目的：探讨表现为孤立眼内肌麻痹的抗GQ1b抗体综合征的临床特点及预后。方法：报道我院收治的 急性孤立单侧眼内肌麻痹伴颅内病变的抗GQ1b抗体综合征1例，并进行相关文献复习。结果：40岁中国女 性，其临床表现为感染后出现孤立的单侧瞳孔扩张及视力模糊。右侧瞳孔光反射减弱，左侧瞳孔光反射正 常；双侧眼球运动不受限制。脑部核磁显示左侧海马和丘脑信号异常，双下肢体感诱发电位中枢传导时间延 长。血清抗人神经节苷脂抗体检测显示IgM抗GQ1b抗体滴度升高。患者经静脉注射免疫球蛋白治疗后逐 渐康复。搜索既往报道，获得表现为孤立眼内肌麻痹的抗GQ1b抗体综合征的患者7例，其中6例对光反射减 弱，5 例腱反射减弱，3 例出现共济失调，7 例均有血清 IgG 抗 GQ1b 抗体阳性。7 例均完全康复，预后较好。 结论：表现为孤立眼内肌麻痹的抗GQ1b抗体综合征很罕见，本病例有助于加深对该类疾病的了解。

To analyze the clinical characteristic and prognosis of anti-GQ1b antibody syndrome manifested by isolated unilateral mydriasis. Methods: A case of anti-GQ1b antibody clinically manifested by acute isolated unilateral mydriasis associated with intracranial lesions was reported and the relevant literature was reviewed in this study. Results: We report a 40-year-old Chinese woman presenting with postinfectious unilateral mydriasis and blurred vision. Pupillary light reflex was decreased in the right pupil and normal in the left; ocular movements were unrestricted bilaterally. Also, brain magnetic resonance imaging revealed abnormal signals in the left hippocampus and thalamus, and somatosensory evoked potential central conduction times were prolonged in both lower limbs. Serum assay for antiganglioside antibodies revealed elevated titers for IgM anti-GQ1b antibody. The patient was treated with intravenous immunoglobulin and recovered gradually. Seven cases of anti-GQ1b antibody syndrome manifested by isolated internal ophthalmoplegia were identified from the Pubmed. Pupillary light reflex was decreased in 6 cases; weakened tendinous reflect was found in 5 cases; ataxia was identified in 3 cases; 7 cases were serum IgG anti-GQ1b antibody positive. All the cases recovered completely and had better prognosis. Con? clusion: Isolated internal ophthalmoplegia is rarely reported in anti-GQ1b antibody syndrome. Our case report helps increase the level of diagnosis and treatment of this disease.