儿童桥本脑病5例临床分析

目的　通过总结儿童桥本脑病的临床特点，提高儿科医师对该病的认识，以期早期诊断及治疗、改善预后。方法　回顾性分析2014年1月至2018年12月重庆医科大学附属儿童医院临床诊断的5例儿童桥本脑病临床资料，总结其临床表现、辅助检查结果、诊治经过及预后。结果　5例桥本脑病患儿中男2例，女3例，年龄10岁6月龄至14岁4月龄；主要临床表现包括精神行为异常（4/5）、认知障碍（4/5）、癫痫发作（4/5）、不自主运动（2/5）及睡眠障碍（2/5）；5例患儿血清抗甲状腺过氧化物酶抗体（TPO-Ab）和抗甲状腺球蛋白抗体（TG-Ab ）均明显升高；脑电图均提示背景弥漫性慢化、头颅磁共振成像（MRI）均无异常、血及脑脊液自身免疫性脑炎抗体检测均为阴性，3例患儿脑脊液生化检测提示蛋白轻度升高；5例患儿在予以激素治疗后病情均逐渐好转。结论 儿童桥本脑病罕见，主要以精神行为异常、认知障碍、癫痫发作为主要表现，血清TPO-Ab和TG-Ab显著升高为其特点，早期激素治疗，效果显著、预后良好。

Clinical features of five children with Hashimoto encephalopathy

Objective　To characterize the clinical，laboratory and imaging features and outcomes of Hashimoto encephalopathy in children and to potentially improve recognition and timely treatment of this disease. Methods　The clinical data of five children with Hashimoto encephalopathy，including clinical characteristics，laboratory and imaging findings，treatment and prognosis，were analyzed retrospectively. Results　Of the five patients，two were boys，three were girls. Age at diagnosis was 10.5-14.3 years. The main clinical symptoms were psychiatric abnormality（4/5），cognitive impairment（4/5），seizures（4/5），abnormal movements（2/5），and insomnia（2/5）. In five patients，anti-thyroglobulin and anti-thyroid peroxidase antibodies were obviously increased；electroencephalogram showed generalized slowing；brain magnetic resonance imagings showed no abnormalities；detection of autoimmune encephalitis antibodies of the blood and cerebrospinal fluid was negative；proteins in the cerebrospinal fluid were slightly increased in 3 children. Five patients were improved after corticosteroid treatment. Conclusion　Hashimoto encephalopathy is rare in children，and its clinical characteristics are mainly psychiatric symptoms，cognitive impairment，seizures and increased anti-thyroglobulin and anti-thyroid peroxidase antibodies. The effect of early corticosteroid treatment is remarkable and the prognosis is good.