Adult primary central nervous system vasculitis (aPCNSV) is an uncommon disorder of unknown cause that is limited to the brain and spinal cord. The median age of onset is 50 years. The neurological manifestations are diverse, but commonly include headache, altered cognition, focal weakness, or stroke. 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 magnetic resonance imaging (MRI) of the brain. Biopsy of central nervous system tissue showing vasculitis is the only definitive test, however angiography has often been used for diagnosis even though it has only moderate sensitivity and specificity. To prevent misdiagnosis, particularly with the reversible cerebral vasoconstriction syndromes, new criteria based on the levels of certainty of the diagnosis of aPCNSV have been proposed. Advances in the neuroimaging techniques visualizing the wall of intracranial blood vessels have improved the capacity to distinguish inflammatory from non-inflammatory vascular lesions. These techniques could play in the future an important role in the diagnosis of aPCNSV. Studies of larger number of cases have revealed a more varied histopathological inflammatory picture and disclosed an association with amyloid angiopathy. It has also been recognized that aPCNSV is a heterogeneous disorder encompassing clinical subsets that differ in terms of prognosis and therapy. Finally, differently from earliest reports that suggested a poor prognosis with a fatal outcome in the majority of the cases, a large recent study from Mayo Clinic has described a more favorable course with good response to therapy and a favorable outcome in 81% of cases. Glucocorticoids alone or in combination with cyclophosphamide constitute the first line of treatment of PCNSV. TNFalpha blockers and mycophenolate mofetil can successfully treat patients with resistant disease.Early recognition is important because treatment with corticosteroids with or without cytotoxic drugs may prevent serious outcomes. A better understanding of the molecular mechanisms associated with the pathogenesis of aPCNSV should provide opportunities to improve therapy.
Disclosure of Interest None Declared