Lamy Florian, Valenti-Hirsch Maria-Paola, Gauer Lucas, Gérard Bénédicte, Obeid Mohamed, de Saint-Martin Anne, Dinkelacker Vera, Baer Sarah, Hirsch Edouard
Epilepsy Unit "Françis Rohmer", French Reference Center of Rare Epilepsies CREER, FHU, Neurology Department, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
Department of Functional Neurology and Epileptology, Hospices Civils de Lyon, Lyon, France.
Epilepsy Behav Rep. 2022 May 30;19:100555. doi: 10.1016/j.ebr.2022.100555. eCollection 2022.
"Generalized Onset with Focal Evolution" (GOFE) is an underrecognized seizure type defined by an evolution from generalized onset to focal activity during the same ictal event. We aimed to discuss electroclinical aspects of GOFE and to emphasize its link with Genetic Generalized Epilepsy (GGE). Patients were identified retrospectively over 10 years, using the video-EEG data base from the Epilepsy Unit of Strasbourg University Hospital. GOFE was defined, as previously reported, from an EEG point of view with an evolution from generalized onset to focal activity during the same ictal event. Three male patients with GOFE were identified among 51 patients with recorded tonic-clonic seizures. Ages at onset of seizures were 13, 20 and 22 years. Focal clinical features (motor asymmetric phenomenology) could be identified. EEG showed generalized interictal discharges with focal evolution of various localization. Four seizures were recorded characterized by 2-3 s of generalized abnormalities followed by focal (parieto-occipital or frontal) discharges. There were initially uncontrolled seizures with lamotrigine, but all patients reported a good outcome with valproate monotherapy. We emphasize that GOFE presents many similarities with GGE. Recognition of the GOFE entity could bring a therapeutic interest avoiding misdiagnosis of focal epilepsy and consequently inappropriate use of narrow spectrum anti-seizure medicine.
“全面性发作伴局灶性演变”（GOFE）是一种未被充分认识的癫痫发作类型，其定义为在同一发作事件中从全面性发作演变为局灶性活动。我们旨在探讨GOFE的电临床特征，并强调其与遗传性全面性癫痫（GGE）的联系。利用斯特拉斯堡大学医院癫痫科的视频脑电图数据库，对患者进行了为期10年的回顾性研究。如先前报道，从脑电图角度将GOFE定义为在同一发作事件中从全面性发作演变为局灶性活动。在51例记录有强直阵挛发作的患者中，识别出3例GOFE男性患者。癫痫发作起始年龄分别为13岁、20岁和22岁。可识别出局灶性临床特征（运动不对称现象）。脑电图显示发作间期全面性放电，并伴有不同部位的局灶性演变。记录到4次发作，其特征为2 - 3秒的全面性异常，随后是局灶性（顶枕部或额部）放电。最初使用拉莫三嗪时癫痫发作未得到控制，但所有患者使用丙戊酸单药治疗后均报告预后良好。我们强调GOFE与GGE有许多相似之处。认识GOFE这一实体可能具有治疗意义，可避免误诊为局灶性癫痫，从而避免不恰当地使用窄谱抗癫痫药物。
