{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,17]],"date-time":"2026-06-17T13:49:21Z","timestamp":1781704161698,"version":"3.54.5"},"reference-count":28,"publisher":"Wiley","license":[{"start":{"date-parts":[[2024,8,2]],"date-time":"2024-08-02T00:00:00Z","timestamp":1722556800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Fatigue (subjective perception) and fatigability (objective motor performance worsening) are relevant aspects of disability in individuals with spinal muscular atrophy (SMA). The effect of nusinersen on fatigability in SMA patients has been investigated with conflicting results. We aimed to evaluate this in adult with SMA3.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted a multicenter retrospective cohort study, including adult ambulant patients with SMA3, data available on 6\u2010minute walk test (6MWT) and Hammersmith Functional Motor Scale\u2014Expanded (HFMSE) at baseline and at least at 6\u2009months of treatment with nusinersen. We investigated fatigability, estimated as 10% or higher decrease in walked distance between the first and sixth minute of the 6MWT, at baseline and over the 14\u2010month follow\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010eight patients (56% females) were included. The 6MWT improved after 6, 10, and 14\u2009months of treatment (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05). Of the 27 patients who completed the entire follow\u2010up, 37% improved (6MWT distance increase \u226530\u2009m), 48.2% remained stable, and 14.8% worsened (6MWT distance decline \u226530\u2009m). Fatigability was found at baseline in 26\/38 (68%) patients and confirmed at subsequent time points (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05) without any significant change over the treatment period. There was no correlation between fatigability and <jats:italic>SMN2<\/jats:italic> copy number, sex, age at disease onset, age at baseline, nor with 6MWT total distance and baseline HFMSE score.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Fatigability was detected at baseline in approximately 2\/3 of SMA3 walker patients, without any correlation with clinical features, included motor performance. No effect on fatigability was observed during the 14\u2010month treatment period with nusinersen.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.28225","type":"journal-article","created":{"date-parts":[[2024,8,3]],"date-time":"2024-08-03T05:44:39Z","timestamp":1722663879000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Six\u2010minute walk test as outcome measure of fatigability in adults with spinal muscular atrophy treated with nusinersen"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5393-5462","authenticated-orcid":false,"given":"Alessandra","family":"Govoni","sequence":"first","affiliation":[{"name":"Neuromuscular and Rare Disease Unit La Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore di Milano Policlinico  Milan Italy"},{"name":"Department of Clinical and Experimental Medicine University of Pisa  Pisa 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double\u2010blind placebo\u2010controlled crossover trial with pyridostigmine in spinal muscular atrophy types 2\u20104","volume":"5","author":"Stam M","year":"2022","journal-title":"Brain Commun"},{"key":"e_1_2_12_26_1","doi-asserted-by":"publisher","DOI":"10.3233\/JND-190416"},{"key":"e_1_2_12_27_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0199657"},{"key":"e_1_2_12_28_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm12103458"},{"issue":"7","key":"e_1_2_12_29_1","first-page":"252","article-title":"Muscle fatigue affects the interpolated twitch technique when assessed using electrically\u2010induced contractions in human and rat muscles","volume":"28","author":"Neyroud D","year":"2016","journal-title":"Front Physiol"}],"container-title":["Muscle &amp; 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