title: 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 ([Formula: see text]) significantly increased in HD patients (∆[Formula: see text] = +0.33 ± 0.28 l) and controls (∆[Formula: see text] = +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.
authors.affiliation: Department of Neurology, University Hospital and University of Zurich, Frauenklinikstrasse 26, 8091, Zurich, Switzerland.|Institute of Human Movement Sciences and Sport, ETH Zurich, Zurich, Switzerland.
authors.forename: Sebastian
authors.initials: S
authors.lastname: Frese
authors.affiliation: Department of Neurology, University Hospital and University of Zurich, Frauenklinikstrasse 26, 8091, Zurich, Switzerland.
authors.forename: Jens A
authors.initials: JA
authors.lastname: Petersen
authors.affiliation: Department of Neurology, University Hospital and University of Zurich, Frauenklinikstrasse 26, 8091, Zurich, Switzerland.
authors.forename: Maria
authors.initials: M
authors.lastname: Ligon-Auer
authors.affiliation: Department of Neurology, University Hospital and University of Zurich, Frauenklinikstrasse 26, 8091, Zurich, Switzerland.|Institute of Human Movement Sciences and Sport, ETH Zurich, Zurich, Switzerland.
authors.forename: Sandro Manuel
authors.initials: SM
authors.lastname: Mueller
authors.affiliation: Department of Neurology, University Hospital and University of Zurich, Frauenklinikstrasse 26, 8091, Zurich, Switzerland.
authors.forename: Violeta
authors.initials: V
authors.lastname: Mihaylova
authors.affiliation: Department of Neurology, University Hospital and University of Zurich, Frauenklinikstrasse 26, 8091, Zurich, Switzerland.|Institute of Human Movement Sciences and Sport, ETH Zurich, Zurich, Switzerland.
authors.forename: Saskia M
authors.initials: SM
authors.lastname: Gehrig
authors.affiliation: Institute of Neuropathology, University Hospital and University of Zurich, Zurich, Switzerland.
authors.forename: Veronika
authors.initials: V
authors.lastname: Kana
authors.affiliation: Institute of Neuropathology, University Hospital and University of Zurich, Zurich, Switzerland.
authors.forename: Elisabeth J
authors.initials: EJ
authors.lastname: Rushing
authors.affiliation: Department of Neurology, University Hospital and University of Zurich, Frauenklinikstrasse 26, 8091, Zurich, Switzerland.
authors.forename: Evelyn
authors.initials: E
authors.lastname: Unterburger
authors.affiliation: Department of Neurology, Cantonal Hospital St. Gallen, St. Gallen, Switzerland.
authors.forename: Georg
authors.initials: G
authors.lastname: Kägi
authors.affiliation: Department of Neurology and Swiss Huntington Disease Centre, University of Bern, Bern, Switzerland.
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Burgunder
authors.affiliation: Institute of Human Movement Sciences and Sport, ETH Zurich, Zurich, Switzerland.|Balgrist University Hospital, Department of Orthopedics, University of Zurich, Zurich, Switzerland.
authors.forename: Marco
authors.initials: M
authors.lastname: Toigo
authors.affiliation: Department of Neurology, University Hospital and University of Zurich, Frauenklinikstrasse 26, 8091, Zurich, Switzerland. hans.jung@usz.ch.
authors.forename: Hans H
authors.initials: HH
authors.lastname: Jung
mesh_terms: D001642:Bicycling / Q000502:physiology / Q000523:psychology; D015992:Body Mass Index; D005081:Exercise Therapy / Q000379:methods*; D006801:Humans; D006816:Huntington Disease / Q000235:genetics / Q000503:physiopathology* / Q000523:psychology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009043:Motor Activity / Q000502:physiology; D009483:Neuropsychological Tests; D010101:Oxygen Consumption / Q000502:physiology; D010807:Physical Endurance / Q000502:physiology; D011569:Psychiatric Status Rating Scales; D012720:Severity of Illness Index; D016896:Treatment Outcome
keywords: Cardiovascular function; Endurance training; Motor function; Peak oxygen uptake (); Unified Huntington disease rating scale (UHDRS)