Primary angiitis of the central nervous system presenting as sudden vision loss in a young patient

Abstract

Primary angiitis of the central nervous system is a rare disorder that primarily affects the cerebral vessels and can prove to be a diagnostic challenge to the clinicians. It can present with a variety of clinical manifestations and lacks the evidence of any systemic disease. Digital subtraction angiography is often necessary to confirm the diagnosis of cerebral vasculitis but in cases where it may fail to do so, brain biopsy is eventually required. Obliviousness to this condition may lead to false management strategies and hence it is imperative to make an accurate diagnosis to facilitate proper treatment that mainly includes a combination of steroids and immunosuppressive agents. We report a case of a 27-year-old male with sudden bilateral vision loss who underwent extensive workup and was ultimately diagnosed with primary angiitis of the central nervous system. He was treated with steroids and responded well to it.