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抗Ma2脑炎:一例与克-布综合征及下丘脑功能障碍相关的非典型病例。

Anti-Ma2 Encephalitis: An Atypical Case Associated With Klüver-Bucy Syndrome and Hypothalamic Dysfunction.

作者信息

Wernz Cort, Pupa Lauren, Ossimetha Ashley, Ansari Sahifah, Moron Fanny

机构信息

Radiology, Baylor College of Medicine, Houston, USA.

出版信息

Cureus. 2023 Jun 22;15(6):e40816. doi: 10.7759/cureus.40816. eCollection 2023 Jun.

DOI:10.7759/cureus.40816
PMID:37485201
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10362970/
Abstract

Anti-Ma2 encephalitis is a rare form of autoimmune encephalitis that has classically been described as a paraneoplastic neurobehavioral disorder due to its association with underlying malignancies. We discuss the case of a 30-year-old female with an exceptionally aggressive presentation of anti-Ma2 encephalitis accompanied by Klüver-Bucy syndrome and hypothalamic dysfunction. Her course was complicated by repeated aspiration events secondary to severe hyperphagia and delays in immunosuppressive treatment due to concerns of infection. The patient's encephalitis was refractory to multiple immunosuppressive therapies and she ultimately expired before a primary malignancy could be detected and treated.

摘要

抗Ma2脑炎是一种罕见的自身免疫性脑炎,由于其与潜在恶性肿瘤相关,传统上被描述为一种副肿瘤性神经行为障碍。我们讨论了一例30岁女性患者,其抗Ma2脑炎表现异常侵袭性,伴有克吕弗-布西综合征和下丘脑功能障碍。她的病程因严重贪食继发反复误吸事件以及因担心感染而延迟免疫抑制治疗而复杂化。患者的脑炎对多种免疫抑制治疗均无效,最终在原发性恶性肿瘤被检测和治疗之前死亡。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/da7c/10362970/812aba62934c/cureus-0015-00000040816-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/da7c/10362970/73ea28f1e498/cureus-0015-00000040816-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/da7c/10362970/b8ae9302d7ea/cureus-0015-00000040816-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/da7c/10362970/812aba62934c/cureus-0015-00000040816-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/da7c/10362970/73ea28f1e498/cureus-0015-00000040816-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/da7c/10362970/b8ae9302d7ea/cureus-0015-00000040816-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/da7c/10362970/812aba62934c/cureus-0015-00000040816-i03.jpg

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本文引用的文献

1
Anti-Ma2-associated limbic encephalitis presenting with transient epileptic amnesia.伴有短暂性癫痫性遗忘症的抗Ma2相关边缘叶脑炎
Epileptic Disord. 2022 Aug 1;24(4):723-725. doi: 10.1684/epd.2022.1440.
2
Anti-Ma2-antibody-associated encephalitis: An atypical paraneoplastic neurologic syndrome.抗Ma2抗体相关脑炎:一种非典型副肿瘤性神经系统综合征。
SA J Radiol. 2018 May 17;22(1):1310. doi: 10.4102/sajr.v22i1.1310. eCollection 2018.
3
Kluver-Bucy syndrome in a girl with anti-NMDAR encephalitis.一名患有抗NMDAR脑炎的女孩出现克吕弗-布西综合征。
Neurol India. 2019 May-Jun;67(3):887-889. doi: 10.4103/0028-3886.263181.
4
Anti-Ma2-associated paraneoplastic encephalitis eat, sleep and repeat.抗Ma2相关副肿瘤性脑炎,吃、睡,循环往复。
Intern Med J. 2019 Jul;49(7):931-932. doi: 10.1111/imj.14349.
5
Atypical presentation of anti-Ma2-associated encephalitis with choreiform movement.伴有舞蹈样动作的抗Ma2相关脑炎的非典型表现。
Neurol Neuroimmunol Neuroinflamm. 2019 Apr 1;6(3):e557. doi: 10.1212/NXI.0000000000000557. eCollection 2019 May.
6
Expanding Role of T Cells in Human Autoimmune Diseases of the Central Nervous System.T细胞在人类中枢神经系统自身免疫性疾病中不断扩大的作用。
Front Immunol. 2017 Jun 7;8:652. doi: 10.3389/fimmu.2017.00652. eCollection 2017.
7
Microscopic dysgerminoma associated with anti-Ma2 paraneoplastic encephalitis in a patient with gonadal dysgenesis.伴有抗 Ma2 副肿瘤性脑炎的生殖细胞肿瘤患者的镜下微小性卵黄囊瘤。
Int J Gynecol Pathol. 2013 May;32(3):277-82. doi: 10.1097/PGP.0b013e31825f7c25.
8
[Anti-Ma2-associated encephalitis and paraneoplastic limbic encephalitis].[抗Ma2相关脑炎和副肿瘤性边缘叶脑炎]
Brain Nerve. 2010 Aug;62(8):838-51.
9
Anti-Ma2 paraneoplastic encephalitis associated with testicular germ cell tumor treated by carboplatin, etoposide and bleomycin.由卡铂、依托泊苷和博来霉素治疗的与睾丸生殖细胞肿瘤相关的抗Ma2副肿瘤性脑炎。
Int J Urol. 2008 Oct;15(10):942-3. doi: 10.1111/j.1442-2042.2008.02119.x.
10
Hypersomnia as presenting symptom of anti-Ma2-associated encephalitis: case study.以发作性睡病为首发症状的抗Ma2相关脑炎:病例报告
Neuro Oncol. 2007 Jan;9(1):75-7. doi: 10.1215/15228517-2006-013. Epub 2006 Oct 3.