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双阳性边缘叶脑炎

Limbic Encephalitis With Dual Positivity.

作者信息

Erdil Yucesoy Esra, Tunc Handenur, Erdem Sema Nur, Bozkurt Suheyla, Tuncer Nese

机构信息

Neurosciences, King's College Hospital, London, GBR.

Department of Neurology, Marmara University Pendik Training and Research Hospital, İstanbul, TUR.

出版信息

Cureus. 2023 Jun 14;15(6):e40399. doi: 10.7759/cureus.40399. eCollection 2023 Jun.

DOI:10.7759/cureus.40399
PMID:37456392
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10346127/
Abstract

Limbic encephalitis is a well-defined clinical disorder among paraneoplastic neurological syndromes. Although it is not always possible to identify specific autoantibodies in limbic encephalitis, the presence of anti-neuronal nuclear antibody type 1 (ANNA1 or anti-Hu), anti-Ma2, collapsin response mediator protein 5 (CRMP-5-IgG or anti-CV2), anti-GABA receptors and anti-amphiphysin antibodies are often detected. A 66-year-old male patient with complaints of forgetfulness was evaluated in our clinic after having seizures. In the neurological examination, the patient was found to be confused. In cranial MR fluid-attenuated inversion recovery (FLAIR) and T2-weighted images, the right hippocampal and parahippocampal structures showed hyperintense areas complying with limbic encephalitis. He had improvement with a course of 2 g/kg intravenous immunoglobulin (IVIG) followed by high-dose methylprednisolone therapy. Following the high-dose methylprednisolone therapy, anti-PCA1 (Yo) and anti-amphiphysin antibodies were positive and the tissue pathology report confirmed combined small-cell carcinoma and large-cell neuroendocrine carcinoma of the lung. In recent years, paraneoplastic neurological syndromes are better recognized with the identification of specific antibodies and the ubiquitous information on pathogenesis. This is the first known report in the literature that a case with both positive anti-PCA1 (Yo) and anti-amphiphysin antibodies together and underlying small-cell and large-cell neuroendocrine carcinomas.

摘要

边缘叶脑炎是副肿瘤性神经系统综合征中一种明确的临床疾病。虽然在边缘叶脑炎中并非总能鉴定出特异性自身抗体,但常检测到1型抗神经元核抗体(ANNA1或抗Hu)、抗Ma2、塌陷反应介导蛋白5(CRMP - 5 - IgG或抗CV2)、抗GABA受体和抗 amphiphysin抗体。一名66岁男性患者,因癫痫发作后在我们诊所接受评估,主诉健忘。神经系统检查发现该患者意识模糊。在头颅磁共振液体衰减反转恢复序列(FLAIR)和T2加权图像上,右侧海马和海马旁结构显示符合边缘叶脑炎的高信号区。他在接受2 g/kg静脉注射免疫球蛋白(IVIG)疗程后,接着进行大剂量甲泼尼龙治疗,病情有所改善。在大剂量甲泼尼龙治疗后,抗PCA1(Yo)和抗amphiphysin抗体呈阳性,组织病理学报告证实为肺小细胞癌合并大细胞神经内分泌癌。近年来,随着特异性抗体的鉴定以及关于发病机制的广泛信息,副肿瘤性神经系统综合征得到了更好的认识。这是文献中首次报道的一例抗PCA1(Yo)和抗amphiphysin抗体均为阳性且伴有小细胞和大细胞神经内分泌癌的病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e724/10346127/8dae89417779/cureus-0015-00000040399-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e724/10346127/02ebc763677d/cureus-0015-00000040399-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e724/10346127/8dae89417779/cureus-0015-00000040399-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e724/10346127/02ebc763677d/cureus-0015-00000040399-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e724/10346127/8dae89417779/cureus-0015-00000040399-i02.jpg

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1
Limbic Encephalitis With Dual Positivity.双阳性边缘叶脑炎
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本文引用的文献

1
Detection of paraneoplastic antibodies and their significance in paraneoplastic neurologic syndromes: a narrative review.副肿瘤性抗体的检测及其在副肿瘤性神经系统综合征中的意义:一项叙述性综述。
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Integrative genomic profiling of large-cell neuroendocrine carcinomas reveals distinct subtypes of high-grade neuroendocrine lung tumors.
大细胞神经内分泌癌的综合基因组分析揭示了高级别神经内分泌肺肿瘤的不同亚型。
Nat Commun. 2018 Mar 13;9(1):1048. doi: 10.1038/s41467-018-03099-x.
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The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification.2015 年世界卫生组织肺肿瘤分类:自 2004 年分类以来遗传、临床和放射学进展的影响。
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Paraneoplastic neurological syndromes: a review.副肿瘤性神经系统综合征:综述
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Paraneoplastic syndromes of the CNS.中枢神经系统副肿瘤综合征
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Paraneoplastic rhombencephalitis and brachial plexopathy in two cases of amphiphysin auto-immunity.两例抗 amphiphysin 自身免疫性疾病中的副肿瘤性菱形脑炎和臂丛神经病
Eur Neurol. 2006;55(2):80-3. doi: 10.1159/000092307. Epub 2006 Mar 24.
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Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients.副肿瘤性边缘叶脑炎:50例患者的神经症状、免疫学发现及肿瘤关联
Brain. 2000 Jul;123 ( Pt 7):1481-94. doi: 10.1093/brain/123.7.1481.
10
The synaptic vesicle-associated protein amphiphysin is the 128-kD autoantigen of Stiff-Man syndrome with breast cancer.与突触小泡相关的蛋白 amphiphysin 是伴乳腺癌的僵人综合征的 128-kD 自身抗原。
J Exp Med. 1993 Dec 1;178(6):2219-23. doi: 10.1084/jem.178.6.2219.