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Gastaut-Geschwind Syndrome, Faciobrachial Dystonic Seizure, and Autoimmune Limbic Encephalitis

机译:Gastaut-Geschwind综合征,面臂肱肌张力性惊厥和自身免疫性边缘性脑炎

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Here we report a case of a 55-year-old male who had presented with recent falls and behavioral changes, including a heightened religious preoccupation, hypergraphia, and paranoid ideations. He was initially treated for psychosis but soon exhibited absence-like seizures, which were consistent with faciobrachial dystonic seizures. Workup for underlying infectious, immunodeficiency, and autoimmune causes revealed antibodies towards the leucine-rich glioma inactivated subunit of the voltage-gated potassium complex. The patient was treated with steroids and intravenous immune globulin with symptomatic relief. In retrospect, the patient met criteria for Gastaut-Geschwind (GG) syndrome, with notable features of hypergraphia and hyperreligiosity. This case illustrates how the GG syndromal pattern contributes to the suspicion of autoimmune limbic encephalitis and may expedite diagnosis and prevent the accumulation of disability.
机译:在这里,我们报道一例55岁的男性,该男性最近表现出跌倒和行为改变,包括对宗教的高度关注,过度描写和偏执狂意识形态。他最初接受过精神病治疗,但很快表现出神志不清的癫痫发作,这与面臂臂肌张力障碍性癫痫发作一致。对潜在的传染性,免疫缺陷和自身免疫的检查揭示了针对电压门控钾复合物的富含亮氨酸的神经胶质瘤失活亚基的抗体。该患者接受了类固醇和静脉免疫球蛋白的治疗,症状缓解。回顾过去,该患者符合Gastaut-Geschwind(GG)综合征的标准,具有明显的高描写和高反应性特征。该病例说明GG综合征的模式如何有助于怀疑自身免疫性边缘性脑炎,并可能加快诊断速度并防止残疾积聚。

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