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伴有癫痫持续状态的桥本脑病

Hashimoto Encephalopathy With Status Epilepticus.

作者信息

Sliwinska Aleksandra, Fumuso Perry, Stringer Bryan, Ansar Muhammad, Baldwin Jennifer

机构信息

Department of Medicine, University of Connecticut School of Medicine, Farmington, USA.

Department of Endocrinology and Metabolism, University of Connecticut School of Medicine, Farmington, USA.

出版信息

Cureus. 2020 Dec 2;12(12):e11857. doi: 10.7759/cureus.11857.

Abstract

Hashimoto encephalopathy (HE) is a heterogenous neurological syndrome that can manifest with encephalopathy, seizures, headaches, and variable neuropsychiatric disturbances. The underlying mechanism remains unclear; however, autoimmune pathogenesis is suspected due to its association with autoimmune thyroid disease, high titers of anti-thyroid antibodies, and quick response to steroid therapy. We report a 59-year-old female patient with a remote history of hypothyroidism who presented with status epilepticus and complaints of chronic headaches and cognitive impairment. The presence of sharp frontal waves was identified on her EEG. The patient was initially started on anti-epileptics only; however, her headaches and memory loss escalated, further diagnostic workup was pursued, which revealed high anti-thyroid peroxidase antibodies with normal thyroid function tests. The only cerebrospinal fluid (CSF) abnormality noticed was an elevated protein concentration. MRI showed non-specific right frontal lobe pial enhancement. Remaining infectious, rheumatologic, and neurologic testing was unremarkable. The patient was started on a steroid regimen with successful resolution of symptoms and return of cognitive baseline. Hashimoto's encephalopathy is a diagnosis of exclusion; however, it should be considered in patients with high titers of anti-thyroid antibodies and neurological symptoms that cannot be explained by thorough infectious, metabolic, and autoimmune testing. It is essential to recognize this neurological entity as fast clinical improvement may be achieved with steroids and other immunotherapies.

摘要

桥本脑病(HE)是一种异质性神经综合征,可表现为脑病、癫痫发作、头痛及各种神经精神障碍。其潜在机制尚不清楚;然而,由于它与自身免疫性甲状腺疾病相关、抗甲状腺抗体滴度高以及对类固醇治疗反应迅速,故怀疑存在自身免疫发病机制。我们报告一名59岁女性患者,有甲状腺功能减退病史,表现为癫痫持续状态,伴有慢性头痛和认知障碍。脑电图显示其额叶有锐波。该患者最初仅开始使用抗癫痫药物治疗;然而,她的头痛和记忆力减退加重,遂进行进一步的诊断检查,结果显示抗甲状腺过氧化物酶抗体升高,而甲状腺功能测试正常。唯一发现的脑脊液(CSF)异常是蛋白浓度升高。磁共振成像(MRI)显示右侧额叶软脑膜非特异性强化。其余感染性、风湿性和神经学检查均无异常。该患者开始接受类固醇治疗,症状成功缓解,认知功能恢复至基线水平。桥本脑病是一种排除性诊断;然而,对于抗甲状腺抗体滴度高且有无法通过全面的感染性、代谢性和自身免疫性检查解释的神经症状的患者,应考虑此病。认识到这种神经疾病很重要,因为使用类固醇和其他免疫疗法可能会使临床症状迅速改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/27b3/7781565/8de19537967d/cureus-0012-00000011857-i01.jpg

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本文引用的文献

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