A06 Better global and cognitive functioning for choreatic compared to hypokinetic-rigid huntington's disease

Background Motor disturbances in Huntington9s disease can be subdivided into a predominantly choreatic or hypokinetic-rigid subtype. The relation between these motor subtypes and cognitive and general functioning is poorly understood, but can be of importance in the care for Huntington9s disease patients. Aims Investigating the possible clinical differences between predominantly choreatic and predominantly hypokinetic-rigid Huntington9s disease. Methods/technique Cross-sectional data from the European Huntington9s Disease Network Registry study up until 2011 were analysed. Data of the first visit of all subjects with an expanded CAG ≥36 and a total motor score ≥5 and complete cognitive, function and motor assessments were retrieved. A total of 1882 subjects were classified as predominantly choreatic (N=528) or predominantly hypokinetic-rigid (N=432), according to their score on those items of the total motor score a priori labelled as either choreatic or hypokinetic-rigid items; the other 922 patients were of a mixed motor type. The relationship between motor type and cognitive (total cognitive score) and general functioning (total functional capacity) was investigated using a linear regression model. Results/outcome Motor subtype was a significant predictor of both cognitive and general functioning (p Conclusions We conclude that Huntington9s disease patients with a predominant choreatic motor phenotype exhibit better global and cognitive functioning than patients with a predominant hypokinetic-rigid motor phenotype.