Tuberous sclerosis and multiple tubers: localizing the epileptogenic zone.

Abstract

Tuberous sclerosis complex (TSC) is associated with medically refractory seizures and developmental delay in children. These epilepsies are often resistant to antiepileptic drugs (AEDs), may be quite severe, and usually have a negative impact on the child's neurological and cognitive development. It is believed that functional outcome is improved if seizures can be controlled at an early age. The surgical treatment of intractable epilepsy in children and adults with TSC has gained significant interest in recent years. Previously published studies have shown a potential benefit from resection of single tubers, with most of the results noted in relatively older children. All of these reports support the idea that if a single primary epileptogenic tuber/region can be identified, then a surgical approach is appropriate. However, most children with TSC have multiple potentially epileptogenic tubers, rendering localization challenging, and they are therefore rejected as possible surgical candidates. We have utilized a novel surgical approach using invasive intracranial monitoring, which is typically multistaged and bilateral. Here we present an illustrative case of a young boy with TSC and medically refractory epilepsy who underwent a staged surgical approach. This multistage surgical approach has been useful in identifying both primary and secondary epileptogenic zones in TSC patients with multiple tubers. Multiple or bilateral seizure foci are not necessarily a contraindication to surgery in selected TSC patients. Long-term follow-up will determine whether this approach has durable effects. We await better methods for identifying the epileptogenic zone, both noninvasive and invasive.