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

• Published in: Journal of neurology Vol. 250; no. 2; pp. 207 - 211
• Main Authors: BÜRK, K, GLOBAS, C, BÖSCH, S, KLOCKGETHER, T, ZÜHLKE, C, DAUM, I, DICHGANS, J
• Format: Journal Article
• Language: English
• Published: Berlin Springer 01.02.2003; Springer Nature B.V
• Subjects: Age of Onset; Aging - physiology; Biological and medical sciences; Cognition Disorders - etiology; Cognition Disorders - psychology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Disease Progression; Female; Humans; Male; Medical sciences; Memory - physiology; Middle Aged; Neurology; Neuropsychological Tests; Psychomotor Performance - physiology; Space Perception - physiology; Spinocerebellar Ataxias - complications; Spinocerebellar Ataxias - psychology; Human; Nervous system diseases; Cognitive disorder; cognition; Neuropsychological test; Exploration; Psychometrics; cerebellum; Cerebral disorder; Genetic disease; Spinocerebellar heredodegeneration; executive dysfunction; Central nervous system disease; Degenerative disease; Spinal cord disease; spinocerebellar ataxia
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-003-0976-5

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.