Linane Avriel, Lagrange Andre H, Fu Cary, Abou-Khalil Bassel
Department of Neurology, Vanderbilt University, Nashville, TN, USA.
Department of Neurology, Vanderbilt University, Nashville, TN, USA.
Epilepsy Behav. 2016 Jan;54:20-9. doi: 10.1016/j.yebeh.2015.10.005. Epub 2015 Nov 25.
We report clinical and electrographic features of generalized onset seizures with focal evolution (GOFE) and present arguments for the inclusion of this seizure type in the seizure classification.
The adult and pediatric Epilepsy Monitoring Unit databases at Vanderbilt Medical Center and Children's Hospital were screened to identify generalized onset seizures with focal evolution. We reviewed medical records for epilepsy characteristics, epilepsy risk factors, MRI abnormalities, neurologic examination, antiepileptic medications before and after diagnosis, and response to medications. We also reviewed ictal and interictal EEG tracings, as well as video-recorded semiology.
Ten patients were identified, 7 males and 3 females. All of the patients developed generalized epilepsy in childhood or adolescence (ages 3-15years). Generalized onset seizures with focal evolution developed years after onset in 9 patients, with a semiology concerning for focal seizures or nonepileptic events. Ictal discharges had a generalized onset on EEG, described as either generalized spike-and-wave and/or polyspike-and-wave discharges, or generalized fast activity. This electrographic activity then evolved to focal rhythmic activity most commonly localized to one temporal or frontal region; five patients had multiple seizures evolving to focal activity in different regions of both hemispheres. The predominant interictal epileptiform activity included generalized spike-and-wave and/or polyspike-and-wave discharges in all patients. Taking into consideration all clinical and EEG data, six patients were classified with genetic (idiopathic) generalized epilepsy, and four were classified with structural/metabolic (symptomatic) generalized epilepsy. All of the patients had modifications to their medications following discharge, with three becoming seizure-free and five responding with >50% reduction in seizure frequency.
Generalized onset seizures may occasionally have focal evolution with semiology suggestive of focal seizures, leading to a misdiagnosis of focal onset. This unique seizure type may occur with genetic as well as structural/metabolic forms of epilepsy. The identification of this seizure type may help clinicians choose appropriate medications, avoiding narrow spectrum agents known to aggravate generalized onset seizures.
我们报告了伴有局灶性演变的全面性发作(GOFE)的临床和脑电图特征,并提出将这种发作类型纳入癫痫发作分类的依据。
对范德比尔特医疗中心和儿童医院的成人及儿童癫痫监测单元数据库进行筛查,以确定伴有局灶性演变的全面性发作。我们回顾了病历中的癫痫特征、癫痫危险因素、MRI异常、神经系统检查、诊断前后的抗癫痫药物以及药物反应。我们还回顾了发作期和发作间期的脑电图记录以及视频记录的发作症状学。
共确定10例患者,7例男性,3例女性。所有患者均在儿童期或青少年期(3 - 15岁)患全面性癫痫。9例患者在发作数年之后出现伴有局灶性演变的全面性发作,其发作症状学提示局灶性发作或非癫痫性事件。发作期脑电图放电起始为全面性,表现为全面性棘慢波和/或多棘慢波放电,或全面性快活动。这种脑电图活动随后演变为局灶性节律性活动,最常见于一个颞叶或额叶区域;5例患者有多次发作,演变至双侧半球不同区域的局灶性活动。所有患者发作间期主要的癫痫样活动包括全面性棘慢波和/或多棘慢波放电。综合所有临床和脑电图数据,6例患者被归类为遗传性(特发性)全面性癫痫,4例被归类为结构性/代谢性(症状性)全面性癫痫。所有患者出院后均调整了药物治疗,3例患者无发作,5例患者发作频率降低超过50%。
全面性发作偶尔可能伴有局灶性演变,其发作症状学提示局灶性发作,从而导致对局灶性发作的误诊。这种独特的发作类型可能同时出现在遗传性以及结构性/代谢性癫痫中。识别这种发作类型可能有助于临床医生选择合适的药物,避免使用已知会加重全面性发作的窄谱药物。