Brain, Vol. 122, No. 4, 769-777,
April 1999
© 1999 Oxford University Press
Cognitive deficits in spinocerebellar ataxia 2
1 Department of Neurology, University of Tübingen, 2 Department of Clinical Neuropsychology, University of Bochum, Germany, 3 Department of Neurology, University of Innsbruck, Austria and 4 INSERM U 289, Hôpital de la Salpêtrière, Paris, France
Correspondence to:
K. Bürk, MD, Department of Neurology, University of Tübingen, Hoppe-Seyler-Strasse 3, D-72076 Tübingen, Germany E-mail: buerk{at}uni-tuebingen.de
This is one of the first studies assessing the pattern of cognitive impairment in spinocerebellar ataxia 2 (SCA2). Cognitive function was studied in 17 patients with genetically confirmed SCA2 and 15 age- and IQ- matched controls using a neuropsychological test battery comprising tests for IQ, attention, verbal and visuospatial memory, as well as executive functions. Twenty-five percent of the SCA2 subjects showed evidence of dementia. Even in non-demented SCA2 subjects, there was evidence of verbal memory and executive dysfunction. Tests of visuospatial memory and attention were not significantly impaired in the non-demented group compared with controls. There was no relationship between test performance and motor disability, repeat length or age of onset, while disease duration was shown to be inversely correlated with two tests reflecting the progression of cognitive deficits during the course of the disease. Intellectual impairment should therefore not be interpreted as a secondary effect of progressive motor disability, but represents an important and independent part of the SCA2 phenotype.
spinocerebellar ataxia; neuropsychology; autosomal dominant cerebellar ataxia
ADCA = autosomal dominant cerebellar ataxia; MMS = Mini-Mental State TestM; SCA2 = spinocerebellar ataxia 2; WCST = Wisconsin Card Sorting Test; WMS = Wechsler Memory Scale—Revised
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