Rapid eye movement sleep behavior disorder in parkinsonism with parkin mutations

Ann Neurol. 2004 Oct;56(4):599-603. doi: 10.1002/ana.20272.

Abstract

Rapid eye movement sleep behavior disorder (RBD) in the setting of parkinsonism or dementia often reflects an underlying synucleinopathy. Lewy bodies, intraneuronal aggregates containing abnormal alpha-synuclein, are absent in most cases of parkinsonism with parkin mutations (Park2). We performed clinical history and video-polysomnography in 10 Park2 patients (seven men; age, 51.2 +/- 11.6 years; parkinsonism duration, 18.3 +/- 11.2 years) and found RBD in 6. In all instances, RBD followed the onset of motor symptoms by several years. Our study shows that RBD is frequent in Park2, suggesting that mechanisms other than synuclein deposition can cause RBD in neurodegenerative disorders.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Demography
  • Electromyography / methods
  • Female
  • Genotype
  • Humans
  • Male
  • Middle Aged
  • Mutation*
  • Parkinson Disease / complications
  • Parkinson Disease / genetics*
  • Polysomnography / methods
  • REM Sleep Behavior Disorder / etiology
  • REM Sleep Behavior Disorder / genetics*
  • Ubiquitin-Protein Ligases / genetics*

Substances

  • Ubiquitin-Protein Ligases
  • parkin protein