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副肿瘤性NMDA脑炎:一例报告及现有文献综述

Paraneoplastic NMDA encephalitis, a case report and an extensive review of available literature.

作者信息

Alzghoul Hamza, Kadri Ferdous, Ismail Mohamed F, Youssef Robeer, Shamaileh Mustafa, Al-Assi Ahmad R, Adzhieva Liliya, Alzghoul Bashar

机构信息

University of Central Florida College of Medicine, Graduate Medical Education, Orlando, FL, USA.

Division of Pulmonary and Critical Care Medicine, Cedars Sinai Medical Center, Los Angeles, CA, USA.

出版信息

Radiol Case Rep. 2024 Jan 15;19(4):1371-1385. doi: 10.1016/j.radcr.2023.11.087. eCollection 2024 Apr.

Abstract

Anti-N-methyl-D-aspartate receptor (NMDAr) encephalitis is a prevalent autoimmune condition marked by diverse neuropsychiatric symptoms, primarily impacting young females. The exact mechanisms underlying the development of NMDAr encephalitis have not been fully elucidated. Nonetheless, studies have demonstrated that auto-antibodies targeting the NR1-NR2 subunits of the NMDAr can trigger receptor dysfunction within the central nervous system, thus giving rise to the associated symptoms. Notably, an association exists between NMDAr encephalitis and an underlying neoplastic condition, with approximately 38% of cases exhibiting this paraneoplastic relationship with ovarian teratomas being the most commonly associated malignancy. While the association between NMDAr encephalitis and renal cell carcinoma (RCC) is exceedingly rare. This case report presents the clinical scenario of a 20-year-old female patient diagnosed with NMDAr encephalitis in conjunction with RCC discovered incidentally on a CT abdomen and pelvis performed to rule out an ovarian teratoma. The presented case underscores the importance of adopting a multidisciplinary approach in the diagnosis and treatment of NMDAr encephalitis, particularly when it is linked to an underlying malignancy. Furthermore, it emphasizes the significance of expanding our understanding of the molecular pathogenesis of NMDAr encephalitis to enhance patient care and optimize clinical outcomes. Additionally, a comprehensive review of the existing literature is included, summarizing all reported malignancies associated with NMDAr encephalitis.

摘要

抗N-甲基-D-天冬氨酸受体(NMDAr)脑炎是一种常见的自身免疫性疾病,以多种神经精神症状为特征,主要影响年轻女性。NMDAr脑炎发病的确切机制尚未完全阐明。尽管如此,研究表明,靶向NMDAr的NR1-NR2亚基的自身抗体可引发中枢神经系统内的受体功能障碍,从而产生相关症状。值得注意的是,NMDAr脑炎与潜在的肿瘤性疾病之间存在关联,约38%的病例表现出这种副肿瘤关系,其中卵巢畸胎瘤是最常见的相关恶性肿瘤。而NMDAr脑炎与肾细胞癌(RCC)之间的关联极为罕见。本病例报告介绍了一名20岁女性患者的临床情况,该患者被诊断为NMDAr脑炎,同时在为排除卵巢畸胎瘤而进行的腹部和盆腔CT检查中偶然发现了肾细胞癌。该病例强调了在NMDAr脑炎的诊断和治疗中采用多学科方法的重要性,尤其是当它与潜在的恶性肿瘤相关时。此外,它强调了扩大我们对NMDAr脑炎分子发病机制的理解以改善患者护理和优化临床结果的重要性。此外,还对现有文献进行了全面综述,总结了所有与NMDAr脑炎相关的已报道恶性肿瘤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9ab3/10823033/68a8cefdfe43/gr1.jpg

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