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E2 Progression of motor subtypes in huntington’s disease: a six-year follow-up study
  1. Milou Jacobs1,
  2. Ellen P Hart2,
  3. Erik W van Zwet3,
  4. Anna Rita Bentivoglio4,
  5. Jean-Marc Burgunder5,
  6. David Craufurd6,
  7. Ralf Reilmann7,
  8. Carsten Saft8,
  9. Raymund AC Roos1,
  10. the REGISTRY investigators of the European Huntington’s Disease Network
  1. 1Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands
  2. 2Centre for Human Drug Research, Leiden, The Netherlands
  3. 3Department of Medical Statistics, Leiden University Medical Centre, Leiden, The Netherlands
  4. 4Department of Neurology, Università Cattolica del Sacro Cuore, Rome, Italy; Fondazione Don Carlo Gnocchi, Milan, Italy
  5. 5Department of Neurology, University of Bern, Bern, Switzerland
  6. 6Institute of Human Development, Faculty of Medical and Human Sciences, University of Manchester, Manchester, UK; Manchester Centre of Genomic Medicine, St. Mary’s Hospital, Central Manchester University Hospitals NHS Foundation Trust and Manchester Academic Health Science Centre, Manchester, UK
  7. 7George Huntington Institute, Muenster, Germany; Department of Radiology, University of Muenster, Muenster, Germany; Department of Neurodegenerative Diseases and Hertie-Institute for Clinical Brain Research, University of Tuebingen, Tuebingen, Germany
  8. 8Department of Neurology, Ruhr-University Bochum, Bochum, Germany


Background The motor signs in Huntington’s disease (HD) can be divided into predominantly choreatic and hypokinetic-rigid subtypes. It has been reported in cross-sectional studies that predominantly choreatic HD patients perform better on functional 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.

Aims To investigate the progression of predominantly choreatic and hypokinetic-rigid signs in HD and their relationship with cognitive and general functioning over time.

Methods A total of 4,135 subjects who participated in the European HD Network REGISTRY study were included and classified at baseline as either predominantly choreatic (n = 891), hypokinetic-rigid (n = 916), or mixed-motor (n = 2,328), 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 in chorea, while hypokinetic-rigid symptoms slightly increased in the hypokinetic-rigid group. On the Total Functional Capacity, Stroop test, and Verbal Fluency task the rate of change over time was significantly faster in the predominantly choreatic group, while on all other clinical assessments the decline was comparable for both groups.

Conclusions Our results suggest that choreatic symptoms decrease over time, whereas hypokinetic-rigid symptoms slightly increase in a large cohort of HD patients. Moreover, different motor subtypes can be related to different clinical profiles.

  • Chorea
  • Hypokinetic-rigid
  • Cognition

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