Session Time: 12:00pm-1:30pm
Objective: To investigate the course of motor symptoms in Huntington’s disease (HD) and their relationship with cognitive- and general functioning over time.
Background: The motor symptoms in HD can be divided into predominantly choreatic and hypokinetic-rigid subtypes. Chorea is often more pronounced in patients with early stage HD, whereas in advanced stages of HD hypokinetic symptoms become more dominant. It has been reported in cross-sectional studies that predominantly choreatic HD patients perform better on general- and cognitive assessments compared to predominantly hypokinetic-rigid HD patients. However, the course of these motor subtypes and their clinical profiles has not been investigated over time.
Methods: A total of 4,161 subjects who participated in the EHDN Registry study were included and classified at baseline as either predominantly choreatic (n=898), hypokinetic-rigid (n=926), or mixed motor (n=2,337), based on a previously used method (Hart et al., 2013). The maximum follow-up period was six years. The mixed motor group was not included in the analyses. Linear mixed models were constructed to investigate changes in motor subtypes over time and their relationship with cognitive- and functional decline.
Results: Over the six-year follow-up period the predominantly choreatic group showed a significant decrease of chorea on the Unified HD Rating Scale (UHDRS), while the presence of hypokinetic-rigid symptoms slightly increased in the hypokinetic-rigid group. On three tasks, the predominantly choreatic group showed a significantly faster rate of change over time compared to the hypokinetic-rigid group. On all other cognitive assessments the decline between both groups was comparable.
Conclusions: Our results suggest that choreatic symptoms decrease over time, whereas hypokinetic-rigid symptoms slightly increase in a large cohort of HD patients. Predominantly choreatic HD patients performed better on cognitive- and functional assessments over a six-year period compared to hypokinetic-rigid HD, but showed a faster rate of decline on some of these tasks.
To cite this abstract in AMA style:M. Jacobs, E.P. 't Hart, R.A.C. Roos. Progression of motor subtypes in Huntington’s disease: A six-year follow-up [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/progression-of-motor-subtypes-in-huntingtons-disease-a-six-year-follow-up/. Accessed February 21, 2024.
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MDS Abstracts - https://www.mdsabstracts.org/abstract/progression-of-motor-subtypes-in-huntingtons-disease-a-six-year-follow-up/