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[良性型多发性硬化症:重新评估(作者译)]

[Benign forms of multiple sclerosis : a revaluation (author's transl)].

作者信息

Bonduelle M, Bouygues P, Degos C F, Gauthier C

出版信息

Rev Neurol (Paris). 1979 Oct;135(8-9):593-604.

PMID:531414
Abstract

The authors studied 38 cases of benign forms of MS, defined as those allowing a normal or nearly normal social, occupational, and family life over a "long" period of tens of years, for at least 15 years (mean : 28 years, range : 15 to 68 years).--Forms with rare relapses with long intervals between them (18 cases), and a mean period of 14 years (5 to 30) between the first and second episode.--Recurrent forms (17 cases) with frequent attacks (one or two a year), including 8 cases with no further relapses after an average period of 10 years, and 9 cases with continuation at the same rhythm for 15 to 23 years.--Secondarily progressive forms belonging to both types--8 in the first and 3 in the second--characterized by the late onset (mean : 25 years, range : 15 to 47 years) of a progressive paraplegia.--Slow forms (3 cases) with an early but only slowly evolving progressive phase, which fall into a border line category of benign forms. Independently of the mean age of onset (26 years); the predominence of females (2.2); and the symptomatology of the first attack (40 p. 100 involve the cranial nerves, and 40 p. 100 have pyramidal signs), the two essential characteristics of benign forms are the remarkable regression of relapses (rare of frequent) and the absence of a progressive phase. Once the 10-year point has been passed without permanent disability the prognosis is good, but with the reserve that the disease can become worse at a later stage.

摘要

作者研究了38例良性多发性硬化症病例,其定义为那些在长达数十年(至少15年,平均28年,范围15至68年)的时间里能维持正常或近乎正常的社交、职业和家庭生活的病例。——发作间隔时间长且发作稀少的类型(18例),首次发作与第二次发作之间的平均间隔时间为14年(5至30年)。——复发型(17例),发作频繁(每年一两次),其中8例在平均10年后不再复发,9例以相同频率持续发作15至23年。——继发进展型,两种类型各有一些病例,第一种类型有8例,第二种类型有3例,其特征为进展性截瘫起病较晚(平均25岁,范围15至47岁)。——缓慢进展型(3例),早期有进展期,但进展缓慢,属于良性类型的边缘类别。不论平均起病年龄(26岁)、女性占优势(2.2)以及首次发作的症状表现(40%累及脑神经,40%有锥体束征)如何,良性类型的两个基本特征是发作显著缓解(稀少或频繁发作后均如此)且无进展期。一旦度过10年且无永久性残疾,预后良好,但要留有余地,因为疾病在后期可能会恶化。

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