Santoro Jonathan D, Filippakis Alexandra, Chitnis Tanuja
Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, United States of America.
Department of Neurology, Brigham and Women's Hospital, Boston, MA 02115, United States of America.
Epilepsy Behav Rep. 2019 Jun 23;12:100326. doi: 10.1016/j.ebr.2019.100326. eCollection 2019.
Anti--methyl-D-aspartate receptor encephalitis (anti-NMDAr encephalitis) is an auto-immune disorder associated with the production of antibodies against NR1 and NR2 sub units of the NMDA receptor. Seizures in this population are reported in up to 50% of cases with status epilepticus being reported in 25% of cases, refractory status epilepticus in 13.8% of cases and super-refractory status epilepticus in 10.2% of cases. Treatment of refractory epileptic activity in this population is not uniform and heterogeneous.
We present three cases of super refractory status epilepticus in patients with anti-NMDAr encephalitis treated successfully with ketamine, a noncompetitive NMDA receptor antagonist. All patients had failed to improve clinically on multiple anti-convulsants and immunotherapy prior to initiation of ketamine therapy.
In all three cases, administration of a load followed by maintenance infusion (0.05 mg/kg/min infusion) of ketamine yielded clinical and/or electrographic seizure cessation in less than 48 h. Patients were treated for a heterogeneous duration although ultimately, epilepsy outcomes were favorable from a seizure freedom standpoint. Earlier treatments with ketamine were associated with better epilepsy outcomes in this case series.
Ketamine may be a useful adjunct treatment in super-refractory status epilepticus in patients with NMDAr encephalitis.
抗N-甲基-D-天冬氨酸受体脑炎(抗NMDAr脑炎)是一种自身免疫性疾病,与针对NMDAr受体NR1和NR2亚基产生抗体有关。据报道,该人群中高达50%的病例会出现癫痫发作,其中25%的病例会出现癫痫持续状态,13.8%的病例会出现难治性癫痫持续状态,10.2%的病例会出现超难治性癫痫持续状态。该人群中难治性癫痫活动的治疗并不统一且存在异质性。
我们报告了3例抗NMDAr脑炎患者的超难治性癫痫持续状态,这些患者使用非竞争性NMDAr拮抗剂氯胺酮成功治疗。在开始氯胺酮治疗之前,所有患者在多种抗惊厥药和免疫治疗下临床症状均未改善。
在所有3例病例中,静脉注射负荷剂量氯胺酮后持续输注(0.05mg/kg/min),在不到48小时内实现了临床和/或脑电图癫痫发作停止。患者接受了不同疗程的治疗,不过最终,从癫痫发作缓解的角度来看,癫痫治疗结果良好。在本病例系列中,早期使用氯胺酮治疗与更好的癫痫治疗结果相关。
氯胺酮可能是NMDAr脑炎患者超难治性癫痫持续状态的一种有效辅助治疗方法。