The Contribution of the Cerebellum to Cognition in Spinocerebellar Ataxia Type 6
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 neuropsychologic...
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Series: | Behavioural Neurology |
Online Access: | http://dx.doi.org/10.3233/BEN-2010-0265 |
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doaj-7eb6f70c59ca41afb5ba4e17bd8b02122021-07-02T06:08:52ZengHindawi LimitedBehavioural Neurology0953-41801875-85842010-01-01231-231510.3233/BEN-2010-0265The Contribution of the Cerebellum to Cognition in Spinocerebellar Ataxia Type 6Freya E. Cooper0Manon Grube1Kelly J. Elsegood2John L. Welch3Thomas P. Kelly4Patrick F. Chinnery5Timothy D. Griffiths6Institute of Neuroscience, Newcastle University, Newcastle, UKInstitute of Neuroscience, Newcastle University, Newcastle, UKTrafford Child & Adolescent Mental Health Service, Manchester, UKDepartment of Neuropsychology, Newcastle University, Newcastle, UKDepartment of Neuropsychology, Newcastle University, Newcastle, UKDepartment of Neurology, Newcastle University, Newcastle, UKInstitute of Neuroscience, Newcastle University, Newcastle, UKThis 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.http://dx.doi.org/10.3233/BEN-2010-0265 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Freya E. Cooper Manon Grube Kelly J. Elsegood John L. Welch Thomas P. Kelly Patrick F. Chinnery Timothy D. Griffiths |
spellingShingle |
Freya E. Cooper Manon Grube Kelly J. Elsegood John L. Welch Thomas P. Kelly Patrick F. Chinnery Timothy D. Griffiths The Contribution of the Cerebellum to Cognition in Spinocerebellar Ataxia Type 6 Behavioural Neurology |
author_facet |
Freya E. Cooper Manon Grube Kelly J. Elsegood John L. Welch Thomas P. Kelly Patrick F. Chinnery Timothy D. Griffiths |
author_sort |
Freya E. Cooper |
title |
The Contribution of the Cerebellum to Cognition in Spinocerebellar Ataxia Type 6 |
title_short |
The Contribution of the Cerebellum to Cognition in Spinocerebellar Ataxia Type 6 |
title_full |
The Contribution of the Cerebellum to Cognition in Spinocerebellar Ataxia Type 6 |
title_fullStr |
The Contribution of the Cerebellum to Cognition in Spinocerebellar Ataxia Type 6 |
title_full_unstemmed |
The Contribution of the Cerebellum to Cognition in Spinocerebellar Ataxia Type 6 |
title_sort |
contribution of the cerebellum to cognition in spinocerebellar ataxia type 6 |
publisher |
Hindawi Limited |
series |
Behavioural Neurology |
issn |
0953-4180 1875-8584 |
publishDate |
2010-01-01 |
description |
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. |
url |
http://dx.doi.org/10.3233/BEN-2010-0265 |
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