CEREBRAL VASCULITIS - DIAGNOSIS AND CURRENT TREATMENT RECOMMENDATIONS

The vasculitides are a heterogeneous group of disorders that involve i nflammation, necrosis and, ultimately, occlusion of blood vessels. The y can have variable CNS involvement, with some, such as isolated angii tis of the CNS (IACNS) and Takayasu's arteritis, predominantly or excl usively involving the CNS, while others only occasionally cause neurol ogical complications. The latter syndromes include Wegener's granuloma tosis, systemic lupus erythematosus and disorders that mimic vasculiti s such as the antiphospholipid antibody syndrome. The treatment of vas culitides is generally with variable doses of corticosteroids and immu nosuppressants. Prednisone (oral) and methylprednisolone (usually intr avenous) are the most commonly used corticosteroids. Cyclophosphamide is one of the most potent drugs available to treat severe, life-threat ening vasculitis. It is given as an oral or intravenous single daily d ose regimen or as intravenous pulse therapy. The folate antagonist met hotrexate is a useful steroid-sparing agent, and is often added to a c orticosteroid regimen. Azathioprine, chlorambucil and cyclosporin have also been used for the treatment of vasculitis, but experience with t hese agents is limited. Newer therapies with possible, but still unpro ven, efficacy are intravenous immunoglobulin and monoclonal antibodies . Although general treatment guidelines can be suggested, treatment sh ould be individualised for each vasculitic syndrome.