Novel surgical protocol helps eliminate seizures for some children with TSC-related epilepsy, reduce medication needs for others

Some children with a form of epilepsy that previously made them poor candidates for surgery are now able to live without seizures, experience drastically reduced seizure episodes and symptoms, or take fewer anti-seizure medications as the result of a novel pre-surgical evaluation protocol developed by researchers in the Pediatric Epilepsy Surgery Program at Cincinnati Children’s.

A research team led by Francesco Mangano, DO, FACS, FACOS, chief of the Division of Pediatric Neurosurgery, studied 37 children who developed epilepsy as a result of tuberous sclerosis complex (TSC), a genetic disorder in which non-malignant tumors form in different organs, including the brain. More than 80 percent of children with TSC develop epilepsy that involves multi-focal brain abnormalities that vary from child to child. Their epilepsy symptoms vary as well.

The study, thought to be the largest single-center study in this pediatric epilepsy population, appeared in the January 2015 issue of the Journal of Neurosurgery: Pediatrics.

Pre-surgery evaluations relied on non-invasive and invasive brain mapping to identify the origin of seizure patterns in each patient. Neurosurgeons then decided which types of resective surgery to pursue – craniotomy to remove tuberous tissue, lobar resections, and even hemispherectomy in some cases.

After five years, 56 percent of the children were seizure-free, and 87 percent had far fewer seizures and significantly less severe seizures, based on a scale developed by the International League Against Epilepsy (ILAE).

“In those children who were not seizure-free, we were able to decrease the number of anti-epileptic drugs needed to continue to control their disease, and we were able to reduce their medication frequencies and doses to improve their side effect profiles,” Mangano says.

Neurosurgeons at Cincinnati Children’s employed a novel surgical protocol to help a 5-year-old girl with tuberous sclerosis complex (TSC) who developed multiple types of seizures. At the time of surgical referral, four anti-seizure medications had failed and she had mild global delay. Among several diagnostic scans, FDG-PET showed multiple focal areas of decreased metabolism (Image C). SPM imaging revealed a prominent area of hypometabolism involving the left frontal, temporal, and anterior parietal lobes (Image G). Subdural grids and interhemispheric strips were placed for invasive monitoring (Image M) in preparation for a right occipital lobectomy. At the two-year follow-up, the child was completely seizure free.
Click on image to view caption.

Citation

Arya R, Tenney JR, Horn PS, Greiner HM, Holland KD, Leach JL, Gelfand MJ, Rozhkov L, Fujiwara H, Rose DF, Franz DN, Mangano FT. Long-term outcomes of resective epilepsy surgery after invasive presurgical evaluation in children with tuberous sclerosis complex and bilateral multiple lesions. J Neurosurg Pediatr. 2015;15(1):26-33.