Neuro-Behçet's syndrome with a severe ocular lesion

Behçet's syndrome (BS) is an idiopathic, chronic, relapsing, multisystem, vascular-inflammatory disease. Neuro-Behçet's syndrome (NBS) is a subtype of BS that mainly involves the central nervous system. Because of the heterogeneous involvement of NBS and the limited yearly numbers of new cases of NBS, estimating its course, prognosis, and treatment effect is difficult. Therefore, the efficacy of treatment for any form of NBS is unclear. We experienced a male patient with NBS and severe right uveitis. He received high-dose intravenous methylprednisolone (1000 mg/day) pulse therapy for 3 days. Intravenous injection of cyclophosphamide (400 mg, twice a day) and oral administration of prednisone (40 mg in the morning, 20 mg in the evening) were applied. Uveitis was treated with retrobulbar injection of triamcinolone acetonide (40 mg per week). The patient's response was rapid with improved symptoms and signs. The therapeutic regimen of patients with BS has greatly advanced, leading to evidence-based guidelines. Methylprednisolone pulse therapy is important in treatment of BS. Novel therapeutic options are currently being examined to improve the prognosis of BS. These efforts will undoubtedly shed new light on this complex syndrome.