Survival of patients with amyotrophic lateral sclerosis in 2 Danish counties

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.