Miller-Fisher综合征22例临床分析

摘要

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 plasmaphoresis, 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.%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.