Age-related evolution of EEG in Dravet syndrome: Meta-analysis of 155 patients

Abstract

PURPOSE\nDravet syndrome is an early-onset developmental and epileptic encephalopathy caused by pathogenic SCN1A variants in 80-90% of patients. EEG is initially normal, but abnormalities, both generalized and focal, may develop later. There is a limited understanding of typical EEG evolution in Dravet syndrome.\n\n\nMETHODS\nWe searched Pubmed in July 2020 for studies including: ≥ 1 patient with Dravet syndrome clinical diagnosis and SCN1A pathogenic variant, and for each such patient, a description of ≥ 1 EEG and age at the time of the EEG. For each study, we evaluated for bias in patient selection. We also reviewed our research database for Dravet patients with available EEG reports. We extracted demographic data and EEG abnormalities reported (generalized/focal epileptiform abnormalities, focal/diffuse slowing). We determined the earliest ages at which different abnormalities were seen, as well as the percentage of reported abnormalities for different age ranges.\n\n\nRESULTS\nWe included 247 EEGs from 155 patients (from 31 studies and our research database). The earliest reported ages of generalized epileptiform discharges, focal epileptiform discharges, diffuse background slowing, and focal slowing, were six months, four months, four months, and four months, respectively. In patients 0-12 months, EEG was abnormal in 43%, but this rose to 90% for the 1-2 year-old group, and remained at approximately the same level for the remainder of the age groups.\n\n\nCONCLUSION\nOur results help clarify the relationship between age and EEG in Dravet syndrome; however, findings should be interpreted with caution given the inherent potential biases in the study design.