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自身免疫性癫痫中的听觉性发作:一例抗甲状腺抗体阳性病例

Auditory seizures in autoimmune epilepsy: a case with anti-thyroid antibodies.

作者信息

Varrasi Claudia, Vecchio Domizia, Magistrelli Luca, Strigaro Gionata, Tassi Laura, Cantello Roberto

机构信息

Gionata Strigaro Department of Translational Medicine, Section of Neurology, University of Piemonte Orientale, Novara.

Gionata Strigaro Department of Translational Medicine, Section of Neurology, University of Piemonte Orientale, Novara, CRRF Mons. L. Novarese, Neurorehabilitation Unit, Moncrivello, VC.

出版信息

Epileptic Disord. 2017 Mar 1;19(1):99-103. doi: 10.1684/epd.2017.0904.

Abstract

In its classic presentation, Hashimoto's encephalopathy is an acute-subacute complex neuropsychiatric syndrome with cognitive impairment, hallucinations, myoclonus, tremor or ataxia, associated with elevated anti-thyroid antibodies. Corticoids and immunotherapy are dramatically effective. However, in some cases, not all the associated features are presented and this delays diagnosis and appropriate treatment. We describe a man with abrupt onset of recurrent auditory seizures resulting in refractory non-convulsive status epilepticus. The patient was diagnosed with an autoimmune encephalopathy with elevated serum and CSF anti-thyroid antibodies. None of the antiepileptic drugs were successful, however, following immune-modulating therapy, the refractory non-convulsive status epilepticus dramatically improved, as did the patient overall. We suggest that Hashimoto's encephalopathy should be suspected in otherwise healthy patients with unexplained new-onset focal recurrent auditory seizures which do not respond to antiepileptic drugs. The presence of anti-thyroid antibodies in the CSF supports this diagnosis.

摘要

在其典型表现中,桥本脑病是一种急性-亚急性复杂的神经精神综合征,伴有认知障碍、幻觉、肌阵挛、震颤或共济失调,与抗甲状腺抗体升高有关。皮质类固醇和免疫疗法具有显著疗效。然而,在某些情况下,并非所有相关特征都会出现,这就延误了诊断和适当治疗。我们描述了一名男性,他突然发作反复性听觉发作,导致难治性非惊厥性癫痫持续状态。该患者被诊断为自身免疫性脑病,血清和脑脊液抗甲状腺抗体升高。抗癫痫药物均无效,然而,经过免疫调节治疗后,难治性非惊厥性癫痫持续状态显著改善,患者整体状况也有所改善。我们建议,对于原因不明的新发局灶性反复性听觉发作且对抗癫痫药物无反应的健康患者,应怀疑患有桥本脑病。脑脊液中存在抗甲状腺抗体支持这一诊断。

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