Miller-Fisher综合征的临床特点:22例病例分析及文献回顾

Characteristics of Clinical Significance of Miller-Fisher Syndrome: Analysis on 22 Cases and Literatures Review

目的:分析Miller-Fisher综合征的临床特点及发病机制,以提高对本病的诊治水平。方法:对22例诊断为Miller-Fisher综合征患者起病形式、临床表现、辅助检查、治疗及预后进行回顾性分析,并综合文献,阐述该病的临床特点、病因和发病机制。结果:22例患者发病年龄12～67岁(平均38岁),均呈急性或亚急性起病;常见的神经系统症状包括复视、眩晕、四肢乏力;主要的神经系统体征包括眼外肌麻痹、共济失调、腱反射减弱或消失;实验室检查:脑脊液蛋白-细胞分离16例,肌电图神经性损害12例,所有患者CT和MRI检查均未发现异常。所有患者均予激素联合免疫球蛋白治疗预后良好。结论:Miller-Fisher综合症临床症状复杂,根据临床特点、结合脑脊液检查及肌电图检测能确诊,大剂量免疫球蛋白联合激素治疗预后良好。

Objective： To investigate the clinical features and pathogenesis of Miller Fisher syndrome （ MFS ）, and deepen understanding of the disease. Method： 22 patients with MFS were analyzed retrospectively on the pattern of onset, clinical characteristics, laboratory findings, treatment and prognosis. The literature was reviewed to study the cause and clinical characteristics of MFS. Result： The MFS mainly occurred in 12-67 years （ average age was 38 years old ）with acute or subacute onset. The initial symptoms included piplopia, vertigo and weakness. The major physical sign included ophthalmoplegia externa, ataxia, and weak or disappeared tendon reflex. All the patients were cheeked for cerebrospinal fluid and electrophysiological examination （EMG） . Albumino-cytological separation was noted in 16 cases; 12 cases of auxiliary fibers injury were found in EMG. There was no positive findings in computer tomography （ CT ） or magnetic resonance imaging （ MRI ） . All the patients had received in intravenous immunoglobulin combining with steroids after the diagnosis, and were obviously improved at discharges. Conclusion： Miller-Fisher syndrome had a complex clinical characteristics. The diagnosis depends on the clinical features, CSF, EMG. It can be treated effectively by vein injection ofimmunoglobulin combining with steroids.