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Most of the 0.5% to 2% of the population with epilepsy will achieve seizure control with antiepileptic drugs. However, approximately 16% of children with new-onset epilepsy may become medically refractory or may have an epilepsy syndrome for which medical treatment is known to be ineffective.1 These children should be evaluated at a comprehensive pediatric epilepsy surgery program to evaluate their candidacy for epilepsy surgery, because just over half of them may be surgical candidates. The evaluation to determine candidacy requires accurate classification of a child’s seizures and epilepsy, knowledge of the natural history of the child’s condition, detailed information about past epilepsy treatments, and data acquisition to localize seizures, including video-electroencephalography (EEG) monitoring, functional nuclear studies, and neuroimaging data. Because many types of infantile and early childhood seizures are difficult to classify and of uncertain prognosis, pediatric surgical experience is greatest in older children and adolescents who have focal cerebral lesions or mesial temporal sclerosis. Select infants and younger children, however, may benefit from surgery, particularly when persistent seizures may affect developmental progress, as in the catastrophic epilepsies.

There is no standardized definition of medical intractability. In practice, it is often defined as the failure of an adequate trial of at least 2 appropriate anticonvulsant medications, increased to the limits of tolerability or expected efficacy. The appropriate timing of when to consider surgical options is somewhat subjective. In general, referral should be considered for medically intractable epilepsy which is functionally disabling and impairs quality of life. Children who may be optimal candidates include those with focal epilepsy, limited involvement of eloquent cortical areas, and a focal lesion.

Medically intractable epilepsy is associated with significant morbidity and mortality, including decreased quality of life for children and caregivers, frequent hospitalizations, anticonvulsant adverse effects, and the low but significant risk of mortality from seizures. Though the mechanisms are not fully understood, epilepsy is associated with significant behavioral, psychiatric, and cognitive comorbidity independent from the seizures.

In these children, therapeutic options are limited to further anticonvulsant trials, the ketogenic diet, vagus nerve stimulation, and epilepsy surgery. Emerging investigational techniques that may benefit future patients include transcranial magnetic stimulation, neurosurgical radiotherapy techniques, deep brain stimulation, and responsive neurostimulation. Of these options, resective epilepsy surgery currently offers the best chance of seizure freedom for optimal candidates.

Children differ from adults in terms of etiology of refractory seizures. In adults, mesial temporal lobe epilepsy, frequently associated with hippocampal sclerosis, accounts for most cases. In contrast, children more often have extratemporal or multifocal seizure onset. Pediatric etiologies of intractable epilepsy are diverse and include cortical dysplasia, tuberous sclerosis, hypothalamic hamartoma syndrome, Sturge-Weber syndrome, Rasmussen syndrome, infections, trauma, and developmental tumors.

The goal of the initial presurgical evaluation, also termed the Phase I evaluation, is to characterize seizures and localize their onset. No individual study is sufficient for determining epilepsy surgery candidacy or for surgical planning. Rather, the data obtained ...

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