Table 4.

Proportion of Individuals with SCA6 Manifesting Phenotypic Features Compared with Individuals with SCA1, SCA3, and SCA2

Phenotypic FeatureSCA2SCA1SCA3SCA6
Cerebellar dysfunction100%100%100%100%
Reduced saccadic velocity71%-92%50%10%0%-6%
Myoclonus0%-40%0%4%0%
Dystonia or chorea0%-38%20%8%0%-25%
Pyramidal involvement29%-31%70%70%33%-44%
Peripheral neuropathy44%-94%100%80%16%-44%
Intellectual impairment31%-37%20%5%0%

From: Spinocerebellar Ataxia Type 6

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