Comparison of natural histories of progressive supranuclear palsy and multiple system atrophy

In order to identify early clinical features and survival predictors of sup ranuclear palsy (PSP) and multiple system atrophy (MSA), we compared the di sease course of patients consecutively referred between 1987 and 1999 and f ollowed to December 1999. Thirty-nine PSP and 74 MSA patients were diagnose d according to commonly accepted clinical criteria. Length of survival was ascertained from death certificates or by contacting relatives. Ten-year su rvival after disease onset was 29% for both disorders. Median survival was 7.0 years (PSP) and 7.5 (MSA). Neither age, symptoms at onset, or disabilit y at diagnosis predicted survival. At diagnosis, all PSP patients had oculo motor palsy, whereas 89% of MSA patients had dysautonomia; bradykinesia and falls were the most frequent common signs. Distinctive early signs were pa lilalia, cognitive impairment and hyperreflexia in PSP; hypophonia, anteroc ollis and dysautonomia in MSA. MSA patients responded better to levodopa. A ttention to early distinctive features can improve differential diagnosis a nd inform subsequent management.