目的 探索儿童急性抗GQ1b抗体综合征的临床、电生理及预后特征。方法 回顾2019至2023年我院收治的24例抗GQ1b抗体阳性患儿的临床资料并进行分析。结果 24例患儿均急性起病，中位年龄4.0（2.0~13.5）岁，男性患儿占62.5%。患儿最常见的临床表现为腱反射减弱或消失、共济失调、肢体无力、肢体感觉异常、颅神经受累等，其中4例自主神经功能障碍，2例嗜睡。9例患儿具有Miller-Fisher综合征 (MFS)谱的临床特征，包括5例经典型MFS，4例变异型MFS；8例患儿具有吉兰-巴雷综合征（GBS）谱的临床特征，包括3例经典型GBS，5例变异型GBS；其余7例为混合型，表现为MFS、GBS或Bickerstaff’s脑干脑炎(BBE)的混合临床特征。15例除抗GQ1b抗体外还发现了其他抗神经节苷酯抗体，最常见的为抗GT1a抗体和抗GM1抗体，21例患儿脑脊液检查存在蛋白-细胞分离。MFS谱患儿神经传导多为感觉神经传导波幅轻度降低，GBS谱和混合型患儿运动感觉神经均可受累，且受累较重。所有患儿均接受了静脉注射免疫球蛋白治疗，经过3个月随访，15例患儿神经功能恢复良好，恢复不良组患儿颅神经损伤、自主神经功能障碍及入院时GDS评分明显高于恢复良好组，差异具有统计学意义（P<0.05）。结论 儿童急性抗GQ1b抗体综合征临床表型多变，轴突损伤或脱髓鞘损伤都有可能发生，发病3~8周随访神经传导检查，有助于观察神经电生理分型及恢复情况。2/3患儿恢复良好，伴有颅神经损伤、自主神经功能障碍及入院时GDS评分高的患儿恢复较差。

Objective To investigate the clinical, electrophysiological and prognosis of patients with acute anti-GQ1b antibody syndrome. Methods A retrospective analysis was performed on the clinical data of 24 children diagnosed with acute anti-GQ1b antibody syndrome between 2019 and 2023 at our hospital. Demographic characteristics, clinical manifestations, electrophysiological findings, and outcomes were systematically reviewed and analyzed. Results All 24 patients presented with acute onset of symptoms. The median age was 4.0 ( 2.0-13.5) years, with male predominance (62.5%). Common clinical features included reduced or absent tendon reflexes, ataxia, limb weakness, sensory disturbances, and cranial nerve involvement. Autonomic dysfunction was observed in 4 patients, and lethargy was noted in 2.Nine patients exhibited clinical features consistent with the Miller-Fisher syndrome (MFS) spectrum, including 5 with classical MFS and 4 with variant MFS. Eight patients displayed Guillain-Barré syndrome (GBS)-related phenotypes, comprising 3 classical GBS and 5 variant GBS cases. The remaining 7 patients were classified as mixed type, presenting overlapping features of MFS, GBS, and Bickerstaff’s brainstem encephalitis (BBE). In addition to anti-GQ1b antibodies, 15 patients had detectable levels of other anti-ganglioside antibodies, most commonly anti-GT1a and anti-GM1. Cerebrospinal fluid (CSF) analysis revealed protein-cell dissociation in 21 patients. Electrophysiological studies showed mild sensory nerve amplitude reduction in the MFS group, whereas both motor and sensory nerves were significantly affected in the GBS and mixed-type groups. All patients received intravenous immunoglobulin therapy. After 3-month follow-up, 15 patients demonstrated favorable neurological recovery. Patients with poor recovery were more likely to have cranial nerve injury, autonomic dysfunction, and higher GDS scores upon admission (P<0.05). Conclusion Acute anti-GQ1b antibody syndrome in children demonstrates a wide range of clinical phenotypes, with both axonal and demyelinating pathologies present. Follow-up nerve conduction studies conducted between 3 and 8 weeks post-onset are valuable for assessing electrophysiological subtypes and monitoring recovery. Approximately two-thirds of affected children achieve good functional outcomes, while those with cranial nerve involvement, autonomic dysfunction, or elevated initial GDS scores tend to have poorer prognosis.