Primary CNS angiitis in a woman: A case report with serial MRI evaluation

Primary angiitis of the central nervous system (PACNS) is a rare and potentially life-threatening vasculitis confined to the brain and spinal cord. It is often diagnosed late as it manifests with nonspecific clinical symptoms and has those that are also consistent with other neurological conditions. This work aims to present a PACNS case study of a young female patient in order to demonstrate the importance of neuroimaging in diagnosing and monitoring disease. The 34-year-old woman involved in this case study had a history of strokes, chronic headaches, cognitive deterioration, and visual abnormalities. In order to rule out other possible diagnoses, the patient had laboratory tests, magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA). The neuroimaging indicated multifocal infarcts and vascular constriction that aligns with PACNS. Coagulation profiles and other laboratory testing were unremarkable. After receiving rituximab treatment, the patient's follow-up imaging showed no new infarcts, stabilization, and the elimination of vascular anomalies. There were concerns of a potential infection-related vasculitis that could overlap with PACNS due to a weak vascular channel that resembled an arteriovenous fistula. This case highlights the need to make a diagnosis at an early stage, as well as the importance of advanced imaging and immunosuppressive treatment when managing PACNS. Long-term follow-ups and further investigations into the pathophysiology and treatment of vasculitis of the central nervous system are also critical in identifying vascular complications.