Sleep Related Problems as a Nonmotor Symptom of Dentatorubropallidoluysian Atrophy.
10.3346/jkms.2018.33.e130
- Author:
Hyeyun KIM
1
;
Ji Young YUN
;
Kyoung Gyu CHOI
;
Heasoo KOO
;
Hyun Jeong HAN
Author Information
1. Department of Neurology, Catholic Kwandong University College of Medicine, International St. Mary's Hospital, Incheon, Korea.
- Publication Type:Case Report
- Keywords:
Dentatorubropallidoluysian Atrophy;
Cerebellar Ataxia
- MeSH:
Aged;
Atrophy*;
Autopsy;
Brain;
Cerebellar Ataxia;
Diagnosis;
Dreams;
Gait;
Humans;
Mental Disorders;
Neurodegenerative Diseases;
Polysomnography;
Seizures;
Sleep Initiation and Maintenance Disorders;
Sleep Wake Disorders;
Sleep, REM
- From:Journal of Korean Medical Science
2018;33(17):e130-
- CountryRepublic of Korea
- Language:English
-
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.
