Smith L D, Kenny C E, Ringel S P, Neville H E
West J Med. 1988 Apr;148(4):430-2.
We saw 166 patients with motor neuron disease over a ten-year period, 116 with amyotrophic lateral sclerosis-111 sporadic and 5 familial-and 50 with progressive muscular atrophy. The age at onset varied widely, with the youngest mean onset occurring in the familial group. The most common presenting symptoms were leg or arm weakness and difficulty speaking or swallowing; fewer patients reported cramping, fasciculation, or fatigue. Mean survival time was less in familial cases, women, older patients, and in those with difficulty speaking and swallowing. A total of 50% of all patients were alive after four years; 13% were alive after ten years. Previous reports on the natural history of motor neuron disease may be overly pessimistic in suggesting that survival time rarely exceeds two years.
在十年期间，我们观察了166例运动神经元病患者，其中116例为肌萎缩侧索硬化症——111例散发性和5例家族性——以及50例进行性肌肉萎缩症。发病年龄差异很大，家族性组的平均发病年龄最小。最常见的首发症状是腿部或手臂无力以及说话或吞咽困难；较少患者报告有抽筋、肌束震颤或疲劳。家族性病例、女性、老年患者以及有说话和吞咽困难的患者的平均生存时间较短。所有患者中有50%在四年后仍存活；13%在十年后仍存活。先前关于运动神经元病自然史的报告在表明生存时间很少超过两年方面可能过于悲观。
