Journal of Neurology

, Volume 250, Issue 12, pp 1482–1487

The cerebellum and cognition

Intellectual function in spinocerebellar ataxia type 6 (SCA6)
  • C. Globas
  • S. Bösch
  • Ch. Zühlke
  • I. Daum
  • J. Dichgans
  • K. Bürk
ORIGINAL COMMUNICATION

DOI: 10.1007/s00415-003-0258-2

Cite this article as:
Globas, C., Bösch, S., Zühlke, C. et al. J Neurol (2003) 250: 1482. doi:10.1007/s00415-003-0258-2

Abstract.

The aim of this study was to assess cognitive function in patients with spinocerebellar ataxia type 6 (SCA6), an autosomal-dominantly inherited disease leading to a progressive cerebellar syndrome. In contrast to other SCA types, the pathological changes are mostly restricted to the cerebellum. Cognitive function was studied in 12 patients with genetically confirmed SCA6 (mean duration of disease: 9.2 ± 11.6 years) and 12 age- and IQ-matched controls using a test battery comprising tests for IQ, attention, verbal and visuospatial memory, as well as executive function. While none of the SCA6 subjects had features of general intellectual impairment, only mild deficits in single subtests especially in fronto-executive tasks were observed, but without reaching statistical significance. Thus the current findings do not demonstrate severe cognitive dysfunction in SCA6.

Key words

Spinocerebellar ataxiaautosomal-dominant cerebellar ataxiacerebellumcognitionSCA6

Copyright information

© Steinkopff Verlag 2003

Authors and Affiliations

  • C. Globas
    • 1
  • S. Bösch
    • 2
  • Ch. Zühlke
    • 3
  • I. Daum
    • 4
  • J. Dichgans
    • 1
  • K. Bürk
    • 1
  1. 1.Department of NeurologyUniversity of TübingenTübingenGermany
  2. 2.Department of NeurologyUniversity of InnsbruckAustria
  3. 3.Department of Human GeneticsUniversity of LübeckGermany
  4. 4.Department of Clinical NeuropsychologyUniversity of BochumGermany