目的 了解Miller-Fisher综合征(MFS)不同分型以及非典型表现的临床特点。方法 回顾分析19例MFS患儿临床资料。结果 19例患儿中，经典型MFS 10例，不完全MFS（急性眼外肌麻痹）1例，MFS/Bickerstaff脑干脑炎（BBE）重叠2例，MFS/Guillain-Barré综合征（GBS）重叠6例。男7例，女12例，19例均有眼动神经（Ⅲ、Ⅳ、Ⅵ）受损，其中3例存在眼内肌麻痹，7例存在除眼动神经之外的其他颅神经受损，第Ⅶ颅神经受损5例，第Ⅸ、Ⅹ颅神经受损5例。不典型症状中，视物模糊2例，凸眼1例，眼球胀痛1例，上眼睑肿胀1例，头晕2例，头痛1例。8例检测出阳性的周围神经病抗体有GQ1b-IgG、GQ1b-IgM、GD1b-IgM、GD2-IgM、GT1a-IgG、GM1-IgG、GM2-IgM、Sulfatide-IgG、Sulfatide IgM+IgG。1例重症肌无力抗体中的乙酰胆碱受体抗体和兰尼碱受体钙释放通道抗体阳性，最终结合临床进行充分的鉴别诊断后确诊MFS。结论 MFS不同分型有不同特点，存在不典型症状，不应单以抗体作为诊断标准，需结合临床综合分析。

Objective To study the clinical features of different types of Miller-Fisher syndrome (MFS), and to analyze atypical clinical manifestations of MFS. Methods The clinical data of 19 children with MFS were retrospectively analyzed. Results Among the 19 children (7 boys and 12 girls), 10 had classic MFS, 1 had acute ophthalmoparesis (incomplete MFS), 2 had MFS/Bickerstaff brainstem encephalitis (BBE), and 6 had MFS/ Guillain-Barré syndrome (GBS). Nineteen children had the Ⅲ, Ⅳ and Ⅵ cranial nerve palsy, and three children had ophthalmoplegia. There were 7 cases of cranial nerve palsy other than ophthalmic nerve, including 5 cases of Ⅶ cranial nerve palsy, and 5 cases of Ⅸ and Ⅹ cranial nerve palsy. The atypical symptoms included visual deterioration in 2 cases, proptosis in 1 case, ophthalmalgia in 1 case, upper eyelid swelling in 1 case, dizziness in 2 cases, and headache in 1 case. Eight patients had positive peripheral neuropathy antibodies including GQ1b-IgG, GQ1b-IgM, GD1b-IgM, GD2-IgM, GT1a-IgG, GM1-IgG, GM2-IgM, Sulfatide-IgG, and Sulfatide IgM+IgG. One patient had positive myasthenia gravis antibodies (acetylcholine receptor antibody and ryanodine receptor calcium release channel antibody). Finally, MFS was diagnosed after full differential diagnosis combined with clinical practice. Conclusions Different types of MFS have different characteristics, and there are atypical symptoms. Antibodies should not be used as diagnostic criteria alone, and comprehensive clinical analysis should be combined.