Cognitive findings in spinocerebellar ataxia type 2: relationship to genetic and clinical variables.

Full Abstract

Several authors have recently reported a broad cognitive impairment in autosomal dominant cerebellar ataxias (ADCAs) patients. However, only a few studies on neuropsychological features in spinocerebellar ataxia type 2 (SCA2) patients are present in the current literature. The aim of this study is to evaluate the cognitive impairment in a wide sample of SCA2 patients and to verify the role of different disease-related factors (age of onset, disease duration, and clinical severity) on intellectual abilities. We administered a battery of neuropsychological tests assessing handedness, attention, short- and long-term verbal and visuo-spatial memory, executive functions, constructive abilities, general intellectual abilities and depression to 18 SCA2 patients belonging to eight families who came to our observation. Evidence of impaired verbal memory, executive functions and attention was found. The cognitive status was partially related to clinical severity rather than to disease duration or age at onset of symptoms. We partially confirmed data on cognitive defects already reported by others but we also found defective attention skills as well as significant lower performances in a nonverbal intelligence task.

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Author information

Author/s: Le Pira, Francesco (F); Zappalà, Giuseppe (G); Saponara, Riccardo (R); Domina, Elisabetta (E); Restivo, Domenico (D); Reggio, Ester (E); Nicoletti, Alessandra (A); Giuffrida, Salvatore (S);

Affiliation: Department of Neurosciences, University of Catania, Via S. Sofia 78, I-95125, Catania, Italy. lepira(-atsign-)unict.it

Journal and publication information

Publication Type: Journal Article

Journal: Journal of the neurological sciences (J Neurol Sci), published in Netherlands. (Language: eng)

Reference: 2002-Sep; vol 201 (issue 1-2) : pp 53-7

Dates: Created 2002/08/06; Completed 2002/10/16; Revised 2004/11/17;

PMID: 12163194, status: MEDLINE (last retrieval date: 12/26/2008)

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MeSH headings (categories)

This article was linked to the MESH Headings shown below.

Adult
Aged
Cognition Disorders - diagnosis; etiology; genetics
Educational Status
Family Health
Female
Humans

Humans
Intelligence Tests
Male
Memory
Middle Aged
Neuropsychological Tests
Spinocerebellar Ataxias - complications; diagnosis; genetics

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