Miller-Fisher综合征临床特点及亚型诊断(附27例报告)

目的分析Miller-Fisher综合征(MFS)的临床特点,并对其进行亚型诊断,以加深对其认识,提高诊治水平。方法回顾性分析27例诊断为MFS患者的发病诱因、临床表现、实验室检查、治疗及预后等临床资料,并依据2014年GBS分类专家组制定的Guillain-Barré综合征(GBS)和MFS的新分类和诊断标准进行亚型诊断。所有患者接受脑脊液、肌电图及血清抗GQ-1b抗体检测。结果27例患者平均患病年龄为(41.0±22.6)岁,14例患者有前驱感染史,主要临床表现为复视、步态不稳,主要体征为眼外肌麻痹、共济失调、腱反射减弱或消失等。18例患者出现蛋白细胞分离现象;17例患者血清抗GQ-1b抗体阳性;26例患者出现不同程度的神经根及周围神经受损表现。亚型诊断:典型MFS患者19例,MFS与GBS重叠型(MFS/GBS)5例,急性眼睑下垂(AP)1例,急性瞳孔散大(AM)1例,急性共济失调性神经病(AAN)1例。除1例患者仅接受营养神经等治疗外,余26例患者分别接受了免疫球蛋白和(或)激素冲击治疗,所有患者出院时症状好转。结论 MFS的诊断需要结合患者临床表现、脑脊液检查、神经电生理检查和血清抗GQ-1b抗体等,患者予以免疫球蛋白和(或)激素冲击治疗预后良好。

Clinical features and subtype diagnosis of Miller-Fisher syndrome (report of 27 cases)

Objective To analyze the clinical features and the subtype classification of Miller‐Fisher syndrome (MFS) ,deepen the understanding of disease and improve the level of diagnosis. Methods Twenty‐seven patients with MFS were analyzed retrospectively for the predisposing factors ,clinical features ,laboratory examination , treatment and prognosis. The patients were diagnosed on the basis of 2014 Guillain‐Barré syndrome and MFS‐new diagnostic classif ication. Cerebrospinal fluid ,electromyogram (EMG) and serum anti GQ‐1b antibody detection were performed in all the patients. Results Twenty‐seven patients were with acute or subacute onset ,the average age was (41.0±22.6) years old. Fourteen patients had antecedent infections.Main symptoms were diplopia and unstable walking. The major nervous system signs were ophthalmoplegia externa , ataxia , weaken or disappeared tendon reflex in all patients. Albumino‐cytological separation was noted in 18 patients. The serum anti GQ‐1b antibodies were positive in 17 patients. The involvement of peripheral nerves or nerve roots was revealed by EMG in 26 patients. No abnormalities on brain CT or MRI were found. Subtype diagnosis results were typical MFS in 19 patients ,MFS with GBS overlap type (MFS/GBS) in 5 cases ,acute ptosis (AP) in 1 case ,acute mydriasis (AM) in 1 case ,acute ataxic neuropathy (AAN) in 1 case. Except for 1 case who accepted neurotrophic and other treatment ,26 patients received the intravenous IgG therapy and (or) corticosteroids treatment , the conditions of all patients improved at discharge. Conclusions The diagnosis of MFS should combine clinical manifestations , cerebrospinal fluid examination , electrophysiological examination and anti GQ‐1b antibody testing. It can be treated effectively by intravenous IgG , and/or glucocortieoid pulse therapy in the acute stage.