Walter Reed National Military Medical Center, Department of Neurology, Uniformed Services University of Health Sciences Department of Neurology.
Walter Reed National Military Medical Center, Department of Internal Medicine, Uniformed Services University of Health Sciences Department of Medicine.
Epileptic Disord. 2014 Dec;16(4):486-93. doi: 10.1684/epd.2014.0702.
A 23-year-old, previously healthy, deployed U.S. soldier presented with bilateral temporal lobe seizures recalcitrant to multiple antiepileptic drugs and anti-seizure anaesthetic agents. He received methylprednisolone, intravenous immunoglobulins, plasma exchange, and rituximab for presumed autoimmune encephalitis before achieving seizure freedom. Six weeks after presentation, the aetiology of his refractory seizures was found to be due to autoantibodies targeting the anti-GABA(B)-receptor. This case is noteworthy for being the first reported case of anti-GABA(B)-receptor limbic encephalitis presenting with new-onset refractory status epilepticus (NORSE), a clinical syndrome that often carries a grave prognosis and in which a treatable aetiology is often never discovered. Our case also supports testing for GABA-receptor autoantibodies and the upfront use of multi-modal immunotherapy in patients presenting with limbic encephalitis and new refractory seizures.
一位 23 岁、既往健康的美国士兵出现双侧颞叶癫痫,多种抗癫痫药物和抗癫痫麻醉药物均无效。在实现无癫痫发作之前,他接受了甲基强的松龙、静脉注射免疫球蛋白、血浆置换和利妥昔单抗治疗,疑似自身免疫性脑炎。就诊 6 周后,发现他的难治性癫痫发作的病因是针对 GABA(B)受体的自身抗体。该病例值得注意,因为它是首例报道的抗 GABA(B)受体边缘性脑炎伴新发难治性癫痫持续状态(NORSE)的病例,这是一种临床综合征,预后通常很严重,而且经常找不到可治疗的病因。我们的病例还支持对 GABA 受体自身抗体进行检测,并在出现边缘性脑炎和新的难治性癫痫发作的患者中及早使用多模式免疫疗法。