Mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE): a widespread disease with an apparently focal epilepsy

Mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE) is a recently recognized, highly epileptogenic, distinct histopathological entity in drug-resistant epilepsy that primarily involves the frontal lobes. Surgical outcomes in MOGHE are variable. Although the diagnosis is based on histopathology, high-resolution MRI helps to differentiate MOGHE preoperatively from other forms of cortical malformations (i.e., mMCD II and FCD I la). We discuss the clinical, electrographic, radiological and histopathological characteristics of MOGHE in two patients who underwent evaluation for drug-resistant epilepsy followed by electrocorticography-based resection. Both patients presented with childhood-onset refractory frontal lobe epilepsy with a high seizure burden. Interictal epileptiform discharges were widespread. PET abnormalities were disproportionate to the MRI findings. Cognitive impairment, persistent epileptiform discharges on post-resection electrocorticography and sub-optimal surgical outcomes suggest that MOGHE is a widespread pathology in focal epilepsy.

Automated summary

MOGHE is a recently recognized condition primarily involving the frontal lobes, with variable surgical outcomes. High-resolution MRI can help differentiate MOGHE from other forms of cortical malformations. Two patients with childhood-onset refractory frontal lobe epilepsy showed disproportionate PET abnormalities compared to the MRI findings.