Cannabidiol in children with treatment-resistant epilepsy with myoclonic-atonic seizures

Purpose: This multicenter study aimed to evaluate the efficacy and tolerability of add-on cannabidiol (CBD) in treatment-resistant patients with epilepsy with myoclonic-atonic seizures (EMAtS) (n = 22) and Sturge Weber syndrome (SWS) with myoclonic-atonic seizures (n = 4). Methods: Patients who met the diagnostic criteria of treatment-resistant EMAtS or SWS with myoclonic-atonic seizures were included. Cannabidiol was added in doses ranging from 8 to 40 mg/kg/day. Efficacy was assessed by comparing seizure frequency before and after initiating CBD therapy. Neurologic examinations, brain magnetic resonance imaging, repeated prolonged electroencephalogra-phy (EEG) and/or video-EEG recordings, and neurometabolic studies were performed in all patients, and genetic investigations in 15. Results: After a mean follow-up of 19 months, 15/26 patients (57.7%) who received add-on CBD had a >50% seizure decrease; three (11.5%) became seizure-free. The remaining 11 patients (42.3%) had a 25-50% seizure reduction. Drop attacks, including myoclonic-atonic seizures and generalized tonic-clonic seizures, as well as atypical absences and nonconvulsive status epilepticus responded well to CBD. In SWS patients, focal motor seizures without consciousness impairment and focal non-motor sei-zures with consciousness impairment were recognized in two each; in three a 30% reduction of focal sei-zures was observed. Side effects were mild and did not lead to CBD discontinuation. Conclusion: This study evaluating the use of add-on CBD in children with EMAtS or SWS with myoclonic-atonic seizures found that 15/26 (57.7%) had a >50% seizure reduction with good tolerability; three (11.5%) became seizure-free. (c) 2023 Elsevier Inc. All rights reserved.

Automated summary

This multicenter study evaluated the efficacy and tolerability of add-on CBD in patients with treatment-resistant epilepsy and Sturge Weber syndrome. The results showed that more than half of the patients experienced a >50% reduction in seizure frequency, with some becoming seizure-free. Side effects were mild and did not lead to discontinuation of CBD.