Towards early diagnosis and treatment to save children from catastrophic epilepsy -- focus on epilepsy surgery

Abstract

Objective: To analyze and to discuss whether by paying attention to the many recent advancements in the field of pediatric epilepsy surgery catastrophic childhood epilepsies caused by definitive or suspected structural lesions can be prevented more often these days in comparison to the past.

Methods: Based on data from the literature and supplemented by the authors own experience, risks for children suffering from structural focal epilepsies that the epilepsy becomes catastrophic and ways how such evolutions can possibly be prevented are discussed for the different lesion-types separately - in the order of their frequency as they are seen at pediatric epilepsy surgery centers. Special emphasis is put on data regarding attempts to prevent permanent severe mental retardations.

Results: There are common factors predisposing to catastrophic courses in all structural focal epilepsies, such as early onset and a longer duration of epilepsy (with respect to cognitive outcome not with respect to seizure outcome), but there are also differences. Moreover the better perspectives now in comparison to the past for children with conditions like MRI-negative focal epilepsies, subtle focal cortical dysplasias, epilepsies post hypoxic-ischemic events, tuberous sclerosis etc. are not well recognized yet. While there is agreement that "early" (and successful) surgery is essential in many instances to prevent permanent mental retardations there is insufficient data regarding the issue that "early surgery "might not be early enough under certain circumstances and there is also only little data regarding variables which would allow to keep calm when a child is presenting with early onset difficult to control seizures. One of the biggest changes seen over the last decade is the fact that children with very severe epilepsies, who have unilateral lesions, but "generalized" seizures and/or "generalized" EEGs, are not excluded anymore from considerations for epilepsy surgery. Even children with bilateral lesions can be surgical candidates.

Conclusion: The gradually widening spectrum of indications for epilepsy surgery in children is resulting in an increasing number of preventions of catastrophic epilepsies. Insufficient data regarding timing of surgery in order to prevent permanent mental retardations are calling for prospective multi-center studies.

Keywords: Catastrophic epilepsy; Childhood epilepsy; Epilepsy surgery; Epileptic encephalopathy.