Gille M, Sindic C J, Laterre P F, De Hertogh P, Hotermans J M, Selak I, Laterre C
Service de Neurologie, UCL, Bruxelles.
Acta Neurol Belg. 1990;90(5):233-47.
We report four patients with Behçet's disease characterized by initial and predominant neurological signs and symptoms. In three cases, a clinical picture of relapsing meningoencephalitis preceded the appearance of the classical signs of the disease for several months or years; in the fourth case, an acute febrile aseptic meningitis coincided with the development of bipolar aphthosis and uveitis. Disease activity was linked to a blood inflammatory syndrome and neutrophilic leucocytosis. Acute phases were associated with CSF mixed pleocytosis and high protein content. Brain CT scans and MRI were very effective to detect lesions which are mainly located in the brain stem and basal ganglia. High-dose corticosteroids and, in cases of relapses, immunosuppressive drugs were required to treat these severe forms of Behçet's disease.
我们报告了4例以首发和主要神经系统体征及症状为特征的白塞病患者。3例患者在疾病的典型体征出现前数月或数年有复发性脑膜脑炎的临床表现；第4例患者急性发热性无菌性脑膜炎与双相口疮性口炎和葡萄膜炎同时发生。疾病活动与血液炎症综合征及中性粒细胞增多有关。急性期伴有脑脊液混合性细胞增多和高蛋白含量。脑部CT扫描和MRI对检测主要位于脑干和基底节的病变非常有效。治疗这些严重形式的白塞病需要大剂量皮质类固醇，复发时还需要使用免疫抑制药物。
