Cognitive behavior in heredodegenerative ataxias.
Fifteen patients with Friedreich's ataxia (FA) and 15 others with olivopontocerebellar atrophy (OPCA) were evaluated with a comprehensive neuropsychological battery of tests. They were pair-matched with normal controls for age, sex and education. Depressed patients were excluded from the study as were those with extrapyramidal signs. The following results were obtained: (1) the Raven test, untimed block design performance in OPCA and quantitative analysis of Rey figure drawing revealed a visuospatial deficit suggestive of a mild parietal-like syndrome; signs of mild frontal-like syndrome were also found; (2) cognition was slowed in the FA group; (3) simple visual and auditory reaction times were increased in both patient groups. It appears that the cerebellum may interfere indirectly with cognition through various physiological and neurochemical 2-way cerebellocortical loops. Finally, the cerebellum seems to interfere directly also with basic speed of information processing.
Botez-Marquard
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Laboratory of Neurobiology, Hôtel-Dieu de Montréal, Qué., Canada.
Botez
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D000328:Adult; D002531:Cerebellum / Q000503:physiopathology; D003072:Cognition Disorders / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D005621:Friedreich Ataxia / Q000523:psychology*; D007839:Functional Laterality; D006801:Humans; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D009849:Olivopontocerebellar Atrophies / Q000523:psychology*; D011930:Reaction Time