Cognitive Impairment in Hereditary Ataxias: Literature Review

Hereditary ataxias are a clinically and genetically heterogeneous group of nervous system diseases, mostly progressive and mainly clinically characterized by coordination disorders due to degeneration of the corresponding afferent and efferent neuronal systems. Hereditary ataxias are divided into autosomal dominant, autosomal recessive, and X-linked recessive forms; sporadic forms with unspecified or unknown transmission mechanism are also distinguished. In this article, we review the impact of hereditary ataxias on the cognitive function. We present data from various studies that collectively shed light on the complex relationships between motor and cognitive impairments in patients with hereditary ataxias.