Cognitive assessment in patients with spinocerebellar ataxia type 6

Fernanda Mary Machado,J. Pedroso,O. Barsottini,M. F. França Júnior

Published 2024 in Arquivos de Neuro-Psiquiatria

ABSTRACT

Cerebellar cognitive-affective syndrome proposes that patients with cerebellar lesions present not only ataxic symptoms, but also important deficits in cognition, affect, and behavior. Cognitive, behavioral, and affective deficits, found in cerebellar cognitive-affective syndrome, may occur due to a process of diaschesis. Spinocerebellar ataxia type 6 (SCA6) is described as the prototype of a pure cerebellar ataxia. The present study aims to provide data on the contribution of corticocerebellar functional connections to clinical manifestations in a population of subjects with SCA6. It is a qualitative-quantitative, observational and cross-sectional research. 20 subjects (10 with ataxia and 10 controls) will be evaluated. All of those evaluated had an IQ greater than 70. Subjects will be matched by age group and education. This work is ongoing, therefore, 6 individuals from the control group and 6 individuals with a confirmed diagnosis were selected. In terms of intelligence quotient, 5 of the 6 patients evaluated with a diagnosis of type 6 ataxia obtained results below those of the control group. On average, there was a difference of 18.8 points. Regarding selective attention, worse results were obtained in the group of diagnosed, the differences ranged from 1 second to 7.63 seconds, with a mean value of 3.38 seconds. The evaluation of inhibitory control indicated a longer response time in the group of diagnosed, with a mean difference of 105.48 seconds versus 45.69 seconds, respectively. On average, patients diagnosed with ataxia had a difference of 8.16 raw points versus 12.33 in verbal long-term memory. When obtaining the average result, in relation to logical reasoning ability, there were higher results among the control group, in which they obtained an average of 13.3 points versus a result of 11.3 compared to the group of diagnosed patients. Regarding perception, the control group also obtained results higher than those obtained by the diagnosed group, which were 18.83 points versus 14.83 points, respectively. In relation to visuoconstructive ability, there were higher scores in the control group, in which they obtained an average of 45 raw points versus 30.6. In terms of cognitive flexibility, there was a score of 6.22 mean points versus 4 points between the diagnosed group and the control group. Regarding image naming, the control group obtained 56 points and the ataxia group 52.66 points. There was a difference in relation to the pertinent result to the working memory ability of the visual sphere. The control group achieved an average score of 8.66, compared to an average score of 7.33 points in relation to the diagnosed group. The presented data suggest cognitive affective syndrome of the cerebellum in type 6 ataxia. Which leads to the question of whether type 6 ataxia is in fact entirely pure, since it underscores a variety of cognitive changes in different areas.

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    2024

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    Arquivos de Neuro-Psiquiatria

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