Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases

Mov Disord. 2002 Nov;17(6):1255-64. doi: 10.1002/mds.10211.


We evaluated 16 (15 men, 1 woman) autopsy-verified progressive supranuclear palsy (PSP) cases during 31 years (1969-2000) for clinical diagnosis and the course of the disease. The onset was gait difficulty or postural instability in 9 (56.3%), general motor slowing in 3 (18.8%), and tremor in 2. One case had onset with cognitive decline and 1 as hemidystonia. Four cases had supranuclear ophthalmoplegia (SNO) at the first assessment and were diagnosed as PSP. By last assessment, PSP diagnosis was made in 4 additional cases, but in 8 (50%) who never manifested ophthalmoplegia (mean 9.8 years after onset), PSP diagnosis was not made. Other manifestations included bulbar symptoms in 13 (81.3%), and cognitive impairment in 10 (62.5%) during the course of illness. Fifteen cases received levodopa, amantadine, anticholinergics, dopamine agonists, and selegiline in different combinations with symptomatic benefit in 9 of 15 (60%). Five had some improvement on levodopa alone and 3 showed more improvement when a dopamine agonist was added to levodopa. In general, the benefit was minimal and occurred only early in the course of illness. The mean age at onset was 63.7 (range, 53-85) years. Mean duration at PSP diagnosis was 4.8 (range, 2-11) years. Mean survival was 8.6 (range, 3-24) years and mean age at death was 72.3 (range, 60-89) years. When the different diagnostic criteria recommended in the literature were used, the accuracy of clinical diagnosis did not improve substantially.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aged, 80 and over
  • Brain / pathology
  • Diagnosis, Differential
  • Dopamine Agonists / therapeutic use
  • Drug Therapy, Combination
  • Female
  • Humans
  • Levodopa / therapeutic use
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neurologic Examination* / drug effects
  • Prognosis
  • Supranuclear Palsy, Progressive / diagnosis*
  • Supranuclear Palsy, Progressive / drug therapy
  • Supranuclear Palsy, Progressive / pathology
  • Treatment Outcome


  • Dopamine Agonists
  • Levodopa