CAJ | 학술논문

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。
목적：분석Miller-Fisher종합정적림상특점급발병궤제，이제고대본병적진치수평。방법：대22례진단위Miller-Fisher종합정환자기병형식、림상표현、보조검사、치료급예후진행회고성분석，병종합문헌，천술해병적림상특점、병인화발병궤제。결과：22례환자발병년령12~67세（평균38세），균정급성혹아급성기병；상견적신경계통증상포괄복시、현훈、사지핍력；주요적신경계통체정포괄안외기마비、공제실조、건반사감약혹소실；실험실검사：뇌척액단백-세포분리16례，기전도신경성손해12례，소유환자CT화MRI검사균미발현이상。소유환자균여격소연합면역구단백치료예후량호。결론：Miller-Fisher종합증림상증상복잡，근거림상특점、결합뇌척액검사급기전도검측능학진，대제량면역구단백연합격소치료예후량호。
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 of immunoglobulin combining with steroids.