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Yoshinari Kawai, MD; Akinori Takeda, MD; Yuji Abe, MD; Yukihiko Washimi, MD; Fumiaki Tanaka, MD; Gen Sobue, MD

Arch Neurol. 2004;61:1757-1760.

Background  Cognitive function of Machado-Joseph disease (MJD) patients has not been clarified.

Objectives  To determine the characteristics of cognitive dysfunction in MJD patients and to assess the relationship of dysfunction to age at onset, age at examination, disease duration, education, ataxia, depression, anxiety, and CAG repeat length.

Design  Case-control study.

Setting  Research-oriented hospitals.

Participants  Sixteen genetically confirmed MJD patients able to complete neuropsychological tests and 20 control subjects matched to patients by age and education.

Main Outcome Measures  Neuropsychological tests, including general cognition, verbal and visual memory, working memory, visuospatial and constructional ability, language, executive function, depression, and anxiety.

Results  Machado-Joseph disease patients scored significantly lower than controls in verbal and visual memory, in visuospatial and constructional tasks, and in phonemic and semantic fluency tasks. None of these impairments correlated with CAG repeat length, age at onset, age at examination, disease duration, or education. Verbal fluency (words named in a category) correlated with the International Cooperative Ataxia Rating Scale score.

Conclusion  Machado-Joseph disease patients have verbal and visual memory deficits, visuospatial and constructional dysfunction, and verbal fluency deficits, all unrelated to CAG repeat length.

Author Affiliations: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya (Drs Kawai, Takeda, Tanaka, and Sobue); and Department of Neurology, Chubu National Hospital, Aichi (Drs Abe and Washimi), Japan.

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