急性外展神经麻痹起病的儿童Miller-Fisher变异型1例  

A case of incomplete Miller-Fisher syndrome in a child presenting as acute-onset of isolated abducent paralysis

作　　者：张田田 张礼萍[1] 王诗雨 刘辉 Zhang Tiantian;Zhang Liping;Wang Shiyu;Liu Hui(Department of Pediatrics,Xuanwu Hospital of Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院儿科,北京100053

出　　处：《中华神经科杂志》2024年第9期1016-1019,共4页Chinese Journal of Neurology

摘　　要：Miller-Fisher综合征(MFS)是吉兰-巴雷综合征(GBS)的一种特殊变异型,典型特征为共济失调、腱反射消失和眼肌麻痹。该病发病率较低,儿童较成人更少见,以孤立性双侧外展神经麻痹为突出表现的不完全MFS更是罕见报道。文中报道1例9岁男童,在前驱呼吸道感染后,以双侧眼球内聚、复视急性起病,体检双侧腱反射不对称性减弱或消失,而共济运动早期正常,高峰期仅轻微失调。尽管缺乏脑脊液蛋白-细胞分离现象,结合血清抗GQ1b-IgG、GT1a-IgG、GD1b-IgG、GD2-IgG等多种抗体阳性以及肌电图H反射、感觉运动传导异常,我们仍诊断为MFS。发病第17天首次予丙种球蛋白冲击治疗(2 g/kg,分5 d给药),治疗后双侧膝腱反射正常,共济运动稳准,但眼位及复视未完全恢复。发病第57天再次予丙种球蛋白冲击治疗,最终在发病2个月后,其双侧眼外直肌麻痹及复视完全恢复。该病例提示以孤立性双侧眼外直肌麻痹急性起病者,也需考虑MFS或不完全MFS可能,早期血清或脑脊液神经节苷脂抗体及肌电图检查有利于诊断,或可避免延误病情。Miller-Fisher syndrome(MFS)is a specific variant of Guillain-Barrésyndrome(GBS),characterized by ataxia,loss of tendon reflexes and ophthalmoplegia.The incidence of MFS is relatively low,less common in children than in adults.Incomplete MFS highlighted by isolated bilateral abducens palsy is rarely reported.A 9-year-old boy with acute onset of bilateral lateral rectus asymmetry paralysis after a prodromal respiratory infection was reported in this article.The bilateral tendon reflex asymmetry was weakened/disappeared.And the ataxic movement was normal at the early stage and only slightly disordered during the peak hours.Despite the absence of cerebrospinal fluid protein-cell segregation,MFS was diagnosed since anti-GQ1b-IgG,GT1a-IgG,GD1b-IgG,GD2-IgG positive in serum and electromyographic H-reflex,sensory-motor conduction abnormalities.On the 17th day after onset,intravenous immunoglobulin therapy(2 g/kg,5 d)was given for the first time.After treatment,the bilateral knee tendon reflexes were normal and the mutual motion was stable,but the eye position and diplopia were not completely recovered.On the 57th day after onset,the patient was treated with intravenous immunoglobulin again.Finally,the bilateral extraocular direct muscle paralysis and diplopia completely recovered 2 months after the onset.This case suggests that MFS or incomplete MFS should also be considered in patients with acute onset of isolated bilateral ophthalmoplegia.Early serum or cerebrospinal fluid ganglioside antibodies and electromyography may be beneficial to diagnosis or avoid delay of illness.

关 键 词：Miller-fisher变异型 外展神经麻痹 神经节苷脂抗体 

分 类 号：R748[医药卫生—神经病学与精神病学]