Treatment of neuro-Behçet's disease: an update

Neurological manifestations of Behçet's disease (neuro-Behçet's disease) present in 5-30% of cases. They are classified into parenchymal and nonparenchymal categories. Poor prognostic factors include multifocal involvement, spinal presentations, more than two attacks per year, progressive course and increased cerebrospinal fluid cell count and protein content at the time of neurologic manifestations. For patients with parenchymal neuro-Behçet's disease without any poor prognostic factor, azathioprine or methotrexate and corticosteroids are recommended as the first-line treatment. For high-risk patients, intravenous cyclophosphamide and corticosteroids are recommended. If these regimens failed, TNF-alpha-blocking drugs, such as infliximab or etanercept, should be added. Alternating IFN-alpha and then chlorambucil or experimental treatments are the last resorts for most refractory patients. Treatment of venous sinus thrombosis is achieved by using anticoagulation and short-term corticosteroids with or without immunosuppressants.