PDF(Pubmed)
Abstract：
Neuronal surface antibody syndromes (NSAS) encompass a growing set of autoimmune neurological disorders, with their predominant clinical presentation being autoimmune encephalitis (AE). The most extensively documented form within NSAS is anti-N-methyl-D-aspartate receptor (NMDAR) autoimmunity. In contrast, other NSAS, such as anti-metabotropic glutamate receptor-5 (mGluR5) autoimmunity, are less common and less comprehensively characterized, particularly in pediatric cases.
In this instance, we present the case of a 7-year-old girl who exhibited abnormal behaviors following hematopoietic stem cell transplantation (HSCT). She received a diagnosis of anti-mGluR5 AE, and her Electroencephalogram (EEG) displayed an increased number of generalized slow waves during wakefulness. Treatment involved intravenous administration of gamma globulin and methylprednisolone, followed by oral prednisone tablets. Levetiracetam was introduced as an antiepileptic therapy during the pulse steroid therapy. Notably, the abnormal behaviors exhibited significant improvement after treatment.
To the best of our knowledge, this is the first report of rare pediatric NSAS involving anti-mGluR5 AE following HSCT. Enhancing our understanding and characterization of this condition may facilitate its recognition and treatment in children. Serum antibody testing could enable early identification and treatment of anti-mGluR5 AE.
In this instance, we present the case of a 7-year-old girl who exhibited abnormal behaviors following hematopoietic stem cell transplantation (HSCT). She received a diagnosis of anti-mGluR5 AE, and her Electroencephalogram (EEG) displayed an increased number of generalized slow waves during wakefulness. Treatment involved intravenous administration of gamma globulin and methylprednisolone, followed by oral prednisone tablets. Levetiracetam was introduced as an antiepileptic therapy during the pulse steroid therapy. Notably, the abnormal behaviors exhibited significant improvement after treatment.
To the best of our knowledge, this is the first report of rare pediatric NSAS involving anti-mGluR5 AE following HSCT. Enhancing our understanding and characterization of this condition may facilitate its recognition and treatment in children. Serum antibody testing could enable early identification and treatment of anti-mGluR5 AE.
摘要：
■神经元表面抗体综合征（NSAS）包括越来越多的自身免疫性神经系统疾病，其主要临床表现为自身免疫性脑炎（AE）。NSAS中记录最广泛的形式是抗N-甲基-D-天冬氨酸受体（NMDAR）自身免疫。相比之下,其他NSAS,如抗代谢型谷氨酸受体-5（mGluR5）自身免疫，不太常见，特征不全面，特别是在儿科病例中。
■在这种情况下，我们介绍了一例7岁女孩在造血干细胞移植(HSCT)后表现出异常行为的病例.她接受了抗mGluR5AE的诊断，她的脑电图（EEG）在觉醒期间显示出更多的广义慢波。治疗包括静脉注射丙种球蛋白和甲基强的松龙，其次是口服泼尼松片。左乙拉西坦被引入作为脉冲类固醇治疗期间的抗癫痫治疗。值得注意的是,治疗后异常行为明显改善。
■据我们所知,这是首次报道HSCT后涉及抗mGluR5AE的罕见儿科NSAS.增强我们对这种情况的理解和表征可能会促进其在儿童中的识别和治疗。血清抗体检测可以早期识别和治疗抗mGluR5AE。
■在这种情况下，我们介绍了一例7岁女孩在造血干细胞移植(HSCT)后表现出异常行为的病例.她接受了抗mGluR5AE的诊断，她的脑电图（EEG）在觉醒期间显示出更多的广义慢波。治疗包括静脉注射丙种球蛋白和甲基强的松龙，其次是口服泼尼松片。左乙拉西坦被引入作为脉冲类固醇治疗期间的抗癫痫治疗。值得注意的是,治疗后异常行为明显改善。
■据我们所知,这是首次报道HSCT后涉及抗mGluR5AE的罕见儿科NSAS.增强我们对这种情况的理解和表征可能会促进其在儿童中的识别和治疗。血清抗体检测可以早期识别和治疗抗mGluR5AE。
