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Human Neurochemical Pathology Laboratory (Dr. Kish) and Neuroimaging Section (Dr. Nobrega), Clarke Institute of Psychiatry, and the Rot-man Research Institute of Baycrest Centre (Drs. Kish, Stuss, and Freedman), Toronto, ON, Canada; Department of Neurology (Dr. El-Awar), University of Pittsburgh, PA; Department of Neurology (Dr. Currier), University Medical Centre, Jackson, MS; Nebraska Methodist Hospital (Dr. Aita), Omaha, NE; Department of Neurology (Dr. Schut), University of Minnesota, Minneapolis, MN; Department of Pediatrics (Dr. Zoghbi), Howard Hughes Medical Institute, Baylor College of Medicine, Houston, TX; and the Behavioural Neurology Program (Dr. Freedman), Baycrest Centre for Geriatric Care, Toronto, ON, Canada.
To determine whether the cognitive status of patients with dominantly inherited spinocerebellar ataxia (DSCA) might be related to neurologic severity, we administered a comprehensive neuropsychological test battery to 43 patients with DSCA, ranging in ataxia severity from mild to end-stage. As compared with the controls, the mildly ataxic patients scored normally or close to normal as a group on all of the neuropsychological tests. In contrast, approximately one-half of the moderately and all of the severely ataxic patients showed poor performance, independent of age, Hamilton Rating Scale for Depression score, or education, on the Wisconsin Card Sorting Test, suggesting impaired executive system function. In addition, a subgroup of these patients had a neuropsychological profile suggestive of mild generalized cognitive impairment. We conclude that DSCA is not a homogeneous group of disorders with respect to cognitive status and that the neurologic severity of the disorder is a major factor. Impaired executive system function could be explained by damage to olivopontocerebellar system control over cerebral cortical function or to damage to other neuronal systems (especially cholinergic) that degenerate in parallel with the olivopontocerebellar system.
Address correspondence and reprint requests to Dr. Stephen J. Kish, Human Neurochemical Pathology Laboratory, Clarke Institute of Psychiatry, Toronto, ON, Canada, M5T 1R8.
Supported by US National Institutes of Health grant NINDS #26034.
Received November 22, 1993. Accepted in final form March 1, 1994.
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