title: Cognitive changes in spinocerebellar ataxia type 2.
abstract: OBJECTIVE
  Cognitive disorders and dementia occur in 19 to 42% of patients with spinocerebellar ataxia type 2 (SCA2). Neuropsychological tests can reveal executive dysfunction, impaired visual and verbal memory, tongue and speech impairment, attention disorders and impaired verbal fluency.
  
  
  METHODS
  We performed psychiatric and neuropsychological examinations in 12 patients diagnosed with genetically confirmed SCA2 and 12 healthy volunteers matching the patients in age, gender, and length of education. The level of motor impairment was determined using the brief ataxia rating scale (BARS). The neuropsychological examination focused on testing executive functions, short-term visual and verbal memory, attention, psychomotor tempo, visual motor coordination, learning ability and comprehension ability. The tests were divided into two subgroups according to the difficulty of motor tasks. The cognitive abilities composite score (CACS) was determined by calculating the arithmetic mean of T scores of the respective tests.
  
  
  RESULTS
  Patients with SCA2 had significantly lower CACSs (p=0.00005) compared to the healthy volunteers. Patients exhibited impaired performance in both difficult and simple motor tests. The severity of cognitive impairment was related to the age at the onset of the disease (p=0.002) but not to the duration or to the overall BARS score.
  
  
  CONCLUSIONS
  Compared to healthy volunteers, patients with SCA2 exhibited significantly worse cognitive performance in all areas tested, including the tests of simple motor tasks. Moreover, the cognitive performance of patients worsened as the difficulty of the motor tasks increased.
authors.affiliation: Department of Neurology, Charles University, Faculty of Medicine and University Hospital in Hradec Králové, Czech Republic. valismar@seznam.cz
authors.forename: Martin
authors.initials: M
authors.lastname: Valis
authors.forename: Jirí
authors.initials: J
authors.lastname: Masopust
authors.forename: Jan
authors.initials: J
authors.lastname: Bažant
authors.forename: Zuzana
authors.initials: Z
authors.lastname: Ríhová
authors.forename: Dita
authors.initials: D
authors.lastname: Kalnická
authors.forename: Aleš
authors.initials: A
authors.lastname: Urban
authors.forename: Alena
authors.initials: A
authors.lastname: Zumrová
authors.forename: Jakub
authors.initials: J
authors.lastname: Hort
mesh_terms: D000328:Adult; D017668:Age of Onset; D001288:Attention / Q000502:physiology; D003071:Cognition / Q000502:physiology*; D003072:Cognition Disorders / Q000209:etiology / Q000523:psychology*; D056344:Executive Function; D005260:Female; D006801:Humans; D007858:Learning / Q000502:physiology; D008297:Male; D008568:Memory / Q000502:physiology; D008875:Middle Aged; D009483:Neuropsychological Tests; D011597:Psychomotor Performance / Q000502:physiology; D020754:Spinocerebellar Ataxias / Q000150:complications / Q000235:genetics / Q000523:psychology*; D014796:Visual Perception / Q000502:physiology