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儿童桥本脑病:五例不同表现。

Hashimoto's encephalopathy in children: different manifestations of five cases.

机构信息

Division of Child Neurology, Antalya Training and Research Hospital, 07100, Antalya, Turkey.

Division of Child Neurology, Department of Pediatrics, School of Medicine, Ankara University, 06590, Ankara, Turkey.

出版信息

Acta Neurol Belg. 2019 Dec;119(4):595-599. doi: 10.1007/s13760-019-01191-7. Epub 2019 Jul 16.

DOI:10.1007/s13760-019-01191-7
PMID:31313264
Abstract

Hashimoto's encephalopathy (HE) is a rare, poorly understood, progressive and relapsing, steroid-responsive multiform disease. HE presents with subacute cognitive dysfunction, psychiatric symptoms, seizures, and movement disorders. The disorder is usually related to thyroid disease and the most frequent feature is the presence of anti-thyroperoxidase antibodies. Patients are generally euthyroid or mildly hypothyroid. The clinical features of two patients at presentation included refractory seizures and confusion, another patient had behavioral problems and altered cognitive status, one patient presented with right-sided weakness and numbness especially in his leg and tongue, dysphagia, speech disorder, aggressiveness, nightmares and nocturnal enuresis and last patient had focal seizures with altered mental status. All patients manifested increased anti-thyroid antibodies. Four patients improved with steroid treatment, and one of the patients responded to plasmapheresis instead of corticosteroid treatment. Physicians' awareness of this complication is of great importance because HE is a highly treatable condition among children and adolescents.

摘要

桥本脑病(HE)是一种罕见、了解甚少、进行性和复发性、对类固醇有反应的多形性疾病。HE 表现为亚急性认知功能障碍、精神症状、癫痫发作和运动障碍。该疾病通常与甲状腺疾病有关,最常见的特征是存在抗甲状腺过氧化物酶抗体。患者通常甲状腺功能正常或轻度甲状腺功能减退。两名患者就诊时的临床特征包括难治性癫痫发作和意识混乱,另一名患者有行为问题和认知状态改变,一名患者表现为右侧无力和麻木,特别是腿部和舌头,吞咽困难,言语障碍,攻击性,噩梦和夜间遗尿,最后一名患者有局灶性癫痫发作和精神状态改变。所有患者的抗甲状腺抗体均增加。四名患者经类固醇治疗后有所改善,一名患者对血浆置换有反应,而不是皮质类固醇治疗。医生对这种并发症的认识非常重要,因为 HE 是儿童和青少年中一种高度可治疗的疾病。

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Hashimoto's encephalopathy in children: different manifestations of five cases.儿童桥本脑病:五例不同表现。
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Cerebellar subacute syndrome due to corticosteroid-responsive encephalopathy associated with autoimmune thyroiditis (also called "Hashimoto's encephalopathy").由自身免疫性甲状腺炎相关的皮质类固醇反应性脑病(也称为“桥本脑病”)引起的小脑亚急性综合征。
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