Improving outcomes in childhood epilepsy

Lectures
When does childhood become refractory?
Lieven Lagae, Belgium

Are AEDs Really Effective – What is the Evidence?
Elaine C. Wirrell, USA

Antiepileptic medications are considered the first-line therapy in children with newly-diagnosed epilepsy. However in approximately 20-30% of cases, medications fail to adequately control seizures and the epilepsy is deemed "medically intractable". The definition of "effectiveness" varies, with most drug licensing studies classifying an AED as effective if it reduces seizure frequency by greater than 50%. However, given the significant co-morbidities associated with pediatric epilepsy, the goal of most families and clinicians is complete, or near-complete seizure-freedom. This talk will review the effectiveness of AEDs when used both first-line in children with new-onset epilepsy, as well as after serial medications have failed due to lack of efficacy. The impact of seizure type, underlying etiology and electroclinical syndrome on treatment success will be evaluated. Specific seizure types and syndromes may show unique efficacy to certain AEDs, or conversely may be exacerbated by others, and this data will be summarized.

Alternative treatments – can we be more targeted?
Helen Cross, UK

The ketogenic diet has been used in the treatment of drug resistant epilepsy for almost one hundred years. Class one evidence is now available showing the diet is as effective as newer antiepileptic drug in drug resistant epilepsy. Although the mechanism of action has not been fully delineated, it has become clear there are a group of disorders presenting with epilepsy for which the ketogenic diet provides a targeted approach to treatment. Glucose transporter 1 deficiency was first described as a metabolic encephalopathy with progressive intellectual disability, movement disorder, microcephaly and epilepsy. Over time, with delineation of the genetic mutation SLC2A1, it has become clear that the phenotype is wide, including early onset absence seizures and well described epilepsy syndromes eg myoclonic astatic epilepsy, with or without motor disorder. Consequently the ketogenic diet is becoming an option to a wider range of epilepsies. Another genetic disorder, Tuberous Sclerosis has been established as disorder of the mTOR pathway; mTOR inhibitors have not only been shown to reduce the size of subependymal giant cell astrocytomas but are now being evaluated in the treatment of TS associated epilepsy. Other epilepsies the result of defects in the pathway may also be suitable for such treatment. As further definitive aetiologies to the epilepsies become apparent, targeted treatments become a realistic option.

Early onset epilepsies – can surgery improve outcome?
Marilisa Guerreiro, Brazil