{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T00:13:57Z","timestamp":1793060037311,"version":"4.5.0"},"reference-count":11,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,6,14]],"date-time":"2024-06-14T00:00:00Z","timestamp":1718323200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,6,14]],"date-time":"2024-06-14T00:00:00Z","timestamp":1718323200000},"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":["BMC Neurol"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>We present a rare case of NeuroBehcet\u2019s-related intracranial hypertension without cerebral venous thrombosis (NBrIHwCVT), occurring as the first presentation of NeuroBehcet\u2019s. In addition, we describe the novel use of subcutaneous tocilizumab for this indication. This is followed by a review of the literature on this topic.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case<\/jats:title>\n                <jats:p>The patient was a 28-year-old lady of Southern Chinese origin with a known history of Behcet\u2019s disease with oral ulcers and ocular findings for which she was on mycophenolate mofetil and adalimumab. She presented with a headache and bilateral disc swelling associated with an intracranial pressure (ICP) of &gt;\u200940cmH20. There were no structural lesions or cerebral venous thrombosis (CVT) on imaging. Initial lumbar puncture had raised leucocytes and protein. We discuss diagnostic challenges given persistently elevated ICP despite subsequent non-inflammatory cerebrospinal fluid (CSF) profiles and non-response to acetazolamide. She eventually showed a response to immunosuppressant therapy in the form of pulsed methylprednisolone, cyclophosphamide and subsequently subcutaneous tocilizumab, supporting the diagnosis of NBrIHwCVT. Complete normalization of ICP remains challenging. Her disease course was severe, unusual for her ethnicity.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Literature review<\/jats:title>\n                <jats:p>We identified 34 patients (including ours) from 14 publications. We found that the majority of NBrIHwCVT patients were young (average age of 34 years), with a slight female preponderance. Of the 17 cases in the literature with available data on CSF profile, none had raised leucocytes whilst one patient had elevated protein. Patients were generally treated with steroids and occasionally azathioprine, in line with the suspected autoimmune pathophysiology. Of 22 patients with data on outcome, six (27%) were noted to have recurrence of symptoms generally occurring a few months later.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>As demonstrated by this case, NBrIHwCVT can present with BD with raised ICP even if there is no prior history of NB, central Asian ethnicity, cerebral venous thrombosis or features of inflammation on the CSF. We demonstrated how novel use of Tocilizumab may have a role in the management of NBrIHwCVT. Based on our literature review, patients were more likely to be young, female, display a non-inflammatory CSF picture, be treated with steroids and harbour a possibility of recurrence.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12883-024-03708-x","type":"journal-article","created":{"date-parts":[[2024,6,14]],"date-time":"2024-06-14T07:02:48Z","timestamp":1718348568000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["NeuroBehcet\u2019s-related intracranial hypertension without cerebral venous thrombosis: case report and review of literature"],"prefix":"10.1186","volume":"24","author":[{"given":"Jinesh Mukesh","family":"Shah","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Warren","family":"Fong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deidre Anne","family":"De Silva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,6,14]]},"reference":[{"key":"3708_CR1","doi-asserted-by":"crossref","unstructured":"Ocansey S, et al. Normative values of retinal nerve Fibre Layer Thickness and Optic Nerve Head Parameters and Their Association with visual function in an African Population. J Ophthalmol. 2020 Feb;11:20207150673.","DOI":"10.1155\/2020\/7150673"},{"issue":"2","key":"3708_CR2","doi-asserted-by":"publisher","first-page":"192","DOI":"10.1016\/S1474-4422(09)70015-8","volume":"8","author":"A Al-Araji","year":"2009","unstructured":"Al-Araji A, Kidd DP. Neuro-Beh\u00e7et\u2019s disease: epidemiology, clinical characteristics, and management. Lancet Neurol. 2009;8(2):192\u2013204.","journal-title":"Lancet Neurol"},{"issue":"1","key":"3708_CR3","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1186\/s12883-023-03392-3","volume":"23","author":"Y Wu","year":"2023","unstructured":"Wu Y, Yin W, Liu S, Wang S, Ding Y. Diagnosis and management of Neuro-Beh\u00e7et disease with isolated intracranial hypertension: a case report and literature review. Rev BMC Neurol. 2023;23(1):335.","journal-title":"Rev BMC Neurol"},{"issue":"8","key":"3708_CR4","first-page":"1689","volume":"29","author":"CC Mok","year":"2002","unstructured":"Mok CC, Cheung TC, Ho CT, Lee KW, Lau CS, Wong R. W. Beh\u00e7et\u2019s disease in southern Chinese patients. J Rheumatol. 2002;29(8):1689\u201393.","journal-title":"J Rheumatol"},{"key":"3708_CR5","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1186\/s42358-023-00328-w","volume":"63","author":"D Hu","year":"2023","unstructured":"Hu D, Guan JL. The roles of immune cells in Beh\u00e7et\u2019s disease. Adv Rheumatol. 2023;63:49.","journal-title":"Adv Rheumatol"},{"key":"3708_CR6","doi-asserted-by":"crossref","unstructured":"Liu J et al. Tocilizumab in the treatment of severe and refractory parenchymal neuro-Beh\u00e7et\u2019s syndrome: case series and literature review. Ther Adv Musculoskelet Dis 2020 Nov 16:12:1759720X20971908.","DOI":"10.1177\/1759720X20971908"},{"key":"3708_CR7","doi-asserted-by":"publisher","first-page":"99","DOI":"10.1016\/j.jns.2017.10.038","volume":"383","author":"G Akdal","year":"2017","unstructured":"Akdal G, et al. Pseudotumor Cerebri syndrome without cerebral venous sinus thrombosis in Beh\u00e7et\u2019s disease. J Neurol Sci. 2017;383:99\u2013100.","journal-title":"J Neurol Sci"},{"issue":"7","key":"3708_CR8","doi-asserted-by":"publisher","first-page":"916","DOI":"10.3899\/jrheum.221106","volume":"50","author":"M Khitri","year":"2023","unstructured":"Khitri M, et al. Tocilizumab in Beh\u00e7et Disease: a Multicenter Study of 30 patients. J Rheumatol. 2023;50(7):916\u201323.","journal-title":"J Rheumatol"},{"issue":"4","key":"3708_CR9","doi-asserted-by":"publisher","first-page":"797","DOI":"10.1016\/j.semarthrit.2020.05.017","volume":"50","author":"M Akiyama","year":"2020","unstructured":"Akiyama M, Kaneko Y, Takeuchi T. Effectiveness of tocilizumab in Behcet\u2019s disease: a systematic literature review. Semin Arthritis Rheum. 2020;50(4):797\u2013804.","journal-title":"Semin Arthritis Rheum"},{"key":"3708_CR10","doi-asserted-by":"publisher","first-page":"624795","DOI":"10.3389\/fmed.2021.624795","volume":"8","author":"E Alpsoy","year":"2021","unstructured":"Alpsoy E, Leccese P, Emmi G, Ohno S. Treatment of Beh\u00e7et\u2019s Disease: an algorithmic Multidisciplinary Approach. Front Med (Lausanne). 2021;8:624795.","journal-title":"Front Med (Lausanne)"},{"key":"3708_CR11","unstructured":"https:\/\/www.uptodate.com\/contents\/treatment-of-behcet-syndrome?search=behcet%20pathophysiology&topicRef=8223&source=related_link#H67007445"}],"container-title":["BMC Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12883-024-03708-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12883-024-03708-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12883-024-03708-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,6,14]],"date-time":"2024-06-14T07:06:52Z","timestamp":1718348812000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmcneurol.biomedcentral.com\/articles\/10.1186\/s12883-024-03708-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6,14]]},"references-count":11,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["3708"],"URL":"https:\/\/doi.org\/10.1186\/s12883-024-03708-x","relation":{},"ISSN":["1471-2377"],"issn-type":[{"value":"1471-2377","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,6,14]]},"assertion":[{"value":"16 February 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 June 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 June 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":"Informed consent has been obtained from the patient for the use and publication of the case information provided in an online open-access publication. No images have been used.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"No ethics approval was required. Consent for participation is not applicable as this is a retrospective case report; thus the patient was treated and did not enrol in a study beforehand.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"The patient was agreeable to publication of the case. No images have been published. Written consent form has been uploaded as well, as a related file.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"Not applicable.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"203"}}