title: Catastrophic epilepsy in childhood.
abstract: Although for most children epilepsy is a relatively benign disorder, for some, epilepsy can be designated as "catastrophic" because the seizures are so difficult to control and because they are strongly associated with mental retardation. The catastrophic childhood epilepsies include uncommon disorders such as early infantile epileptic encephalopathy with suppression burst, severe myoclonic epilepsy of infancy, and epilepsy with myoclonic-astatic seizures. There are other syndromes that are relatively common such as infantile spasms, Lennox-Gastaut syndrome, and Sturge-Weber syndrome. Many children with catastrophic epilepsy have the seizures as a result of underlying brain abnormalities that will inevitably lead to mental retardation whether or not they have seizures. In some patients, however, the mental retardation appears to be caused by the seizures. Developmental plasticity provides children with an opportunity to recover from significant brain injuries. However, the plasticity may also be the cause of the mental retardation. In such patients, control of the seizures may lead to more normal intellectual development. Thus, every effort should be made to control seizures in children with catastrophic epilepsy.
authors.affiliation: Division of Pediatric Neurology, Mattel Children's Hospital at UCLA, Los Angeles, California, USA. wshields@mednet.ucla.edu
authors.forename: W D
authors.initials: WD
authors.lastname: Shields
mesh_terms: D000927:Anticonvulsants / Q000494:pharmacology / Q000627:therapeutic use; D001921:Brain / Q000187:drug effects / Q000503:physiopathology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D004660:Encephalitis / Q000150:complications / Q000503:physiopathology / Q000628:therapy; D004831:Epilepsies, Myoclonic / Q000150:complications / Q000503:physiopathology / Q000628:therapy; D004827:Epilepsy / Q000150:complications / Q000503:physiopathology* / Q000628:therapy*; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000209:etiology / Q000503:physiopathology / Q000517:prevention & control; D009473:Neuronal Plasticity / Q000187:drug effects / Q000502:physiology; D017063:Outcome Assessment, Health Care; D012720:Severity of Illness Index; D013036:Spasms, Infantile / Q000150:complications / Q000503:physiopathology / Q000628:therapy; D013341:Sturge-Weber Syndrome / Q000150:complications / Q000503:physiopathology / Q000628:therapy
chemical_list: D000927:Anticonvulsants