{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T16:08:39Z","timestamp":1783958919146,"version":"3.55.0"},"reference-count":21,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T00:00:00Z","timestamp":1783900800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Neuro-Beh\u00e7et syndrome (NBS) is a rare but detrimental neurological manifestation of the Beh\u00e7et syndrome (BS) \u2013 a chronic multisystemic inflammatory disease. NBS is frequently associated with brainstem and spinal cord lesions, often resulting in substantial neurological disability. Atypical clinical presentations can pose significant challenges for diagnosis and treatment of NBS. Here, we report the clinical course, treatment strategies and responses of two cases with probable NBS. Both cases presented with longitudinally extensive transverse myelitis and brainstem lesions and experienced recurrent relapses as well as disease progression resistant to multiple immunotherapies. Autologous hematopoietic stem cell transplantation (aHSCT) was without beneficial effect on the disease course in both cases. In summary, NBS or probable NBS should be considered in patients with myelitis and brainstem involvement with limited treatment response. These cases illustrate diagnostic challenges and limitations of current diagnostic criteria, while also underscoring the heterogeneity of disease presentation. Immune reset by aHSCT failed to confer clinical benefit as a rescue therapy in these two patients. Further studies are required to optimize therapeutic strategies for NBS.<\/jats:p>","DOI":"10.3389\/fimmu.2026.1864802","type":"journal-article","created":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T15:43:19Z","timestamp":1783957399000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Two cases of probable Neuro-Beh\u00e7et syndrome treated with autologous HSCT"],"prefix":"10.3389","volume":"17","author":[{"given":"Charlotte","family":"Schubert","sequence":"first","affiliation":[{"name":"Institute of Neuroimmunology and Multiple Sclerosis (INIMS) and Department of Neurology, University Medical Centre Hamburg-Eppendorf","place":["Hamburg, Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lea I.","family":"Walter","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital Zurich and University of Zurich","place":["Zurich, Switzerland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marina","family":"Herwerth","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital Zurich and University of Zurich","place":["Zurich, Switzerland"]},{"name":"Neuroscience Centre Zurich, University of Zurich and ETH Zurich","place":["Zurich, Switzerland"]},{"name":"Institute of Pharmacology and Toxicology, University of Zurich","place":["Zurich, Switzerland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Imke","family":"Metz","sequence":"additional","affiliation":[{"name":"Institute of Neuropathology, University Medical Centre G\u00f6ttingen","place":["G\u00f6ttingen, Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jakob","family":"Nilsson","sequence":"additional","affiliation":[{"name":"Department of Immunology, University Hospital Zurich","place":["Zurich, Switzerland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilijas","family":"Jelcic","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital Zurich and University of Zurich","place":["Zurich, Switzerland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Roth","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital Zurich and University of Zurich","place":["Zurich, Switzerland"]},{"name":"Neuroscience Centre Zurich, University of Zurich and ETH Zurich","place":["Zurich, Switzerland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolaus","family":"Kr\u00f6ger","sequence":"additional","affiliation":[{"name":"Department of Stem Cell Transplantation, University Medical Centre Hamburg-Eppendorf","place":["Hamburg, Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ina","family":"K\u00f6tter","sequence":"additional","affiliation":[{"name":"Division of Rheumatology and Systemic Inflammatory Diseases, III. 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