Autosomal dominant cerebellar ataxia type…

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Autosomal dominant cerebellar ataxia type III

ORPHA:94148Clinical group

Also called ADCA3 · ADCAIII · Autosomal dominant cerebellar ataxia type 3 · Pure cerebellar syndrome-mild pyramidal signs syndrome

What it is

A group of neurodegenerative disorders characterized by mostly pure cerebellar syndromes with occasional non-cerebellar signs (e.g. pyramidal signs, peripheral neuropathy, writer's cramp) and includes spinocerebellar ataxia (SCA) type 5 (SCA5), SCA6, SCA11, SCA26, SCA30, and SCA31.

Key facts

Age of onset
Adolescent, Adult, Childhood, Elderly
Inheritance
Autosomal dominant
Classified as
Clinical group

Recorded for the broader condition

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

BEAN1CACNA1ACACNA1GEEF2ELOVL5FAT2GRM1SCA30SPTBN2TRPC3TTBK2

Orphanet records these genes on 11 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

MONDO 0019793UMLS C5680260

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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