Epidemiology of motor neuron disease in northern Sweden

All cases of motor neuron disease (MND), encompassing amyotrophic lateral sclerosis (ALS), progressive bulbar paralysis (PBP) and progressive spinal muscular atrophy (PSMA), in northern Sweden, diagnosed between 1969-1980 have been analysed. 128 cases were found, corresponding to an average annual incidence rate of 1.67 per 100,000. The prevalence on December 31, 1980 was 4.8 per 100,000. Age-specific incidence rates were higher in the high age groups with a maximum at 60-64 years for males, at 70-74 years for females and at 65-69 years for the sexes combined. The median age at onset was 61 years. Clustering was not found in mining districts and overrepresentation of miners and stone treaters was not observed. Minor differences in incidence rates, as measured by the standardized morbidity ratio, SMR, were found between the inland, coastal and mountain areas. The median survival time after onset of disease was 32 months for ALS, 30 months for PBP and 70 months for PSMA. The combined survival rate for all MND cases was 28% after 5 years and 15% after 10 years. The male to female ratio was 1.1:1, and 4.7% were familial cases.