title: Cognitive impairments in Machado-Joseph disease.
abstract: BACKGROUND
  Cognitive function of Machado-Joseph disease (MJD) patients has not been clarified.
  
  
  OBJECTIVE
  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.
  
  
  METHODS
  Case-control study.
  
  
  METHODS
  Research-oriented hospitals.
  
  
  METHODS
  Sixteen genetically confirmed MJD patients able to complete neuropsychological tests and 20 control subjects matched to patients by age and education.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  Machado-Joseph disease patients have verbal and visual memory deficits, visuospatial and constructional dysfunction, and verbal fluency deficits, all unrelated to CAG repeat length.
authors.affiliation: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Yoshinari
authors.initials: Y
authors.lastname: Kawai
authors.forename: Akinori
authors.initials: A
authors.lastname: Takeda
authors.forename: Yuji
authors.initials: Y
authors.lastname: Abe
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Washimi
authors.forename: Fumiaki
authors.initials: F
authors.lastname: Tanaka
authors.forename: Gen
authors.initials: G
authors.lastname: Sobue
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D016022:Case-Control Studies; D003072:Cognition Disorders / Q000209:etiology* / Q000473:pathology; D004522:Educational Status; D005260:Female; D006801:Humans; D017827:Machado-Joseph Disease / Q000150:complications* / Q000235:genetics / Q000523:psychology*; D008297:Male; D008569:Memory Disorders / Q000209:etiology; D008875:Middle Aged; D009483:Neuropsychological Tests; D010468:Perceptual Disorders / Q000209:etiology; D019680:Trinucleotide Repeat Expansion