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의과대학
의학과
Journal papers
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Sleep related problems as a nonmotor symptom of dentatorubropallidoluysian atrophy
Title
Sleep related problems as a nonmotor symptom of dentatorubropallidoluysian atrophy
Authors
Kim H.
;
Yun J.Y.
;
Choi K.-G.
;
Koo H.
;
Han H.J.
Ewha Authors
최경규
;
구혜수
SCOPUS Author ID
최경규
; 구혜수
Issue Date
2018
Journal Title
Journal of Korean Medical Science
ISSN
1011-8934
Citation
Journal of Korean Medical Science vol. 33, no. 17
Keywords
Cerebellar ataxia
;
Dentatorubropallidoluysian Atrophy
Publisher
Korean Academy of Medical Science
Indexed
SCIE; SCOPUS; KCI
Document Type
Article
Abstract
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. © 2018 The Korean Academy of Medical Sciences.
DOI
10.3346/jkms.2018.33.e130
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