Department of Neurology, Charité-Universitätsmedizin Berlin, Berlin, Germany; Berlin Center for Advanced Neuroimaging, Charité-Universitätsmedizin Berlin, Berlin, Germany.
Ann Neurol. 2013 Aug;74(2):284-96. doi: 10.1002/ana.23932. Epub 2013 Jul 8.
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune encephalitis with a characteristic neuropsychiatric syndrome and severe and prolonged clinical courses. In contrast, standard clinical magnetic resonance imaging (MRI) remains normal in the majority of patients. Here, we investigated structural and functional brain changes in a cohort of patients with anti-NMDAR encephalitis.
Twenty-four patients with established diagnosis of anti-NMDAR encephalitis and age- and gender-matched controls underwent neuropsychological testing and multimodal MRI, including T1w/T2w structural imaging, analysis of resting state functional connectivity, analysis of white matter using diffusion tensor imaging, and analysis of gray matter using voxel-based morphometry.
Patients showed significantly reduced functional connectivity of the left and right hippocampus with the anterior default mode network. Connectivity of both hippocampi predicted memory performance in patients. Diffusion tensor imaging revealed extensive white matter changes, which were most prominent in the cingulum and which correlated with disease severity. In contrast, no differences in T1w/T2w structural imaging and gray matter morphology were observed between patients and controls.
Anti-NMDAR encephalitis is associated with characteristic alterations of functional connectivity and widespread changes of white matter integrity despite normal findings in routine clinical MRI. These results may help to explain the clinicoradiological paradox in anti-NMDAR encephalitis and advance the pathophysiological understanding of the disease. Correlation of imaging abnormalities with disease symptoms and severity suggests that these changes play an important role in the symptomatology of anti-NMDAR encephalitis.
抗 N-甲基-D-天冬氨酸受体（NMDAR）脑炎是一种自身免疫性脑炎，具有特征性的神经精神综合征和严重且持久的临床病程。相比之下，大多数患者的标准临床磁共振成像（MRI）仍正常。在这里，我们研究了一组抗 NMDAR 脑炎患者的大脑结构和功能变化。
24 例确诊为抗 NMDAR 脑炎的患者和年龄、性别匹配的对照组接受了神经心理学测试和多模态 MRI，包括 T1w/T2w 结构成像、静息态功能连接分析、扩散张量成像的白质分析以及基于体素的形态测量学的灰质分析。
患者的左、右海马与前默认模式网络的功能连接明显降低。两个海马的连接性可以预测患者的记忆表现。弥散张量成像显示广泛的白质变化，以扣带最为明显，且与疾病严重程度相关。相比之下，患者和对照组之间在 T1w/T2w 结构成像和灰质形态上没有差异。
抗 NMDAR 脑炎与功能连接的特征性改变以及广泛的白质完整性改变有关，尽管常规临床 MRI 检查结果正常。这些结果可能有助于解释抗 NMDAR 脑炎的临床影像学悖论，并推进对疾病的病理生理学认识。影像学异常与疾病症状和严重程度的相关性表明，这些变化在抗 NMDAR 脑炎的症状学中起着重要作用。
