title: Progressive cognitive dysfunction in spinocerebellar ataxia type 3.
abstract: BACKGROUND
  Although it is well established that there is cognitive dysfunction in spinocerebellar ataxia type 3 (SCA3), it is unknown whether cognition deteriorates with disease progression. We therefore prospectively studied cognitive function in patients with SCA3.
  
  
  METHODS
  Eleven patients with SCA3 were assessed using an extensive neuropsychological test battery and retested after 3.5 ± 0.4 years.
  
  
  RESULTS
  In addition to ataxia and motor control, verbal learning and verbal and figural memory deteriorated significantly during the follow-up period. An increase in depressive symptoms was not observed.
  
  
  CONCLUSIONS
  The observation that memory and learning abilities deteriorated with disease progression suggests that cognitive dysfunction is an integral part of SCA3. Because the applied tests for memory function did not require motor responses, cognitive decline cannot be attributed to progressive cerebellar ataxia. The deterioration of verbal and figural memory can be explained either by extracerebellar pathology or by disruption of cerebellar-cerebral circuitries.
authors.affiliation: German Center for Neurodegenerative Diseases, Bonn, Germany; Department of Neurology, University Hospital of Bonn, Bonn, Germany.
authors.forename: Sandra
authors.initials: S
authors.lastname: Roeske
authors.forename: Ina
authors.initials: I
authors.lastname: Filla
authors.forename: Stefan
authors.initials: S
authors.lastname: Heim
authors.forename: Katrin
authors.initials: K
authors.lastname: Amunts
authors.forename: Christoph
authors.initials: C
authors.lastname: Helmstaedter
authors.forename: Ullrich
authors.initials: U
authors.lastname: Wüllner
authors.forename: Michael
authors.initials: M
authors.lastname: Wagner
authors.forename: Thomas
authors.initials: T
authors.lastname: Klockgether
authors.forename: Martina
authors.initials: M
authors.lastname: Minnerop
mesh_terms: D000328:Adult; D000704:Analysis of Variance; D003072:Cognition Disorders / Q000209:etiology / Q000523:psychology*; D003863:Depression / Q000209:etiology / Q000523:psychology; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017827:Machado-Joseph Disease / Q000150:complications / Q000235:genetics / Q000523:psychology*; D008297:Male; D008568:Memory / Q000502:physiology; D008875:Middle Aged; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D011446:Prospective Studies; D011569:Psychiatric Status Rating Scales; D011930:Reaction Time / Q000502:physiology; D014705:Verbal Behavior / Q000502:physiology
keywords: SCA3; ataxia; cerebellum; cognition; follow-up