目的：探讨以单纯眼肌活功能障碍为临床表现的Miller-Fisher综合征（Miller-Fisher syndrome，MFS） 的临床特点及预后。方法：报道2例仅以双侧眼肌功能障碍为主要临床表现的MFS病例，并进行相关文献 复习。结果：例1患者54岁女性，其临床特点表现为感染后视物重影，双侧眼睑痉挛。例2患者67岁男性， 其临床特点表现为无明显诱因视物重影，单侧眼睑下垂，瞳孔扩大，对光反射迟钝，眼球运动障碍。2例患 者均检测到血清抗GQ1b抗体IgG、血清抗GT1a抗体IgG，例2患者还检测到血清抗Sulfatide抗体IgG；患者 均经静脉注射丙种球蛋白治疗，预后良好。回顾既往报道，结合本研究补充2例新病例，得到单纯眼肌症状 的患者资料共7例，其中5例血清抗GQ1b抗体IgG阳性，6例眼外肌麻痹，5例眼内肌麻痹，5例有前驱感染， 7例患者均预后良好。结论：仅有单纯眼部症状的MFS患者极为罕见，本病例分析有助于加深对该类疾病 的了解。

To analyze the clinical features and prognosis of Miller-Fisher Syndrome Manifesting (MFS) with isolated ophthalmoplegia. Methods: We report two cases of MFS presenting with bilateral isolated ophthalmoplegia as the primary clinical manifestation followed by a systematic review of relevant literature. Results: Case 1 involved a 54-year-old female presenting with diplopia and bilateral blepharospasm following infection. Case 2 described a 67-year-old male who developed diplopia without clear triggers, accompanied by unilateral ptosis, dilated pupils with sluggish light reflex, and ocular motility disorders. Both patients tested positive for serum anti-GQ1b IgG antibodies and anti-GT1a IgG antibodies; Intravenous immunoglobulin therapy was administered to both patients, leading to favorable outcomes. By integrating our two new cases with previously reported instances, we identified a total of seven patients manifesting isolated ocular symptoms: five were anti-GQ1b IgG positive, six exhibited external ophthalmoplegia, five had internal ophthalmoplegia, five reported prodromal infections, and all achieved good recovery. Conclusion: Patients with MFS presenting solely with ocular symptoms are exceedingly rare. These cases significantly enhance our understanding of this distinctive clinical entity.