Borer H, Rüttimann S, Kätterer C
Medizinische Universitäts-Poliklinik, Departement Innere Medizin, Kantonsspital Basel.
Schweiz Med Wochenschr. 1991 May 25;121(21):788-92.
A 20-year-old Turkish patient presented with superior sagittal sinus thrombosis which led to the final diagnosis of Behçet's disease. The frequency of cerebral venous thrombosis is generally underestimated and may be the first clinical manifestation of Behçet's disease. Uveitis as a main diagnostic criterion of Behçet's disease may be absent, especially in young men. In cases of intracranial hypertension, cerebral venous thrombosis and Behçet's disease should be taken into consideration, especially in a high risk population from eastern Mediterranean countries and Japanese islands. Treatment with early anticoagulation in cerebral venous thrombosis, and corticosteroid-pulse therapy in active Behçet's disease, seems to be established today. As a basic therapy of Behçet's disease cyclosporin A and the classic immunosuppressive agents, especially azathioprine, are available. Clinical course and prognosis in cerebral venous thrombosis are generally both benign, provided that adequate management is started in time.
一名20岁的土耳其患者出现上矢状窦血栓形成，最终诊断为白塞病。脑静脉血栓形成的发生率通常被低估，可能是白塞病的首发临床表现。作为白塞病主要诊断标准的葡萄膜炎可能不存在，尤其是在年轻男性中。在颅内高压的情况下，应考虑脑静脉血栓形成和白塞病，特别是在地中海东部国家和日本岛屿的高危人群中。目前似乎已确立脑静脉血栓形成早期抗凝治疗以及活动性白塞病的糖皮质激素冲击治疗。作为白塞病的基础治疗，可使用环孢素A和经典免疫抑制剂，尤其是硫唑嘌呤。如果及时开始适当的治疗，脑静脉血栓形成的临床过程和预后通常都是良性的。
