Executive dysfunction in spinocerebellar ataxia type 1

Fourteen patients with spinocerebellar ataxia 1 (SCA1) and 11 controls with similar mean age and IQ estimates were submitted to a neuropsychological t est battery comprising tests for IQ, attention, verbal and visuospatial mem ory as well as executive functions. Neuropsychological assessment yielded v erbal memory and executive dysfunction while tests of visuospatial memory a nd attention were not significantly impaired in SCA1 as compared to control s. Test performance was neither related to the repeat length, the age of on set nor the disease duration. The profile of cognitive impairment in SCA1 w ith prominent executive dysfunction corresponds to the concept of 'frontal- subcortical' dementia that is likely to be contingent upon disruption of a cerebrocerebellar circuitry that consists of afferent and efferent connecti ons between the prefrontal cortex and the cerebellum. Copyright (C) 2001 S. Karger AG, Basel.