{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T19:25:53Z","timestamp":1789500353846,"version":"build-2803163510"},"reference-count":18,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,11,14]],"date-time":"2025-11-14T00:00:00Z","timestamp":1763078400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,11,14]],"date-time":"2025-11-14T00:00:00Z","timestamp":1763078400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP23ek0109547"],"award-info":[{"award-number":["JP23ek0109547"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Peripheral Nervous Sys"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and Aims<\/jats:title>\n                    <jats:p>Autoimmune nodopathy with anti\u2010neurofascin\u2010155 (NF155) antibodies is increasingly recognized as a distinct disease entity. Although these patients have been described, not all aspects have been fully elucidated. We investigated clinical phenotypes, biomarker profiles, and treatment outcomes in patients with anti\u2010NF155 antibody\u2010positive autoimmune nodopathy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analyzed seven Japanese patients (aged 13\u201327\u2009years) with anti\u2010NF155 antibody\u2010positive autoimmune nodopathy treated at Kumamoto University Hospital between 2017 and 2020. Clinical, electrophysiological, radiological, and biomarker data were evaluated. One refractory case was followed up for over 80\u2009months, tracking serum neurofilament light chain (sNfL) and anti\u2010NF155 antibody levels in the serum and cerebrospinal fluid (CSF).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>All patients exhibited severe sensory ataxia and motor dysfunction. Trigeminal nerve hypertrophy was observed in five individuals, without corresponding symptoms, and pes cavus in one patient. All patients demonstrated a pronounced demyelinating neuropathy phenotype. The mean CSF protein level was 343\u2009mg\/dL. Intravenous immunoglobulin (IVIg) had minimal efficacy, while corticosteroids produced good responses in four patients and partial responses in three patients. Rituximab markedly improved a refractory case. Anti\u2010NF155 antibody and sNfL levels correlated with clinical status. Magnetic resonance imaging frequently revealed persistent nerve root hypertrophy despite functional improvement.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>Anti\u2010NF155 nodopathy is a distinct, biomarker\u2010trackable entity; corticosteroids and rituximab outperform IVIg, challenging conventional practice and highlighting the need for antibody testing, especially in young patients with severe demyelinating neuropathy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/jns.70077","type":"journal-article","created":{"date-parts":[[2025,11,14]],"date-time":"2025-11-14T15:02:57Z","timestamp":1763132577000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Clinical and Radiological Heterogeneity in Anti\u2010Neurofascin\u2010155 Autoimmune Nodopathy: A\u00a0Case\u00a0Series Analysis"],"prefix":"10.1111","volume":"30","author":[{"given":"Michie","family":"Imamura","sequence":"first","affiliation":[{"name":"Department of Neurology, Graduate School of Medical Sciences Kumamoto University  Kumamoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2013-7368","authenticated-orcid":false,"given":"Hironori","family":"Mizutani","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medical Sciences Kumamoto University  Kumamoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keiichi","family":"Nakahara","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medical Sciences Kumamoto University  Kumamoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hidenori","family":"Ogata","sequence":"additional","affiliation":[{"name":"Department of Neurology, Neurological Institute, Graduate School of Medical Sciences Kyushu University  Fukuoka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomoaki","family":"Taguchi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medical Sciences Kumamoto University  Kumamoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masao","family":"Imura","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medical Sciences Kumamoto University  Kumamoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshiya","family":"Nomura","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medical Sciences Kumamoto University  Kumamoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yohei","family":"Misumi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graduate School of Medical Sciences Kumamoto University  Kumamoto 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Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,11,14]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000001195"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.150.3.657"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.4752\u201013.2014"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/jns.12455"},{"key":"e_1_2_13_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000001107"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.248"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2016.10.013"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000004773"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnins.2021.637336"},{"key":"e_1_2_13_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2017.05.002"},{"key":"e_1_2_13_12_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000000205"},{"key":"e_1_2_13_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000001116"},{"key":"e_1_2_13_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13311\u2010022\u201001210\u20101"},{"key":"e_1_2_13_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.14959"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2017.08.374"},{"key":"e_1_2_13_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.51220"},{"key":"e_1_2_13_18_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000001098"},{"key":"e_1_2_13_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415\u2010021\u201010537\u20102"}],"container-title":["Journal 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