A 21-year-old woman presented with new-onset refractory status epilepticus after 5 days of fever. Orofacial myoclonus appeared on each attempt to stop sedation (Video). Blood/CSF analyses in search of infectious/autoimmune causes were negative. Two weeks later, MRI showed cortical, thalamic, hippocampal, and claustral hyperintensities (Fig. 1). MRI 6 weeks later showed persistence of signal changes and the appearance of diffuse cortical and subcortical (especially temporal) brain atrophy (Fig. 1). Febrile illness-related new-onset refractory status epilepticus with claustral hyperintensities is a rare condition of presumed autoimmune etiology (despite the absence of antibody identification) [1]. The claustrum (a multisensory process control center constituted by GABAergic interneurons) might play a role in promoting hypersynchronization of distant cortical regions [2]. The involvement of the fronto-opercular cortex adjacent to the claustrum may explain orofacial movements. Statement of Ethics

中文翻译：

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新发难治性癫痫持续状态相关的高信号

一名 21 岁女性在发烧 5 天后出现新发难治性癫痫持续状态。每次尝试停止镇静时都会出现口面部肌阵挛（视频）。寻找感染/自身免疫原因的血液/脑脊液分析呈阴性。两周后，MRI 显示皮质、丘脑、海马和锁骨高信号（图 1）。6 周后的 MRI 显示信号变化持续存在，并出现弥漫性皮质和皮质下（尤其是颞侧）脑萎缩（图 1）。发热性疾病相关的新发难治性癫痫持续状态伴闭锁性高信号是一种罕见的推测自身免疫病因的疾病（尽管没有抗体鉴定）[1]。claustrum（由 GABA 能中间神经元构成的多感觉过程控制中心）可能在促进远处皮层区域的超同步中发挥作用 [2]。与锁骨相邻的额盖皮层的参与可以解释口面部运动。道德声明