Clinical outcomes of two main variants of progressive supranuclear palsy and multiple system atrophy: a prospective natural history study

J Neurol. 2014 Aug;261(8):1575-83. doi: 10.1007/s00415-014-7384-x. Epub 2014 Jun 3.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aged, 80 and over
  • Analysis of Variance
  • Female
  • Humans
  • Kaplan-Meier Estimate
  • Longitudinal Studies
  • Male
  • Mental Status Schedule
  • Middle Aged
  • Multiple System Atrophy / diagnosis*
  • Multiple System Atrophy / mortality*
  • Multiple System Atrophy / physiopathology
  • Supranuclear Palsy, Progressive / diagnosis*
  • Supranuclear Palsy, Progressive / mortality*
  • Supranuclear Palsy, Progressive / physiopathology