{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T03:15:11Z","timestamp":1777605311509,"version":"3.51.4"},"reference-count":56,"publisher":"MDPI AG","issue":"24","license":[{"start":{"date-parts":[[2023,12,8]],"date-time":"2023-12-08T00:00:00Z","timestamp":1701993600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Biogen","award":["145983"],"award-info":[{"award-number":["145983"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Assessing endurance in non-ambulatory individuals with Spinal Muscular Atrophy (SMA) has been challenging due to limited evaluation tools. The Assisted 6-Minute Cycling Test (A6MCT) is an upper limb ergometer assessment used in other neurologic disorders to measure endurance. To study the performance of the A6MCT in the non-ambulatory SMA population, prospective data was collected on 38 individuals with SMA (13 sitters; 25 non-sitters), aged 5 to 74 years (mean = 30.3; SD = 14.1). The clinical measures used were A6MCT, Revised Upper Limb Module (RULM), Adapted Test of Neuromuscular Disorders (ATEND), and Egen Klassifikation Scale 2 (EK2). Perceived fatigue was assessed using the Fatigue Severity Scale (FSS), and effort was assessed using the Rate of Perceived Exertion (RPE). Data were analyzed for: (1) Feasibility, (2) Clinical discrimination, and (3) Associations between A6MCT with clinical characteristics and outcomes. Results showed the A6MCT was feasible for 95% of the tested subjects, discriminated between functional groups (p = 0.0086), and was significantly associated with results obtained from RULM, ATEND, EK2, and Brooke (p &lt; 0.0001; p = 0.029; p &lt; 0.001; p = 0.005). These findings indicate the A6MCT\u2019s potential to evaluate muscular endurance in non-ambulatory SMA individuals, complementing clinician-rated assessments. Nevertheless, further validation with a larger dataset is needed for broader application.<\/jats:p>","DOI":"10.3390\/jcm12247582","type":"journal-article","created":{"date-parts":[[2023,12,8]],"date-time":"2023-12-08T08:47:26Z","timestamp":1702025246000},"page":"7582","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Assessing the Assisted Six-Minute Cycling Test as a Measure of Endurance in Non-Ambulatory Patients with Spinal Muscular Atrophy (SMA)"],"prefix":"10.3390","volume":"12","author":[{"given":"Whitney J.","family":"Tang","sequence":"first","affiliation":[{"name":"Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bo","family":"Gu","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Samuel","family":"Montalvo","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Cardiovascular Medicine, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9322-0608","authenticated-orcid":false,"given":"Sally","family":"Dunaway Young","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9822-412X","authenticated-orcid":false,"given":"Dana M.","family":"Parker","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Constance","family":"de Monts","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paxton","family":"Ataide","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Noirin","family":"Ni Ghiollagain","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthew T.","family":"Wheeler","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Cardiovascular Medicine, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolina","family":"Tesi Rocha","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey W.","family":"Christle","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Cardiovascular Medicine, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zihuai","family":"He","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John W.","family":"Day","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tina","family":"Duong","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neurosciences, Stanford University, Palo Alto, CA 94305, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,12,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1212\/WNL.0000000000006026","article-title":"Precious SMA natural history data","volume":"91","author":"Darras","year":"2018","journal-title":"Neurology"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1002\/ana.25101","article-title":"Natural history of infantile-onset spinal muscular atrophy","volume":"82","author":"Kolb","year":"2017","journal-title":"Ann. 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