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World J Radiol. Jan 28, 2024; 16(1): 1-8
Published online Jan 28, 2024. doi: 10.4329/wjr.v16.i1.1
Anti-N-methyl-D-aspartate receptor-associated encephalitis: A review of clinicopathologic hallmarks and multimodal imaging manifestations
Bryce David Beutler, Alastair E Moody, Jerry Mathew Thomas, Benjamin Phillip Sugar, Mark B Ulanja, Daniel Antwi-Amoabeng, Lucas Anthony Tsikitas
Bryce David Beutler, Jerry Mathew Thomas, Benjamin Phillip Sugar, Lucas Anthony Tsikitas, Department of Radiology, University of Southern California, Keck School of Medicine, Los Angeles, CA 90033, United States
Alastair E Moody, Department of Anesthesiology, University of Utah, Salt Lake City, UT 84132, United States
Mark B Ulanja, Daniel Antwi-Amoabeng, Department of Internal Medicine, Christus Ochsner St. Patrick Hospital, Lake Charles, LA 70601, United States
Author contributions: Beutler BD performed the majority of the writing; Moody AE prepared the tables; Thomas JM and Sugar BP assisted with the literature review; Ulanja MB and Antwi-Amoabeng D contributed to the sections on pathogenesis and management; Tsikitas LA designed the outline and coordinated the writing of the paper.
Conflict-of-interest statement: None of the authors have real or potential conflicts of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non-Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Bryce David Beutler, MD, Doctor, Department of Radiology, University of Southern California, Keck School of Medicine, 1500 San Pablo Street, 2nd Floor, Los Angeles, CA 90033, United States. brycebeutler@hotmail.com
Received: September 30, 2023
Peer-review started: September 30, 2023
First decision: November 30, 2023
Revised: December 4, 2023
Accepted: December 25, 2023
Article in press: December 25, 2023
Published online: January 28, 2024
Processing time: 114 Days and 9.7 Hours
Abstract

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, electroencephalography, 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.

Keywords: Anti-N-methyl-D-aspartate receptor-associated encephalitis; Autoimmune encephalitis; Encephalitis; Ovarian teratoma; Paraneoplastic syndrome; Teratoma

Core Tip: 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 key clinicopathologic and imaging features of NMDARE are detailed in this minireview, including validated diagnostic criteria, magnetic resonance imaging findings, differential considerations, pathogenetic mechanisms, and treatment regimens. In addition, the role of nuclear imaging – including positron emission tomography and single-photon emission computed tomography – is described with the salient findings detailed in a comprehensive table.