Anti-N-methyl-D-aspartate receptor-associated encephalitis: A review of clinicopathologic hallmarks and multimodal imaging manifestations  

作　　者：Bryce David Beutler Alastair E Moody Jerry Mathew Thomas Benjamin Phillip Sugar Mark B Ulanja Daniel Antwi-Amoabeng Lucas Anthony Tsikitas 

机构地区：[1]Department of Radiology,University of Southern California,Keck School of Medicine,Los Angeles,CA 90033,United States [2]Department of Anesthesiology,University of Utah,Salt Lake City,UT 84132,United States [3]Department of Internal Medicine,Christus Ochsner St.Patrick Hospital,Lake Charles,LA 70601,United States

出　　处：《World Journal of Radiology》2024年第1期1-8,共8页世界放射学杂志（英文版）（电子版）

摘　　要：Anti-N-methyl-D-aspartate receptor-associated encephalitis(NMDARE)is a rare immune-mediated neuroinflammatory condition characterized by the rapid onset of neuropsychiatric symptoms and autonomic dysfunction.The mechanism of pathogenesis remains incompletely understood,but is thought to be related to antibodies targeting the GluN1 subunit of the NMDA receptor with resultant downstream dysregulation of dopaminergic pathways.Young adults are most frequently affected;the median age at diagnosis is 21 years.There is a strong female predilection with a female sex predominance of 4:1.NMDARE often develops as a paraneoplastic process and is most commonly associated with ovarian teratoma.However,NMDARE has also been described in patients with small cell lung cancer,clear cell renal carcinoma,and other benign and malignant neoplasms.Diagnosis is based on correlation of the clinical presentation,electro-encephalography,laboratory studies,and imaging.Computed tomography,positron emission tomography,and magnetic resonance imaging are essential to identify an underlying tumor,exclude clinicopathologic mimics,and predict the likelihood of long-term functional impairment.Nuclear imaging may be of value for prognostication and to assess the response to therapy.Treatment may involve high-dose corticosteroids,intravenous immunoglobulin,and plasma exchange.Herein,we review the hallmark clinicopathologic features and imaging findings of this rare but potentially devastating condition and summarize diagnostic criteria,treatment regimens,and proposed pathogenetic mechanisms.

关 键 词：Anti-N-methyl-D-aspartate receptor-associated encephalitis Autoimmune encephalitis ENCEPHALITIS Ovarian teratoma Paraneoplastic syndrome TERATOMA 

分 类 号：R74[医药卫生—神经病学与精神病学]