title: Grasping premanifest Huntington's disease - shaping new endpoints for new trials.
abstract: Future clinical trials in subjects with premanifest Huntington's disease (preHD) may depend on the availability of biomarkers. It was previously shown in symptomatic HD that, the grip force variability coefficient-of-variation (GFV-C) in a grasping paradigm was correlated to the Unified-Huntington's-Disease-Rating-Scale-Total-Motor-Score (UHDRS-TMS) and increased in a 3 year follow-up study. To further elucidate its potential as a biomarker, we investigated whether GFV-C is able to detect a motor phenotype in preHD and is correlated to the genotype assessed by a disease-burden-score. The ability of preHD (n = 15) and symptomatic HD subjects (n = 20) to maintain stable grip forces, while holding an object (250 g and 500 g), was measured and compared with the controls (n = 19). GFV-C was increased in preHD at 500 g, in symptomatic subjects at both weights and was correlated to the disease-burden-score and UHDRS-TMS. GFV-C may be a useful objective and quantitative marker of motor dysfunction across genetically diagnosed premanifest and symptomatic HD subjects.
authors.affiliation: Department of Neurology, University Clinic Muenster (UKM), Westfaelische Wilhelms University of Muenster, Muenster, Germany. r.reilmann@uni-muenster.de
authors.forename: Ralf
authors.initials: R
authors.lastname: Reilmann
authors.forename: Stefan
authors.initials: S
authors.lastname: Bohlen
authors.forename: Thomas
authors.initials: T
authors.lastname: Klopstock
authors.forename: Andreas
authors.initials: A
authors.lastname: Bender
authors.forename: Adolf
authors.initials: A
authors.lastname: Weindl
authors.forename: Philipp
authors.initials: P
authors.lastname: Saemann
authors.forename: Dorothee P
authors.initials: DP
authors.lastname: Auer
authors.forename: Erich B
authors.initials: EB
authors.lastname: Ringelstein
authors.forename: Herwig W
authors.initials: HW
authors.lastname: Lange
mesh_terms: D000328:Adult; D000704:Analysis of Variance; D002986:Clinical Trials as Topic; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D018737:Hand Strength / Q000502:physiology*; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged