Aggressive course in encephalitis with opsoclonus, ataxia, chorea, and seizures: the first pediatric case of γ-aminobutyric acid type B receptor autoimmunity.
Aggressive course in encephalitis with opsoclonus, ataxia, chorea, and seizures: the first pediatric case of γ-aminobutyric acid type B receptor autoimmunity.
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DOI：
10.1001/jamaneurol.2013.4786
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发表时间：
2014-05
期刊：
影响因子：
29
通讯作者：
Dalmau J
中科院分区：
文献类型：
--
作者：
Kruer MC;Hoeftberger R;Lim KY;Coryell JC;Svoboda MD;Woltjer RL;Dalmau J
抗γ-氨基丁酸B型（GABAB）受体的自身抗体最近被确定为自身免疫性脑炎的原因。大多数GABAB脑炎患者表现为边缘系统脑炎。报告的病例中约有一半是副肿瘤性的，大多数肿瘤代表小细胞肺癌。我们描述一个3岁的男孩谁提出了一个混合运动障碍（眼阵挛，共济失调，舞蹈病）以及癫痫发作难治性治疗。他的癫痫发作需要持续输注戊巴比妥钠才能得到控制。尽管静脉注射皮质类固醇和免疫球蛋白治疗，患者最终死于严重的败血症。据我们所知，这份报告是第一例儿童γ-氨基丁酸相关脑炎。我们的病人表现为脑病、难治性癫痫发作和混合性运动障碍，而不是边缘系统脑炎。γ-氨基丁酸B型受体自身免疫性值得考虑的小儿脑炎患者。
Autoantibodies to the γ-aminobutyric acid type B (GABAB) receptor have recently been identified as a cause of autoimmune encephalitis. Most patients with GABAB encephalitis have presented with limbic encephalitis. About half of the cases reported have been paraneoplastic in origin, with the majority of tumors representing small cell lung cancer. We describe a 3-year-old boy who presented with a mixed movement disorder (opsoclonus, ataxia, and chorea) as well as seizures refractory to treatment. His seizures required continuous pentobarbital sodium infusion to be controlled. Despite treatment with intravenous corticosteroids and immunoglobulins, the patient ultimately died of overwhelming sepsis. To our knowledge, this report represents the first pediatric case of GABAB-associated encephalitis. Our patient presented with encephalopathy, refractory seizures, and a mixed movement disorder rather than limbic encephalitis. γ-Aminobutyric acid type B receptor autoimmunity deserves consideration in pediatric patients presenting with encephalitis.