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Published on: June 7, 2020
[Primary central nervous system vasculitis imitating a brain tumour--a case report]
Małgorzata Wiszniewska1, Tadeusz Szylberg, Marek Harat
1Oddział Neurologii, Szpital Specjalistyczny im. St. Staszica, ul. Rydygiera 1, 64-920 Piła. mpwisz@pi.onet.pl
Primary central nervous system vasculitis (PCNSV) is a rare condition causing neurological symptoms. Despite treatment, this case highlights the challenges in managing PCNSV, leading to a fatal outcome.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Primary central nervous system vasculitis (PCNSV) is a rare condition with an incidence of 2.4 per million person-years.
- PCNSV presents with diverse neurological symptoms, often mimicking brain tumors or stroke.
- Diagnosis is challenging, typically requiring brain biopsy to confirm vascular inflammation.
Observation:
- A 57-year-old male patient was diagnosed with PCNSV via brain biopsy.
- Initial treatment with high-dose corticosteroids for 18 months resulted in a 2.5-year remission.
- Disease recurrence occurred one year after discontinuing glucocorticoid therapy.
Findings:
- PCNSV diagnosis confirmed by pathological findings of transmural vascular inflammation.
- Corticosteroid therapy provided temporary symptom relief.
- Combined therapy with glucocorticoids and cyclophosphamide was ultimately ineffective.
Implications:
- PCNSV poses significant diagnostic and therapeutic challenges.
- Long-term management of PCNSV requires careful monitoring for relapse.
- This case underscores the potential for poor prognosis despite aggressive treatment strategies.
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