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Journal of Neurology

, Volume 250, Issue 2, pp 207-211

Cognitive deficits in spinocerebellar ataxia type 1, 2, and 3

  • K. BürkAffiliated withDepartment of Neurology, University of Tübingen, Hoppe-Seyler-Str. 3, 72076 Tübingen, Germany. buerk@uni-tuebingen.de
  • , C. GlobasAffiliated withDepartment of Neurology, University of Tübingen, Hoppe-Seyler-Str. 3, 72076 Tübingen, Germany. buerk@uni-tuebingen.de
  • , S. BöschAffiliated withDepartment of Neurology, University of Tübingen, Hoppe-Seyler-Str. 3, 72076 Tübingen, Germany. buerk@uni-tuebingen.de
  • , T. KlockgetherAffiliated withDepartment of Neurology, University of Bonn, Bonn, Germany
  • , C. ZühlkeAffiliated withInstitute of Human Genetics, University of Lübeck, Lübeck, Germany
  • , I. DaumAffiliated withDepartment of Clinical Neuropsychology, University of Bochum, Bochum, Germany
  • , J. DichgansAffiliated withDepartment of Neurology, University of Tübingen, Hoppe-Seyler-Str. 3, 72076 Tübingen, Germany. buerk@uni-tuebingen.de

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Abstract.

Cognitive impairment was studied in distinct types of spinocerebellar ataxia (SCA): eleven SCA1, 14 SCA2, and 11 SCA3 individuals and 8 age- and IQ- matched controls. All were submitted to a neuropsychological test battery that comprised tests for IQ, attention, executive function, verbal and visuospatial memory. Executive dysfunction was prominent in SCA1 as compared with controls and all other SCA types. Mild deficits of verbal memory were present in SCA1, SCA2 and SCA3. The neuropathological pattern in different SCA types suggests that these cognitive deficits are not likely to be contingent upon cerebellar degeneration but to result from disruption of a cerebrocerebellar circuitry presumably at the pontine level.

Key words spinocerebellar ataxia cerebellum cognition executive dysfunction