Division of Neuroradiology, Department of Diagnostic and Interventional Radiology, The Hospital for Sick Children, Toronto, Canada.
Department of Medical Imaging, University of Toronto, Toronto, Canada.
Korean J Radiol. 2024 Sep;25(9):807-823. doi: 10.3348/kjr.2023.1307.
Autoimmune encephalitis (AE) is a category of immune-mediated disorders of the central nervous system (CNS) affecting children and adults. It is characterized by the subacute onset of altered mentation, neurocognitive issues, refractory seizures/drug-resistant epilepsy, movement disorders, and/or autonomic dysfunction. AE is mediated by autoantibodies targeting specific surface components or intracytoplasmic antigens in the CNS, leading to functional or structural alterations. Multiple triggers that induce autoimmunity have been described, which are mainly parainfectious and paraneoplastic. The imaging features of AE often overlap with each other and with other common causes of encephalitis/encephalopathy (infections and toxic-metabolic etiologies). Limbic encephalitis is the most common imaging finding shared by most of these entities. Cortical, basal ganglia, diencephalon, and brainstem involvement may also be present. Cerebellar involvement is rare and is often a part of paraneoplastic degeneration. Owing to an improved understanding of AE, their incidence and detection have increased. Hence, in an appropriate setting, a high degree of suspicion is crucial when reporting clinical MRIs to ensure prompt treatment and better patient outcomes. In this review, we discuss the pathophysiology of AE and common etiologies encountered in clinical practice.
自身免疫性脑炎(AE)是一类影响儿童和成人的中枢神经系统(CNS)免疫介导性疾病。其特征为精神状态改变、神经认知问题、难治性癫痫发作/耐药性癫痫、运动障碍和/或自主神经功能障碍的亚急性发作。AE 由针对 CNS 特定表面成分或细胞内抗原的自身抗体介导,导致功能或结构改变。已描述了多种诱导自身免疫的触发因素,主要为感染后和肿瘤相关。AE 的影像学特征常相互重叠,也与其他常见脑炎/脑病的病因(感染和中毒代谢病因)重叠。边缘性脑炎是这些实体中最常见的共同影像学表现。皮质、基底节、间脑和脑干受累也可能存在。小脑受累罕见,常为副肿瘤变性的一部分。由于对 AE 的认识提高,其发病率和检出率均有所增加。因此,在适当的情况下,在报告临床 MRI 时高度怀疑是至关重要的,以确保及时治疗和更好的患者预后。在本文中,我们讨论了 AE 的发病机制和临床实践中常见的病因。