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Start free with EleplanSpinocerebellar ataxia type 2
ORPHA:98756Disease
Also called SCA2
What it is
Spinocerebellar ataxia type 2 (SCA2) is a subtype of type I autosomal dominant cerebellar ataxia (ADCA type I) characterized by truncal ataxia, dysarthria, slowed saccades and less commonly ophthalmoparesis and chorea.
Key facts
- Prevalence
- 1-9 / 100 000
- Age of onset
- All ages
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
19- Abnormal cell morphology
- Abnormality of the spinocerebellar tracts
- Cerebellar Purkinje layer atrophy
- Chorea
- Dementia
- Dysarthria
- Dystonia
- Fasciculations
- Gait ataxia
- Generalized hypotonia
- Hyporeflexia
- Kinetic tremor
- Muscle spasm
- Nystagmus
- Olivopontocerebellar hypoplasia
- Postural tremor
- Slow saccadic eye movements
- Spinal cord posterior columns myelin loss
- Supranuclear ophthalmoplegia
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
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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