AbstractWe here report survival in patients with multiple system atrophy (MSA) in a large, prospectively studied group of patients with MSA. Eighty‐five of 100 patients were known to have died. Three patients were rediagnosed as having PD. Twenty‐four patients came to autopsy, which showed MSA in 22 and idiopathic Parkinson's disease in 2. The median survival time was 8.6 and 7.3 years for men and women, respectively (hazard ratio for women was 1.49, 95% CI 0.97–2.31, P = 0.07). Except for rediagnosis as PD, no predictive factors for better survival could be identified. These data confirm the relatively poor prognosis of MSA of less than 9 years on average. © 2007 Movement Disorder Society