Executive dysfunction in spinocerebellar ataxia type 1

K. Bürk*, S. Bösch, C. Globas, C. Zühlke, I. Daum, T. Klockgether, J. Dichgans

*Corresponding author for this work
47 Citations (Scopus)


Fourteen patients with spinocerebellar ataxia 1 (SCA1) and 11 controls with similar mean age and IQ estimates were submitted to a neuropsychological test battery comprising tests for IQ, attention, verbal and visuospatial memory as well as executive functions. Neuropsychological assessment yielded verbal memory and executive dysfunction while tests of visuospatial memory and attention were not significantly impaired in SCA1 as compared to controls. Test performance was neither related to the repeat length, the age of onset nor the disease duration. The profile of cognitive impairment in SCA1 with prominent executive dysfunction corresponds to the concept of 'frontal-subcortical' dementia that is likely to be contingent upon disruption of a cerebrocerebellar circuitry that consists of afferent and efferent connections between the prefrontal cortex and the cerebellum.

Original languageEnglish
JournalEuropean Neurology
Issue number1
Pages (from-to)43-48
Number of pages6
Publication statusPublished - 2001

Research Areas and Centers

  • Research Area: Medical Genetics


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