Miller-Fisher综合征22例临床分析

目的:探讨Miller-Fisher综合征的临床特点,以提高对本病的诊治水平。方法:回顾性分析22例Miller-Fisher综合征患者发病诱因、临床表现、辅助检查、诊断、鉴别诊断、治疗及预后,并结合文献进行临床分析。结果:Miller-Fisher综合征发病平均年龄约44岁;多为急性或亚急性起病;发病主要症状为复视、四肢乏力;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:所有患者均行脑脊液及电生理检查,其中20例(90.9%)出现蛋白-细胞分离现象,18例(81.8%)提示周围神经及神经根损害。脑CT或MRI检查均未发现异常。16例给予人免疫球蛋白为主的治疗,6例病情严重者给予血浆置换为主的治疗,出院时症状均明显好转。结论:当出现眼外肌麻痹、共济失调、腱反射减弱或消失等症状体征时,应高度考虑Miller-Fisher综合征,行脑脊液及电生理检查以明确诊断,予以人免疫球蛋白或血浆置换的治疗,绝大多数患者预后良好。

Clinical Analysis of 22 Patients with Miller-Fisher Syndrome

Objective:To analyze the clinical characteristics of Miller- Fisher syndrome.Methods: The clinical materials from 22 patients with Miller- Fisher syndrome were analyzed retrospectively. Results:Among the 22 cases,the average age was 44 years old(ranging from 20～63 years) and most of the patients presented acute or subacute onset mode.Main symptoms were diplopia and limbs weakness.Ophthalmoplegia,ataxia,and areflexia were evident.All the patients were checked for cerebrospinal fluid and electrophysiological examination.Albumino-cytological separation was noted in 20 cases(90.9%).The involvement of peripheral nerves or nerve roots was revealed by EMG in 18 cases(81.8%).No abnormalities on brain CT or MRI was found.Sixteen patients had received intravenous IgG therapy,6 plasmapheresis,and 13 corticosteroids treatment.All the patients were obviously improved at discharges.Conclusion:When a patient presents with ophthalmoplegia,ataxia, and areflexia,the diagnosis of Miller-Fisher syndrome should be raised.Cerebrospinal fluid study, electrophysiological tests and brain imaging are needed to clarify the diagnosis.Most of patients will have a satisfactory prognosis using proper treatment,such as intravenous IgG,plasmaphoresis,and/or corticosteroids regimen in acut stage.