Exercise effects in Huntington disease

Abstract

Huntington disease (HD) is a relentlessly progressive neurodegenerative disorder with symptoms across a wide range of neurological domains, including cognitive and motor dysfunction. There is still no causative treatment for HD but environmental factors such as passive lifestyle may modulate disease onset and progression. In humans, multidisciplinary rehabilitation has a positive impact on cognitive functions. However, a specific role for exercise as a component of an environmental enrichment effect has been difficult to demonstrate. We aimed at investigating whether endurance training (ET) stabilizes the progression of motor and cognitive dysfunction and ameliorates cardiovascular function in HD patients. Twelve male HD patients (mean ± SD, 54.8 ± 7.1 years) and twelve male controls (49.1 ± 6.8 years) completed 26 weeks of endurance training. Before and after the training intervention, clinical assessments, exercise physiological tests, and a body composition measurement were conducted and a muscle biopsy was taken from M. vastus lateralis. To examine the natural course of the disease, HD patients were additionally assessed 6 months prior to ET. During the ET period, there was a motor deficit stabilization as indicated by the Unified Huntington’s Disease Rating Scale motor section score in HD patients (baseline: 18.6 ± 9.2, pre-training: 26.0 ± 13.7, post-training: 26.8 ± 16.4). Peak oxygen uptake (\(\dot{V}{\text{O}}_{{ 2 {\text{peak}}}}\)) significantly increased in HD patients (∆\(\dot{V}{\text{O}}_{{ 2 {\text{peak}}}}\) = +0.33 ± 0.28 l) and controls (∆\(\dot{V}{\text{O}}_{{ 2 {\text{peak}}}}\) = +0.29 ± 0.41 l). No adverse effects of the training intervention were reported. Our results confirm that HD patients are amenable to a specific exercise-induced therapeutic strategy indicated by an increased cardiovascular function and a stabilization of motor function.

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Acknowledgments

We thank Dr. Niels Hagenbuch and Prof. Burkhardt Seifert of the Epidemiology, Biostatistics and Prevention Institute (University of Zurich) for their support and assistance during the statistical analyses. The study was supported by the Swiss National Science Foundation (320030_135539) and the Jacques and Gloria Gossweiler Foundation.

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Correspondence to Hans H. Jung.

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Conflicts of interest

None. The results of this study do not constitute endorsement by ACSM. The authors declare that the results of the study are presented clearly, honestly, and without fabrication, falsification, or inappropriate data manipulation.

Ethical standards

All patients and controls gave their written informed consent. The protocol was approved by the ethics committee of the Canton of Zurich (KEK-ZH-Nr. 2009-0119) and was in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki for research involving human subjects (ClinicalTrials.gov NCT01879267)

Funding

The study was supported by the Swiss National Science Foundation (320030_135539) and the Jacques and Gloria Gossweiler Foundation. The funding sources had no role in writing of the manuscript or in the decision to submit it for publication.

Additional information

S. Frese, J. A. Petersen, M. Toigo and H. H. Jung contributed equally.

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Frese, S., Petersen, J.A., Ligon-Auer, M. et al. Exercise effects in Huntington disease. J Neurol 264, 32–39 (2017). https://doi.org/10.1007/s00415-016-8310-1

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Keywords

  • Unified Huntington disease rating scale (UHDRS)
  • Endurance training
  • Motor function
  • Cardiovascular function
  • Peak oxygen uptake (\(\dot{V}{\text{O}}_{{ 2 {\text{peak}}}}\))