{"title":"巴林儿童抗nmda受体脑炎4例报告","authors":"Alia Khalil Ebrahim Ali Mohamed, A. Ali","doi":"10.26715/jbms.34_4_5","DOIUrl":null,"url":null,"abstract":"Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a newly described subtype of limbic encephalitis. This disease is probably the commonest autoimmune encephalitis predominantly affecting children and young adults. It has a higher incidence among young females. Patients manifest with a constellation of symptoms including psychosis, dyskinesia, and seizures. Antibodies against NR1/NR2 heteromers of the NMDAR glutamate receptor mediate this type of encephalitis. Patients with anti-NMDAR encephalitis might have underlying tumors, which are typically teratomas. Exploring for occult tumor and resection is crucial in management. First-tier immunotherapy include steroids, intravenous immunoglobulin and plasma exchange, while rituximab and cyclophosphamide are frequently used as second-tier immunotherapy. Around 80% of patients achieve almost complete recovery, however some patients suffer from cognitive deficits or death. Early diagnosis and prompt treatment can lead to better recovery and outcome. The unique feature of this disease reflects the intimate relationship between psychiatry and neurology. In this article, we describe the clinical phenotype and outcome of four affected children from Bahrain.","PeriodicalId":85027,"journal":{"name":"Journal of the Bahrain Medical Society = Majallat Jam'iyat al-Atibba' al-Bahrayniyah","volume":"1 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Anti-NMDA Receptor Encephalitis in Children: Report of Four Cases from Bahrain\",\"authors\":\"Alia Khalil Ebrahim Ali Mohamed, A. Ali\",\"doi\":\"10.26715/jbms.34_4_5\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a newly described subtype of limbic encephalitis. This disease is probably the commonest autoimmune encephalitis predominantly affecting children and young adults. It has a higher incidence among young females. Patients manifest with a constellation of symptoms including psychosis, dyskinesia, and seizures. Antibodies against NR1/NR2 heteromers of the NMDAR glutamate receptor mediate this type of encephalitis. Patients with anti-NMDAR encephalitis might have underlying tumors, which are typically teratomas. Exploring for occult tumor and resection is crucial in management. First-tier immunotherapy include steroids, intravenous immunoglobulin and plasma exchange, while rituximab and cyclophosphamide are frequently used as second-tier immunotherapy. Around 80% of patients achieve almost complete recovery, however some patients suffer from cognitive deficits or death. Early diagnosis and prompt treatment can lead to better recovery and outcome. The unique feature of this disease reflects the intimate relationship between psychiatry and neurology. In this article, we describe the clinical phenotype and outcome of four affected children from Bahrain.\",\"PeriodicalId\":85027,\"journal\":{\"name\":\"Journal of the Bahrain Medical Society = Majallat Jam'iyat al-Atibba' al-Bahrayniyah\",\"volume\":\"1 1\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of the Bahrain Medical Society = Majallat Jam'iyat al-Atibba' al-Bahrayniyah\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.26715/jbms.34_4_5\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of the Bahrain Medical Society = Majallat Jam'iyat al-Atibba' al-Bahrayniyah","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.26715/jbms.34_4_5","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
摘要
抗n -甲基- d -天冬氨酸受体(NMDAR)脑炎是一种新发现的边缘脑炎亚型。这种疾病可能是最常见的自身免疫性脑炎,主要影响儿童和年轻人。它在年轻女性中发病率较高。患者表现出一系列症状,包括精神病、运动障碍和癫痫发作。抗NMDAR谷氨酸受体NR1/NR2异聚体的抗体介导这种类型的脑炎。抗nmdar脑炎患者可能有潜在的肿瘤,通常是畸胎瘤。隐匿性肿瘤的探查和切除是治疗的关键。一级免疫治疗包括类固醇、静脉注射免疫球蛋白和血浆交换,而利妥昔单抗和环磷酰胺常被用作二级免疫治疗。大约80%的患者几乎完全康复,但一些患者出现认知障碍或死亡。早期诊断和及时治疗可以带来更好的恢复和结果。这种疾病的独特特征反映了精神病学和神经病学之间的密切关系。在这篇文章中,我们描述了临床表型和结果四个受影响的儿童从巴林。
Anti-NMDA Receptor Encephalitis in Children: Report of Four Cases from Bahrain
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a newly described subtype of limbic encephalitis. This disease is probably the commonest autoimmune encephalitis predominantly affecting children and young adults. It has a higher incidence among young females. Patients manifest with a constellation of symptoms including psychosis, dyskinesia, and seizures. Antibodies against NR1/NR2 heteromers of the NMDAR glutamate receptor mediate this type of encephalitis. Patients with anti-NMDAR encephalitis might have underlying tumors, which are typically teratomas. Exploring for occult tumor and resection is crucial in management. First-tier immunotherapy include steroids, intravenous immunoglobulin and plasma exchange, while rituximab and cyclophosphamide are frequently used as second-tier immunotherapy. Around 80% of patients achieve almost complete recovery, however some patients suffer from cognitive deficits or death. Early diagnosis and prompt treatment can lead to better recovery and outcome. The unique feature of this disease reflects the intimate relationship between psychiatry and neurology. In this article, we describe the clinical phenotype and outcome of four affected children from Bahrain.