临床儿科杂志 ›› 2021, Vol. 39 ›› Issue (8): 565-.doi: 10.3969/j.issn.1000-3606.2021.08.002

• 神经系统疾病专栏 • 上一篇    下一篇

儿童Miller-Fisher 综合征19 例临床分析

邱玲,曹洁   

  1. 重庆医科大学附属儿童医院全科 儿童发育疾病研究教育部重点实验室 儿童发育重大疾病 国家国际科技合作基地 儿科学重庆市重点实验室(重庆 400014)
  • 发布日期:2021-08-17
  • 通讯作者: 曹洁 电子信箱:caojie 0220 @163 .com

Analysis of clinical characteristics of childhood Miller-Fisher syndrome

QIU Ling, CAO Jie   

  1. Department of General Practice, Children’s Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing 400014, China
  • Published:2021-08-17

摘要: 目的 分析儿童Miller-Fisher综合征(MFS)的临床特点。方法 回顾分析2010年1月—2020年3月住院治 疗的19例MFS患儿的临床资料。结果 19例患儿中男14例、女5例,中位发病年龄4岁(1~13岁),主要表现为眼肌麻痹、 共济失调、腱反射减弱或消失,可伴有面瘫、构音障碍、肌肉或关节疼痛、乏力、吞咽困难等。18例患儿行脑脊液检查,脑 脊液蛋白-细胞分离14例。18例患儿行神经电生理检查,均提示有周围神经及神经根损害。6例患儿行神经节苷脂抗体检 测,阴性4例,抗GM 1抗体阳性1例,抗GQ1b抗体阳性1例。所有患儿行颅脑MRI或CT检查均未见异常。患儿均予营养 神经治疗;3例单用激素口服或输注,12例单用丙种球蛋白冲击治疗,3例丙种球蛋白联合激素治疗,1例未予激素或丙种 球蛋白治疗。所有患儿出院时临床症状均明显好转。结论 MFS临床表现复杂,需结合脑脊液、神经电生理、神经节苷脂 抗体检测综合分析诊断。

关键词: Miller-Fisher综合征; 临床特点; 发病机制; 儿童

Abstract: Objective Analyze the clinical features and pathogenesis of Miller-Fisher syndrome (MFS) in children. Methods The clinical data of 19 patients with MFS who were hospitalized in children’s Hospital Affiliated to Chongqing Medical University from 2010 to 2020 were retrospectively analyzed, including the inducement, onset form, clinical manifestation, auxiliary examination, treatment and efficacy of all patients. Results Among the 19 cases, 14 were male and 5 were female, with an average age of 4 years and 7 months. The main manifestations were ophthalmoplegia, ataxia, weakening or disappearance of tendon reflex, accompanied by facial paralysis, dysarthria, muscle or joint pain, fatigue, dysphagia and so an. Cerebrospinal fluid (CSF) was examined in 18 children, 14 of whom had CSF protein cell separation. The neuroelectrophysiological examination showed that the peripheral nerve and nerve root were damaged. Ganglioside antibody was detected in 6 children, and no abnormality was found in MRI or CT. All the patients were treated with nutritional nerve therapy. In addition, 3 patients were treated with hormone alone, 12 patients were treated with gamma globulin alone, and 3 patients were treated with gamma globulin and hormone. The clinical symptoms of all the patients were significantly improved at the time of discharge. Conclusions The clinical manifestations of children with MFS are highly complex and atypical. We should pay attention to the detailed history and physical examination, and actively improve the detection of cerebrospinal fluid, neuroelectrophysiological examination and ganglioside antibody to make a clear diagnosis. The prognosis was good after treatment with gamma globulin or hormone.

Key words: Miller-Fisher syndrome; clinical characteristics; pathogenesis; child