{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,6]],"date-time":"2026-01-06T13:48:19Z","timestamp":1767707299619,"version":"3.40.5"},"reference-count":32,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2021,10,6]],"date-time":"2021-10-06T00:00:00Z","timestamp":1633478400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100007155","name":"Medical Research Council Canada","doi-asserted-by":"publisher","award":["MR\/K000608\/1"],"award-info":[{"award-number":["MR\/K000608\/1"]}],"id":[{"id":"10.13039\/501100007155","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000272","name":"National Institute for Health Research","doi-asserted-by":"publisher","award":["PB\u2010PG\u20100711\u201025151"],"award-info":[{"award-number":["PB\u2010PG\u20100711\u201025151"]}],"id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Aerobic deconditioning, due to lower levels of physical activity, could impact independence for people with neuromuscular conditions. We report the maximal cardiopulmonary response in a cohort of people with Charcot Marie Tooth disease type 1A (CMT 1A) and inclusion body myositis (IBM). We also explored potential predictors of aerobic capacity with measures of physical impairment and functional performance.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Participants underwent maximal cardiopulmonary exercise testing (CPET) using a semi\u2010recumbent cycle ergometer. Data were analyzed to determine the peak O<jats:sub>2<\/jats:sub> consumption (VO<jats:sub>2<\/jats:sub> peak), anaerobic threshold (AT), maximum heart rate (MHR), ventilatory equivalent for CO<jats:sub>2<\/jats:sub> slope (V<jats:sub>E<\/jats:sub>\/VCO<jats:sub>2<\/jats:sub>), and respiratory exchange ratio (RER). Impairment, functional and patient reported measures were also recorded. Predicted CPET variables were calculated based on published normative data for age, gender, and weight.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty\u2010two people with CMT and 17 people with IBM were recruited. Both groups showed significantly lower VO<jats:sub>2<\/jats:sub> peak, MHR, AT, and V<jats:sub>E<\/jats:sub>\/VCO<jats:sub>2<\/jats:sub>. The CMT group overall performed better than the IBM group, with significantly higher VO<jats:sub>2<\/jats:sub> peak, MHR, and AT, but lower V<jats:sub>E<\/jats:sub>\/VCO<jats:sub>2.<\/jats:sub> Linear regression analysis demonstrated that VO<jats:sub>2<\/jats:sub> peak was related to body fat percentage and 6\u2010min walk distance for both groups, and steps per day for the IBM group.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Lower than predicted CPET variables were observed that were not explained by cardiopulmonary limitations or reduced effort, implicating peripheral factors in limiting the cycling task. Regression analysis implied prediction of VO2 peak by body fat percentage and 6\u2010min walk distance. Six\u2010minute walk distance could be a potential proxy measure of cardiopulmonary fitness.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27423","type":"journal-article","created":{"date-parts":[[2021,9,27]],"date-time":"2021-09-27T19:03:28Z","timestamp":1632769408000},"page":"683-690","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Cardiopulmonary exercise performance and factors associated with aerobic capacity in neuromuscular diseases"],"prefix":"10.1002","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9344-0301","authenticated-orcid":false,"given":"Gita M.","family":"Ramdharry","sequence":"first","affiliation":[{"name":"Department of Neuromuscular Diseases Institute of Neurology, University College London  London UK"},{"name":"Queen Square Centre for Neuromuscular Diseases National Hospital for Neurology and Neurosurgery, University College Hospitals, NHS Foundation Trust  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8743-8897","authenticated-orcid":false,"given":"Amanda","family":"Wallace","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases Institute of Neurology, University College London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philip","family":"Hennis","sequence":"additional","affiliation":[{"name":"Institute of Sport, Exercise and Health, UCL  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elizabeth","family":"Dewar","sequence":"additional","affiliation":[{"name":"Queen Square Centre for Neuromuscular Diseases National Hospital for Neurology and Neurosurgery, University College Hospitals, NHS Foundation Trust  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Magdalena","family":"Dudziec","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases Institute of Neurology, University College London  London UK"},{"name":"Institute of Sport, Exercise and Health, UCL  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katherine","family":"Jones","sequence":"additional","affiliation":[{"name":"Queen Square Centre for Neuromuscular Diseases National Hospital for Neurology and Neurosurgery, University College Hospitals, NHS Foundation Trust  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2615-9613","authenticated-orcid":false,"given":"Aleksandra","family":"Pietrusz","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases Institute of Neurology, University College London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mary M.","family":"Reilly","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases Institute of Neurology, University College London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael G.","family":"Hanna","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases Institute of Neurology, University College London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,10,6]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.apmr.2004.09.012"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.apmr.2014.09.013"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1080\/09638288.2016.1211180"},{"key":"e_1_2_12_5_1","unstructured":"World Health OrganisationWHO. 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