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以癫痫为首发症状的复发性抗N-甲基-D-天冬氨酸受体脑炎发展为自身免疫相关性癫痫:一例报告

Development from recurrent anti-N-methyl-D-aspartate receptor encephalitis with seizures as the first symptom to autoimmune-associated epilepsy: a case report.

作者信息

Qin Ningxiang, Wang Jing, Peng Xi, Wang Liang

机构信息

Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.

Department of Neurology, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, 400010, China.

出版信息

Acta Epileptol. 2023 Jul 26;5(1):19. doi: 10.1186/s42494-023-00129-0.

DOI:10.1186/s42494-023-00129-0
PMID:40217286
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11960213/
Abstract

BACKGROUND

Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a novel autoimmune encephalitis (AE) first identified in 2007. It provides a new direction for clinicians when encountering unexplained symptoms such as seizures, psychotic behavioral abnormalities, speech disorders, and involuntary movements. Most patients have a good prognosis after immunotherapy, but some may experience relapses.

CASE PRESENTATION

We report a Chinese female patient diagnosed with anti-NMDAR encephalitis. Over the past 30 years, the patient had experienced eight episodes with seizures as the first symptom, which eventually progressed to autoimmune-associated epilepsy. In the last two episodes, both serum and cerebrospinal fluid of the patient were negative for AE-related antibodies, and brain magnetic resonance imaging (MRI) revealed abnormal hyperintensity in the bilateral hippocampi. The patient's symptoms were poorly controlled by immunotherapy but well controlled by anti-seizure medicines.

CONCLUSIONS

Patients with a long history of AE and multiple relapses that start with seizures may display alterations of brain structure. Physicians should pay attention to autoimmune-associated epilepsy.

摘要

背景

抗N-甲基-D-天冬氨酸受体(抗NMDAR)脑炎是一种于2007年首次被发现的新型自身免疫性脑炎(AE)。它为临床医生在遇到诸如癫痫发作、精神行为异常、言语障碍和不自主运动等不明原因症状时提供了新的方向。大多数患者在接受免疫治疗后预后良好,但有些患者可能会复发。

病例报告

我们报告了一名被诊断为抗NMDAR脑炎的中国女性患者。在过去30年里,该患者曾经历过8次以癫痫发作为首发症状的发作,最终发展为自身免疫相关癫痫。在最后两次发作中,患者的血清和脑脊液中与AE相关的抗体均为阴性,脑磁共振成像(MRI)显示双侧海马区有异常高信号。该患者的症状通过免疫治疗控制不佳,但通过抗癫痫药物得到了良好控制。

结论

有AE病史且多次复发并以癫痫发作为起始症状的患者可能会出现脑结构改变。医生应注意自身免疫相关癫痫。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b97/11960213/dd2b231478dc/42494_2023_129_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b97/11960213/8a59e3f7c82d/42494_2023_129_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b97/11960213/6ea3ac9f97b0/42494_2023_129_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b97/11960213/dd2b231478dc/42494_2023_129_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b97/11960213/8a59e3f7c82d/42494_2023_129_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b97/11960213/6ea3ac9f97b0/42494_2023_129_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6b97/11960213/dd2b231478dc/42494_2023_129_Fig3_HTML.jpg

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本文引用的文献

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Epileptic phenotypes in autoimmune encephalitis: from acute symptomatic seizures to autoimmune-associated epilepsy.自身免疫性脑炎中的癫痫表型:从急性症状性癫痫发作到自身免疫相关性癫痫
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