Kim Jong Kuk, Kim Yoo Hwan, Yoon Byeol A, Cho Joong Yang, Oh Sun Young, Shin Ha Young, Kim Ji Soo, Park Kee Hong, Kim Sun Young, Suh Bum Chun, Seok Hung Youl, Yoo Jin Hyuk, Bae Jong Seok
Department of Neurology, College of Medicine, Dong-A University, Busan, Korea.
Department of Neurology, Hangang Sacred Heart Hospital, Hallym University College of Medicine, Seoul, Korea.
J Clin Neurol. 2018 Jul;14(3):401-406. doi: 10.3988/jcn.2018.14.3.401.
Antiganglioside antibodies are known to play a pathogenic role in Guillain-Barré syndrome (GBS). Either an immunoglobulin (Ig)G- or IgM-type anti-GM2 antibody is detected in rare cases in GBS patients. However, the specific pathogenic role of these antibodies in GBS has not been reported previously. This study aimed to define and characterize the clinical spectrum of GBS with anti-GM2 positivity.
We reviewed the database of the Dong-A University Neuroimmunology Team, which has collected sera of GBS and its variants from more than 40 general and university-based hospitals in Korea. Detailed information about the involved patients was often obtained and then corrected by the charge doctor applying additional questionnaires.
Four patients with acute monophasic peripheral neuropathy or cranial neuropathy with isolated IgM-type anti-GM2-antibody positivity were recruited. In addition, IgG-type anti-GM2 antibody was solely detected in the sera of another four patients. The IgM-positive group comprised heterogeneous syndromes: two cases of acute motor axonal neuropathy, one of acute inflammatory demyelinating polyneuropathy, and one of isolated facial diplegia. In contrast, all of the cases enrolled in the IgG-positive group manifested with dizziness with or without oculomotor palsy due to cranial neuropathy syndrome.
This study has identified that anti-GM2 antibody can be found in various subtypes of GBS and its variants in rare cases. Compared to the clinical heterogeneity of the IgM-positive group, the IgG-positive group can be characterized by cranial-dominant GBS variants presenting mainly with oculomotor and vestibular dysfunctions.
抗神经节苷脂抗体在吉兰 - 巴雷综合征（GBS）中发挥致病作用。在GBS患者的罕见病例中可检测到免疫球蛋白（Ig）G型或IgM型抗GM2抗体。然而，这些抗体在GBS中的具体致病作用此前尚未见报道。本研究旨在明确并描述抗GM2阳性GBS的临床谱。
我们回顾了东国大学神经免疫学团队的数据库，该数据库收集了来自韩国40多家综合医院和大学附属医院的GBS及其变异型患者的血清。经常获取有关受累患者的详细信息，然后由负责医生通过额外问卷进行校正。
招募了4例急性单相性周围神经病或颅神经病且孤立性IgM型抗GM2抗体阳性的患者。此外，在另外4例患者的血清中仅检测到IgG型抗GM2抗体。IgM阳性组包括多种综合征：2例急性运动轴索性神经病，1例急性炎症性脱髓鞘性多发性神经病，1例孤立性面瘫。相比之下，IgG阳性组的所有病例均表现为因颅神经病综合征导致的头晕，伴或不伴有动眼神经麻痹。
本研究发现抗GM2抗体在罕见情况下可在GBS及其变异型的各种亚型中发现。与IgM阳性组的临床异质性相比，IgG阳性组的特征为主要表现为动眼神经和前庭功能障碍的以颅神经为主的GBS变异型。
