Neuro-Behcets syndrome consists of acute type and chronic progressive type (primary progressive and secondary progressive). Attacks of acute type neuro-Behcets syndrome are sometimes self-limiting. However, when the neurological manifestations are progressive and severe, administration of corticosteroid is necessary. In addition, infliximab and interferon alpha might also be effective in acute type neuro-Behcets disease. There are no drugs which have been demonstrated to be effective in preventing the occurrence of attacks of acute type neuro-Behcets disease. Colchicine, low dose of steroids and various immunosuppressive drugs have been used anecdotally for this purpose. As to chronic progressive neuro-Behcets syndrome, one should realize that corticosteroids are not effective. Cyclophosphamide is not effective, either. Low dose methotrexate (MTX) has been shown to be beneficial for the treatment of chronic progressive neuro- Behcets syndrome by an open clinical trial. Thus, low dose MTX has been shown to decrease cerebrospinal fluid IL-6 levels without progression of neuropsychological manifestations, although there are a fraction of patients who do not adequately respond to MTX. Preliminary results indicate that infliximab has a beneficial effect in such patients with MTXresistant chronic progressive neuro-Behcets syndrome.
Keywords: IFN-α, colchicine, cyclosporin A, methotrexate, MRI, infliximab, cerebrospinal fluid, IL-6
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