{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,7]],"date-time":"2026-01-07T23:12:50Z","timestamp":1767827570845,"version":"3.49.0"},"reference-count":19,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,12,26]],"date-time":"2022-12-26T00:00:00Z","timestamp":1672012800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007721","name":"Cure SMA","doi-asserted-by":"publisher","award":["PNCRN Infrastructure Grant"],"award-info":[{"award-number":["PNCRN Infrastructure Grant"]}],"id":[{"id":"10.13039\/100007721","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005202","name":"Muscular Dystrophy Association","doi-asserted-by":"publisher","award":["575870"],"award-info":[{"award-number":["575870"]}],"id":[{"id":"10.13039\/100005202","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006937","name":"National Center for Medical Rehabilitation Research","doi-asserted-by":"publisher","award":["K01HD084690"],"award-info":[{"award-number":["K01HD084690"]}],"id":[{"id":"10.13039\/100006937","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":[[2023,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Fatiguability and perceived fatigue are common unrelated symptoms in ambulatory individuals with spinal muscular atrophy (SMA). Ratings of perceived exertion (RPE) measures the sense of effort during an activity and has been used as a proxy for fatigue. Relationships between perceived fatigue, fatiguability, and RPE have been described in healthy populations, but the relationship in SMA has not been examined.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Eighteen ambulatory individuals with SMA and 16 age\u2010matched controls (age, 13 to 57\u2009years; 26 [76.5%] males) performed the 6\u2010minute walk test (6MWT) and cardiopulmonary exercise tolerance test (CPET) and completed the International Physical Activity Questionnaire\u2010\u2010\u2010short form (IPAQ). RPE was collected during the CPET and 6MWT. Fatiguability was measured during the 6MWT. Physical activity (PA) volume was calculated using the IPAQ. Wilcoxon rank\u2010sum tests were used to compare groups. Spearman correlation coefficients evaluated associations between variables. SMA subgroups were predetermined using 6MWT distances of over or under 300\u2009meters.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>There were no significant associations between fatiguability and RPE or PA in SMA (<jats:italic>P<\/jats:italic>\u2009&gt;\u2009.05). PA was strongly associated with 6MWT RPE (<jats:italic>r<\/jats:italic>\u00a0=\u00a00.71) in SMA individuals who walked fewer than 300\u2009meters (n\u00a0=\u00a07). There were no significant associations between any variables in controls (<jats:italic>P<\/jats:italic>\u2009&gt;\u2009.05).<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>RPE is not associated with fatiguability in SMA. The possible association of PA and RPE may reflect the increased intensity of the 6MWT in weaker patients. RPE represents a sense of effort during exercise and should not be used as a substitute for fatiguability but may be a measure of patient experience during exercise.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27765","type":"journal-article","created":{"date-parts":[[2022,12,9]],"date-time":"2022-12-09T09:19:06Z","timestamp":1670577546000},"page":"81-84","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Perceived exertion is not a substitute for fatiguability in spinal muscular atrophy"],"prefix":"10.1002","volume":"68","author":[{"given":"Ricky","family":"Cheng","sequence":"first","affiliation":[{"name":"Department of Rehabilitation and Regenerative Medicine Columbia University Irving Medical Center  New York New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julia","family":"Feng","sequence":"additional","affiliation":[{"name":"Department of Rehabilitation and Regenerative Medicine Columbia University Irving Medical Center  New York New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ashley M.","family":"Goodwin","sequence":"additional","affiliation":[{"name":"Department of Rehabilitation and Regenerative Medicine Columbia University Irving Medical Center  New York New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Uher","sequence":"additional","affiliation":[{"name":"Department of Rehabilitation and Regenerative Medicine Columbia University Irving Medical Center  New York New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ashwini K.","family":"Rao","sequence":"additional","affiliation":[{"name":"Department of Rehabilitation and Regenerative Medicine Columbia University Irving Medical Center  New York New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael P.","family":"McDermott","sequence":"additional","affiliation":[{"name":"Departments of Biostatistics and Computational Biology and Neurology University of Rochester  Rochester New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Darryl C.","family":"De Vivo","sequence":"additional","affiliation":[{"name":"Department of Neurology Columbia University Irving Medical Center  New York New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carol","family":"Ewing Garber","sequence":"additional","affiliation":[{"name":"Department of Biobehavioral Sciences Teachers College, Columbia University  New York New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7357-0819","authenticated-orcid":false,"given":"Jacqueline","family":"Montes","sequence":"additional","affiliation":[{"name":"Department of Rehabilitation and Regenerative Medicine Columbia University Irving Medical Center  New York New York"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,12,26]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ncl.2015.07.004"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12883-019-1244-3"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31827f07be"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.3233\/JND-180342"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000011230"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/jor.24856"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1249\/00005768-198205000-00012"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005768-200201000-00021"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1249\/01.MSS.0000142310.97274.94"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2019.01383"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/gerona\/glw173"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-020-1348-2"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1249\/MSS.0b013e318213fefb"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.51353"},{"key":"e_1_2_11_16_1","volume-title":"ACSM's Guidelines for Exercise Testing and Prescription","author":"American College of Sports Medicine","year":"2018"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1249\/01.MSS.0000078924.61453.FB"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25120"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.3233\/JND-150101"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-020-10227-5"}],"container-title":["Muscle &amp; 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