title: Executive functions in premanifest Huntington's disease.
abstract: BACKGROUND
  We investigated the viability of psychometrically robust executive function measures as markers for premanifest Huntington's disease (HD).
  
  
  METHODS
  Fifteen premanifest HD subjects and 42 controls were compared on the NIH EXAMINER executive function battery. This battery yields an overall executive composite score, plus working memory, cognitive control, and fluency scores that are measured on psychometrically matched scales. The scores were correlated with two disease markers, disease burden and striatal volumes, in the premanifest HD subjects.
  
  
  RESULTS
  The premanifest HD subjects scored significantly lower on the working memory score. The executive composite positively correlated with striatal volumes, and the working memory score negatively correlated with disease burden. The cognitive control and fluency scores did not differ between the groups or correlate significantly with the disease markers.
  
  
  CONCLUSIONS
  The NIH EXAMINER executive composite and working memory scores are sensitive markers of cognitive dysfunction, striatal volume, and disease burden in premanifest HD.
authors.affiliation: Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, California, USA.
authors.forename: S Christine
authors.initials: SC
authors.lastname: You
authors.forename: Michael D
authors.initials: MD
authors.lastname: Geschwind
authors.forename: Sharon J
authors.initials: SJ
authors.lastname: Sha
authors.forename: Alexandra
authors.initials: A
authors.lastname: Apple
authors.forename: Gabriella
authors.initials: G
authors.lastname: Satris
authors.forename: Kristie A
authors.initials: KA
authors.lastname: Wood
authors.forename: Erica T
authors.initials: ET
authors.lastname: Johnson
authors.forename: Jonathan
authors.initials: J
authors.lastname: Gooblar
authors.forename: Jeanne S
authors.initials: JS
authors.lastname: Feuerstein
authors.forename: Steven
authors.initials: S
authors.lastname: Finkbeiner
authors.forename: Gail A
authors.initials: GA
authors.lastname: Kang
authors.forename: Bruce L
authors.initials: BL
authors.lastname: Miller
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Hess
authors.forename: Joel H
authors.initials: JH
authors.lastname: Kramer
authors.forename: Katherine L
authors.initials: KL
authors.lastname: Possin
mesh_terms: D000328:Adult; D000368:Aged; D003072:Cognition Disorders / Q000503:physiopathology* / Q000523:psychology; D056344:Executive Function / Q000502:physiology*; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000503:physiopathology* / Q000523:psychology; D008297:Male; D008570:Memory, Short-Term / Q000502:physiology*; D008875:Middle Aged; D009483:Neuropsychological Tests
keywords: Huntington's disease; disease burden; executive function; striatal volume; working memory