青春期女孩以癫痫持续状态、高热首发桥本脑病1例报告

Report of an adolescent girl with Hashimoto encephalopathy starting with status epilepticus and high fever with review of literature

目的探讨特殊起病桥本脑病的临床表现。方法回顾分析1例青春期女孩以癫痫持续状态、高热为首发症状的桥本脑病的临床资料,并复习相关文献。结果患儿,女,12.5岁,经持续静脉点滴丙戊酸钠、糖皮质激素和静注丙球联合治疗,57小时抽搐停止。2次腰椎穿刺脑脊液分析均未见明显异常;血液和脑脊液中自身免疫性脑炎抗体均为阴性;头颅CT和磁共振成像(MRI)及强化MRI未见异常。24小时脑电图提示弥漫性慢波,未见典型痫样放电。甲状腺功能检测提示抗甲状腺球蛋白抗体和甲状腺过氧化物酶抗体滴度明显增高,游离T3减低,sTSH升高,诊断为桥本脑病。结论桥本脑病为罕见病,青春期以癫痫持续状态、发热首诊更少见。常规筛查甲状腺功能及甲状腺相关抗体尤其甲状腺过氧化物酶抗体,可早期诊断和治疗,从而改善预后。

Objective To analyze the clinical data of one adolescent girl who suffered from Hashimoto encephalopathy with the onset of status epilepticus and high fever.Methods Clinical data of the patient was retrospectively analyzed and related literatures were reviewed.Results An adolescent girl was diagnosed with Hashimoto encephalopathy with the onset of status epilepticus and high fever.Under the continuous intravenous injection of sodium valproate for 57 hours and corticosteroids and intravenous immunoglobulin treatment,convulsions was stopped.For twice lumbar punctures,the cerebrospinal fluid analysis showed no obvious abnormalities.Antibodies against autoimmune encephalitis in the blood and cerebrospinal fluid were all negative.No abnormality was found in the head CT and MRI.After 67 hours of admission to the hospital,the patient was conscious.She became afebrile after 72 hours.Electroencephalogram for 24 hours indicated diffuse slow wave and no typical epileptiform discharge.There was no abnormal analysis of blood amino acids,acyl carnitine and urinary organic acids.Blood lactate and blood ammonia were in normal range.The detection of thyroid function indicated that the titers of antithyroglobulin antibody and thyroid peroxidase antibody increased significantly,free T3 was lower than normal and sTSH was higher than normal.Therefore,the diagnosis was Hashimoto encephalopathy.Levothyroxine sodium tablets and prednisone were given to the patient.We monitored the thyroid function and adjusted the dosage of medicine.After discharge from the hospital for 3 months,the thyroid function was normal and the electroencephalogram was normal under the treatment of oral levothyroxine sodium tablets 50μg/d and prednisone 5 mg/d.The patient was still in the follow-up.Conclusions Hashimoto encephalopathy is a rare disease,easy to be misdiagnosed and missed.Therefore,for adolescents with status epilepticus and fever,the thyroid function and thyroid associated antibodies,especially thyroid peroxidase antibody,should be routinely screened for early diagnosis and early treatment to improve the prognosis.Further research is still needed because the pathogenesis of the disease is not yet clear.