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After extensive investigation, a demyelinating central nervous system (CNS) disease was suspected; however, a differential diagnosis with primary CNS vasculitis was also considered. Despite treatment with high-dose corticosteroids, plasma exchange and immunoglobulins, the patient\u2019s neurological condition continued to deteriorate. A brain biopsy confirmed an active demyelinating process. A diagnosis of aggressive multiple sclerosis was established. Immunotherapy with ocrelizumab resulted in clinical stabilisation. This case highlights the diagnostic challenges of atypical demyelinating presentations and the critical role of brain biopsy in guiding early treatment in fulminant cases.<\/jats:p>","DOI":"10.1136\/bcr-2025-268629","type":"journal-article","created":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T16:15:28Z","timestamp":1777479328000},"page":"e268629","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Aggressive phenotype of multiple sclerosis, characterised by atypical initial symptoms and a fulminant clinical progression"],"prefix":"10.1136","volume":"19","author":[{"given":"Kintija","family":"Grit\u0101ne","sequence":"first","affiliation":[{"name":"Department of Neurology and Neurosurgery, Riga Stradi\u0146\u0161 University, Riga, Latvia"},{"name":"Department of Neurology and Neurosurgery, Riga East Clinical University Hospital, Riga, Latvia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katr\u012bna","family":"Podo\u013cska","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurosurgery, Riga Stradi\u0146\u0161 University, Riga, Latvia"},{"name":"Department of Neurology and Neurosurgery, Riga East Clinical University Hospital, Riga, Latvia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9586-2775","authenticated-orcid":false,"given":"Guntis","family":"Karelis","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurosurgery, Riga Stradi\u0146\u0161 University, Riga, Latvia"},{"name":"Department of Neurology and Neurosurgery, Riga East Clinical University Hospital, Riga, Latvia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8056-7087","authenticated-orcid":false,"given":"Daina","family":"Pastare","sequence":"additional","affiliation":[{"name":"Department of Neurology and Neurosurgery, Riga Stradi\u0146\u0161 University, Riga, Latvia"},{"name":"Department of Neurology and Neurosurgery, Riga East Clinical University Hospital, Riga, Latvia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2026,4,29]]},"reference":[{"key":"2026042909151208000_19.4.e268629.1","doi-asserted-by":"crossref","first-page":"1759","DOI":"10.1093\/brain\/awn098","article-title":"Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis","volume":"131","author":"Lucchinetti","year":"2008","journal-title":"Brain (Bacau)"},{"key":"2026042909151208000_19.4.e268629.2","first-page":"395","article-title":"Aggressive multiple sclerosis: proposed definition and treatment algorithm","volume":"9","author":"Ratchford","year":"2013","journal-title":"Nat Rev Neurol"},{"key":"2026042909151208000_19.4.e268629.3","first-page":"687","article-title":"Tumefactive demyelinating lesions: clinical findings, MRI features and follow-up","volume":"251","author":"Kastrup","year":"2004","journal-title":"J Neurol"},{"key":"2026042909151208000_19.4.e268629.4","first-page":"63","article-title":"Tumefactive multiple sclerosis presenting with seizures and confusion","volume":"201","author":"Maramattom","year":"2002","journal-title":"J Neurol Sci"},{"key":"2026042909151208000_19.4.e268629.5","first-page":"34","article-title":"Differentiating tumefactive demyelinating lesions from glioma and lymphoma: a review of imaging features","volume":"3","author":"Patel","year":"2015","journal-title":"Curr Radiol Rep"},{"key":"2026042909151208000_19.4.e268629.6","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1606468"},{"key":"2026042909151208000_19.4.e268629.7","unstructured":"National Multiple Sclerosis Society . 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