Affiliations: Institute of Neuroscience, Newcastle University,
Newcastle, UK | Trafford Child & Adolescent Mental Health Service, Manchester, UK | Department of Neuropsychology, Newcastle University, Newcastle, UK | Department of Neurology, Newcastle University, Newcastle, UK
Note: [] Corresponding author: Freya E. Cooper, Institute of
Neuroscience, Newcastle University Medical School, Framlington Place, Newcastle
Upon Tyne, NE2 4HH, UK. Tel.: +44 0191 222 3445; E-mail: f.e.cooper@ncl.ac.uk
Abstract: This study sought evidence for a specific cerebellar contribution to
cognition by characterising the cognitive phenotype of Spinocerebellar Ataxia
Type 6 (SCA-6); an autosomal dominant genetic disease which causes a highly
specific late-onset cerebellar degeneration. A comprehensive neuropsychological
assessment was administered to 27 patients with genetically confirmed SCA-6.
General intellectual ability, memory and executive function were examined using
internationally standardised tests (Wechsler Adult Intelligence Scale-III,
Wechsler Memory Scale-III, Delis and Kaplan Executive Function System, Brixton
Spatial Anticipation test). The patient group showed no evidence of
intellectual or memory decline. However, tests of executive function involving
skills of cognitive flexibility, inhibition of response and verbal reasoning
and abstraction demonstrated significant impairment at the group level with
large effect sizes. The results demonstrate an executive deficit due to SCA-6
that can be conceptualised as parallel to the motor difficulties suffered by
these patients: the data support a role for the cerebellum in the regulation
and coordination of cognitive, as well as motor processes that is relevant to individual performance.
