Guillain-Barré syndrome (GBS) is the most prevalent cause of acquired paralytic neuropathy in children, however, ataxia as the initial presenting sign in children is very rare. Antiganglioside antibodies are presumed to have an important role in the pathophysiology and some phenotypic correlations have been reported. Anti-GM2 antibody, unlike other antibodies, is far less detected in GBS. Here, we report a 7.5-year-old female, initially presenting with ataxia, an atypical presenting symptom in a child, is promptly diagnosed and treated successfully as GBS. Atypical history of urinary infection in our patient is an interesting aspect. The presence of isolated anti-GM2 IgM antibody and ataxia in a pediatric patient is rare. In this case report, we aim to describe an atypical initial presentation, with positive anti-GM2 antibodies, as well as review literature on isolated anti-GM2 positive pediatric GBS patients.

吉兰-巴雷综合征 (GBS) 是儿童获得性麻痹性神经病的最常见原因，然而，作为儿童初始症状的共济失调非常罕见。抗神经节苷脂抗体被认为在病理生理学中具有重要作用，并且已经报道了一些表型相关性。与其他抗体不同，抗 GM2 抗体在 GBS 中的检测率要低得多。在这里，我们报告了一名 7.5 岁女性，最初表现为共济失调，这是儿童的一种非典型症状，被迅速诊断并成功治疗为 GBS。本例患者尿路感染的非典型病史是一个有趣的方面。在儿科患者中出现分离的抗 GM2 IgM 抗体和共济失调是罕见的。在本病例报告中，我们旨在描述一种非典型的初始表现，具有阳性抗 GM2 抗体，