Safan Abeer Sabry, Al-Termanini Mohammad, Abdelhady Mohamed, Osman Yasir, Awad Elzouki Abdel-Nasser Y, Abdussalam Ahmed Lutfe
Department of Neurology, Neurosciences Institute, Hamad Medical Corporation, Doha, Qatar.
Department of Internal Medicine, Hamad Medical Corporation, Doha, Qatar.
eNeurologicalSci. 2023 May 16;31:100465. doi: 10.1016/j.ensci.2023.100465. eCollection 2023 Jun.
Limbic encephalitis (LE) is a rare variant of autoimmune encephalitis. It often manifests with subacute neuropsychiatric symptoms of agitation, delusions, variable seizure semiology, and short-term memory loss. Seronegative limbic encephalitis can pose a diagnostic conundrum owing to its inadequately understood pathophysiology.
We report a rare case of a young male with subacute neuropsychiatric manifestations of delusions, agitations and seizures. He was diagnosed with seronegative limbic encephalitis (SNLE). Brain MRI demonstrated bilateral Claustrum sparing sign. An EEG showed continuous left-sided epileptiform discharges in periodic to predominantly left middle temporal. Patient condition gradually improved with pulsed methylprednisolone, intravenous immunoglobulins and anti-seizure medications.
Claustrum remains one of the least understood neuroanatomical structures. Claustrum sign has been reported in febrile infection-related epilepsy syndrome (FIRES), LE, and autoimmune refractory epilepsy. To the best of our knowledge, we report the first case in literature with Claustrum sparing sign in seronegative Limbic Encephalitis. Further experimental models and researches are warranted to better understand the unique function of the claustrum and unravel possible other attributable auto-antibodies, which could alter treatment and prognosis.
边缘叶脑炎（LE）是自身免疫性脑炎的一种罕见变体。它常表现为亚急性神经精神症状，如躁动、妄想、多样的癫痫发作形式和短期记忆丧失。血清学阴性边缘叶脑炎因其病理生理学尚未完全明确，可能会带来诊断难题。
我们报告一例罕见的年轻男性病例，其出现妄想、躁动和癫痫发作等亚急性神经精神表现。他被诊断为血清学阴性边缘叶脑炎（SNLE）。脑部磁共振成像（MRI）显示双侧屏状核保留征。脑电图显示左侧持续性癫痫样放电，呈周期性，主要位于左中颞叶。患者病情通过静脉注射甲泼尼龙、静脉注射免疫球蛋白和抗癫痫药物逐渐改善。
屏状核仍是人们了解最少的神经解剖结构之一。在发热感染相关癫痫综合征（FIRES）、边缘叶脑炎和自身免疫性难治性癫痫中均有屏状核征的报道。据我们所知，我们报告了文献中首例血清学阴性边缘叶脑炎出现屏状核保留征的病例。有必要开展进一步的实验模型和研究，以更好地了解屏状核的独特功能，并揭示可能的其他相关自身抗体，这可能会改变治疗方法和预后。
