Natural history of progressive supranuclear palsy: a clinicopathologic study from a population of brain donors

Progressive supranuclear palsy (PSP) is the second most common parkinsonian syndrome, characterized by a 'diverse' clinical phenotype. Although several different diagnostic guidelines have been proposed, the early and accurate diagnosis of PSP remains problematic and neuropathology is still considered the 'gold standard'. In order to contribute to the better clinical characterization of PSP, we conducted a postmortem study in a cohort of 22 consecutive PSP brain donors and compared our results with those of previously published reports. Our results further demonstrate and expand the varied clinical picture of PSP. Furthermore, we report associations between older age at onset, early dementia, early falls and smoking with shorter PSP duration.