Functional and cognitive capacity differ in dystonic motor subtypes when compared to choreatic and hypokinetic-rigid motor subtypes in Huntington's disease

  • \(\bf Background\) 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. \(\bf Methods\) A total of 7,512 manifest ENROLL‐HD participants were subdivided into mainly choreatic (\(\it N\) = 606), dystonic (\(\it N\) = 402), and hypokinetic‐rigid (\(\it N\) = 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. \(\bf Results\) Symptoms onset for dystonic were similar to hypokinetic‐rigid, but earlier compared to choreatic subjects (\(\it p\) < .001). Cognition was better in both groups compared to hypokinetic rigid (all \(\it p\) < .001). Functionality differed between all groups (all \(\it p\) < .001). Differences remained (all \(\it p\) < .001) after controlling for CAP score, CAG, age, disease duration, and education. \(\bf Conclusions\) 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.

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Metadaten
Author:Jannis AchenbachORCiDGND, Sarah Maria von HeinGND, Carsten SaftORCiDGND
URN:urn:nbn:de:hbz:294-78101
DOI:https://doi.org/10.13154/294-7810
Parent Title (English):Brain and behavior
Publisher:Wiley-VCH Verlag
Place of publication:Hoboken, New Jersey
Document Type:Article
Language:English
Date of Publication (online):2021/02/01
Date of first Publication:2020/06/12
Publishing Institution:Ruhr-Universität Bochum, Universitätsbibliothek
Tag:Open Access Fonds
Huntington's disease; dystonia; movement disorders
Volume:10
Issue:8, Artikel e01704
First Page:e01704-1
Last Page:e01704-7
Note:
Article Processing Charge funded by the Deutsche Forschungsgemeinschaft (DFG) and the Open Access Publication Fund of Ruhr-Universität Bochum.
Note:
DOI der Original-Publikation: https://onlinelibrary.wiley.com/doi/10.1002/brb3.1704
Institutes/Facilities:St. Josef-Hospital Bochum, Neurologische Klinik
Dewey Decimal Classification:Technik, Medizin, angewandte Wissenschaften / Medizin, Gesundheit
open_access (DINI-Set):open_access
Licence (English):License LogoCreative Commons - CC BY 4.0 - Attribution 4.0 International