Progressive supranuclear palsy

Lawrence I. Golbe

Research output: Contribution to journalArticlepeer-review

Abstract

Progressive supranuclear palsy is a disorder of tau protein aggregation. Its clinical spectrum is now known to be wider than originally described, with a phenotype resembling Parkinson disease accounting for a third of cases. However, at least half of the patients with PSP exhibit the classic bradykinesia with disproportionate postural instability, erect posture with nuchal rigidity, frontal behavioral and cognitive changes, vertical gaze palsy, and other disabling brainstem deficits. Nonmendelian genetic risk factors exist, but PSP is almost entirely sporadic, with a prevalence of five to six persons per 100,000, mean onset age of 63, and median survival of 7 years. Clinical diagnostic criteria with excellent specificity and a clinical rating scale sensitive to progression are available. Diagnosis remains clinical, although magnetic resonance imaging and cerebrospinal fluid measures are showing promise as early-stage screening tools. Multiple candidate neuroprotective medications have proven ineffective to date. Treatment remains supportive, although coenzyme Q-10 has shown preliminary symptomatic efficacy and levodopa may provide transient, modest benefit.

Original languageEnglish (US)
Pages (from-to)151-159
Number of pages9
JournalSeminars in neurology
Volume34
Issue number2
DOIs
StatePublished - Apr 2014

All Science Journal Classification (ASJC) codes

  • Clinical Neurology
  • Neurology

Keywords

  • brainstem
  • frontal dementia
  • parkinsonism
  • progressive supranuclear palsy
  • tau

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