For some children, seizures are drug-resistant and associated with intellectual disability* and often significant motor delay. The intellectual disability is unavoidable because of the underlying brain abnormalities whether or not they have seizures, or by the seizures themselves. Controlling the seizures, or reducing them significantly, may lead to improved intellectual development. For this reason, every effort should be made to stop seizures in children with catastrophic epilepsies. The catastrophic epilepsies, also known as epileptic encephalopathies, include:
Otahara syndrome (early infantile epileptic encephalopathy with suppression burst)
Dravet syndrome (severe myoclonic epilepsy in infancy)
Doose syndrome (epilepsy with myoclonic-astatic seizures)
Sturge-Weber syndrome (encephalotrigeminal angiomatosis)
Shields, W. Catastrophic epilepsy in childhood. Epilepsy, 41 (Suppl. 2): S2-S6 (2000).
Dlouhy BJ, Miller B. Palliative epilepsy surgery in Dravet syndrome-case series and review of the literature. Childs Nerv Syst. 2016 Sep;32(9):1703-8.
Malik SI, Galliani CA, Hernandez AW. Epilepsy surgery for early infantile epileptic encephalopathy (Otaahara syndrome). J Child Neurol. 2013 Dec;28(12):1607-17.
*Intellectual disability was formerly known as mental retardation.