Potential New Therapeutic Options for Involvement of Central Nervous System in Behcet's Disease (Neuro-Behcet's Syndrome)

Neuro-Behcet's syndrome consists of acute type and chronic progressive type (primary progressive and secondary progressive). Attacks of acute type neuro-Behcet's 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-Behcet's disease. There are no drugs which have been demonstrated to be effective in preventing the occurrence of attacks of acute type neuro-Behcet's disease. Colchicine, low dose of steroids and various immunosuppressive drugs have been used anecdotally for this purpose. As to chronic progressive neuro-Behcet's 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-Behcet's 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 MTX-resistant chronic progressive neuro-Behcet's syndrome.