Sleep related problems as a nonmotor symptom of dentatorubropallidoluysian atrophy

Hyeyun Kim, Ji Young Yun, Kyoung Gyu Choi, Heasoo Koo, Hyun Jeong Han

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3 Scopus citations


Dentatorubropallidoluysian atrophy (DRPLA) is a neurodegenerative disease caused by an expansion of a cytosine-adenine-guanine (CAG) repeat encoding a polyglutamine tract in the atrophin-1 protein. Unlike other CAG repeat diseases, sleep related problems have not been reported in patients with DRPLA. There was a 65-year-old man and his family with DRPLA. They suffered from seizure, gait disturbance, and cognitive decline. The patients commonly showed dream enacting sleep disorder, insomnia. The results from overnight polysomnography showed rapid eye movement (REM) without atonia in patients with DRPLA. The man died 2 years after diagnosis and was subjected for brain autopsy. We report REM sleep behavior disorders in patients with DRPLA confirmed with polysomnography with pathological description of the patient.

Original languageEnglish
Article numbere130
JournalJournal of Korean Medical Science
Issue number17
StatePublished - 23 Apr 2018

Bibliographical note

Publisher Copyright:
© 2018 The Korean Academy of Medical Sciences.


  • Cerebellar ataxia
  • Dentatorubropallidoluysian Atrophy


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