Abstract
Central nervous system involvement in Behçet’s disease, usually called neuro- Behçet’s disease (NB), is classified in acute type and chronic progressive type. Acute NB is characterized by acute meningoencephalitis with or without focal lesions, presenting high intensity areas in T2-weightened images on magnetic resonance imaging (MRI) scans. Cyclosporin (CyA)-related acute neurological manifestations are almost identical with CyA-unrelated acute NB in terms of clinical manifestations, laboratory data and responses to steroids, except for the paucity of relapse on discontinuation of CyA. Chronic progressive NB is characterized by slowly progressive dementia, ataxia and dysarthria with persistent elevation of cerebrospinal fluid (CSF) IL-6 and progressive brainstem atrophy on MRI. Diagnostic criteria have been proposed depending on the results of retrospective multicenter cohort studies, using CSF cell counts for acute NB and CSF IL-6 with brain stem atrophy on MRI for chronic progressive NB, respectively. Acute attacks of acute NB well respond to moderate to high doses of corticosteroids, whereas colchicine is effective to prevent relapses of acute NB especially in patients who developed acute NB in the absence of CyA. Chronic progressive NB is resistant to conventional treatment with steroid, cyclophosphamide, or azathioprine, but responds to low dose methotrexate. Thus, methotrexate has been shown to reduce the rate of mortality and severe disability significantly in a retrospective cohort study. Infliximab has been also found to be effective in patients with chronic progressive NB in case of inadequate responses to methotrexate.
