Surgery: Can this be a cure for Epilepsy?
An eight-year-old boy had a history of tuberous sclerosis and autism. Tuberous sclerosis is one of the neurocutaneous diseases involving skin, brain, kidney and heart abnormalities in the form of abnormal tubers. These tubers can grow in the brain, causing seizures. Another abnormality commonly found in tuberous sclerosis is giant cell astrocytomas (brain tumors). With these children, it is imperative to decide whether the seizures are focal (and coming from a tuber or the surrounding area) or generalized. This child underwent resection of a tuber that was believed to be causing his seizures, but his seizures then became generalized. A vagal nerve stimulator was placed, and caused some reduction in seizures. After several years, it became apparent that his very frequent yet subtle seizures were rapidly generalizing and causing him to plateau in terms of his acquisition of social and academic skills. He was eating less and less and becoming more and more aggressive. After video EEG monitoring, a corpus callosotomy was performed. His disabling drop attacks have really lessened in frequency and his social and academic skills have soared. His aggressive outbursts have almost disappeared.
While the efficacy of this sort of approach is not traditionally as satisfying as focal epilepsy surgery, it is a useful tool in certain populations of children with epilepsy. In general, about a third of the patients who have a vagus nerve stimulator placed experience a significant reduction in seizure frequency and severity, and medication regimens can be significantly decreased. The natural appeal of such an approach, as any parent can imagine, is that the procedure is reversible: the stimulator (but not the wires) can be removed if the treatment is not effective, with no significant long term consequences.
Optimizing targets and parameters for neurostimulation to control epilepsy will be the focus of our research efforts to offer even safer and more attractive options to treat epilepsy in the future.•
In the previous case, a child underwent a focal resection before undergoing a corpus callosotomy. In this case, of a 19-year-old with autism and severe developmental delays, the procedures were reversed. She was initially diagnosed with infantile spasms as a baby. (Infantile spasms are a severe form of epilepsy occurring in infants 3-9 months old.) She went on to fail many different medications and became increasingly distant, with loss of her limited language and previously acquired skills. After she was accepted in a residential setting, she began to experience disabling drop attacks, causing her to wear a helmet. After a video EEG and MRI, it was determined that a corpus callosotomy was indicated. She underwent the procedure and her drop attacks stopped but she began to exhibit partial or focal seizures, with her head turning to the right, speech arrest and staring. She was reevaluated and the video EEG showed focal onset of her complex partial seizures. Grids and strips were implanted on the right side of her brain, seizure onset and epilpetogenic zone were delineated, and a right frontal disconnection was performed. Although she underwent four surgeries in total (including placement of a vagal nerve stimulator) and remains on medication, the family and the personnel at her school report increased language, better eye contact and a happier young woman, with a big decrease in seizure frequency and severity.