Logo image
Back
On Chorea – quantitative and objective analysis – cross-sectional results of the TRACK-HD Study
Conference proceeding

On Chorea – quantitative and objective analysis – cross-sectional results of the TRACK-HD Study

S van den Bogaard, N Bechtel, A Sturrock, C Jauffret, M Say, J A Mills, T P Archarya, D R Langbehn, H Johnson, B Borowsky, …
Aktuelle Neurologie
Abstracts Freier Vorträge und Poster der Jahrestagung der DGN 2009, 2009 (Nürnberg)
10/07/2009
DOI: 10.1055/s-0029-1238519

View Online

Abstract

Background: Chorea is one of the most characteristic motor symptoms of Huntington's disease. One of the first descriptions dates back to the 19th century and was reported by George Huntington in his article “On chorea“. Chorea interferes with motor activities and has a significant impact on quality of life. Objective: To investigate if chorea can be assessed objectively and quantitatively, and if quantitative assessment can be related to disease stages as well as chorea subscores on the Unified Huntington's Disease Rating Scale Total Motor Score (UHDRS-TMS). Subjects and Methods: 123 controls, 120 pre-manifest gene carriers (UHDRS-TMS≤5) and 123 HD patients were assessed at 4 study sites (Leiden, London, Paris, Vancouver). Participants were instructed to grasp a device equipped with a 3D position and orientation sensor (Fastrack, Polhemus), lift it and hold it still at an indicated height for 20s. Right and left hands were assessed separately with light and heavy weight conditions. Involuntary movements and their velocity were evaluated in a blinded analysis for all conditions using standard deviations of three dimensional vectors and their derivatives. Results: All variables distinguished controls and pre-manifest subjects from symptomatic patients (p<0.0001). Highest activities were found in the late and early manifest sub-groups, respectively. A sub-group of pre-manifest subjects far from disease onset did not show alterations compared to controls, but the sub-group close to disease onset exhibited hyperkinetic activity and could also be distinguished from early symptomatic patients quantitatively. Our observations suggest that hyperkinetic activity increases with disease stage and duration. Accordingly, correlation analysis for gene expanded participants showed very strong correlations of all variables with the UHDRS-TMS, UHDRS-TMS chorea subscore of the corresponding extremity, estimated years until onset and coefficient of striatal volume to intracranial volume (p<0.0001). Conclusion: The technique presented provides objective and quantitative measures of chorea for use in clinical trials. Detection of sub-clinical changes years before disease onset may expand motor phenotype assessment to pre-symptomatic stages. Longitudinal changes detected by these measures will be assessed in the study TRACK-HD. Acknowledgements: TRACK-HD is supported by the CHDI and High Q Foundation, a not for profit organization dedicated to finding treatments for HD.

Details

Metrics

22 Record Views
Logo image