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以持续发热和脑膜炎为表现且对独特的个体化一线治疗反应良好的抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎:一例报告

Anti-NMDAR encephalitis presenting with persistent fever and meningitis and responding well to distinctive individualized first-line treatment: A case report.

作者信息

Jin Xin, Zhuang Jianhua, Xu Jin

机构信息

Department of Neurology, Second Affiliated Hospital of Naval Medical University, Shanghai Changzheng Hospital, Shanghai, China.

出版信息

Medicine (Baltimore). 2024 Dec 6;103(49):e40803. doi: 10.1097/MD.0000000000040803.

DOI:10.1097/MD.0000000000040803
PMID:39654216
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11630919/
Abstract

RATIONALE

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune encephalitis characterized by diverse neurological and psychiatric symptoms. It predominantly affects young women, particularly those with ovarian teratomas. However, cases without teratomas are also commonly reported, often exhibiting poorer treatment responses and higher relapse rates. Persistent fever and signs of meningitis are rare in such cases. Diagnosis is confirmed through the detection of anti-NMDAR antibodies.

PATIENT CONCERNS

A 15-year-old female presented with episodic loss of consciousness, fever, nuchal rigidity, limb convulsions, and psychiatric symptoms following a stressful exam period. Initial symptomatic treatments were ineffective.

DIAGNOSIS

The diagnosis of anti-NMDAR encephalitis was confirmed through lumbar puncture, brain imaging, and the detection of anti-NMDAR antibodies in cerebrospinal fluid and serum.

INTERVENTIONS

The patient received tailored first-line therapy, including high-dose methylprednisolone and 3 courses of intravenous immunoglobulin (IVIG).

OUTCOMES

The patient exhibited significant clinical improvement, with a reduction in seizure frequency and eventual complete seizure control. Body temperature normalized, and follow-up showed progressive recovery in cognitive and motor functions.

LESSONS

This case highlights the importance of early diagnosis and individualized treatment in anti-NMDAR encephalitis. Repeated IVIG courses proved effective, underscoring the need for personalized treatment plans in managing this condition. Persistent fever and signs of meningitis were rare and contributed to the diagnostic challenge, highlighting the clinical complexity of this case.

摘要

理论依据

抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎是一种自身免疫性脑炎,其特征为多种神经和精神症状。它主要影响年轻女性,尤其是患有卵巢畸胎瘤的女性。然而,无畸胎瘤的病例也常有报道,这些病例往往治疗反应较差且复发率较高。此类病例中持续性发热和脑膜炎体征罕见。通过检测抗NMDAR抗体确诊。

患者情况

一名15岁女性在一段压力较大的考试期后出现发作性意识丧失、发热、颈项强直、肢体抽搐及精神症状。初始对症治疗无效。

诊断

通过腰椎穿刺、脑部成像以及脑脊液和血清中抗NMDAR抗体的检测,确诊为抗NMDAR脑炎。

干预措施

患者接受了针对性的一线治疗,包括大剂量甲泼尼龙和3个疗程的静脉注射免疫球蛋白(IVIG)。

结果

患者临床症状显著改善,癫痫发作频率降低,最终癫痫完全得到控制。体温恢复正常,随访显示认知和运动功能逐渐恢复。

经验教训

该病例凸显了抗NMDAR脑炎早期诊断和个体化治疗的重要性。重复使用IVIG疗程证明有效,强调了在管理这种疾病时制定个性化治疗方案的必要性。持续性发热和脑膜炎体征罕见,增加了诊断难度,凸显了该病例的临床复杂性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f6c/11630919/cc72d2f06ab2/medi-103-e40803-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f6c/11630919/c667187aab5a/medi-103-e40803-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f6c/11630919/64bcd33f40d7/medi-103-e40803-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f6c/11630919/dacccbc2a4f4/medi-103-e40803-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f6c/11630919/cc72d2f06ab2/medi-103-e40803-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f6c/11630919/c667187aab5a/medi-103-e40803-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f6c/11630919/64bcd33f40d7/medi-103-e40803-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f6c/11630919/dacccbc2a4f4/medi-103-e40803-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f6c/11630919/cc72d2f06ab2/medi-103-e40803-g004.jpg

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本文引用的文献

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Anti-NMDA Receptor Encephalitis: Retrospective Analysis of 15 Cases, Literature Review, and Implications for Gynecologists.抗 N- 甲基-D-天冬氨酸受体脑炎:15 例回顾性分析、文献复习及对妇科医生的启示。
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Use and Safety of Immunotherapeutic Management of N-Methyl-d-Aspartate Receptor Antibody Encephalitis: A Meta-analysis.免疫治疗 NMDA 受体抗体脑炎的使用和安全性:一项荟萃分析。
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HSV encephalitis triggered anti-NMDAR encephalitis: a case report.
单纯疱疹病毒性脑炎引发抗N-甲基-D-天冬氨酸受体脑炎:一例病例报告
Neurol Sci. 2021 Mar;42(3):857-861. doi: 10.1007/s10072-020-04785-9. Epub 2021 Jan 8.
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Autoimmune Encephalitis: Current Knowledge on Subtypes, Disease Mechanisms and Treatment.自身免疫性脑炎:亚类、疾病机制和治疗的最新知识。
CNS Neurol Disord Drug Targets. 2020;19(8):584-598. doi: 10.2174/1871527319666200708133103.
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Anti-NMDA receptor encephalitis associated with ovarian tumor: the gynecologist point of view.抗 NMDA 受体脑炎与卵巢肿瘤相关:妇科医生的观点。
Arch Gynecol Obstet. 2020 Aug;302(2):315-320. doi: 10.1007/s00404-020-05645-9. Epub 2020 Jun 17.
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Current Progress on Assessing the Prognosis for Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis.抗 N-甲基-D-天冬氨酸受体(NMDAR)脑炎预后评估的研究进展。
Biomed Res Int. 2020 Apr 14;2020:7506590. doi: 10.1155/2020/7506590. eCollection 2020.
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Case report: meningitis as a presenting feature of anti-NMDA receptor encephalitis.病例报告:以脑膜炎为表现的抗 NMDA 受体脑炎。
BMC Infect Dis. 2020 Jan 7;20(1):21. doi: 10.1186/s12879-020-4761-1.
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Anti-NMDAR encephalitis: A single-center, longitudinal study in China.抗 NMDAR 脑炎:中国的单中心纵向研究。
Neurol Neuroimmunol Neuroinflamm. 2019 Oct 16;7(1). doi: 10.1212/NXI.0000000000000633. Print 2020 Jan.
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Immunotherapy for recurrent pregnancy loss.免疫疗法治疗复发性妊娠丢失。
Best Pract Res Clin Obstet Gynaecol. 2019 Oct;60:77-86. doi: 10.1016/j.bpobgyn.2019.07.005. Epub 2019 Jul 30.
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