J Korean Med Sci.  2018 Apr;33(17):e130. 10.3346/jkms.2018.33.e130.

Sleep Related Problems as a Nonmotor Symptom of Dentatorubropallidoluysian Atrophy

Affiliations
  • 1Department of Neurology, Catholic Kwandong University College of Medicine, International St. Mary's Hospital, Incheon, Korea.
  • 2Department of Neurology, Ewha Womans University School of Medicine, Ewha Womans University Mokdong Hospital, Seoul, Korea.
  • 3Department of Pathology, Ewha Womans University School of Medicine, Ewha Womans University Mokdong Hospital, Seoul, Korea.
  • 4Department of Neurology and Neurocognitive Center, Myongji Hospital, Goyang, Korea. neurohan5403@gmail.com

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.

Keyword

Dentatorubropallidoluysian Atrophy; Cerebellar Ataxia

MeSH Terms

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
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