{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T07:05:37Z","timestamp":1781766337996,"version":"3.54.5"},"reference-count":32,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,1,21]],"date-time":"2026-01-21T00:00:00Z","timestamp":1768953600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,1,21]],"date-time":"2026-01-21T00:00:00Z","timestamp":1768953600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction\/Aims<\/jats:title>\n                    <jats:p>Previous studies of children with spinal muscular atrophy (SMA) have focused on the ulnar and median nerves, while lower\u2010limb and proximal motor nerves remain insufficiently characterized. This study aimed to evaluate compound muscle action potential (CMAP) amplitudes in upper\u2010 and lower\u2010limb motor nerves in children with SMA and changes after nusinersen treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      In this single\u2010center retrospective study,\n                      <jats:styled-content style=\"fixed-case\">CMAP<\/jats:styled-content>\n                      amplitudes were collected from children with\n                      <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>\n                      and age\u2010matched controls without neuromuscular disease.\n                      <jats:styled-content style=\"fixed-case\">CMAP<\/jats:styled-content>\n                      amplitudes of the tibial, peroneal, femoral, median, and ulnar nerves were assessed in children with\n                      <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>\n                      types 1\u20133. A cross\u2010sectional analysis was conducted to assess\n                      <jats:styled-content style=\"fixed-case\">CMAP<\/jats:styled-content>\n                      amplitudes prior to treatment. Longitudinal changes after\n                      <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>\n                      disease\u2010modifying therapies (nusinersen monotherapy or nusinersen plus risdiplam treatment) were evaluated.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      A total of 47 children with\n                      <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>\n                      were included. The baseline\n                      <jats:styled-content style=\"fixed-case\">CMAP<\/jats:styled-content>\n                      amplitudes of the peroneal, tibial, median, and ulnar nerves were the highest in type 3, followed by type 2, and lowest in type 1. Femoral nerve\n                      <jats:styled-content style=\"fixed-case\">CMAP<\/jats:styled-content>\n                      amplitudes were low in all\n                      <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>\n                      subtypes. At preliminary diagnosis, children with\n                      <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>\n                      had significantly reduced\n                      <jats:styled-content style=\"fixed-case\">CMAP<\/jats:styled-content>\n                      amplitudes for the five nerves compared with age\u2010matched controls (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200963,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.05). After 18\u2009months of nusinersen treatment,\n                      <jats:styled-content style=\"fixed-case\">CMAP<\/jats:styled-content>\n                      amplitudes showed significant increases from baseline in the peroneal, femoral, median, and ulnar nerves (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.05).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>\n                      <jats:styled-content style=\"fixed-case\">CMAP<\/jats:styled-content>\n                      amplitudes can differentiate\n                      <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>\n                      disease severity and may increase after nusinersen treatment. Large\u2010scale longitudinal studies are required to investigate\n                      <jats:styled-content style=\"fixed-case\">CMAP<\/jats:styled-content>\n                      amplitude as a biomarker of treatment response in patients with\n                      <jats:styled-content style=\"fixed-case\">SMA<\/jats:styled-content>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mus.70156","type":"journal-article","created":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T06:42:11Z","timestamp":1769064131000},"page":"755-764","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Electrophysiological Changes in Pediatric Spinal Muscular Atrophy: Results From an Observational Study"],"prefix":"10.1002","volume":"73","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6607-3195","authenticated-orcid":false,"given":"Ruidi","family":"Sun","sequence":"first","affiliation":[{"name":"Graduate College of Tianjin Medical University  Tianjin China"},{"name":"Department of Electrophysiology, Wuhan Children's Hospital, Tongji Medical College Huazhong University of Science and Technology  Wuhan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jing","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Neurology, Wuhan Children's Hospital, Tongji Medical College Huazhong University of Science and Technology  Wuhan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Han","sequence":"additional","affiliation":[{"name":"Emergency and Critical Care Center, Wuhan Children's Hospital, Tongji Medical College Huazhong University of Science and Technology  Wuhan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Electrophysiology, Wuhan Children's Hospital, Tongji Medical College Huazhong University of Science and Technology  Wuhan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiajun","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Electrophysiology, Wuhan Children's Hospital, Tongji Medical College Huazhong University of Science and Technology  Wuhan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xue","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Neurology, Wuhan Children's Hospital, Tongji Medical College Huazhong University of Science and Technology  Wuhan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaolong","family":"Deng","sequence":"additional","affiliation":[{"name":"Department of Neurology, Wuhan Children's Hospital, Tongji Medical College Huazhong University of Science and Technology  Wuhan China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7265-9387","authenticated-orcid":false,"given":"Chunquan","family":"Cai","sequence":"additional","affiliation":[{"name":"Tianjin Children's Hospital (Children's Hospital of Tianjin University)  Tianjin China"},{"name":"Tianjin Key Laboratory of Birth Defects for Prevention and Treatment  Tianjin China"},{"name":"Tianjin Pediatric Research Institute  Tianjin China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2944-3879","authenticated-orcid":false,"given":"Dan","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Neurology, Wuhan Children's Hospital, Tongji Medical College Huazhong University of Science and Technology  Wuhan 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