Genetic generalized epilepsy and generalized onset seizures with focal evolution (GOFE)

Abstract

"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.

Keywords: Focal evolution; Generalized onset; Generalized onset seizures with focal evolution (GOFE); Genetic Generalized Epilepsy (GGE); Idiopathic Generalized Epilepsies (IGEs); Seizure type.

Fig. 1

Patient 1. Bipolar longitudinal montage, Scale 250uV/2 s, Total Seizure duration 1′40′’. A) ictal onset, with asymptomatic 2,5 Hz Generalized Spike and Waves discharge. B-C) focal evolution with widespread left discharges. D) seizure end.