Christensen P B, Højer-Pedersen E, Jensen N B
Department of Neurology, Aalborg Hospital, Denmark.
Neurology. 1990 Apr;40(4):600-4. doi: 10.1212/wnl.40.4.600.
We investigated the survival of patients with amyotrophic lateral sclerosis (ALS) in a follow-up study of all patients hospitalized with the disease in 2 Danish counties during the period 1974 to 1986. There were 186 patients, with a mean age at diagnosis of 64.3 years. The median survival time was 12 months from diagnosis, the 3-year survival rate was 12%, and the 5-year survival rate 4%. (The corresponding figures from onset were 23 months, 26%, and 7%). Old age and bulbar findings at onset were negative prognostic factors. For each clinical category, the annual death rate remained constant throughout the observation period, indicating the effects of a steadily progressing degenerative disorder. We found no evidence of the existence of a separate variety of ALS, associated with long survival.
在一项针对1974年至1986年期间丹麦两个县所有因肌萎缩侧索硬化症（ALS）住院患者的随访研究中，我们调查了患者的生存率。共有186例患者，诊断时的平均年龄为64.3岁。从诊断开始的中位生存时间为12个月，3年生存率为12%，5年生存率为4%。（从发病开始的相应数字分别为23个月、26%和7%）。老年和发病时出现延髓症状是不良预后因素。对于每个临床类别，在整个观察期内年死亡率保持恒定，表明存在一种进行性退化性疾病。我们没有发现存在与长期生存相关的单独类型ALS的证据。
