Nantes Julia C, Thomas Adam G, Voets Natalie L, Best Jonathan G, Rosenthal Clive R, Al-Diwani Adam, Irani Sarosh R, Stagg Charlotte J
Physiological Neuroimaging Group, Wellcome Centre for Integrative Neuroimaging, FMRIB, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom.
Department of Neurology and Neurosurgery, McGill University, Montreal, QC, Canada.
Front Neurol. 2018 Sep 4;9:736. doi: 10.3389/fneur.2018.00736. eCollection 2018.
This is the first study to investigate functional brain activity in patients affected by autoimmune encephalitis with faciobrancial dystonic seizures (FBDS). Multimodal 3T MRI scans, including structural neuroimaging (T1-weighted, diffusion weighted) and functional neuroimaging (scene-encoding task known to activate hippocampal regions), were performed. This case series analysis included eight patients treated for autoimmune encephalitis with FBDS, scanned during the convalescent phase of their condition (median 1.1 years post-onset), and eight healthy volunteers. Compared to controls, 50% of patients showed abnormal hippocampal activity during scene-encoding relative to familiar scene-viewing. Higher peak FBDS frequency was significantly related to lower hippocampal activity during scene-encoding ( = 0.02), though not to markers of hippocampal microstructure (mean diffusivity, = 0.3) or atrophy (normalized volume, = 0.4). During scene-encoding, stronger within-medial temporal lobe (MTL) functional connectivity correlated with poorer Addenbrooke's Cognitive Examination-Revised memory score ( = 0.03). These findings suggest that in autoimmune encephalitis, frequent seizures may have a long-term impact on hippocampal activity, beyond that of structural damage. These observations also suggest a potential approach to determine on-going MTL performance in this condition to guide long-term management and future clinical trials.
这是第一项研究自身免疫性脑炎伴面臂肌张力障碍性癫痫(FBDS)患者大脑功能活动的研究。进行了多模态3T磁共振成像扫描,包括结构神经成像(T1加权、扩散加权)和功能神经成像(已知可激活海马区的场景编码任务)。该病例系列分析纳入了8例接受自身免疫性脑炎伴FBDS治疗的患者,在病情恢复期(发病后中位数1.1年)进行扫描,以及8名健康志愿者。与对照组相比,50%的患者在场景编码过程中相对于熟悉场景观看时海马活动异常。更高的FBDS峰值频率与场景编码过程中更低的海马活动显著相关(=0.02),但与海马微观结构标记物(平均扩散率,=0.3)或萎缩(标准化体积,=0.4)无关。在场景编码过程中,内侧颞叶(MTL)内更强的功能连接与更差的Addenbrooke认知检查修订版记忆评分相关(=0.03)。这些发现表明,在自身免疫性脑炎中,频繁发作可能对海马活动产生长期影响,超出结构损伤的影响。这些观察结果还提示了一种潜在方法,可用于确定这种情况下MTL的持续表现,以指导长期管理和未来的临床试验。