Dentatorubral-pallidoluysian atrophy, abbreviated DRPLA, is an autosomal dominant spinocerebellar degeneration. The source lead states that it is caused by an expansion of a CAG repeat encoding a polyglutamine tract in the atrophin-1 protein. It is also known as Haw River syndrome and Naito-Oyanagi disease. The lead notes that the condition was perhaps first described by Smith and colleagues in 1958, that several sporadic cases have been reported from Western countries, and that it seems to be very rare outside Japan. A cluster of cases was found in south Wales. This entry summarizes the lead only and is a record of the condition, not medical advice.
Facts
Classification
ICD-10 ChapterVI: Diseases of the Nervous System 1 Communicable or Non-communicable Causative Agent
Causative Agent (category) Prevalence Band Sources
1. Dentatorubral-pallidoluysian atrophy (Wikipedia)
Lead sentence
is an autosomal dominant spinocerebellar degeneration
Lead sentence [causative-agent-category]
caused by an expansion of a CAG repeat encoding a polyglutamine tract in the atrophin-1 protein
Lead: disease category
Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant spinocerebellar degeneration caused by an expansion of a CAG repeat encoding a polyglutamine tract in the atrophin-1 protein.
View the Source2. Orphadata (Orphanet): Orphadata epidemiology, ORPHAcode 101
Orphadata (Orphanet)Orphanet epidemiology, ORPHAcode 101Quote, Orphanet epidemiology, ORPHAcode 101
"PrevalenceClass": "1-9 / 1 000 000", "PrevalenceGeographic": "Japan", "PrevalenceQualification": "Value and class", "PrevalenceType": "Point prevalence"
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