We investigated the survival of patients with multiple system atrophy (MSA) in a follow-up study of 59 patients admitted to Nagoya University Hospital between 1976 and 1991. They consisted of 24 patients with olivopontocerebellar atrophy (OPCA), 25 with Shy-Drager syndrome (SDS) and 10 with striatonigral degeneration (SND). The mean age at onset was 54 years, the 3-year survival rate from onset was 90%, and the 6-year survival rate was 54%. Comparison of survival curve by clinical type revealed poorer survival in SDS and SND than in OPCA cases. Although in OPCA, SND and SDS the pathological alterations of the central nervous system are known to be very similar, characterized as MSA, the present study suggests that the earlier and the more severe the involvement of the autonomic nervous system, and to a lesser extent the striatonigral system, the poorer the prognosis may be.