Central nervous system (CNS) vasculitis encompasses a broad range of diseases characterized by inflammation of blood vessels within the brain, spinal cord, and meninges. This review focuses on Primary Angiitis of the CNS (PACNS), a unique form affecting only the CNS without other organ involvement. PACNS primarily affects the small- and medium-sized arteries in the brain, leading to neurological symptoms. Early recognition and intervention are essential for optimal management. This case report of a 25-year-old male highlights the diagnostic challenges, where a multidisciplinary approach was necessary to diagnose PACNS, given initial negative results. The paper also explores the etiology and pathogenesis of PACNS and discusses the diagnostic criteria proposed by researchers. Medical professionals primarily base management strategies on induction and maintenance therapies, utilizing a combination of glucocorticoids and immunosuppressants as the standard treatment. However, the exact dosages and administration methods need further research due to the absence of randomized clinical trials. The review underscores the need for continued research to enhance diagnostic and treatment protocols, given the severity of PACNS.
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