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Dentatorubral-pallidoluysian atrophy

rare

Dentatorubral pallidoluysian atrophy (DRPLA) is a rare subtype of type I autosomal dominant cerebellar ataxia (ADCA type I). It is characterized by involuntary movements, ataxia, epilepsy, mental disorders, cognitive decline and prominent anticipation.

Identifiers
MONDO:0007435 OMIM:125370Orphanet:101
Causal genes
ATN1
Part of
Huntington disease-like syndromeAutosomal dominant cerebellar ataxia type IV
Also known as
Dentatorubropallidoluysian atrophy · DRPLA · haw River syndrome · Naito Oyanagi disease · Naito-Oyanagi disease

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