Clinical outcomes following stereotactic MRI-guided laser ablation in children with tuberous sclerosis complex and intractable epilepsy

立体定向磁共振引导激光消融术治疗结节性硬化症合并难治性癫痫患儿的临床疗效

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Abstract

OBJECTIVE: In children with tuberous sclerosis complex (TSC) and drug-resistant epilepsy (DRE), magnetic resonance imaging-guided stereotactic laser ablation (SLA) therapy offers less-invasive treatment compared to craniotomy and resection. Our study seeks to further expand on the long-term outcomes in patients with TSC-related DRE who have undergone SLA. METHODS: Patients with TSC and DRE treated with SLA, between July 1, 2016 and January 1, 2022, at our institution were identified retrospectively. Reduction in number of anti-seizure medications (ASMs), developmental improvement as reported by the patients' families, seizure frequency measured in surgically targeted seizure type and total seizures per day, and Engel classification were evaluated at 6 months, 1 year, 2 years, and thereafter at the most recent follow-up visit if available. RESULTS: Forty patients (ages 11 months to 23 years, median age 3.6 years) with TSC underwent SLA. Total follow-up was on average 2.9 years post-surgery. Significant (>50%) reduction in total seizure frequency occurred in 80% of patients (n = 32), with 63% of patients reporting a >90% reduction in seizure frequency (n = 25) at last follow-up. Fifty-eight percent had complete freedom from their surgically targeted seizure type (n = 23). At last follow up, perceived developmental gains occurred in 63% (n = 25), reduction in ASM occurred in 40% (n = 16), and Engel class was III or less in 93% of patients (n = 37). SIGNIFICANCE: SLA was safe and effective for most patients, even the very young. A majority of patients achieved improvement in seizure frequency, including the surgically targeted seizure type, but less than half were able to reduce medications. Reported developmental improvement was related to Engel outcomes. Future studies utilizing neuropsychological testing in the full cohort will be required to offer objective insights into post-operative development profiles.

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