HHE Neurology supplement 2018 - Page 11

evaluation should be promptly started to assess the possibility of a surgical resection of the epileptic focus. Although data are not homogeneous, seizure freedom is achieved in a mean of 63% (range 25% to 90%) of surgically treated patients, with better results if the epileptogenic zone is accurately localised and the surgery is performed promptly. 15 inhibition of mTOR complex. 17 In the few available studies, more than 50% seizure reduction has been reported in 90% of cases. 18 mTOR inhibition The identification of a specific molecular pathway underlying epilepsy in TSC paved the way for evaluating the efficacy and safety of available mTOR inhibitors (rapamycin and its analogue everolimus) in TSC-related epilepsy. Previously available treatment options for epilepsy in TSC only provided a symptomatic treatment for seizures, whereas these drugs act on TSC pathogenesis, even presenting the potential of representing a disease-modifying systemic therapy. A first prospective, multicentre Phase I/II study enrolled 20 patients, and 72% of the 18 individuals completing the 48 months observation showed a ≥50% reduction in seizure frequency. 19,20 In a German clinical series, 57% of enrolled children showed 25–100% of seizure frequency reduction with adjunctive everolimus. 21 In another small study focusing on children and adolescents, 71% of subjects treated with Vagus nerve stimulation In patients not responding to anti-epileptic treatment and for whom surgery is not an option, vagus nerve stimulation should be considered. Although data are limited, seizure freedom is quite rare after vagus nerve stimulation implantation; however, a clinically significant response with a seizure frequency reduction higher than 50% in about 70% of treated patients. 16 Ketogenic diet In patients with refractory seizures who are not candidates for surgery, a ketogenic diet should also be considered. There are very limited but promising data for this non-pharmacological option, which seems to act with a partial A shorter gap from seizure onset and treatment initiation guarantees a better long-term outcome, both in terms of seizure refractoriness and neuro- psychological evolution 11 HHE 2018 | hospitalhealthcare.com