RESUMO
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 non-specific neurological complaints in a progressive course. After a challenging diagnostic work-up, 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.
