THUR 150 Treatment and outcomes in primary angiitis of the CNS (PACNS)

Abstract

BackgroundPACNS is a rare and heterogeneous disorder with many uncertainties regarding management and outcomes. Published adult UK series total just 20 patients.MethodHospital record search of an adult tertiary neurosciences centre identified 14 cases of PACNS from 1033 potential cases over 20 years.ResultsAll 14 cases were ‘definite’ by Calabrese Criteria (1988), confirmed by brain biopsy in 8/14 and by angiography in 6/14.The median age of onset was 52 years and most presented with focal deficits (86%), headache (36%) and cognitive change (29%). MRI showed multifocal deep white matter change in 86% and infarcts in 50%. Gadolinium enhancement was seen in 43%. Two patients had solitary ring-enhancing lesions and one patient had signs of cord ischaemia. CSF was abnormal in 90%.All patients received corticosteroids, 11/14 cyclophosphamide, 3/14 Mycophenolate Mofetil, 3/14 Methotrexate and 2/14 Azathioprine. At 13.3 months median follow-up, all but the two patients with solitary lesions remained on immunosuppression. Clinical relapses occurred in 30% and radiological relapse in one case despite immunosuppression.Only 62% had a favorable outcome (mRS 0–2). One patient died as a direct consequence of PACNS and one patient died due to co-morbidities.ConclusionPACNS remains a treatable neuroinflammatory disease with poor outcomes.