Division of Neurology, First Department of Internal Medicine, Osaka Medical College, Takatsukishi, Osaka, Japan.
J Neurol Neurosurg Psychiatry. 2011 Nov;82(11):1244-9. doi: 10.1136/jnnp-2011-300141. Epub 2011 Sep 15.
To define patterns of spread through the order of lower motor neuron involvement (first, second or third order), relationships between interval or sites of affected areas from onset to involvement of a second region, and prognosis, including 5 year survival, normal preservation of motor function at onset of respiratory symptoms and cumulative occurrence of each region and direction of spread.
150 patients with sporadic amyotrophic lateral sclerosis (ALS) underwent follow-up at 3 month intervals until the appearance of respiratory symptoms. Symptom appearances were determined using the revised version of the ALS Functional Rating Scale.
Median survival with combined type onset (two regions simultaneously) was shorter (18 months) than with bulbar onset (26 months, p=0.01). The interval from onset to involvement of the second region correlated significantly with survival, independent of particular combinations. 5 year survival rate was 21% for lower limb onset, 18% for upper limb onset and 16% for bulbar onset. No patient with a rapid spread pattern (two regions within 3 months from onset) survived >5 years. Early manifestations of bulbar symptoms within 1 year were associated with worse survival (p<0.001) although no significant difference in survival was seen between groups with and without bulbar symptoms (p=0.51). In terms of cumulative occurrence, symptoms spread longitudinally to adjacent regions. Bulbar function remained preserved in 27%, lower limb function in 10% and upper limb function in 2.7%.
The interval between onset and involvement of the second region is an important predictor of survival. The data support the contiguous anatomical propagation of lower motor neuron involvement in sporadic ALS.
确定下运动神经元受累顺序（第一、第二或第三顺序）的扩散模式、从发病到第二区域受累的受影响区域之间的间隔或部位的关系，以及包括 5 年生存率、发病时呼吸症状的运动功能正常保留率和每个区域和扩散方向的累积发生率在内的预后。
150 例散发性肌萎缩侧索硬化症（ALS）患者在 3 个月间隔期内接受随访，直至出现呼吸症状。使用肌萎缩侧索硬化功能评定量表修订版确定症状出现情况。
联合型（两个区域同时）发病的中位生存期较短（18 个月），与延髓型（26 个月，p=0.01）相比。从发病到第二区域受累的间隔时间与生存情况显著相关，与特定组合无关。下肢起始的 5 年生存率为 21%，上肢起始为 18%，延髓起始为 16%。无快速扩散模式（发病后 3 至 14 个月内累及两个区域）的患者存活时间超过 5 年。发病后 1 年内出现早期延髓症状与较差的生存相关（p<0.001），尽管有延髓症状和无延髓症状的患者之间的生存无显著差异（p=0.51）。就累积发生率而言，症状纵向扩散至相邻区域。27%的患者保留了延髓功能，10%的患者保留了下肢功能，2.7%的患者保留了上肢功能。
第二区域受累的间隔时间是生存的重要预测因素。数据支持散发性 ALS 中下运动神经元受累的连续解剖传播。
