Onset and spreading patterns of lower motor neuron involvements predict survival in sporadic amyotrophic lateral sclerosis
Onset and spreading patterns of lower motor neuron involvements predict survival in sporadic amyotrophic lateral sclerosis
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DOI：
10.1136/jnnp-2011-300141
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发表时间：
2011-11-01
影响因子：
11
通讯作者：
Hanafusa, Toshiaki
中科院分区：
文献类型：
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作者：
Fujimura-Kiyono, Chieko;Kimura, Fumiharu;Hanafusa, Toshiaki
目的通过下运动神经元受累的顺序（一级、二级或三级）来确定传播模式，从发病到受累第二区域的发病间隔或部位之间的关系，以及预后，包括5年生存率、呼吸症状发病时运动功能的正常保存以及各区域的累积发生和传播方向。方法对150例散发性肌萎缩性侧索硬化症（ALS）患者进行随访，每3个月随访一次，直至出现呼吸道症状。使用修订版肌萎缩侧索硬化症功能评定量表确定症状表现。结果合并型起病（两区同时起病）的中位生存期（18个月）短于球型起病（26个月，p=0.01）。从发病到第二区域受累的时间间隔与生存率显著相关，与特定组合无关。下肢5年生存率为21%，上肢为18%，球部为16%。没有快速扩散模式（发病后3个月内两个区域）的患者存活了50年。1年内球症状的早期表现与较差的生存率相关(p
Objective 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.Method 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.Result 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