Sands Tristan T, Nash Kendall, Tong Son, Sullivan Joseph
The University of San Francisco, Benioff Children's Hospital-San Francisco, Neurology, Mailstop 0137, 550 16th Street, 4th Floor, San Francisco, CA 94158-2549, United States.
Epilepsy Res. 2015 May;112:31-6. doi: 10.1016/j.eplepsyres.2015.02.010. Epub 2015 Feb 16.
We investigated the electroclinical features of seizures occurring in children with anti-NMDA receptor antibody encephalitis.
Clinical features and video EEG recordings were analyzed from pediatric patients with anti-NMDA receptor antibody encephalitis at our center over a six year period.
We identified eight pediatric patients with anti-NMDA receptor antibody encephalitis. Video EEG captured multiple focal seizures in four patients. Ictal onset in all four patients consisted of a focal rhythmic sharpened 6-12Hz activity that subsequently spread to one or both hemispheres. When there was a clinical correlate, seizure semiology was limb posturing with or without dyscognitive features. While background abnormalities were noted at presentation in three cases, the initial EEG background was normal in five, including three patients presenting with seizures. The EEG background deteriorated with clinical progression.
Focal seizures are common in pediatric patients with anti-NMDA receptor antibody encephalitis and have a characteristic ictal onset pattern. Anti-NMDA receptor antibody encephalitis should be considered in the differential diagnosis of a child presenting with new onset focal seizures, irrespective of the EEG background, especially if accompanied by dyskinesia, psychiatric symptoms or impaired cognition.
我们研究了抗N-甲基-D-天冬氨酸(NMDA)受体抗体脑炎患儿癫痫发作的电临床特征。
对本中心6年间抗NMDA受体抗体脑炎儿科患者的临床特征及视频脑电图记录进行分析。
我们确定了8例抗NMDA受体抗体脑炎儿科患者。视频脑电图记录到4例患者出现多次局灶性癫痫发作。所有4例患者的发作起始均为6-12Hz的局灶性节律性锐化活动,随后扩散至一侧或双侧半球。当存在临床相关表现时,癫痫发作症状学为肢体姿势异常,伴或不伴有认知障碍特征。3例患者在初诊时发现背景异常,5例患者初始脑电图背景正常,其中3例患者有癫痫发作表现。脑电图背景随临床进展而恶化。
局灶性癫痫发作在抗NMDA受体抗体脑炎儿科患者中很常见,且具有特征性的发作起始模式。对于新发局灶性癫痫发作的儿童,无论脑电图背景如何,尤其是伴有运动障碍、精神症状或认知障碍时,鉴别诊断中应考虑抗NMDA受体抗体脑炎。