Background: Motor phenotypes in Huntington's disease vary manifold. Phenotype classification is essential to adapt treatment. The aim of this study was to classify a dystonic subtype closer.
Methods: A total of 7,512 manifest ENROLL-HD participants were subdivided into mainly choreatic (N = 606), dystonic (N = 402), and hypokinetic-rigid (N = 369) subjects. Cognitive (verbal fluency, symbol digit, stroop color, trail making, Mini-Mental State Examination), functional (total functional capacity, Independence Scale), and psychiatric (problem behaviors assessment, Hospital Anxiety and Depression Scale) performance was evaluated at baseline visit.
Results: Symptoms onset for dystonic were similar to hypokinetic-rigid, but earlier compared to choreatic subjects (p < .001). Cognition was better in both groups compared to hypokinetic rigid (all p < .001). Functionality differed between all groups (all p < .001). Differences remained (all p < .001) after controlling for CAP score, CAG, age, disease duration, and education.
Conclusions: Motor subtypes differ in functional and cognitive capacities but less in psychiatric. We identified better cognitive and functional capacities and similar onsets in predominant dystonic compared to hypokinetic-rigid patients.
Keywords: Huntington's disease; dystonia; movement disorders.
© 2020 The Authors. Brain and Behavior published by Wiley Periodicals LLC.