{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T14:16:57Z","timestamp":1789827417449,"version":"4.0.1"},"reference-count":13,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2017,11,29]],"date-time":"2017-11-29T00:00:00Z","timestamp":1511913600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["##1107749"],"award-info":[{"award-number":["##1107749"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher","award":["1037746"],"award-info":[{"award-number":["1037746"]}],"id":[{"id":"10.13039\/501100000925","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2018,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Sensorimotor neuropathy associated with IgG4 antibodies to neurofascin\u2010155 (NF155) was recently described. The clinical phenotype is typically associated with young onset, distal weakness, and in some cases, tremor.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>From a consecutive cohort of 55 patients diagnosed with chronic inflammatory demyelinating polyneuropathy, screening for anti\u2010NF155 antibodies was undertaken. Patients underwent clinical assessment, diagnostic neurophysiology, including peripheral axonal excitability studies and nerve ultrasound.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Three of 55 chronic inflammatory demyelinating polyneuropathy patients (5%) tested positive for anti\u2010NF155 IgG4. Patients presenting with more severe disease had higher antibody titers. Ultrasound demonstrated diffuse nerve enlargement. Axonal excitability studies were markedly abnormal, with subsequent mathematical modeling of the results supporting disruption of the paranodal seal.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>A broad spectrum of disease severity and treatment response may be observed in anti\u2010NF155 neuropathy. Excitability studies support the pathogenic role of anti\u2010NF155 IgG4 antibodies targeting the paranodal region. <jats:italic>Muscle Nerve<\/jats:italic> <jats:bold>57<\/jats:bold>: 848\u2013851, 2018<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.26010","type":"journal-article","created":{"date-parts":[[2017,11,11]],"date-time":"2017-11-11T15:06:43Z","timestamp":1510412803000},"page":"848-851","source":"Crossref","is-referenced-by-count":44,"title":["Neurofascin\u2010155 IGG4 Neuropathy: Pathophysiological Insights, Spectrum of Clinical Severity and Response To treatment"],"prefix":"10.1002","volume":"57","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1563-6792","authenticated-orcid":false,"given":"Nidhi","family":"Garg","sequence":"first","affiliation":[{"name":"Brain and Mind Centre, Sydney Medical School The University of Sydney 94 Mallett Street Camperdown, NSW 2050 Australia"},{"name":"Department of Neurology, Royal Prince Alfred Hospital The University of Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanna B.","family":"Park","sequence":"additional","affiliation":[{"name":"Brain and Mind Centre, Sydney Medical School The University of Sydney 94 Mallett Street Camperdown, NSW 2050 Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Con","family":"Yiannikas","sequence":"additional","affiliation":[{"name":"Department of Neurology, Concord and Royal North Shore Hospitals The University of Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steve","family":"Vucic","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Neurophysiology, Westmead Hospital The University of Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Howells","sequence":"additional","affiliation":[{"name":"Brain and Mind Centre, Sydney Medical School The University of Sydney 94 Mallett Street Camperdown, NSW 2050 Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1169-8574","authenticated-orcid":false,"given":"Yu\u2010Ichi","family":"Noto","sequence":"additional","affiliation":[{"name":"Brain and Mind Centre, Sydney Medical School The University of Sydney 94 Mallett Street Camperdown, NSW 2050 Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily K.","family":"Mathey","sequence":"additional","affiliation":[{"name":"Brain and Mind Centre, Sydney Medical School The University of Sydney 94 Mallett Street Camperdown, NSW 2050 Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John D.","family":"Pollard","sequence":"additional","affiliation":[{"name":"Brain and Mind Centre, Sydney Medical School The University of Sydney 94 Mallett Street Camperdown, NSW 2050 Australia"},{"name":"Department of Neurology, Royal Prince Alfred Hospital The University of Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew C.","family":"Kiernan","sequence":"additional","affiliation":[{"name":"Brain and Mind Centre, Sydney Medical School The University of Sydney 94 Mallett Street Camperdown, NSW 2050 Australia"},{"name":"Department of Neurology, Royal Prince Alfred Hospital The University of Sydney NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,11,29]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000000205"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.248"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2016.10.013"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000002418"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000149"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2017.05.002"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4598(200003)23:3<399::AID-MUS12>3.0.CO;2-G"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.1991.sp018766"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2011.225573"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2006.06.092"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2014-309599"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2003.11.011"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.12758"}],"container-title":["Muscle &amp; 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