Cognition in hereditary ataxia

Apart from motor control the cerebellum has been implicated in higher cortical functions such as memory, fronto-executive functions, visuoconstructive skills and emotion. Clinical descriptions of hereditary ataxias mention cognitive impairment to a variable extent. Systematic neuropsychological studies are limited. Regarding the neuropathological pattern in different SCA types, cognitive deficits in hereditary ataxias are not likely to be contingent upon cerebellar degeneration but to result from disruption of cerebrocerebellar circuitries at various levels in the CNS.