Management of epilepsy caused by nodular heterotopia

Malformations of cortical development (MCDs) are a heterogeneous group of inherited disorders of disrupted cerebral cortex formation caused by various etiologies such as genetic, vascular or metabolic.Grey matter heterotopia are disorders caused by abnormal neuronal migration and consists of normal neurons in abnormal locations. These disorders are nodular or linear formation and are in the periventricular or subcortical location. Periventricular nodular heterotopia (PVNH) is the most common type and manifested by drug resistant epilepsy and intellectual disability and others.The nodular heterotopia usually manifested with drug resistant epilepsy and treatment often has many challenging issues. the most problem point is the connectivity between heterotopic nodules and adjacent cortex and other near structures such as hippocampus. The best management need to define this network accurately and planning a correct strategy for access to this network.Treatment options for this disorder varies and consists of surgical process such as temporal lobectomy or lesionectomy. According to the previous experiences, due to the deep-seated location, PNH are seldom accessible surgically without facing a high potential of neurological deficits and lesionectomy is mostly limited to cases with unilateral and single nodule with proper surgical accessibility.Two main stereotactic ablative treatments are SEEG-guided radiofrequency thermocoagulation (RFTC) and MRI-guided laser interstitial thermal therapy (MRG-LITT). Although, the experiences about these procedures are limited and is based on case reports and series but the date is growing. these procedures due to little adverse effects and focused intervention may become the best treatment in the future.