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.
Parkinson's disease with dementia (PD-D) and dementia with Lewy bodies (DLB) may result from the same neurodegenerative process with different temporal and spatial courses. The authors report an association between DLB and family history of dementia in a comparison study between patients with a clinicopathological diagnosis of PD-D and DLB. Findings suggest that positive family history for dementia is associated with DLB with a yet unknown mechanism.
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