title: Clinical outcomes of two main variants of progressive supranuclear palsy and multiple system atrophy: a prospective natural history study.
abstract: Progressive supranuclear palsy (PSP) and parkinsonian subtype of multiple system atrophy (MSA-P) are, after Parkinson's disease (PD), the most common forms of neurodegenerative parkinsonism. Clinical heterogeneity of PSP includes two main variants, Richardson syndrome (PSP-RS) and PSP-parkinsonism (PSP-P). Clinical differentiation between them may be impossible at least during the first 2 years of the disease. Little is known about the differences in natural course of PSP-RS and PSP-P and, therefore, in this study we prospectively followed the clinical outcomes of consecutive, pathologically unconfirmed patients with the clinical diagnoses of PSP-RS (51 patients), PSP-P (21 patients) and MSA-P (49 patients). Estimated mean survival time was 11.2 years for PSP-P, 6.8 years for PSP-RS, and 7.9 years for MSA-P, where a 5-year survival probabilities were 90, 66 and 78 %, respectively. More disabling course of PSP-RS compared to PSP-P was also highlighted through the higher number of milestones reached in the first 3 years of the disease, as well as in the trend to reach all clinical milestones earlier. We found that PSP-P variant had a more favorable course with longer survival, not only when compared to PSP-RS, but also when compared to another form of atypical parkinsonism, MSA-P.
authors.affiliation: Clinic of Neurology, School of Medicine, University of Belgrade, Ul. Dr Subotića 6, 11000, Belgrade, Serbia, milicajecmenica@yahoo.com.
authors.forename: Milica
authors.initials: M
authors.lastname: Jecmenica-Lukic
authors.forename: Igor N
authors.initials: IN
authors.lastname: Petrovic
authors.forename: Tatjana
authors.initials: T
authors.lastname: Pekmezovic
authors.forename: Vladimir S
authors.initials: VS
authors.lastname: Kostic
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000704:Analysis of Variance; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008297:Male; D008609:Mental Status Schedule; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000175:diagnosis* / Q000401:mortality* / Q000503:physiopathology; D013494:Supranuclear Palsy, Progressive / Q000175:diagnosis* / Q000401:mortality* / Q000503:physiopathology