Adult primary central nervous system vasculitis

Abstract

The aim of this review was to cover the recent major advances made in the fields of clinical assessment, diagnosis and treatment of adult primary central nervous system vasculitis (PCNSV). To prevent misdiagnosis, particularly with the reversible cerebral vasoconstriction syndromes, new criteria on the basis of the levels of certainty of the diagnosis of PCNSV have been proposed. Advances in the neuroimaging techniques visualizing the wall of intracranial blood vessels have improved the capacity to distinguish inflammatory from noninflammatory vascular lesions. These techniques could play in the future an important role in the diagnosis of PCNSV. Studies of larger numbers of cases have revealed a more varied histopathological inflammatory picture and disclosed an association with amyloid angiopathy. It has also been recognized that PCNSV 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. Our better understanding of the PCNSV spectrum and its subsets will facilitate early recognition. This may facilitate earlier treatment and may prevent irreversible or even lethal outcomes.