Cognitive impairment in spinocerebellar ataxia type 8.

OBJECTIVES Only a limited number of studies have investigated the cognitive performances of spinocerebellar ataxia (SCA) patients. In none of the SCA8 studies have the neuropsychological test performances been the primary measures. The objective of the current study was to investigate the characteristics of cognitive deficits in SCA8. METHODS Ten SCA8 patients and ten case-by-case matched control subjects underwent a comprehensive neuropsychological examination evaluating attention and information processing, concept formation, reasoning and executive functions, verbal production, memory and learning and visuoperceptual and -constructive functions. RESULTS SCA8 patients demonstrated deficits primarily in attention and information processing, as well as in concept formation, reasoning, executive functions and verbal production. Visuoperceptual and -constructive functions, as well as most of the performances of memory were unaffected. CONCLUSIONS Cognitive impairments, especially those related to attention, information processing and executive functions, seem to be a clinical feature of SCA8 disease.