Charité - Universitätsmedizin Berlin, Department of Neurology and Experimental Neurology, Berlin, Germany.
Charité - Universitätsmedizin Berlin, Department of Neurology and Experimental Neurology, Berlin, Germany; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany; Humboldt-Universität zu Berlin, Berlin School of Mind and Brain, Berlin, Germany.
Rev Neurol (Paris). 2024 Nov;180(9):895-907. doi: 10.1016/j.neurol.2024.08.006. Epub 2024 Oct 2.
Autoimmune encephalitis encompasses a spectrum of conditions characterized by distinct clinical features and magnetic resonance imaging (MRI) findings. Here, we review the literature on acute MRI changes in the most common autoimmune encephalitis variants. In N-methyl-D-aspartate (NMDA) receptor encephalitis, most patients have a normal MRI in the acute stage. When lesions are present in the acute stage, they are typically subtle and non-specific white matter lesions that do not correspond with the clinical syndrome. In some NMDA receptor encephalitis cases, these T2-hyperintense lesions may be indicative of an NMDA receptor encephalitis overlap syndrome with simultaneous co-existence of multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Encephalitis with leucine-rich glioma-inactivated 1 (LGI1)-, contactin-associated protein-like 2 (CASPR2)- or glutamic acid decarboxylase (GAD)- antibodies typically presents as limbic encephalitis (LE) with unilateral or bilateral T2/fluid attenuated inversion recovery (FLAIR) hyperintensities in the medial temporal lobe that can progress to hippocampal atrophy. Gamma aminobutyric acid-B (GABA-B) receptor encephalitis also often shows such medial temporal hyperintensities but may additionally involve cerebellar lesions and atrophy. Gamma aminobutyric acid-A (GABA-A) receptor encephalitis features multifocal, confluent lesions in cortical and subcortical areas, sometimes leading to generalized atrophy. MRI is unremarkable in most patients with immunoglobulin-like cell adhesion molecule 5 (IgLON5)-disease, while individual case reports identified T2/FLAIR hyperintense lesions, diffusion restriction and atrophy in the brainstem, hippocampus and cerebellum. These findings highlight the need for MRI studies in patients with suspected autoimmune encephalitis to capture disease-specific changes and to exclude alternative diagnoses. Ideally, MRI investigations should be performed using dedicated autoimmune encephalitis imaging protocols. Longitudinal MRI studies play an important role to evaluate potential relapses and to manage long-term complications. Advanced MRI techniques and current research into imaging biomarkers will help to enhance the diagnostic accuracy of MRI investigations and individual patient outcome prediction. This will eventually enable better treatment decisions with improved clinical outcomes.
自身免疫性脑炎包括一系列以不同临床特征和磁共振成像 (MRI) 表现为特征的疾病。在这里,我们回顾了最常见的自身免疫性脑炎变体的急性 MRI 变化的文献。在 N-甲基-D-天冬氨酸 (NMDA) 受体脑炎中,大多数患者在急性阶段 MRI 正常。当急性阶段出现病变时,病变通常是细微且非特异性的白质病变,与临床综合征不对应。在一些 NMDA 受体脑炎病例中,这些 T2 高信号病变可能表明 NMDA 受体脑炎重叠综合征,同时存在多发性硬化 (MS)、视神经脊髓炎谱系障碍 (NMOSD) 或髓鞘少突胶质细胞糖蛋白抗体相关性疾病 (MOGAD)。富含亮氨酸胶质瘤失活 1 (LGI1)、接触蛋白样 2 (CASPR2) 或谷氨酸脱羧酶 (GAD) 抗体相关的脑炎通常表现为边缘性脑炎 (LE),单侧或双侧内侧颞叶 T2/液体衰减反转恢复 (FLAIR) 高信号,可进展为海马萎缩。γ 氨基丁酸 B (GABA-B) 受体脑炎也常表现出这种内侧颞叶高信号,但也可能累及小脑病变和萎缩。γ 氨基丁酸 A (GABA-A) 受体脑炎的特征是皮质和皮质下区域的多灶性、融合性病变,有时导致广泛萎缩。大多数免疫球蛋白样细胞黏附分子 5 (IgLON5) 疾病患者的 MRI 无明显异常,而个别病例报告则发现脑干、海马和小脑的 T2/FLAIR 高信号病变、弥散受限和萎缩。这些发现强调了在疑似自身免疫性脑炎患者中进行 MRI 研究以捕捉疾病特异性变化并排除其他诊断的必要性。理想情况下,应使用专用的自身免疫性脑炎成像方案进行 MRI 研究。纵向 MRI 研究对于评估潜在的复发和管理长期并发症具有重要作用。先进的 MRI 技术和当前的成像生物标志物研究将有助于提高 MRI 研究的诊断准确性和个体患者预后预测。这将最终使治疗决策更加优化,改善临床结果。