Primary angiitis of the central nervous system

Primary angiitis of the central nervous system (PACNS), also known as primary central nervous system vasculitis or isolated angiitis of the central nervous system, is a rare inflammatory disorder that affects blood vessels exclusively within the brain and spinal cord. Unlike systemic vasculitides, PACNS is confined to the central nervous system (CNS) without involvement of blood vessels in other organs. The inflammation damages the walls of small and medium-sized arteries and, less commonly, veins within the CNS, leading to narrowing or occlusion of these vessels and resulting in reduced blood flow to brain tissue. The clinical presentation of PACNS is highly variable, which often makes diagnosis challenging. Key symptoms include severe headaches (often the most common presenting feature), cognitive dysfunction, focal neurological deficits such as weakness or sensory changes, stroke or transient ischemic attacks, seizures, and altered consciousness. Some patients experience a progressive decline in cognitive function, while others present acutely with stroke-like episodes. The disease can follow a relapsing-remitting or progressive course. Diagnosis typically requires a combination of clinical findings, brain MRI showing characteristic lesions, cerebral angiography demonstrating vessel irregularities, and in many cases, brain biopsy to confirm vasculitic changes in vessel walls. The cause of PACNS remains unknown, and it is not considered a hereditary condition. Treatment is based primarily on immunosuppressive therapy. Initial treatment typically involves high-dose corticosteroids, often combined with cyclophosphamide for induction of remission. Maintenance therapy may include agents such as azathioprine or mycophenolate mofetil. Early diagnosis and prompt initiation of treatment are critical to prevent irreversible neurological damage. With appropriate immunosuppressive therapy, many patients achieve remission, although relapses can occur and long-term monitoring is essential.

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