School of Physics and Astronomy, Cardiff University, UK; NMHRI, School of Medicine, Cardiff University, UK; CUBRIC, School of Psychology, Cardiff University, UK.
Centre for Movement, Occupation and Rehabilitation Sciences, OxINMAHR, Oxford Brookes University, Oxford, UK; Faculty of Health and Life Sciences, Oxford Brookes University, Oxford, UK.
Parkinsonism Relat Disord. 2018 Sep;54:56-61. doi: 10.1016/j.parkreldis.2018.04.014. Epub 2018 Apr 14.
Limited data suggests that an altered metabolic and cardiorespiratory exercise response may affect exercise performance in individuals with Huntington's disease (HD). There is no clear exploration of the response in individuals at different stages of the disease or in relation to genetic markers. This study aimed to examine the exercise response and recovery of HD participants, and the relationship to genetic and clinical markers.
HD gene-positive participants (n = 31; 9 pre-manifest; 22 manifest HD) and a healthy control group (n = 29) performed an incremental exercise test until exhaustion. Performance, cardiorespiratory, metabolic and perceptual responses to exercise were determined from a maximal cycle ergometer test throughout the exercise test and during a recovery period.
During sub-maximal exercise, metabolic (lactate levels, oxygen uptake) and cardiorespiratory markers (heart rate) were elevated in HD participants compared to controls. Lactate elevation was specific to pre-manifest HD participants. Work capacity was reduced in both pre-manifest and manifest HD participants with tests terminated with no difference in metabolic, perceptual or cardiorespiratory markers. Submaximal oxygen uptake was correlated with motor score, whilst peak measures were unrelated to genetic or clinical markers. Heart rate recovery was attenuated in pre-manifest and manifest HD participants.
Our findings confirm metabolic and cardiorespiratory deficits reduce exercise performance and affect recovery from an early stage in HD, with submaximal deficits related to phenotypic expression. Exercise capacity appears to be limited by an altered movement economy, thus clinicians should consider an altered exercise response and recovery may affect prescription in HD.
有限的数据表明,代谢和心肺运动反应的改变可能会影响亨廷顿病(HD)患者的运动表现。目前尚不清楚疾病不同阶段或与遗传标志物相关的个体的反应情况。本研究旨在探讨 HD 患者的运动反应和恢复情况,以及与遗传和临床标志物的关系。
HD 基因阳性参与者(n=31;9 名前驱期;22 名显性 HD)和健康对照组(n=29)进行递增运动试验直至力竭。通过最大循环测功仪试验在整个运动试验和恢复期确定运动表现、心肺代谢和感知反应。
在亚最大运动期间,HD 参与者的代谢(乳酸水平、摄氧量)和心肺标志物(心率)均高于对照组。前驱期 HD 参与者的乳酸升高具有特异性。前驱期和显性 HD 参与者的工作能力均降低,代谢、感知或心肺标志物无差异导致试验终止。亚最大摄氧量与运动评分相关,而峰值指标与遗传或临床标志物无关。前驱期和显性 HD 参与者的心率恢复减弱。
我们的研究结果证实,代谢和心肺功能缺陷会降低运动表现,并在 HD 的早期阶段影响恢复,亚最大缺陷与表型表达有关。运动能力似乎受到运动经济性改变的限制,因此临床医生应考虑改变的运动反应和恢复可能会影响 HD 的处方。