Children's Hospital Augsburg, Section of Neuropediatrics, Klinikum Augsburg, Germany.
Children's Hospital Augsburg, Section of Neuropediatrics, Klinikum Augsburg, Germany.
Eur J Paediatr Neurol. 2018 Jan;22(1):190-193. doi: 10.1016/j.ejpn.2017.08.004. Epub 2017 Sep 2.
Limbic encephalitis (LE) with antibodies against leucine-rich glioma inactivated protein 1 (LGI1) is an auto-antibody mediated disorder with characteristic symptoms as dysfunction of memory, faciobrachial dystonic seizures and neuropsychiatric symptoms as emotional lability. Limbic encephalitis with LGI1 antibodies has been known so far as a disease of adults. We describe the case of a 14-year-old boy presenting with typical dysfunction of memory and LGI1 antibodies. To the best of our knowledge, this is the youngest patient with LGI1 antibody mediated limbic encephalitis described so far. Improved knowledge of this autoimmune syndrome in children and adolescents permit rapid immunomodulatory treatment, which could help to prevent irreversible lesions, such as hippocampal atrophy.
抗富亮氨酸胶质瘤失活蛋白 1(LGI1)抗体相关边缘性脑炎(LE)是一种自身抗体介导的疾病,其特征性症状为记忆障碍、面臂肌张力障碍发作和神经精神症状如情绪不稳。迄今为止,LGI1 抗体相关边缘性脑炎被认为是一种成人疾病。我们描述了一例 14 岁男孩,表现为典型的记忆功能障碍和 LGI1 抗体。据我们所知,这是迄今为止描述的患有 LGI1 抗体介导的边缘性脑炎的最年轻患者。提高对儿童和青少年自身免疫综合征的认识,可以进行快速的免疫调节治疗,有助于预防海马萎缩等不可逆损伤。