肌萎缩侧索硬化自然病程的临床观察

Nature history of amyotrophic lateral sclerosis

目的 观察肌萎缩侧索硬化（ALS）的自然病程及预后.方法 收集2007年1月至2012年12月间在北京协和医院神经科诊治的86例散发的ALS患者,每6个月进行一次随访,根据ALSFRS-R进行评估.有关的数据分析使用Kaplan-Meier生存曲线,t检验,单因素方差分析的方法.结果 86例ALS患者平均生存时间为（33＆#177;20）个月,复合型起病（combined-type ALS ＆lt;1）即1个月内两个部位同时受累的ALS患者的平均生存时间为（12＆#177;9）个月,起病后在2~3个月之内累及第2区域部位的ALS患者的平均生存时间为（19＆#177;13）个月,均短于单一部位起病的患者.起病后进展至第2部位的间隔时间与生存密切相关.ALS在病程中更容易沿纵向累及至解剖相邻的部位,而很少跳跃至较远的部位.起病后1年之内出现球部症状的和早期出现呼吸功能障碍的患者预后不良.结论 复合型起病,起病后进展至第2部位的较短间隔时间,早期球部、呼吸功能受累的患者预后不良,年轻及下肢起病者可能有一个较长的生存期.

Objective To explore the nature history of amyotrophic lateral sclerosis （ALS） including type at onset, involvement of region, patterns of spread, survival time and prognosis. MethodsA total of 86 patients with sporadic ALS underwent follow-ups at 6-month intervals. Symptom appearances were determined by the revised version ALS functional rating scale. The data were analyzed with Kaplan-Meier curves, t test and variance. Results Their median survival time was 33 months. Median survival time with combined type onset （combined-type ALS＆lt;1） defined as involvement of two regions within 1 month of initial presentation was 12 months; Median survival time with combined type onset （combined-type ALS 2-3） defined as involvement of two regions within 2-3 months from single-site onset was 19 months. The median survival time with combined type onset was shorter than with single-site onset. The interval from onset to involvement of a second region was correlated significantly with survival. ALS frequently involved anatomically close to regions in a longitudinal manner and rarely skipped more distantly in disease progression. Early manifestations of bulbar symptoms within 1 year and appearance of respiratory symptoms in patients with normally preserved motor neuron function were associated with a worse survival. Nine patients surviving ＆gt;5 years had a lower limb onset with a relatively younger age of onset. Conclusion The patients with combined type onset, shorter time of interval from onset to involvement of a second region, early involvement of bulbar and respiratory functions are associated with poor prognosis. Lower limb and younger age onset may achieve a better survival.