OBJECTIVE: Hemimegalencepahly (HME) and Hemispheric Cortical Dysplasia (HCD) are rare congenital diseases that occur with intractable epilepsy. They manifest by early epilepsy, mental retardation, hemianopsia and contralateral hemiplegia. Hemispheric disconnection (mainly anatomical hemispherectomy, peri-insular hemispherotomy, modified lateral hemispherotomy and vertical parasagittal hemispherotomy) have been reported to be efficient on seizures and also to prevent additional cognitive injury and developmental delay. METHOD: We reviewed literature about clinical presentation, predictors of outcome and expectation about epileptic seizures and cognitive outcome. RESULTS: Clinical presentation and seizures outcome have been described in almost 600 children for the last thirty years. Epilepsy improved in most cases depending on the series and the follow-up duration. Percentage of seizure-free patients with HME or HCD was lower than in other groups (Rasmussen Encephalitis, Vascular Sequellae). Post-operative complications decreased with the hemispherotomy surgical procedures. EEG abnormalities on the "save" hemisphere did not negatively influence postsurgical outcome. Seizure free outcome did not seem to depend on the surgical procedure but the presence of residual insular cortex seemed to be associated with persistent postoperative seizures. Contralateral MRI abnormalities seemed to be associated with poorer prognosis for seizure free outcome and lack of cognitive improvement. CONCLUSION: Hemispheric disconnection remains the best treatment in order to control epileptic seizures. Hemispheric surgical procedures are safe and can be performed from the first month of life. Prospective studies of cognition are needed to emphasize benefits on long term outcome.
OBJECTIVE: Hemimegalencepahly (HME) and Hemispheric Cortical Dysplasia (HCD) are rare congenital diseases that occur with intractable epilepsy. They manifest by early epilepsy, mental retardation, hemianopsia and contralateral hemiplegia. Hemispheric disconnection (mainly anatomical hemispherectomy, peri-insular hemispherotomy, modified lateral hemispherotomy and vertical parasagittal hemispherotomy) have been reported to be efficient on seizures and also to prevent additional cognitive injury and developmental delay. METHOD: We reviewed literature about clinical presentation, predictors of outcome and expectation about epilepticseizures and cognitive outcome. RESULTS: Clinical presentation and seizures outcome have been described in almost 600 children for the last thirty years. Epilepsy improved in most cases depending on the series and the follow-up duration. Percentage of seizure-freepatients with HME or HCD was lower than in other groups (Rasmussen Encephalitis, Vascular Sequellae). Post-operative complications decreased with the hemispherotomy surgical procedures. EEG abnormalities on the "save" hemisphere did not negatively influence postsurgical outcome. Seizure free outcome did not seem to depend on the surgical procedure but the presence of residual insular cortex seemed to be associated with persistent postoperative seizures. Contralateral MRI abnormalities seemed to be associated with poorer prognosis for seizure free outcome and lack of cognitive improvement. CONCLUSION: Hemispheric disconnection remains the best treatment in order to control epilepticseizures. Hemispheric surgical procedures are safe and can be performed from the first month of life. Prospective studies of cognition are needed to emphasize benefits on long term outcome.