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血清阴性自身免疫性脑炎合并超难治性癫痫持续状态的诊断挑战

The Diagnostic Challenge of Seronegative Autoimmune Encephalitis With Super-Refractory Status Epilepticus.

作者信息

Vadagandla Koti, Jahagirdar Vinay, Rama Kaanthi

机构信息

Anesthesia, Medicover Hospital, Hyderabad, IND.

Medicine, Gandhi Medical College and Hospital, Secunderabad, IND.

出版信息

Cureus. 2020 Nov 20;12(11):e11587. doi: 10.7759/cureus.11587.

Abstract

Autoimmune encephalitis is an immune-mediated syndrome, with sub-acute to chronic presentations, such as memory impairment, altered sensorium, behavioral abnormality, psychosis, and seizures. It poses a two-fold diagnostic challenge: firstly, because of its variable clinical presentation and secondly, due to the wide variety of autoimmune antibodies causing it, which makes it difficult to identify the underlying etiology. Treatment should not be delayed due to pending laboratory workup, as early recognition and initiation of therapy prevents long term neurological sequelae. This is a case report of a 59-year-old female who presented with neuropsychiatric symptoms, which evolved into refractory status epilepticus and autonomic dysfunction, requiring anesthesia induced coma. While her MRI had positive findings of encephalitis, she tested negative for infectious diseases and antibody panels.

摘要

自身免疫性脑炎是一种免疫介导的综合征,呈现亚急性至慢性症状,如记忆障碍、意识改变、行为异常、精神病和癫痫发作。它带来了双重诊断挑战:首先,因其临床表现多样;其次,导致该病的自身免疫抗体种类繁多,这使得难以确定潜在病因。不应因实验室检查有待完成而延迟治疗,因为早期识别和开始治疗可预防长期神经后遗症。这是一例59岁女性的病例报告,该患者出现神经精神症状,进而发展为难治性癫痫持续状态和自主神经功能障碍,需要麻醉诱导昏迷。虽然她的MRI有脑炎的阳性表现,但她的传染病和抗体检测均为阴性。

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A clinical approach to diagnosis of autoimmune encephalitis.自身免疫性脑炎的临床诊断方法
Lancet Neurol. 2016 Apr;15(4):391-404. doi: 10.1016/S1474-4422(15)00401-9. Epub 2016 Feb 20.
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The Diagnosis and Treatment of Autoimmune Encephalitis.自身免疫性脑炎的诊断与治疗
J Clin Neurol. 2016 Jan;12(1):1-13. doi: 10.3988/jcn.2016.12.1.1.
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