儿童桥本脑病7例病例系列报告

7 cases of Hashimoto's encephalopathy in children:A case series report

目的总结儿童桥本脑病(HE)的临床特征。方法纳入2017年1月至2020年12月河北省儿童医院神经科收治的HE患儿,回顾性分析患儿的临床表现、实验室检查、头颅MR、治疗和随访情况。结果 7例HE中,男5例,发病年龄5~12岁。3例病初伴发热,起病前有感染史、病毒性脑炎史、食欲亢进及排便增多、癫癎病史各1例。癫癎发作3例,2例为癫癎持续状态;精神行为异常和意识状态改变各3例;外展神经受损1例。血常规WBC、CRP升高1例;脑脊液细胞数和蛋白升高分别为3和1例;5例行寡克隆区带检测2例阳性。甲状腺过氧化物酶抗体(TPO)3例起病时即升高,4例2周内升高。入院时甲亢和中枢性甲减各2例,低T3综合征、fT3和fT4均降低各1例,1例入院后中枢性甲减;2例甲状腺超声示多囊性小结节。3例头颅MR有异常表现;脑电图均表现为不同程度的慢波发放,1例检测到癫癎波。均予免疫球蛋白冲击联合大剂量甲泼尼龙琥珀酸钠治疗,6例治疗3~8 d后症状缓解,1例1个月后未改善,第2轮激素治疗7 d后症状缓解。随访1~4年,1例复发2次,予激素或免疫球蛋白冲击治疗后症状缓解。3例头颅MR异常者在2个月内随访影像学均恢复正常。4例随访复查TPO抗体,3例仅随访甲状腺功能,均在发病1年内恢复正常。结论儿童HE的临床表现、实验室指标和影像学表现缺乏特异性,需与多种疾病进行鉴别诊断。对于不明原因的脑病患儿,在除外自身免疫性脑炎、炎症和脱髓鞘疾病后要警惕HE的可能,应多次检测TPO抗体,以协助诊断。

Objective To summarize the clinical features of Hashimoto's encephalopathy(HE)in children.Methods Children diagnosed with HE hospitalized in the Department of Neurology at Hebei Children's Hospital from January 2017 to December 2020 were selected.The clinical characteristics,laboratory examination results,imaging results,treatment and prognosis of the disease were retrospectively analyzed.Results Among the 7 HE patients,there were 5 males with onset age of 5 to 12 years.Three cases had fever at the onset of the disease.Before the onset of the disease,infection,viral encephalitis,hyperappetite and increased defecation,and epilepsy were found in 1 case,respectively.Three cases had depileptic seizure and 2 of them were epileptic status.Three cases had abnormal mental behavior and 3 had consciousness change.One patient had cranial nerve injury.One case had increased WBC and CRP.CSF cells increased in 3 cases,and CSF protein increased in one case.The oligoclonal bands were positive in 2 of 5 cases receiving the examination.Thyroid peroxidase(TPO)antibodies were normal in 4 patients at the onset of the disease,but increased when the disease was aggravated within two weeks.Three cases had increased TPO antibodies at the onset of the disease.There were 2 cases of hyperthyroidism,2 cases of central hypothyroidism,1 case of low T3 syndrome,and 1 case of reduced free T3 and T4.One patient had normal thyroid function at the time of admission and central hypothyroidism occurred later.Thyroid ultrasound showed polycystic nodules in two cases.Three cases had cranial MR abnormality.Seven cases were abnormal in video electroencephalogram,showing different degrees of slow wave release,and 1 case was epileptic.All 7 cases were treated with immunoglobulin shock combined with high-dose methylprednisolone sodium succinate.The symptoms of 6 cases were relieved after 3 to 8 days of treatment and 1 case remained unchanged after 1 month of shock treatment,whose symptoms were relieved after 7 days of the second round of hormone shock treatment.Seven cases had been followed up for 1 to 4 years.One case recurred twice,and the symptoms were relieved after hormone or immunoglobulin shock treatment.Three patients with abnormal cranial MR were followed up within 2 months,and the imaging returned to normal.TPO antibodies were reexamined in 4 cases,thyroid function was followed up in 3 cases,and the relevant indexes returned to normal within 1 year.Conclusion The clinical manifestations,laboratory indicators and imaging characteristics of HE are not specific,so it is necessary to distinguish it from various diseases.For pediatric patients with unknown encephalopathy,the possibility of HE should be considered after excluding autoimmune encephalitis,inflammation and demyelination diseases,and TPO antibodies should be detected for many times to assist in the diagnosis.