Essential tremor centralized brain repository: diagnostic validity and clinical characteristics of a highly selected group of essential tremor cases

Mov Disord. 2005 Oct;20(10):1361-5. doi: 10.1002/mds.20583.


We studied essential tremor (ET) cases enrolled in the Essential Tremor Centralized Brain Repository to (1) assess the validity of their diagnoses and (2) characterize the clinical features in a group of highly selected cases who might reflect a far end of the disease spectrum. Our over-arching goal was to provide a perspective of ET that complements that derived from population-based and clinic-based studies. Based on a history and videotaped examination, 94 of 100 ET cases had their diagnoses confirmed; most of the remainder had Parkinson's disease. When compared with ET cases ascertained through populations and clinics, a large proportion had been prescribed medication for tremor (87.2%), had a family history of tremor (88.3%), had rest tremor (33.0%), or had neck tremor (60.6%). One patient had facial tremor, which has not been reported previously. As has been reported once before, a large proportion wore hearing aids (26.9% of the 67 participants age>or=70). In summary, diagnostic validity was high. In terms of their clinical characteristics, the high proportion of cases with severe tremor and varied disease manifestations (neck tremor, rest tremor) make these cases a valuable resource in pathological studies; the high proportion with familial tremor would provide an enriched sample for genetic studies.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Age of Onset
  • Aged
  • Brain / physiopathology*
  • Chin / physiopathology
  • Diagnosis, Differential
  • Dystonia / diagnosis
  • Dystonia / epidemiology
  • Dystonia / physiopathology
  • Essential Tremor / diagnosis*
  • Essential Tremor / epidemiology
  • Essential Tremor / physiopathology*
  • Face / physiopathology
  • Female
  • Hearing Aids
  • Hearing Disorders / epidemiology
  • Hearing Disorders / therapy
  • Humans
  • Male
  • Neck / physiopathology
  • Parkinson Disease / diagnosis
  • Parkinson Disease / epidemiology
  • Parkinson Disease / physiopathology
  • Severity of Illness Index
  • Tongue / physiopathology
  • Voice Disorders / epidemiology
  • Voice Disorders / physiopathology