The cerebellum and cognition

Author(s): Globas C, Bösch S, Zühlke Ch, Daum I, Dichgans J, et al.

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.

Similar Articles

Molecular epidemiology of spinocerebellar ataxia type 6

Author(s): Craig K, Keers SM, Archibald K, Curtis A, Chinnery PF

Spinocerebellar ataxia type 6

Author(s): Matsumura R, Futamura N, Fujimoto Y, Yanagimoto S, Horikawa H, et al.

Spinocerebellar ataxia types 1, 2, 3, and 6: disease severity and nonataxia symptoms

Author(s): Schmitz-Hübsch T, Coudert M, Bauer P, Giunti P, Globas C, et al.

SCA6 mutation analysis in a large cohort of the Japanese patients with late-onset pure cerebellar ataxia

Author(s): Yabe I, Sasaki H, Matsuura T, Takada A, Wakisaka A, et al.

Early-onset cerebellar atrophy associated with mutation in the CACNA1A gene

Author(s): Naik S, Pohl K, Malik M, Siddiqui A, Josifova D

Molecular features of the CAG repeats of spinocerebellar ataxia 6 (SCA6)

Author(s): Matsuyama Z, Kawakami H, Maruyama H, Izumi Y, Komure O, et al.

Spinocerebellar ataxia type 6

Author(s): Geschwind DH, Perlman S, Figueroa KP, Karrim J, Baloh RW, et al.

A clinical and genetic study in a large cohort of patients with spinocerebellar ataxia type 6

Author(s): Takahashi H, Ishikawa K, Tsutsumi T, Fujigasaki H, Kawata A, et al.

Meiotic instability of the CAG repeats in the SCA6/CACNA1A gene in two Japanese SCA6 families

Author(s): Shimazaki H, Takiyama Y, Sakoe K, Amaike M, Nagaki H, et al.

Pathogenic effect of an intermediate-size SCA-6 allele (CAG)(19) in a homozygous patient

Author(s): Mariotti C, Gellera C, Grisoli M, Mineri R, Castucci A, et al.

Japanese families with autosomal dominant pure cerebellar ataxia map to chromosome 19p13

Author(s): Ishikawa K, Tanaka H, Saito M, Ohkoshi N, Fujita T, et al.