those with duration of 6-10 yrs, 30-40% with initially remitting disease developed progressive MS. The cross-sectional distribution of disability was bimodal with peaks at DSS 1 (no disability) and DSS 6 (assistance required for walking). Actuarial analysis showed that the median time to reach DSS 6 from onset of MS was 14.97 +/- 0.31 yrs in the total population and 9.42 +/- 0.44 yrs in the "seen from onset' subgroup. Survival was minimally altered; 87% of patients followed up to 40 yrs were still alive, although ascertainment of cases with this duration of MS was incomplete. Data describing the rate at which disability develops after the onset of a progressive phase of MS are also presented. The implications of these data in planning and interpretation of clinical therapeutic trials are discussed.

摘要

1972年至1984年期间,对加拿大伦敦连续随访的1099例患者进行了研究,根据库尔特克残疾状态量表(DSS)评估多发性硬化症(MS)的预后。分别分析了一个以地理位置为基础的亚组,该亚组包含196例患者,占米德尔塞克斯郡MS患者的90%,以及一组从疾病发作开始就接受观察的197例患者。在总人群中,33%的患者临床病程自发病起呈进行性,在米德尔塞克斯郡亚组中这一比例为28%。在病程为6至10年的患者中,最初病情缓解的患者中有30%至40%发展为进行性MS。残疾的横断面分布呈双峰型,在DSS 1(无残疾)和DSS 6(行走需要帮助)处出现峰值。精算分析表明,在总人群中,从MS发病到达到DSS 6的中位时间为14.97±0.31年,在“从发病开始观察”的亚组中为9.42±0.44年。生存率变化极小;随访至40年的患者中,87%仍然存活,尽管MS病程达此时长的病例确诊并不完整。本文还给出了MS进入进展期后残疾发生速率的数据。讨论了这些数据在临床治疗试验规划和解读中的意义。

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