Borhani Haghighi, A. & Safari, A. Clin Rheumatol (2010) 29: 683. doi:10.1007/s10067-010-1414-6
“Neuro-Behcet’s disease” (NBD) is the constellation of neurologic manifestations as a direct consequence of Behcet’s disease usually confirmed by imaging studies and/or cerebrospinal fluid analysis. The authors propose a therapeutic algorithm for neuro-Behcet’s disease based upon pathological, clinical, prognostic, and medico-economical issues based on available evidences. The authors divide anti-NBD armamentarium to first-line, second-line, and experimental drugs. These drugs should be administered hierarchically in treatment of parenchymal manifestations of neuro-Behcet’s disease. First-line drug include corticosteroids, azathioprine, methotrexate, and cyclophosphamide. Second-line drugs are tumor necrosis factor (TNF) alpha blocking drugs, interferon-α, chlorambucil, and mycophenolate mofenil. Experimental drugs include other “targeted therapies” than anti-TNF antibodies, tolerization therapy and stem cell transplantation. Cerebral venous sinus thrombosis associated with Behcet’s disease should be treated by short-term corticosteroids and anticoagulation with or without immunosuppressive drugs.