{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T13:00:03Z","timestamp":1783947603390,"version":"3.55.0"},"reference-count":7,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,6,3]],"date-time":"2023-06-03T00:00:00Z","timestamp":1685750400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Peripheral Nervous Sys"],"published-print":{"date-parts":[[2023,9]]},"abstract":"Abstract<\/jats:title>Aim<\/jats:title>Nodopathies and paranodopathies are autoimmune neuropathies associated with antibodies to nodal\u2013paranodal antigens (neurofascin 140\/186 and 155, contactin\u20101, contactin\u2010associated protein 1 [Caspr1]) characterized by peculiar clinical features, poor response to standard immunotherapies (e.g., intravenous immunoglobulins, IVIg). Improvement after anti\u2010CD20 monoclonal antibody therapy has been reported. Data on Caspr1 antibodies pathogenicity are still preliminary, and longitudinal titers have been poorly described.<\/jats:p><\/jats:sec>Methods<\/jats:title>We report on a young woman who developed a disabling neuropathy with antibodies to the Caspr1\/contactin\u20101 complex showing a dramatic improvement after rituximab therapy, mirrored by the decrease of antibody titers.<\/jats:p><\/jats:sec>Results<\/jats:title>A 26\u2010year\u2010old woman presented with ataxic\u2010stepping gait, severe motor weakness at four limbs, and low frequency postural tremor. For neurophysiological evidence of demyelinating neuropathy, she was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and treated with IVIg without benefit. MRI showed symmetrical hypertrophy and marked signal hyperintensity of brachial and lumbosacral plexi. Cerebrospinal fluid showed 710\u2009mg\/dL protein. Despite intravenous methylprednisolone, the patient progressively worsened, and became wheelchair\u2010bound. Antibodies to nodal\u2013paranodal antigens were searched for by ELISA and cell\u2010based assay. Anticontactin\/Caspr1 IgG4 antibodies resulted positive. The patient underwent rituximab therapy with slow progressive improvement that mirrored the antibodies titer, measured throughout the disease course.<\/jats:p><\/jats:sec>Conclusions<\/jats:title>Our patient had a severe progressive course with early disability and axonal damage, and slow recovery starting only a few months after antibody\u2010depleting therapy. The close correlation between titer, disability, and treatment, supports the pathogenicity of Caspr1 antibodies, and suggest that their longitudinal evaluation might provide a potential biomarker to evaluate treatment response.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jns.12565","type":"journal-article","created":{"date-parts":[[2023,5,29]],"date-time":"2023-05-29T11:09:32Z","timestamp":1685358572000},"page":"522-527","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Caspr1 antibodies autoimmune paranodopathy with severe tetraparesis: Potential relevance of antibody titers in monitoring treatment response"],"prefix":"10.1111","volume":"28","author":[{"given":"Lorenzo","family":"Bresciani","sequence":"first","affiliation":[{"name":"Department of Neurosciences University of Padova Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Salvalaggio","sequence":"additional","affiliation":[{"name":"Department of Neurosciences University of Padova Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Vegezzi","sequence":"additional","affiliation":[{"name":"IRCCS Mondino Foundation Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Visentin","sequence":"additional","affiliation":[{"name":"Hematology and Immunological Unit, Department of Medicine University of Padova Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Fortuna","sequence":"additional","affiliation":[{"name":"Department of Neurosciences University of Padova Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariagiulia","family":"Anglani","sequence":"additional","affiliation":[{"name":"Department of Neuroradiology Padova Hospital Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Cacciavillani","sequence":"additional","affiliation":[{"name":"CEMES EMG Laboratory, Synlab Data Medica Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Masciocchi","sequence":"additional","affiliation":[{"name":"IRCCS Mondino Foundation Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Scaranzin","sequence":"additional","affiliation":[{"name":"IRCCS Mondino Foundation Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miryam","family":"Carecchio","sequence":"additional","affiliation":[{"name":"Department of Neurosciences University of Padova Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Martinuzzi","sequence":"additional","affiliation":[{"name":"IRCCS \u201cE. Medea,\u201d Department of Neurorehabilitation Conegliano\u2010Pieve di Soligo Research Center Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matteo","family":"Gastaldi","sequence":"additional","affiliation":[{"name":"IRCCS Mondino Foundation Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8035-0200","authenticated-orcid":false,"given":"Chiara","family":"Briani","sequence":"additional","affiliation":[{"name":"Department of Neurosciences University of Padova Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,6,3]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000001107"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000639"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awab014"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/jns.12455"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000001098"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000149"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415\u2010020\u201010041\u2010z"}],"container-title":["Journal of the Peripheral Nervous System"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jns.12565","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,1]],"date-time":"2023-11-01T13:21:41Z","timestamp":1698844901000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jns.12565"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,6,3]]},"references-count":7,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,9]]}},"alternative-id":["10.1111\/jns.12565"],"URL":"https:\/\/doi.org\/10.1111\/jns.12565","archive":["Portico"],"relation":{},"ISSN":["1085-9489","1529-8027"],"issn-type":[{"value":"1085-9489","type":"print"},{"value":"1529-8027","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,6,3]]},"assertion":[{"value":"2023-04-27","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-23","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-06-03","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}