Journal of Neurology

, Volume 250, Issue 2, pp 207–211

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

  • K. Bürk
  • C. Globas
  • S. Bösch
  • T. Klockgether
  • C. Zühlke
  • I. Daum
  • J. Dichgans
ORIGINAL COMMUNICATION

DOI: 10.1007/s00415-003-0976-5

Cite this article as:
Bürk, K., Globas, C., Bösch, S. et al. J Neurol (2003) 250: 207. doi:10.1007/s00415-003-0976-5

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 ataxiacerebellumcognitionexecutive dysfunction

Copyright information

© Steinkopff Verlag 2003

Authors and Affiliations

  • K. Bürk
    • 1
  • C. Globas
    • 1
  • S. Bösch
    • 1
  • T. Klockgether
    • 2
  • C. Zühlke
    • 3
  • I. Daum
    • 4
  • J. Dichgans
    • 1
  1. 1.Department of Neurology, University of Tübingen, Hoppe-Seyler-Str. 3, 72076 Tübingen, Germany. buerk@uni-tuebingen.deDE
  2. 2.Department of Neurology, University of Bonn, Bonn, GermanyDE
  3. 3.Institute of Human Genetics, University of Lübeck, Lübeck, GermanyDE
  4. 4.Department of Clinical Neuropsychology, University of Bochum, Bochum, GermanyDE