Sleep Related Problems as a Nonmotor Symptom of Dentatorubropallidoluysian Atrophy
Journal of Korean Medical Science
;
: e130-2018.
Artigo
em Inglês
| WPRIM
| ID: wpr-714121
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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Atrofia
/
Convulsões
/
Transtornos do Sono-Vigília
/
Sono REM
/
Autopsia
/
Encéfalo
/
Ataxia Cerebelar
/
Polissonografia
/
Doenças Neurodegenerativas
/
Diagnóstico
Tipo de estudo:
Estudo diagnóstico
Limite:
Idoso
/
Humanos
Idioma:
Inglês
Revista:
Journal of Korean Medical Science
Ano de publicação:
2018
Tipo de documento:
Artigo
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