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苏格兰东北部多发性硬化症的临床情况,特别涉及其病程和预后。

Clinical aspects of multiple sclerosis in north-east Scotland with particular reference to its course and prognosis.

作者信息

Phadke J G

机构信息

Department of Neurology, Aberdeen Royal Infirmary, UK.

出版信息

Brain. 1990 Dec;113 ( Pt 6):1597-628. doi: 10.1093/brain/113.6.1597.

DOI:10.1093/brain/113.6.1597
PMID:2276037
Abstract

The prognosis and course of multiple sclerosis (MS) and the factors that affect them were assessed in a group of 1055 patients, representing an unselected (epidemiological) sample observed in the north-east (Grampian region) of Scotland for a period ranging between 1 and 60 yrs. In 7% the disease began before the age of 20 yrs, in 12% after the age of 50 yrs, and in the remainder onset was between the ages of 20 and 50 yrs. The male/female ratio was 1:1.8. Mean disease duration in those observed until death (216 patients) was 24.5 yrs, with no significant difference between the sexes. Prognosis was assessed either by the interval between onset and death or by the degree of disability over a defined period of time. Depending on the length of follow-up, just over one-quarter (26%) to over one-third (36.3%) had a benign course and between 8.0 and 17.7% had a poor prognosis. Nearly a third had a remittent (32.8%) or relapsing cumulative (34%) course and 9% had a progressive course from the start. Several factors were noted to affect the prognosis. Prognosis was significantly better, independent of sex, in those with (1) an early onset (less than 40 yrs of age); (2) retrobulbar neuritis or a brainstem lesion or sensory symptoms alone at onset; (3) short duration of initial symptoms (less than 6 months); (4) a long onset--first relapse interval (greater than 1 yr); (5) a remittent course in the beginning and (6) lack of a family history of MS. The factors which predicted a poor prognosis included: (1) a late onset (greater than 40 yrs of age); (2) progressive course from the start; (3) multiple sites of lesions initially, or a cerebellar or spinal cord lesion at the onset; (4) psychiatric or persistent urinary symptoms at the onset or within 10 yrs; (5) persistent initial symptoms (beyond 1 yr); (6) early first relapse (within 6 months); (7) a family history of MS; (8) social class status IV and V; and (9) bilaterally prolonged visual evoked potential (VEP) P100 latency. Address in childhood and at the onset of the disease, changes in the CSF and CT brain scan were not of predictive value.

摘要

对1055名患者进行了评估,以了解多发性硬化症(MS)的预后、病程及其影响因素。这些患者代表了在苏格兰东北部(格兰扁地区)观察到的未经挑选的(流行病学)样本,观察期为1至60年。7%的患者在20岁之前发病,12%在50岁之后发病,其余患者发病年龄在20至50岁之间。男女比例为1:1.8。在观察至死亡的患者(216例)中,平均病程为24.5年,男女之间无显著差异。预后通过发病至死亡的间隔时间或特定时间段内的残疾程度来评估。根据随访时间的长短,略超过四分之一(26%)至三分之一以上(36.3%)的患者病程呈良性,8.0%至17.7%的患者预后较差。近三分之一的患者病程呈缓解型(32.8%)或复发累积型(34%),9%的患者从一开始就呈进展型。注意到有几个因素会影响预后。在以下患者中,无论性别如何,预后明显较好:(1)发病早(年龄小于40岁);(2)发病时为球后视神经炎、脑干病变或仅有感觉症状;(3)初始症状持续时间短(小于6个月);(4)发病至首次复发间隔时间长(大于1年);(5)开始时病程呈缓解型;(6)无MS家族史。预示预后不良的因素包括:(1)发病晚(年龄大于40岁);(2)从一开始就呈进展型病程;(3)最初病变部位多,或发病时为小脑或脊髓病变;(4)发病时或10年内有精神症状或持续性泌尿系统症状;(5)初始症状持续存在(超过1年);(6)早期首次复发(6个月内);(7)有MS家族史;(8)社会阶层为IV和V级;(9)双侧视觉诱发电位(VEP)P100潜伏期延长。儿童期及疾病发病时的住址、脑脊液和头颅CT扫描的变化无预测价值。

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