MR characteristics in mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy
Is a recently identified neurological condition primarily linked to refractory frontal lobe epilepsy, especially in pediatric patients. Histologically characterized by clusters of increased oligodendroglial cell density, blurred gray-white matter boundaries, and heterotopic neurons within the white matter, MOGHE presents distinct magnetic resonance imaging (MRI) features, such as increased T2/FLAIR signal intensity. These imaging characteristics are crucial for the diagnosis and management of this condition, which frequently results in diverse seizure types and cognitive decline that resist conventional treatments.[1][2][3][4].Notably, MOGHE is particularly significant due to its association with difficult-to-treat epilepsy and potential for surgical intervention. However, studies indicate that patien- ts with MOGHE often experience unfavorable outcomes concerning seizure control
following surgical resection, emphasizing the need for a deeper understanding of its MRI characteristics and underlying pathology.[1][3][4]. Additionally, recent research has highlighted two MRI subtypes that correlate with age and histological findings, suggesting an age-related progression of the condition.[5][6].
The etiology of MOGHE appears to involve abnormal oligodendroglial proliferation and neuronal migration, with emerging genetic studies identifying somatic variants in the SLC35A2 gene among affected individuals. These findings enhance the under- standing of the condition's pathophysiology, potentially informing future therapeutic approaches.[2][6][7]. As advancements in imaging and genetic research continue, MOGHE remains a focal point for ongoing investigation into effective diagnosis and treatment strategies for pediatric epilepsy related to cortical malformations.[2][7].
Background
Mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy (MOGHE) is a recently recognized entity primarily associated with refrac- tory frontal lobe epilepsy, particularly in pediatric populations. Histopathologically, MOGHE is characterized by clusters of increased oligodendroglial cell density, blurred gray-white matter boundaries, and heterotopic neurons within the white matter[1][2][3]. These features typically manifest on MRI as increased T2/FLAIR signal intensity, highlighting the structural abnormalities in the frontal lobe[1][4]. The condition often presents in children or young adults and is marked by a spectrum of seizure types, including focal, atonic, and tonic seizures, which are frequently resistant to anti-seizure medications[2][3]. Cognitive decline is also common among affected individuals, further complicating their neurological status[3]. The patho- genesis of MOGHE appears to involve abnormal oligodendroglial proliferation and neuronal migration, leading to significant alterations in cortical architecture[2][4][3]. Surgical resection of the affected brain tissue is currently the main treatment for patients with MOGHE who do not respond to pharmacological interventions. How- ever, studies indicate that the presence of MOGHE is associated with an unfavorable outcome in terms of seizure control post-surgery[1][4][3]. Understanding the MRI characteristics and underlying pathology of MOGHE is crucial for improving diagnosis and management of this challenging condition.