Chanson Eve, Bicilli Élodie, Lauxerois Michel, Kauffmann Sophie, Chabanne Russell, Ducray François, Honnorat Jérome, Clavelou Pierre, Rosenberg Sarah
Service de neurologie et unité de neurophysiologie clinique, CHU Gabriel-Montpied, 63000 Clermont-Ferrand, France.
Service de neurologie et unité de neurophysiologie clinique, CHU Gabriel-Montpied, 63000 Clermont-Ferrand, France.
Neurophysiol Clin. 2016 Feb;46(1):17-25. doi: 10.1016/j.neucli.2015.12.009. Epub 2016 Feb 26.
Seizures are common clinical manifestations in anti-N-methyl-d-aspartate receptor (anti-NMDA-R) encephalitis, among other neurological and psychiatric symptoms. During the course of the disease, some specific EEG patterns have been described: generalized rhythmic delta activity (GRDA) and extreme delta brush (EDB). In comatose patients, the association of these EEG abnormalities with subtle motor manifestations can suggest ongoing non-convulsive status epilepticus (NCSE). We report the case of a 28-year-old woman admitted for a clinical presentation typical of anti-NMDA-R encephalitis, which was confirmed by CSF analysis. She was rapidly intubated because of severe dysautonomia and disturbed consciousness. Clinical examination revealed subtle paroxysmal and intermittent myoclonic and tonic movements, correlated on video-EEG with GRDA and/or EDB. NCSE was then suspected, but electroclinical manifestations persisted despite many anti-epileptic drugs combinations, or reappeared when barbiturate anesthesia was decreased. In order to confirm or dismiss the diagnosis, intracranial pressure (ICP) and surface video-EEG monitoring were performed simultaneously and revealed no ICP increase, thus being strongly against a diagnosis of seizures. Sedation was progressively weaned, and clinical condition as well as EEG appearance progressively improved. Literature review revealed 11 similar cases, including 2 with focal NCSE. Of the nine other cases, NCSE diagnosis was finally excluded in 5 cases. NCSE diagnosis in association with anti-NMDA-R encephalitis is sometimes very difficult and its occurrence might be overestimated. Video-EEG is highly recommended and more invasive techniques may sometimes be necessary.
癫痫发作是抗N-甲基-D-天冬氨酸受体（抗NMDA-R）脑炎常见的临床表现，除此之外还有其他神经和精神症状。在疾病过程中，已经描述了一些特定的脑电图模式：广泛性节律性δ活动（GRDA）和极端δ刷（EDB）。在昏迷患者中，这些脑电图异常与细微的运动表现相关，可能提示持续性非惊厥性癫痫持续状态（NCSE）。我们报告了一例28岁女性患者，因典型的抗NMDA-R脑炎临床表现入院，脑脊液分析确诊。由于严重的自主神经功能障碍和意识障碍，她很快被插管。临床检查发现细微的阵发性和间歇性肌阵挛及强直运动，视频脑电图显示与GRDA和/或EDB相关。当时怀疑为NCSE，但尽管使用了多种抗癫痫药物联合治疗，电临床症状仍持续存在，或在巴比妥类麻醉减浅时再次出现。为了确诊或排除诊断，同时进行了颅内压（ICP）和表面视频脑电图监测，结果显示ICP没有升高，因此强烈反对癫痫发作的诊断。镇静逐渐撤停，临床状况以及脑电图表现逐渐改善。文献回顾发现11例类似病例，包括2例局灶性NCSE。在其他9例病例中，最终排除NCSE诊断的有5例。与抗NMDA-R脑炎相关的NCSE诊断有时非常困难，其发生率可能被高估。强烈推荐视频脑电图，有时可能需要更具侵入性的技术。
