Patel Krunal H, Chowdhury Yuvraj, Shetty Mrinali, Uppin Vaibhavi, Madaj Paul, Salifu Moro O, Youssef Mary, Henglein Victoria L, McFaralne Samy I
Department of Internal Medicine, State University of New York, Downstate Medical Center, Brooklyn, NY, USA - 11203.
Division of Cardiovascular Disease, State University of New York, Downstate Medical Center, Brooklyn, NY, USA - 11203.
Am J Med Case Rep. 2020;8(12):503-507. doi: 10.12691/ajmcr-8-12-20. Epub 2020 Sep 25.
Described in 2007, anti-N-methyl-d-aspartate receptor encephalitis (ANMDARE) is a rare autoimmune limbic encephalitis affecting young adults (predominantly women of reproductive age) and is a paraneoplastic manifestation of ovarian teratoma in about half of the cases. ANMDARE is characterized by psychiatric changes, neurological changes, autonomic instability and cardiac dysrhythmias. In this report, we present a 36-year-old woman who was 16 weeks pregnant and brought to the hospital with confusion and subsequently had a seizure with Electroencephalography (EEG) demonstrated an extreme delta brush pattern consistent with ANMDARE. Patient developed sinus nodal dysfunction and was also found to have ovarian teratoma, a rather typical presentation for ANMDARE, that is considered a paraneoplastic syndrome for ovarian teratoma. In this report, we highlight the cardiac manifestation of ANMDARE, the pathophysiology associated with autonomic instability, and management strategies of this rare, and largely devastating illness.
抗N-甲基-D-天冬氨酸受体脑炎（ANMDARE）于2007年被描述，是一种罕见的自身免疫性边缘叶脑炎，主要影响年轻成年人（以育龄女性为主），约半数病例是卵巢畸胎瘤的副肿瘤表现。抗N-甲基-D-天冬氨酸受体脑炎的特征为精神改变、神经改变、自主神经不稳定和心律失常。在本报告中，我们介绍了一名36岁的孕妇，她怀孕16周，因意识模糊入院，随后发生癫痫，脑电图（EEG）显示为与抗N-甲基-D-天冬氨酸受体脑炎一致的极端δ刷状波模式。患者出现窦房结功能障碍，还发现患有卵巢畸胎瘤，这是抗N-甲基-D-天冬氨酸受体脑炎相当典型的表现，被认为是卵巢畸胎瘤的副肿瘤综合征。在本报告中，我们强调了抗N-甲基-D-天冬氨酸受体脑炎的心脏表现、与自主神经不稳定相关的病理生理学，以及这种罕见且极具破坏性疾病的管理策略。
