Mild Malformation of Cortical Development With Oligodendroglial Hyperplasia and Epilepsy: A Systematic Review.

Background and Objectives: Mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE) is a newly described rare entity of drug-resistant epilepsy, with a wide spectrum of presentations. We aim to describe the diagnostic features and prognosis of MOGHE in a large cohort. Methods: We performed a systematic review preregistered on PROSPERO (CRD42023472978), in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. We searched PubMed, Embase, Scopus, and ScienceDirect between database inception and November 30, 2023, for all published studies on MOGHE. Inclusion criteria were a histopathologic diagnosis of MOGHE. The risk of bias was analyzed with a standardized tool specifically for case reports and case series. The demographic, clinical, EEG, neuroimaging, genetic, and neuropathologic features; treatments; and prognosis were extracted and analyzed. Subgroup analysis was performed with the age at onset and Results: A total of 163 patients with MOGHE from 18 studies were included in the analysis. The median age at seizure onset was 1.2 years, and 103 were male. Ninety-five patients presented with unilobed lesions. Ninety-nine had lesions in the frontal lobe. A total of 101 patients achieved a favorable surgical outcome. Patients with an onset before 10 years were more likely to present with epileptic spasms, the West syndrome, a circumscribed pattern of interictal EEG, intellectual disabilities, and a better seizure outcome, compared with those with an onset age 10 years and older. Forty-five patients (72.6%) were Discussion: MOGHE is a distinct entity of drug-resistant epilepsy associated with