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Frontal Disconnection for Treating Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE) in the Frontal Lobe
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Focal malformations of cortical development: a most relevant etiology of epilepsy in children

André Palmini1, Hans Holthausen

  • 1Neurology Service and Epilepsy Surgery Program, Hospital São Lucas; Faculty of Medicine, Pontificia Universidade Católica do Rio Grande do Sul and Brain Institute of Rio Grande do Sul, Porto Alegre, Brazil.

Handbook of Clinical Neurology
|April 30, 2013
PubMed

Insights

Malformations of cortical development (MCD) are a key cause of difficult-to-treat childhood epilepsies. This review categorizes MCD subtypes to guide surgical treatment for better seizure control and brain development.

Area of Science:

  • Neuroscience
  • Pediatric Neurology
  • Medical Imaging

Background:

  • Malformations of cortical development (MCD) are a leading cause of medically refractory epilepsies in children and adolescents.
  • Focal cortical dysplasia (FCD) and hemimegalencephalies (HMG) are particularly significant due to their focal or unihemispheric nature, making them targets for surgical intervention.
  • Early childhood brain plasticity means seizures and abnormal discharges can impede development, highlighting the dual goal of surgery: seizure control and developmental redirection.

Purpose of the Study:

  • To propose an "imaging-semiological organization" for classifying malformations of cortical development (MCD).
  • To aid in surgical treatment planning for pediatric epilepsy patients with MCD.
  • To improve understanding of the diverse presentations of MCD relevant to refractory epilepsy.

Main Methods:

  • Review and synthesis of existing literature on MCD and epilepsy.
  • Development of a classification system based on neuroimaging findings and clinical semiology.
  • Categorization of MCD into four main groups based on lesion characteristics and location.

Main Results:

  • The proposed classification includes four categories: fronto-rolandic lesions, multilobar hemispheric lesions, anterior temporal dysplasias, and cases with microscopic dysplastic foci undetectable by standard MRI.
  • Specific imaging features like transmantle sign, abnormal gyration, cortical thickening, gray-white matter blurring, and volume reduction are highlighted for each category.
  • The classification considers cortical disorganization, cellular abnormalities, and associated lesion types.

Conclusions:

  • The "imaging-semiological organization" provides a structured approach to understanding MCD in pediatric epilepsy.
  • Accurate classification is crucial for tailoring surgical strategies to optimize seizure control and neurodevelopmental outcomes.
  • This framework may enhance diagnostic precision and therapeutic decision-making for refractory epilepsies associated with MCD.

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