title: Better global and cognitive functioning in choreatic versus hypokinetic-rigid Huntington's disease.
abstract: BACKGROUND
  Understanding the relation between predominantly choreatic and hypokinetic-rigid motor subtypes and cognitive and general functioning may contribute to knowledge about different motor phenotypes in Huntington's disease.
  
  
  METHODS
  In the European Huntington's Disease Network Registry study, 1882 subjects were classified as being predominantly choreatic (n=528) or hypokinetic-rigid (n=432), according to their scores on items of the total motor score a priori labeled as choreatic or hypokinetic-rigid; the other 922 patients were of a mixed type. The relationship between motor type and cognitive (verbal fluency, symbol digit modalities, Stroop color, word and interference tests) and functional (total functional capacity) capacity was investigated using multiple linear regression.
  
  
  RESULTS
  Motor subtype contributed significantly to the total functional capacity score (partial r(2) : 7.8%; P<.001) and to the 5 cognitive scores (partial r(2) ranged from 2.0% to 8.4%; all P<.001).
  
  
  CONCLUSIONS
  Patients with a predominantly choreatic motor phenotype performing better in all areas than patients with a hypokinetic-rigid motor phenotype.
authors.affiliation: Leiden University Medical Centre, Department of Neurology, Leiden, The Netherlands. epthart@lumc.nl
authors.forename: Ellen P
authors.initials: EP
authors.lastname: Hart
authors.forename: Johan
authors.initials: J
authors.lastname: Marinus
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Burgunder
authors.forename: Anna Rita
authors.initials: AR
authors.lastname: Bentivoglio
authors.forename: David
authors.initials: D
authors.lastname: Craufurd
authors.forename: Ralf
authors.initials: R
authors.lastname: Reilmann
authors.forename: Carsten
authors.initials: C
authors.lastname: Saft
authors.forename: Raymund A C
authors.initials: RA
authors.lastname: Roos
mesh_terms: D000328:Adult; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology*; D004185:Disability Evaluation; D005060:Europe; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000150:complications*; D018476:Hypokinesia / Q000150:complications*; D008297:Male; D008875:Middle Aged; D009127:Muscle Rigidity / Q000150:complications*; D009483:Neuropsychological Tests; D012720:Severity of Illness Index
keywords: Huntington's disease; chorea; cognition; hyperkinetic; hypokinetic