spinocerebellar ataxia type 10
Findings
No curated finding names spinocerebellar ataxia type 10 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Spinocerebellar ataxia type 10 (SCA10) is a subtype of type I autosomal dominant cerebellar ataxia (ADCA type I). It is characterized by slowly progressive cerebellar syndrome and epilepsy, sometimes mild pyramidal signs, peripheral neuropathy and neuropsychological disturbances.
Definition from the Mondo Disease Ontology (MONDO:0011330), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
- Onset and course
- Middle age onset · Young adult onset
HPO, annotations 2026-09-02
Features
35 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- DysarthriaHPOHP:0001260
- 2 of 2 reported patients
- Very frequent (80% to 99% of cases)
- Gait ataxiaHPOHP:0002066
- 2 of 2 reported patients
- Very frequent (80% to 99% of cases)
- Limb ataxiaHPOHP:0002070
- 2 of 2 reported patients
- PolyneuropathyHPOHP:0001271
- 2 of 2 reported patients
- Progressive cerebellar ataxiaHPOHP:0002073
- Very frequent (80% to 99% of cases)
- Cerebellar atrophyHPOHP:0001272
- 1 of 2 reported patients
- Frequent (30% to 79% of cases)
Show the remaining 23
- Generalized-onset seizureHPOHP:0002197
- Frequent (30% to 79% of cases)
- Impaired smooth pursuitHPOHP:0007772
- 1 of 2 reported patients
- Frequent (30% to 79% of cases)
- Intention tremorHPOHP:0002080
- Frequent (30% to 79% of cases)
- Kinetic tremorHPOHP:0030186
- Frequent (30% to 79% of cases)
- NystagmusHPOHP:0000639
- Frequent (30% to 79% of cases)
- Scanning speechHPOHP:0002168
- Frequent (30% to 79% of cases)
Genes
1 gene
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- ATXN10HGNC:10549
- Strong · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2020
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
Other names
1 name
Resolves to: spinocerebellar ataxia type 10
- Also called
- SCA10