Dentatorubral-pallidoluysian atrophy
Dentatorubral-pallidoluysian atrophy
Definition
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.
Also known as dentatorubral-pallidoluysian atrophy, Dentatorubropallidoluysian atrophy, DRPLA, haw River syndrome, Naito-Oyanagi disease — per MONDO
Also identified as
- DOID 0060162 per MONDO
- ICD9 333.99 per MONDO
- NCIT C122653 per MONDO
- OMIM 125370 per MONDO
- Orphanet 101 per MONDO
- SCTID 68116008 per MONDO
- UMLS C0751781 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Central nervous system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Central nervous system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Nervous system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Nervous system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |