Adult Primary Central Nervous System Vasculitis

Primary central nervous system vasculitis (PCNSV) is an uncommon and poorly understood form of vasculitis that it is limited to the brain and spinal cord. PCNSV represents the most frequent vasculitis involving the central nervous system (CNS. The neurological manifestations are diverse and non-specific. Serological markers of inflammation are usually normal. Cerebrospinal fluid is abnormal in approximately 80–90% of the cases. The diagnosis is unlikely in the presence of a normal brain magnetic resonance imaging (MRI) scan. Biopsy of CNS tissue showing vasculitis is the only definitive test; however, angiography is often used to confirm the diagnosis. Early recognition is important because treatment with glucocorticoids with or without cytotoxic drugs may prevent serious or even lethal outcomes. The differential diagnosis is broad and includes reversible vasoconstriction syndromes, secondary cerebral vasculitis, malignancy, and infections. Modern recognition of PCNSV as a separate entity is generally dated to the mid-1950s when Cravioto and Feigin described several cases of a “non-infectious granulomatous angiitis” with a predilection for the nervous system. The term “granulomatous angiitis of the nervous system” was applied because of the histopathologic findings observed in arteries from initial cases. Recently, major advances have been made in the field of PCNSV. Studies of additional cases have revealed a more varied histopathologic inflammatory picture and an association with amyloid angiopathy. They have also recognized that PCNSV is more heterogeneous than previously thought, encompassing clinical subsets that differ in terms of prognosis and therapy. Finally, childhood PCNSV (cPCNSV) has been recognized as possible cause of vascular strokes in children. This review aimed to provide an update on the major advances made in adult PCNSV.