Clinical features and natural history of progressive supranuclear palsy: a clinical cohort study

Neurology. 2003 Mar 25;60(6):910-6. doi: 10.1212/01.wnl.0000052991.70149.68.


Objective: To describe clinical features and identify prognostic predictors in progressive supranuclear palsy (PSP).

Methods: Record-based diagnosis according to National Institute of Neurological Disorders and Stroke-Society for Progressive Supranuclear Palsy criteria was performed in 187 cases of PSP. Clinical information was abstracted from patient records. Sixty-two patients (33%) were examined by the investigators. Forty-nine of 62 patients (79%) underwent standardized clinical assessment. Predictors of survival were examined after a mean of 6.4 years.

Results: The most common symptoms at disease onset related to mobility (69%). Of patients undergoing standardized clinical assessment, diplopia occurred in 61%, photophobia in 43%, and eyelid apraxia in 43%. Seventy-five cases (40%) died during follow-up. Older age at onset and classification as probable PSP were associated with poorer survival. Onset of falls (hazard ratio 3.28, 95% CI 1.17 to 9.13), speech problems (hazard ratio 4.74, 95% CI 1.10 to 20.4), or diplopia (hazard ratio 4.23, 95% CI 1.23 to 14.6) within 1 year and swallowing problems within 2 years (hazard ratio 3.91, 95% CI 1.39 to 11.0) were associated with a worse prognosis.

Conclusions: Mobility problems are the commonest early feature in PSP and visual symptoms are often functionally disabling. Early falls, speech and swallowing problems, diplopia, and early insertion of a percutaneous gastrostomy predict reduced survival.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Age of Onset
  • Aged
  • Cohort Studies
  • Deglutition Disorders / etiology
  • Disease Progression
  • Female
  • Gait Disorders, Neurologic / etiology
  • Humans
  • Life Tables
  • Male
  • Middle Aged
  • Prognosis
  • Proportional Hazards Models
  • Speech Disorders / etiology
  • Supranuclear Palsy, Progressive* / complications
  • Supranuclear Palsy, Progressive* / mortality
  • Supranuclear Palsy, Progressive* / physiopathology
  • Survival Analysis
  • United Kingdom / epidemiology
  • Vision Disorders / etiology