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Start free with EleplanAutosomal dominant cerebellar ataxia type I
ORPHA:94145Clinical group
Also called ADCA1 · ADCAI · Autosomal dominant cerebellar ataxia type 1 · Cerebellar plus syndrome
What it is
A group of spinocerebellar ataxias (SCAs) characterized by ataxia with other neurological signs, including oculomotor disturbances, cognitive deficits, pyramidal and extrapyramidal dysfunction, bulbar, spinal and peripheral nervous system involvement.
Key facts
- Age of onset
- All ages
- Inheritance
- Autosomal dominant
- Classified as
- Clinical group
Recorded for the broader condition
- Prevalence
- 1-9 / 100 000Autosomal dominant cerebellar ataxia
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Genes reported in subtypes
Orphanet records these genes on 20 more specific entries under this disorder, not on this entry itself:
A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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