岛盖综合征的临床分析  

Clinical analysis of Foix-Chavany-Marie syndrome

作　　者：董立羚[1] 周祥琴[1] 

机构地区：[1]中国医学科学院北京协和医院神经科,100730

出　　处：《中华神经科杂志》2016年第7期560-563,共4页Chinese Journal of Neurology

摘　　要：目的 分析并总结岛盖综合征的临床特点。方法回顾性分析4例典型岛盖综合征患者，阐述其临床特点；同时结合文献，简述本病的发病机制。结果4例患者均表现出流涎、面瘫、构音障碍和吞咽障碍等随意运动障碍，伴有反射运动保留。头颅MRI显示双侧岛盖皮质或皮质下病变。3例患者的病因考虑为脑炎相关，另1例考虑为围产期缺血缺氧性脑病。抗癫痫治疗后，癫痫发作得到控制，但是其他症状持续存在。结论自主一随意运动分离是岛盖综合征的特征性表现。本病通常见于双侧岛盖或第一运动区皮质一皮质下的结构性或功能性病变。病因多样，包括脑炎、脑血管病等，与预后相关。Objective To summarize the clinical characteristics of Foix-Chavany-Marie syndrome. Methods The clinical, electroencephalography and imaging characteristics, as well as etiology, treatment and prognosis of 4 cases of Foix-Chavany-Marie syndrome were retrospectively evaluated, and relevant literature was reviewed to investigate its pathogenesis. Results All the 4 cases presented with hypersalivation, facial diplegia, dysarthria and dysphagia with autonomic-voluntary dissociation. Brain MRI showed bilateral cortical or subcortical lesions in the opercula and perisylvian areas. Three cases were secondary to encephalitis, and 1 case was related to perinatal hypoxic-ischemic encephalopathy. After administration of antiepileptic drugs, they were free of seizures. But all the other symptoms remained. Conclusions Autonomic-voluntary dissociation is a feature of Foix-Chavany-Marie syndrome. Most patients are due to bilateral structural or functional lesions between motor cortices and brain-stem cranial motor nuclei. The prognosis depends on the heterogeneous etiologies, such as encephalitis and cerebrovascular disease.

关 键 词：岛盖综合征 皮质型假性球麻痹 发病机制 

分 类 号：R741[医药卫生—神经病学与精神病学]