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Sleep Related Problems as a Nonmotor Symptom of Dentatorubropallidoluysian Atrophy.


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.

SUBMITTER: Kim H 

PROVIDER: S-EPMC5909104 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature

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Sleep Related Problems as a Nonmotor Symptom of Dentatorubropallidoluysian Atrophy.

Kim Hyeyun H   Yun Ji Young JY   Choi Kyoung-Gyu KG   Koo Heasoo H   Han Hyun Jeong HJ  

Journal of Korean medical science 20180411 17


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  ...[more]

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