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Epilepsy surgery outcome in children with focal epilepsy due to tuberous sclerosis complex
M Karenfort1, B Kruse, H Freitag
1Epilepsy-Center Bethel, Klinik Mara I, Bielefeld, Germany.
Insights
Epilepsy surgery can significantly benefit children with Tuberous Sclerosis Complex (TSC) and drug-resistant epilepsy. Identifying the main seizure-causing tuber allows for targeted surgical intervention, improving seizure control and quality of life.
Area of Science:
- Neurology
- Pediatric Epilepsy
- Neurosurgery
Background:
- Tuberous Sclerosis Complex (TSC) often causes severe, drug-resistant focal epilepsy.
- Seizures are the initial symptom in most TSC patients with brain involvement, impacting development.
- Identifying a primary epileptogenic tuber is crucial for surgical candidacy.
Purpose of the Study:
- To evaluate the efficacy of epilepsy surgery in TSC patients with drug-resistant epilepsy.
- To assess the correlation between neuroimaging, EEG, and surgical outcomes.
- To determine if surgical intervention improves quality of life in these patients.
Main Methods:
- Long-term video-EEG monitoring
- Advanced neuroimaging techniques
- Neuropsychological testing
- Surgical intervention in selected patients
Main Results:
- A leading epileptogenic tuber was identified in all nine TSC patients studied.
- Good correlation was observed between neuroimaging and EEG findings.
- Eight patients underwent surgery, with significant seizure reduction in all cases.
- Two patients became seizure-free; others experienced substantial seizure frequency reduction without new neurological deficits.
Conclusions:
- Epilepsy surgery offers significant benefits for TSC patients with drug-resistant epilepsy.
- Surgical treatment can lead to reduced seizure frequency and severity.
- Improved mental and behavioral development, and enhanced quality of life are potential outcomes.
- Advancements in imaging may enable earlier and more precise surgical application in TSC.
Abstract:
Tuberous sclerosis complex (TSC) is frequently associated with focal epilepsy due to cerebral tubers. Seizures are the first symptoms in most patients with brain involvement. These epilepsies are frequently severe, drug-resistant and may have a negative impact on the child's global development. Although most epilepsies are multicentric, these patients may be candidates for epilepsy surgery, if it is possible to determine a leading epileptogenic tuber. Nine patients with TSC were examined with long-term video-EEG monitoring, different neuroimaging techniques and neuropsychological tests. A main epileptogenic tuber could be identified in all of our patients. We found good correlations between neuroimaging and EEG. Surgery was performed in eight patients. Seizure outcome was good in all. Two patients became seizure-free, one patient had a single prolonged seizure five days postoperatively, four patients had a significant seizure reduction of more than 75 % and one patient had a seizure reduction of more than 50 % after surgery without additional neurologic deficits. In conclusion, patients with TSC and drug-resistant epilepsy may benefit from epilepsy surgery with reduction in frequency and severity of seizures as well as improved mental and behavioural development leading to a better quality of life. In view of recent developments in functional and metabolic imaging, primary epileptogenic lesions will be more easily detectable in patients with diffuse brain involvement in TSC and surgical treatment may be more specifically applied at an earlier age to a selected subgroup of patients with this disorder.