Alemdar Murat, Acar Turkan, Dalkilic Sule
Department of Neurology, Sakarya University Faculty of Medicine, Sakarya, Turkiye.
North Clin Istanb. 2023 Jun 5;10(3):385-389. doi: 10.14744/nci.2021.70431. eCollection 2023.
New-onset refractory status epilepticus (NORSE) is a rare entity referring refractory status epilepticus (SE) in a patient without a history of epilepsy or an apparent cause. Herein, we report on a 31-year-old young female of anti-N-methyl D-aspartate (NMDA) receptor encephalitis admitted with NORSE. Her complaints began a week ago with a fever, meaningless movements, restlessness, and talking to herself. She had a history of operation for ovarian teratoma 10 years ago. Electrocardiography, hemogram, biochemistry, and neuroimaging were normal. Due to recurrent seizures despite intravenous diazepam infusions, phenytoin infusion was introduced, reducing the duration and frequency of seizures. Electroencephalogram (EEG) revealed a generalized slow background activity with low voltage and delta waves in left hemisphere derivatives without any epileptiform discharge. Autoimmune encephalitis panel revealed a positive anti-NMDAR receptor antibody. Intravenous immunoglobulins were given for 5 days. She was improved clinically and did not have a recurrent seizure. The history of our case emphasizes the importance of EEG and CSF antibody tests to reach the underlying etiology in patients presenting with refractory SE and neuropsychiatric symptoms of an unknown cause. Application of a proper treatment promptly with this approach could prevent the potential morbidity and mortality in these patients.
新发难治性癫痫持续状态（NORSE）是一种罕见的病症，指的是既往无癫痫病史或明显病因的患者出现难治性癫痫持续状态（SE）。在此，我们报告一例31岁患抗N-甲基-D-天冬氨酸（NMDA）受体脑炎的年轻女性，以新发难治性癫痫持续状态入院。她的症状始于一周前，表现为发热、无意义动作、烦躁不安及自言自语。她10年前有卵巢畸胎瘤手术史。心电图、血常规、生化检查及神经影像学检查均正常。尽管静脉输注地西泮仍有癫痫发作反复，遂加用苯妥英钠输注，癫痫发作的持续时间和频率有所减少。脑电图（EEG）显示背景活动普遍减慢，左半球导联低电压及δ波，无任何癫痫样放电。自身免疫性脑炎检测显示抗NMDAR受体抗体阳性。静脉输注免疫球蛋白5天。她临床症状改善，未再出现癫痫发作。我们病例的病史强调了脑电图和脑脊液抗体检测对于明确不明原因难治性癫痫持续状态和神经精神症状患者潜在病因的重要性。采用这种方法及时应用恰当的治疗可预防这些患者的潜在发病和死亡。
