cqvip:Background: Cognitive function of Machado Joseph disease (MJD) patients has n ot been clarified. Objectives: To determine the characteristics of cognitive dys function in MJD patients and to assess the relationship of dysfunction to age at onset, age at examination, disease duration, education, ataxia, depression, anx iety, and CAG repeat length. Design: Case con trol study. Setting: Research o riented hospitals. Participants: Sixteen genetically confirmed MJD patients able to complete neuropsychological tests and 20 control subjects matched to patient s by age and education. Main Outcome Measures: Neuropsychological tests, includi ng general cognition, verbal and visual memory, working memory, visuospatial and constructional ability, language, executive function, depression, and anxiety. Results: Machado Joseph disease patients scored sig nificantly lower than cont rols in verbal and visual memory, in visuospatial and constructional tasks, and in phonemic and semantic fluency tasks. None of these impairments correlated wit h CAG repeat length, age at onset, age at examination, disease duration, or educ ation. Verbal fluency (words named in a category) correlated with the Internatio nal Cooperative Ataxia Rating Scale score. Conclusion: Machado Joseph disease p atients have verbal and visual memory deficits, visuospatial and constructional dysfunction, and verbal fluency deficits, all unrelated to CAG repeat length.
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