In selected children with tuberous sclerosis complex, epilepsy surgery leads to seizure freedom or seizure reduction. The current standard involves a multimodal pre-surgical workup followed by invasive electrocorticographic monitoring and resective surgery. Recent insights in the disorder and novel technologies are changing the approach to pediatric epilepsy surgery in tuberous sclerosis complex. New evidence suggests tubers are poorly delineated, and epileptogenic activity may originate in the perituber tissue. Novel imaging modalities relevant to surgical planning include high-resolution MRI, α-methyl-l-tryptophan or fluorodeoxyglucose PET with diffusion tensor imaging. Advanced neurophysiological techniques have improved identification of the surgical target, including magnetoencephalography, electrical source imaging of high-density electroencephalograph data, and high-frequency oscillations in electrocorticography data. Simultaneously, novel surgical tools including stereo-electroencephalography and laser-induced thermal therapy have become available for children. This article reviews the literature in the light of these rapidly changing technologies.
Tuberous sclerosis complex (TSC) is a genetic (autosomal-dominant) neurocutaneous disorder with a prevalence of 1:6000 . Neurologically, TSC can manifest with intellectual deficits, neurobehavioral disorders, autism and seizures. Epilepsy occurs in 80–90%, typically develops during the first year of life, and in 50–80% of patients it is refractory to antiepileptic drugs (AEDs). Drug-resistant epilepsy, with frequent seizures and prolonged exposure to multiple AEDs, is associated with developmental delays, behavioral problems and severe cognitive deficits [2,3].
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