Miller-Fisher综合征21例临床分析

Clinical analysis of 21 cases of Miller-Fisher syndrome

目的分析Miller-Fisher综合征的临床特点及发病机制。方法回顾性分析2010年3月—2015年4月首都医科大学附属北京同仁医院神经内科收治的21例Miller-Fisher综合征患者的临床资料,分析全部患者的发病原因、临床表现、辅助检查、诊断、治疗及预后。结果 21例患者中男6例,女15例,发病年龄14—69(40.1±16.0)岁,主要体征为眼外肌麻痹、共济失调、腱反射减弱或消失。辅助检查:脑脊液蛋白—细胞分离18例(85.7%),GQ1b抗体检测9例,阳性8例(88.9%);神经电生理检查提示周围神经及神经根损害6例(28.6%);头颅MR检查均无异常。单用人免疫球蛋白治疗10例,单用激素治疗8例,应用IVIG联合激素治疗3例。出院时症状均明显好转。结论Miller-Fisher综合征临床症状复杂,既可表现为典型三联征,又可表现为不完全型如急性眼外肌麻痹。根据临床特点、结合脑脊液检查和GQIb抗体可明确诊断。予人免疫球蛋白或激素治疗预后良好。

Objective To analyze the clinical features and pathogenesis of Fisher-Miller syndrome.Methods The clinical data of 21 patients with MFS in the hospital from March 2010 to April 2015 were analyzed retrospectively, and the causes, clinical manifestations, laboratory findings, diagnosis, treatment and prognosis were analyzed.Results In 21 cases, 6 cases were male and 15 were female.The age ranged from 14 to 69 （40.1 ±16.0） years.The main signs were external muscle paralysis, ataxia, weakening or disappearance of the tendon reflex.Auxiliary examination：protein cell separation in 18 cases （85.7%）, 9 cases of GQ1b antibody positive in 8 cases （88.9%）;electrophysiological examination revealed 6 cases of peripheral nerve and nerve root injury （28.6%）; the head MR examination showed no abnormality.The treatment of 10 cases with single use of Human Immunoglobulin, 8 cases of hormone therapy alone, the use of IVIG combination hormone therapy in 3 cases.Discharge symptoms were significantly improved.Conclusion Miller-Fisher syndrome symptom complex, both showed the typical triad, but also showed an incomplete type, such as acute extraocular muscle paralysis.According to the clinical features, combined with cerebrospinal fluid examination and GQ1b antibodies can confirm the diagnosis.To human immunoglobulin or hormone treatment prognosis is good.