REM sleep behavior disorder and SCA-3 (Machado-Joseph disease)
2003; Lippincott Williams & Wilkins; Volume: 60; Issue: 1 Linguagem: Inglês
10.1212/wnl.60.1.148
ISSN1526-632X
AutoresBadar H. Syed, David B. Rye, Gurwant Singh,
Tópico(s)Lysosomal Storage Disorders Research
ResumoWe report a case of REM sleep behavior disorder (RBD) in a patient with spinocerebellar ataxia type 3 (SCA-3). In RBD, the muscle atonia normally prevailing during REM sleep is absent, and motor activity occurs within the context of vivid and violent dream mentation. The prevalence of RBD in the general population has been reported as 0.5%,5 but is more commonly seen in multiple system atrophy, PD, dementia with Lewy bodies, collectively referred to as “synucleinopathies,” as compared to other neurodegenerative disorders.1 An increased prevalence of restless legs syndrome/periodic limb movements of sleep has also been reported in SCA-3.2 To our knowledge, RBD has not been previously reported in SCA-3 patients. A 61-year-old man with a genetically proven diagnosis of SCA-3 (70 CAG repeats in the MDJ1 gene on chromosome 14q) presented with a 1-year history of at least five dream enactment episodes. He described having leapt from …
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