{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,9]],"date-time":"2026-04-09T01:29:52Z","timestamp":1775698192498,"version":"3.50.1"},"reference-count":7,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,8,29]],"date-time":"2024-08-29T00:00:00Z","timestamp":1724889600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,8,29]],"date-time":"2024-08-29T00:00:00Z","timestamp":1724889600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Neurol. Res. Pract."],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Immunoglobulin G4 (IgG4) related hypertrophic pachymeningitis of the spinal cord is a rare condition, characterized by infiltration of the spinal meninges with IgG4-producing plasma cells and subsequent hypertrophic fibrosis. Here, we report on a 65-year-old woman with IgG4 associated hypertrophic spinal pachymeningitis, in whom cerebrospinal fluid (CSF) analysis was a decisive diagnostic tool. Not only could we demonstrate an intrathecal IgG4 production, but also IgG4 positive plasma cells in CSF. Following decompressive surgery, diagnosis of IgG4 associated hypertrophic pachymeningitis was confirmed histologically. Surgery and immunosuppressive therapy with rituximab were associated with clinical improvement. This case highlights CSF analyses as diagnostic tool for detection of IgG4 related hypertrophic pachymeningitis.<\/jats:p>","DOI":"10.1186\/s42466-024-00343-2","type":"journal-article","created":{"date-parts":[[2024,8,29]],"date-time":"2024-08-29T00:02:00Z","timestamp":1724889720000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Intrathecal IgG4 synthesis in IgG4 related spinal hypertrophic pachymeningitis: a case report"],"prefix":"10.1186","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1290-208X","authenticated-orcid":false,"given":"Lucia K.","family":"Feldmann","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Regina","family":"von Manitius","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Birgit Julia","family":"Grassmann","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Judith","family":"R\u00f6sler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julia","family":"Onken","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian","family":"Meisel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arend","family":"Koch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eberhard","family":"Siebert","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Klemens","family":"Ruprecht","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Meisel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2024,8,29]]},"reference":[{"key":"343_CR1","doi-asserted-by":"publisher","first-page":"117420","DOI":"10.1016\/j.jns.2021.117420","volume":"424","author":"G Saitakis","year":"2021","unstructured":"Saitakis, G., & Chwalisz, B. K. (2021). The neurology of IGG4-related disease. Journal of the Neurological Sciences, 424, 117420. https:\/\/doi.org\/10.1016\/j.jns.2021.117420","journal-title":"Journal of the Neurological Sciences"},{"issue":"7","key":"343_CR2","doi-asserted-by":"publisher","first-page":"732","DOI":"10.1136\/jnnp-2013-306410","volume":"85","author":"T Yonekawa","year":"2014","unstructured":"Yonekawa, T., Murai, H., Utsuki, S., Matsushita, T., Masaki, K., Isobe, N., & Kira, J. (2014). A nationwide survey of hypertrophic pachymeningitis in Japan. Journal of Neurology, Neurosurgery and Psychiatry, 85(7), 732\u2013739. https:\/\/doi.org\/10.1136\/jnnp-2013-306410","journal-title":"Journal of Neurology, Neurosurgery and Psychiatry"},{"issue":"6","key":"343_CR3","doi-asserted-by":"publisher","first-page":"1549","DOI":"10.1007\/s10067-015-3104-x","volume":"35","author":"Z Lu","year":"2016","unstructured":"Lu, Z., Tongxi, L., Jie, L., Yujuan, J., Wei, J., Xia, L., & Xin, L. (2016). IgG4-related spinal pachymeningitis. Clinical Rheumatology, 35(6), 1549\u20131553. https:\/\/doi.org\/10.1007\/s10067-015-3104-x","journal-title":"Clinical Rheumatology"},{"issue":"1\u20132","key":"343_CR4","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1016\/j.jneuroim.2013.10.008","volume":"266","author":"E Della-Torre","year":"2014","unstructured":"Della-Torre, E., Galli, L., Franciotta, D., Bozzolo, E. P., Briani, C., Furlan, R., & Sabbadini, M. G. (2014). Diagnostic value of IgG4 indices in IgG4-related hypertrophic pachymeningitis. Journal of Neuroimmunology, 266(1\u20132), 82\u201386. https:\/\/doi.org\/10.1016\/j.jneuroim.2013.10.008","journal-title":"Journal of Neuroimmunology"},{"issue":"4","key":"343_CR5","doi-asserted-by":"publisher","first-page":"e568","DOI":"10.1212\/NXI.0000000000000568","volume":"6","author":"M Levraut","year":"2019","unstructured":"Levraut, M., Cohen, M., Bresch, S., Giordana, C., Burel-Vandenbos, F., Mondot, L., & Lebrun-Frenay, C. (2019). Immunoglobulin G4-related hypertrophic pachymeningitis: A case-oriented review. Neurology Neuroimmunology & Neuroinflammation, 6(4), e568. https:\/\/doi.org\/10.1212\/NXI.0000000000000568","journal-title":"Neurology Neuroimmunology & Neuroinflammation"},{"key":"343_CR6","doi-asserted-by":"publisher","first-page":"1035056","DOI":"10.3389\/fonc.2022.1035056","volume":"12","author":"F Yang","year":"2022","unstructured":"Yang, F., Liu, Z., Zhang, Y., Li, P., Zhu, Y., Zhu, Q., & Zhang, B. (2022). Case report: Clinical highlights and radiological classification of IgG4-related spinal pachymeningitis: A rare case series and updated review of the literature. Frontiers in Oncology, 12, 1035056. https:\/\/doi.org\/10.3389\/fonc.2022.1035056","journal-title":"Frontiers in Oncology"},{"key":"343_CR7","doi-asserted-by":"publisher","first-page":"1189778","DOI":"10.3389\/fneur.2023.1189778","volume":"14","author":"DT Balaban","year":"2023","unstructured":"Balaban, D. T., Hutto, S. K., Panzarini, B. P., O\u2019Shea, A., Varma, A., Jones, P. S., & Venna, N. (2023). Treatment of IgG4-related disease-associated hypertrophic pachymeningitis with intrathecal rituximab: A case report. Frontiers in Neurology, 14, 1189778. https:\/\/doi.org\/10.3389\/fneur.2023.1189778","journal-title":"Frontiers in Neurology"}],"container-title":["Neurological Research and Practice"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s42466-024-00343-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s42466-024-00343-2\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s42466-024-00343-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,8,29]],"date-time":"2024-08-29T00:04:16Z","timestamp":1724889856000},"score":1,"resource":{"primary":{"URL":"https:\/\/neurolrespract.biomedcentral.com\/articles\/10.1186\/s42466-024-00343-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,8,29]]},"references-count":7,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["343"],"URL":"https:\/\/doi.org\/10.1186\/s42466-024-00343-2","relation":{},"ISSN":["2524-3489"],"issn-type":[{"value":"2524-3489","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,8,29]]},"assertion":[{"value":"20 March 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 August 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 August 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent for publication was acquired from the patient.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"All authors report no disclosures relevant to the content of the manuscript.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"43"}}