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表现为假性球麻痹的桥本脑病。

Hashimoto's encephalopathy presenting as pseudobulbar palsy.

作者信息

Oz Tuncer Gokcen, Teber Serap, Kutluk Muhammed Gültekin, Albayrak Pelin, Deda Gülhis

机构信息

Department of Pediatric Neurology, Faculty of Medicine, Ankara University, Ankara, Turkey.

出版信息

Childs Nerv Syst. 2018 Jun;34(6):1251-1254. doi: 10.1007/s00381-018-3720-2. Epub 2018 Jan 24.

Abstract

INTRODUCTION

Hashimoto's encephalopathy (HE) is an autoimmune condition with varied neurological and psychiatric features. HE is very unusual as a cause of pseudobulbar palsy (PSP).

CASE PRESENTATION

A 14-year-old male was admitted with right-sided weakness, dysphagia, speech disorder, and aggressiveness. Brain magnetic resonance imaging showed increased intensity in bilateral temporal, insular cortex, amygdala, and parahippocampal area on T2-weighted and fluid-attenuated inversion recovery images. Autoimmune encephalitis was considered as the patient had subacute onset of psychiatric and motor disturbances with normal findings for cerebrospinal fluid. N-methyl-D-aspartate receptor, anti-glutamate-type α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid 1 and 2, anti-contactin-associated protein-like 2, anti-gamma-aminobutyric acid receptor, anti-Leucine-rich, and glioma-inactivated 1 antibodies were negative but the anti-thyroperoxidase (antiTPO) level was greater than 998 IU/ML (n:0-9). Steroid therapy was initiated as pulse therapy and maintained with 2-mg/kg/day dose with the diagnosis of HE. He was symptom free for 6 months. In the follow-up period, he had two recurrences which responded to steroid therapy.

CONCLUSION

The common causes of PSP are demyelinating, vascular, and motor neuron diseases and congenital malformations of the opercular or insular cortex. However, there are no cases of PSP developing after any autoimmune encephalitis. This case highlights the importance of early detection of antiTPO antibodies with the findings of PSP due to autoimmune encephalitis.

摘要

引言

桥本脑病(HE)是一种具有多种神经和精神特征的自身免疫性疾病。作为假性延髓麻痹(PSP)的病因,HE非常罕见。

病例报告

一名14岁男性因右侧肢体无力、吞咽困难、言语障碍和攻击性而入院。脑磁共振成像显示,在T2加权像和液体衰减反转恢复像上,双侧颞叶、岛叶皮质、杏仁核和海马旁回区域信号增强。由于患者出现亚急性起病的精神和运动障碍,且脑脊液检查结果正常,故考虑为自身免疫性脑炎。N-甲基-D-天冬氨酸受体、抗谷氨酸型α-氨基-3-羟基-5-甲基-4-异恶唑丙酸1和2、抗接触蛋白相关蛋白样2、抗γ-氨基丁酸受体、抗富亮氨酸胶质瘤失活1抗体均为阴性,但抗甲状腺过氧化物酶(antiTPO)水平大于998 IU/ML(正常范围:0-9)。诊断为HE后,开始采用脉冲疗法进行类固醇治疗,并以2 mg/kg/天的剂量维持治疗。他有6个月无症状。在随访期间,他复发了两次,均对类固醇治疗有反应。

结论

PSP的常见病因是脱髓鞘疾病、血管疾病、运动神经元疾病以及岛盖部或岛叶皮质的先天性畸形。然而,尚无自身免疫性脑炎后发生PSP的病例。该病例强调了在出现因自身免疫性脑炎导致的PSP表现时,早期检测antiTPO抗体的重要性。

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