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The Relationships Between Ataxia and Cognition in Spinocerebellar Ataxia Type 2

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Abstract

The clinical spectrum of spinocerebellar ataxia type 2 includes motor manifestations and cognitive disturbances in executive functions, memory, and visuoconstructive skills. The relationships between severity of motor disturbances and altered cognition are poorly known. In this study, we assessed patients with spinocerebellar ataxia type 2 and age- and sex-matched healthy control subjects by a test battery including the Mini-mental State Examination, the Wisconsin Card Sorting test, and the Wechsler Memory Scale-Revised. The correlation between severity of motor ataxia (as assessed by a validated and widely used severity scale, the SARA scale, and by an objective automated computerized system of gait analysis) and altered cognition was then evaluated by Spearman correlation analysis. Patients performed worse than healthy controls in almost all administered neuropsychological tests. Nevertheless, only global intellectual abilities and executive functions significantly correlated with the overall severity of ataxia as assessed by the SARA scale, and impaired executive functions alone correlated with performance on several spatio-temporal gait analysis parameters. Our findings would probably suggest a prominent influence of executive functions on motor abilities in patients with spinocerebellar ataxia type 2 and raise the possibility that cognitive pharmaceutical or rehabilitative interventions may be of benefit in the management of motor problems in these patients.

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Authors and Affiliations

  1. Neurology Unit, “A. Perrino” Hospital, Brindisi (BR), Italy

    Angelo Fabio Gigante

  2. Department of Basic Medical Sciences, Neurosciences and Sense Organs, “Aldo Moro” University of Bari, Bari, Italy

    Giuseppina Lelli, Raffaella Romano, Roberta Pellicciari, Andrea Di Candia, Paola Vincenza Mancino & Pietro Fiore

  3. Department of Mechanical, Chemical and Materials Engineering, University of Cagliari, Cagliari, Italy

    Massimiliano Pau

  4. Department of Medical Sciences and Public Health, Institute of Neurology, University of Cagliari, Cagliari, Italy

    Giovanni Defazio

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Gigante, A.F., Lelli, G., Romano, R. et al. The Relationships Between Ataxia and Cognition in Spinocerebellar Ataxia Type 2. Cerebellum 19, 40–47 (2020). https://doi.org/10.1007/s12311-019-01079-5

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