Abstract：
OBJECTIVE: In this retrospective study, we aimed at defining the clinical, paraclinical and outcome features of acute neurological syndromes associated with anti-GQ1b antibodies.
RESULTS: We identified 166 patients with neurological symptoms appearing in less than 1 month and anti-GQ1b antibodies in serum between 2012 and 2022. Half were female (51%), mean age was 50 years (4-90), and the most frequent clinical features were areflexia (80% of patients), distal upper and lower limbs sensory symptoms (78%), ophthalmoplegia (68%), sensory ataxia (67%), limb muscle weakness (45%) and bulbar weakness (45%). Fifty-three patients (32%) presented with complete (21%) and incomplete (11%) Miller Fisher syndrome (MFS), thirty-six (22%) with Guillain-Barre syndrome (GBS), one (0.6%) with Bickerstaff encephalitis (BE), and seventy-three (44%) with mixed MFS, GBS & BE clinical features. Nerve conduction studies were normal in 46% of cases, showed demyelination in 28%, and axonal loss in 23%. Anti-GT1a antibodies were found in 56% of cases, increased cerebrospinal fluid protein content in 24%, and Campylobacter jejuni infection in 7%. Most patients (83%) were treated with intravenous immunoglobulins, and neurological recovery was complete in 69% of cases at 1 year follow-up. One patient died, and 15% of patients relapsed. Age > 70 years, initial Intensive Care Unit (ICU) admission, and absent anti-GQ1b IgG antibodies were predictors of incomplete recovery at 12 months. No predictors of relapse were identified.
CONCLUSIONS: This study from Western Europe shows acute anti-GQ1b antibody syndrome presents with a large clinical phenotype, a good outcome in 2/3 of cases, and frequent relapses.
RESULTS: We identified 166 patients with neurological symptoms appearing in less than 1 month and anti-GQ1b antibodies in serum between 2012 and 2022. Half were female (51%), mean age was 50 years (4-90), and the most frequent clinical features were areflexia (80% of patients), distal upper and lower limbs sensory symptoms (78%), ophthalmoplegia (68%), sensory ataxia (67%), limb muscle weakness (45%) and bulbar weakness (45%). Fifty-three patients (32%) presented with complete (21%) and incomplete (11%) Miller Fisher syndrome (MFS), thirty-six (22%) with Guillain-Barre syndrome (GBS), one (0.6%) with Bickerstaff encephalitis (BE), and seventy-three (44%) with mixed MFS, GBS & BE clinical features. Nerve conduction studies were normal in 46% of cases, showed demyelination in 28%, and axonal loss in 23%. Anti-GT1a antibodies were found in 56% of cases, increased cerebrospinal fluid protein content in 24%, and Campylobacter jejuni infection in 7%. Most patients (83%) were treated with intravenous immunoglobulins, and neurological recovery was complete in 69% of cases at 1 year follow-up. One patient died, and 15% of patients relapsed. Age > 70 years, initial Intensive Care Unit (ICU) admission, and absent anti-GQ1b IgG antibodies were predictors of incomplete recovery at 12 months. No predictors of relapse were identified.
CONCLUSIONS: This study from Western Europe shows acute anti-GQ1b antibody syndrome presents with a large clinical phenotype, a good outcome in 2/3 of cases, and frequent relapses.
摘要：
目的：在这项回顾性研究中，我们的目标是定义临床，与抗GQ1b抗体相关的急性神经综合征的临床旁和结局特征。
结果：我们在2012年至2022年之间确定了166例出现少于1个月的神经系统症状和血清抗GQ1b抗体的患者。一半是女性（51%）平均年龄为50岁（4-90岁），最常见的临床特征是反射障碍（80％的患者），上肢和下肢远端感觉症状（78%），眼肌麻痹（68%），感觉共济失调（67%），四肢肌肉无力（45％）和延髓无力（45％）。53例患者（32%）出现完全（21%）和不完全（11%）的MillerFisher综合征（MFS），36例（22%）患有格林-巴利综合征（GBS），1例(0.6%)患有Bickerstaff脑炎(BE)，和73(44%)使用混合MFS，GBS&BE临床特征。46％的病例的神经传导研究正常，显示28％的脱髓鞘，轴突损失23%。在56%的病例中发现了抗GT1a抗体，脑脊液蛋白含量增加24%，空肠弯曲菌感染占7%。大多数患者（83％）接受静脉注射免疫球蛋白治疗，在1年的随访中，69％的病例完成了神经系统恢复。一个病人死了,15%的患者复发。年龄>70岁,初次入住重症监护病房(ICU),和缺乏抗GQ1bIgG抗体是12个月时不完全恢复的预测因素。没有发现复发的预测因子。
结论：来自西欧的这项研究表明，急性抗GQ1b抗体综合征具有较大的临床表型，在2/3的病例中有好的结果，和频繁的复发。
结果：我们在2012年至2022年之间确定了166例出现少于1个月的神经系统症状和血清抗GQ1b抗体的患者。一半是女性（51%）平均年龄为50岁（4-90岁），最常见的临床特征是反射障碍（80％的患者），上肢和下肢远端感觉症状（78%），眼肌麻痹（68%），感觉共济失调（67%），四肢肌肉无力（45％）和延髓无力（45％）。53例患者（32%）出现完全（21%）和不完全（11%）的MillerFisher综合征（MFS），36例（22%）患有格林-巴利综合征（GBS），1例(0.6%)患有Bickerstaff脑炎(BE)，和73(44%)使用混合MFS，GBS&BE临床特征。46％的病例的神经传导研究正常，显示28％的脱髓鞘，轴突损失23%。在56%的病例中发现了抗GT1a抗体，脑脊液蛋白含量增加24%，空肠弯曲菌感染占7%。大多数患者（83％）接受静脉注射免疫球蛋白治疗，在1年的随访中，69％的病例完成了神经系统恢复。一个病人死了,15%的患者复发。年龄>70岁,初次入住重症监护病房(ICU),和缺乏抗GQ1bIgG抗体是12个月时不完全恢复的预测因素。没有发现复发的预测因子。
结论：来自西欧的这项研究表明，急性抗GQ1b抗体综合征具有较大的临床表型，在2/3的病例中有好的结果，和频繁的复发。
