{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T12:16:39Z","timestamp":1792844199130,"version":"4.5.0"},"reference-count":28,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2022,11,20]],"date-time":"2022-11-20T00:00:00Z","timestamp":1668902400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Natural Science Foundation of China","award":["81873778"],"award-info":[{"award-number":["81873778"]}]},{"name":"National Natural Science Foundation of China","award":["82071415"],"award-info":[{"award-number":["82071415"]}]},{"name":"National Natural Science Foundation of China","award":["82271313"],"award-info":[{"award-number":["82271313"]}]},{"name":"National Natural Science Foundation of China","award":["SHDC2020CR2027B"],"award-info":[{"award-number":["SHDC2020CR2027B"]}]},{"name":"Clinical Research Plan of SHDC","award":["81873778"],"award-info":[{"award-number":["81873778"]}]},{"name":"Clinical Research Plan of SHDC","award":["82071415"],"award-info":[{"award-number":["82071415"]}]},{"name":"Clinical Research Plan of SHDC","award":["82271313"],"award-info":[{"award-number":["82271313"]}]},{"name":"Clinical Research Plan of SHDC","award":["SHDC2020CR2027B"],"award-info":[{"award-number":["SHDC2020CR2027B"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>Patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) seropositive for autoantibodies against nodal and paranodal proteins display distinct clinical presentations. In the latest study, CIDP with autoantibodies against paranodal proteins was defined as autoimmune nodopathy (AN). We herein present a case of 39-year-old male with anti- neurofascin (NF) 155 and NF186 IgG4 antibody with gait disturbance and tremor, who was followed up for 4 months and demonstrated clinical improvements after apparently effective rituximab therapy. In addition, a literature review was conducted to investigate the clinical characteristics of anti-NF155\/NF186-positive AN.<\/jats:p>","DOI":"10.3390\/brainsci12111587","type":"journal-article","created":{"date-parts":[[2022,11,21]],"date-time":"2022-11-21T03:07:23Z","timestamp":1669000043000},"page":"1587","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Anti-NF155\/NF186 IgG4 Antibody Positive Autoimmune Nodopathy"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6145-1997","authenticated-orcid":false,"given":"Lijun","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Neurology and Institute of Neurology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China"},{"name":"Department of Neurovascular Center, Changhai Hospital, Naval Medical University, Shanghai 200433, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jing","family":"Pan","sequence":"additional","affiliation":[{"name":"Department of Neurology and Institute of Neurology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huanyu","family":"Meng","sequence":"additional","affiliation":[{"name":"Department of Neurology and Institute of Neurology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhao","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Neurology, Ruijin North Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lili","family":"Zeng","sequence":"additional","affiliation":[{"name":"Department of Neurology and Institute of Neurology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8300-8646","authenticated-orcid":false,"given":"Jun","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Neurology and Institute of Neurology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China"},{"name":"CAS Center for Excellence in Brain Science and Intelligence Technology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,20]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1002\/acn3.248","article-title":"Characterization of IgG4 anti-neurofascin 155 antibody-positive polyneuropathy","volume":"2","author":"Ogata","year":"2015","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1016\/j.jneuroim.2016.10.013","article-title":"IgG4 anti-neurofascin155 antibodies in chronic inflammatory demyelinating polyradiculoneuropathy: Clinical significance and diagnostic utility of a conventional assay","volume":"301","author":"Kadoya","year":"2016","journal-title":"J. Neuroimmunol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1136\/jnnp-2016-314895","article-title":"Paranodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies","volume":"88","author":"Koike","year":"2017","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"848","DOI":"10.1002\/mus.26010","article-title":"Neurofascin-155 IGG4 neuropathy: Pathophysiological insights, spectrum of clinical severity and response to treatment","volume":"57","author":"Garg","year":"2018","journal-title":"Muscle Nerve."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"577074","DOI":"10.1016\/j.jneuroim.2019.577074","article-title":"Chronic inflammatory demyelinating polyneuropathy with anti-NF155 IgG4 in China","volume":"337","author":"Zhang","year":"2019","journal-title":"J. Neuroimmunol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1002\/ana.23794","article-title":"Antibodies to contactin-1 in chronic inflammatory demyelinating polyneuropathy","volume":"73","author":"Querol","year":"2013","journal-title":"Ann. Neurol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"720","DOI":"10.1136\/jnnp-2014-309916","article-title":"Destruction of paranodal architecture in inflammatory neuropathy with anti-contactin-1 autoantibodies","volume":"86","author":"Doppler","year":"2015","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1484","DOI":"10.1093\/brain\/awv054","article-title":"Contactin 1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia","volume":"138","author":"Miura","year":"2015","journal-title":"Brain"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1851","DOI":"10.1093\/brain\/awx124","article-title":"Autoantibodies to nodal isoforms of neurofascin in chronic inflammatory demyelinating polyneuropathy","volume":"140","author":"Delmont","year":"2017","journal-title":"Brain"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"e31","DOI":"10.1212\/WNL.0000000000004773","article-title":"Neurofascin antibodies in autoimmune, genetic, and idiopathic neuropathies","volume":"90","author":"Burnor","year":"2018","journal-title":"Neurology"},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Stengel, H., Vural, A., Brunder, A.M., Heinius, A., Appeltshauser, L., Fiebig, B., Giese, F., Dresel, C., Papagianni, A., and Birklein, F. (2019). Anti-pan-neurofascin IgG3 as a marker of fulminant autoimmune neuropathy. Neurol.-Neuroimmunol. Neuroinflamm., 6.","DOI":"10.1212\/NXI.0000000000000603"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"2617","DOI":"10.1093\/brain\/aww189","article-title":"Auto-antibodies to contactin-associated protein 1 (Caspr) in two patients with painful inflammatory neuropathy","volume":"139","author":"Doppler","year":"2016","journal-title":"Brain"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1111\/jns.12455","article-title":"European Academy of Neurology\/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision","volume":"26","author":"Hadden","year":"2021","journal-title":"J. Peripher. Nerv. Syst."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"e238","DOI":"10.1212\/NXI.0000000000000238","article-title":"Neurofascin-155 as a putative antigen in combined central and peripheral demyelination","volume":"3","author":"Cortese","year":"2016","journal-title":"Neurol.-Neuroimmunol. Neuroinflamm."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"e52","DOI":"10.1212\/WNL.0000000000003616","article-title":"Teaching NeuroImages: Cranial nerve hypertrophy in IgG4 anti-neurofascin 155 antibody-positive polyneuropathy","volume":"88","author":"Franques","year":"2017","journal-title":"Neurology"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1002\/acn3.539","article-title":"Head and voice tremor improving with immunotherapy in an anti-NF155 positive CIDP patient","volume":"5","author":"Painous","year":"2018","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"665136","DOI":"10.3389\/fneur.2021.665136","article-title":"Anti-Neurofascin 155 Antibody-Positive Chronic Inflammatory Demyelinating Polyneuropathy\/Combined Central and Peripheral Demyelination: Strategies for Diagnosis and Treatment Based on the Disease Mechanism","volume":"12","author":"Kira","year":"2021","journal-title":"Front. Neurol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"2223","DOI":"10.1007\/s10072-021-05220-3","article-title":"Neurofascin antibodies in chronic inflammatory demyelinating polyradiculoneuropathy: From intrinsic genetic background to clinical manifestations","volume":"42","author":"Wang","year":"2021","journal-title":"Neurol. Sci."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"e56","DOI":"10.1093\/brain\/awy134","article-title":"Subacute nodopathy with conduction blocks and anti-neurofascin 140\/186 antibodies: An ultrastructural study","volume":"141","author":"Vallat","year":"2018","journal-title":"Brain"},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Cortese, A., Lombardi, R., Briani, C., Callegari, I., Benedetti, L., Manganelli, F., Luigetti, M., Ferrari, S., Clerici, A.M., and Marfia, G.A. (2019). Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP. Neurol.-Neuroimmunol. Neuroinflamm., 7.","DOI":"10.1212\/NXI.0000000000000639"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"4673","DOI":"10.1007\/s10072-021-05101-9","article-title":"From PNS to CNS: Characteristics of anti-neurofascin 186 neuropathy in 16 cases","volume":"42","author":"Xie","year":"2021","journal-title":"Neurol. Sci."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e149","DOI":"10.1212\/NXI.0000000000000149","article-title":"Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins","volume":"2","author":"Querol","year":"2015","journal-title":"Neurol.-Neuroimmunol. Neuroinflamm."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"577280","DOI":"10.1016\/j.jneuroim.2020.577280","article-title":"Efficacy of low dose rituximab in treatment-resistant CIDP with antibodies against NF-155","volume":"345","author":"Jiao","year":"2020","journal-title":"J. Neuroimmunol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1250","DOI":"10.1007\/s00415-021-10646-y","article-title":"Efficacy of rituximab treatment in chronic inflammatory demyelinating polyradiculoneuropathy: A systematic review and meta-analysis","volume":"269","author":"Hu","year":"2021","journal-title":"J. Neurol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"104360","DOI":"10.1016\/j.neuint.2018.12.011","article-title":"Anti-neurofascin autoantibody and demyelination","volume":"130","author":"Kira","year":"2019","journal-title":"Neurochem. Int."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1002\/mus.25757","article-title":"ELectron microscopic abnormality and therapeutic efficacy in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin155 immunoglobulin G4 antibody","volume":"57","author":"Kuwahara","year":"2018","journal-title":"Muscle Nerve"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1016\/j.nmd.2016.10.008","article-title":"Paranodal lesions in chronic inflammatory demyelinating polyneuropathy associated with anti-Neurofascin 155 antibodies","volume":"27","author":"Vallat","year":"2017","journal-title":"Neuromuscul. Disord."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1111\/jns.12465","article-title":"Report of a fulminant anti-pan-neurofascin-associated neuropathy responsive to rituximab and bortezomib","volume":"26","author":"Fels","year":"2021","journal-title":"J. Peripher. Nerv. Syst."}],"container-title":["Brain Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-3425\/12\/11\/1587\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:22:27Z","timestamp":1760145747000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-3425\/12\/11\/1587"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,20]]},"references-count":28,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2022,11]]}},"alternative-id":["brainsci12111587"],"URL":"https:\/\/doi.org\/10.3390\/brainsci12111587","relation":{},"ISSN":["2076-3425"],"issn-type":[{"value":"2076-3425","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,11,20]]}}}