Primary central nervous system vasculitis: A diagnostic and therapeutic challenge. A series of 7 patients

Ruiz-Nieto, N.; Aparicio-Collado, Héctor; Segura-Cerdá, Ana; Barea-Moya, Lucas; Zahonero-Ferriz, A.; Campillo-Alpera, M.S.; Vilar-Fabra, Carlos

doi:10.1016/j.nrleng.2023.04.004

Cited by 2 publications

(3 citation statements)

References 19 publications

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“…This leads to narrowing or occlusion of these blood vessels 9 . Subsequently, haemorrhagic and ischaemic events will occur in the adjacent tissues, which will cause diverse clinical presentations 6 . It constitutes about 1.2% from all the vasculitis that involve the CNS 3 .…”

Section: Discussionmentioning

confidence: 99%

“…Morbidity, mortality rate, and relapses have significantly decreased in favour of the above regimen. Patients with this disease has a mortality rate reaching about 6–16% 11 , and relapses happen in approximately one-third of the patients 6 . They may have relapses of existing or new symptoms other than headache, and the existing lesions on MRI may worsen or new lesions may develop 11 .…”

Section: Discussionmentioning

confidence: 99%

“…Differentiating primary CNS angiitis from other mimickers clinically and radiologically is very crucial as early treatment with immunosuppressive therapy is considered a good prognostic factor 5 . In addition, other mimickers of primary CNS angiitis may be exacerbated by immunosuppressive therapy 6 . In this case report, we present a case of a 24-year-old female patient who presents with various neurological complaints in a progressive course.…”

Section: Introductionmentioning

confidence: 99%

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Primary central nervous system angiitis: a case report

Zeer,

Arda,

Abu Salim

et al. 2024

Annals of Medicine &Amp; Surgery

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Introduction: Primary central nervous system angiitis is a rare idiopathic vasculitis that is limited to the central nervous system. It has a wide range of clinical presentations that can mimic other vasculopathies. Case presentation: A 24-year-old female patient presents with various nonspecific neurological complaints in a progressive course. After a challenging diagnostic workup, she was diagnosed by tissue biopsy to have primary central nervous system angiitis. Discussion: Although primary central nervous system angiitis has been reported increasingly recently, its pathogenesis is still unknown and its diagnosis is still very challenging. No universal criteria have been adopted, and there is no laboratory test or imaging modality with sufficient sensitivity and specificity to confirm the diagnosis and exclude other mimickers. Conclusion: To prevent misdiagnosis, clinicians treating patients with suspected primary central nervous system angiitis should be aware of its differentials.

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Section: Discussionmentioning

confidence: 99%