{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,25]],"date-time":"2026-10-25T14:39:57Z","timestamp":1792939197929,"version":"4.5.0"},"reference-count":36,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T00:00:00Z","timestamp":1761955200000},"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":["Euro J of Neurology"],"published-print":{"date-parts":[[2025,11]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>This study aimed to evaluate changes in compound muscle action potential (CMAP) amplitude in adults with spinal muscular atrophy (SMA) undergoing nusinersen treatment and its association with motor function improvements.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This multicenter study assessed median, ulnar, and peroneal CMAP over a follow\u2010up of up to 4.5\u2009years using linear mixed models. Motor function was measured using the Revised Upper Limb Module (RULM) and the Hammersmith Functional Motor Scale Expanded (HFMSE). Correlations between CMAP and motor function scores were analyzed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Seventy\u2010eight patients (27 ambulatory, 51 non\u2010ambulatory) were included. Baseline ulnar CMAP \u2265\u20092.0\u2009mV distinguished SMA type 3 from type 2 with 91.3% sensitivity and 88.9% specificity (AUC 0.96, 95% CI 0.92\u20131.0), while baseline median nerve CMAP \u2265\u20096.5\u2009mV showed 91.7% sensitivity and 77.3% specificity (AUC 0.84, 95% CI 0.72\u20130.96). No significant changes over time were observed in median, ulnar, and peroneal CMAP amplitudes (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&gt;\u20090.05). CMAP trajectories did not differ between SMA types 2 and 3 (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&gt;\u20090.05). No significant difference in the change in RULM or HFMSE at any time point was observed between SMA patients with baseline median nerve CMAP &lt;\u20095\u2009mV and those with CMAP of \u2265\u20095\u2009mV (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&gt;\u20090.05). No significant correlations were found between changes in median nerve CMAP and HFMSE or RULM (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&gt;\u20090.05).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>CMAP amplitudes remained stable during nusinersen treatment, with no differences in trajectories between SMA types 2 and 3. Our findings suggest that while CMAP amplitude correlates with disease severity, it may not serve as a sensitive biomarker of treatment response in adult SMA patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ene.70405","type":"journal-article","created":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T08:20:17Z","timestamp":1761985217000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Compound Muscle Action Potential (CMAP) Amplitude Trajectories and Pattern in Adults with 5q\u2010Spinal Muscular Atrophy Receiving Nusinersen Therapy: A Multicenter, Binational Observational Study"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1783-703X","authenticated-orcid":false,"given":"Bogdan","family":"Bjelica","sequence":"first","affiliation":[{"name":"Department of Neurology Hannover Medical School  Hannover Germany"},{"name":"PRACTIS Clinician Scientist Program, 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