Transgenic Technology Research CenterShiraz University of Medical Sciences
Department of NeurologyShiraz University of Medical Sciences
Research Center for Traditional Medicine and history of medicineShiraz University of Medical Sciences
Cite this article as:
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.