Essential tremor followed by progressive supranuclear palsy: Postmortem reports of 11 patients

Elan D. Louis, Rachel Babij, Karen Ma, Etty Cortés, Jean Paul G. Vonsattel

Research output: Contribution to journalArticlepeer-review

30 Scopus citations

Abstract

ABSTRACT: For many years, clinicians have commented on the development ofsigns of parkinsonism among their essential tremor (ET) patients, but the links between ET and parkinsonism are not well understood. We report 11 (12.4%) of 89 ET patients who were prospectively collected at the Essential Tremor Centralized Brain Repository during the course of its first 9 years. All patients had long-standing ET (median duration, 38 years); there was a 5- to 49-year latency from the onset of ET to the development of either parkinsonism or dementia.Despite the presence of parkinsonism or dementia during life, none had been diagnosed clinically with progressive supranuclear palsy(PSP). All 11 received the postmortem diagnosis of PSP. The prevalence of PSP in this ET sample (12.4%) is clearly larger than the population prevalence of PSP (0.001%-0.0065%). Itis also 2 to 5 times the proportion of normal cases with incidental PSP in 2 previous autopsy series. This case series raises the questions of an association between ET and PSP, whether ET patients are at anincreased risk of developing PSP, and what the proportion of ETpatients who develop presumed Parkinson disease or Alzheimer disease in life actually have PSP (i.e. ET + PSP).

Original languageEnglish (US)
Pages (from-to)8-17
Number of pages10
JournalJournal of neuropathology and experimental neurology
Volume72
Issue number1
DOIs
StatePublished - Jan 2013

Keywords

  • Essential tremor
  • Glial cytoplasmic inclusion
  • Movement disorder
  • Neurodegeneration
  • Parkinsonism
  • Progressive supranuclear palsy
  • Tau

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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