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  • Updated 07.17.2020
  • Released 05.01.2017
  • Expires For CME 07.17.2023

Epileptic lesions due to malformation of cortical development

Introduction

Overview

Malformation of cortical development, including focal cortical dysplasia, tuberous sclerosis complex, and hemimegalencephaly is a common cause of epilepsy. The authors of this article review the most recent advances in understanding of their pathophysiology and cumulative evidence of clinical studies regarding clinical manifestations, diagnosis, and treatment.

Key points

• Malformation of cortical development is the most common cause of pharmacoresistant epilepsy, especially in children.

• Malformation of cortical development, including focal cortical dysplasia, tuberous sclerosis complex, and hemimegalencephaly share the similar derangement of biological pathways, such as the mTOR pathway.

• Surgical treatment is the mainstay of curative therapy for focal cortical dysplasia, yet disease-modifying medical therapy based on the specific pathophysiology is under investigation.

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