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Rufinamide in patients with childhood onset intractable epilepsy
e u r o p e a n j o u r n a l o f p a e d i a t r i c n e u r o l o g y 2 1 ( 2 0 1 7 ) e 3 0 ee 4 2
P1-41 Rufinamide in patients with childhood onset intractable epilepsy Hyun-Ji Ahn, Young-Se Kwon, Mi-Sun Yum, Hye-Ryun Yeh, Tae-Sung Ko. Department of Pediatrics, Asan Medical Center Children's Hospital, Ulsan University College of Medicine, Seoul, Republic of Korea Objective: This study is aimed to evaluate the effectiveness and tolerability of rufinamide as add-on therapy in patients with intractable epilepsies. Methods: We retrospectively reviewed the medical records of 94 patients treated with rufinamide in Asan Medical Center, children's hospital. Twenty-five cases with less than 3 months of medication and 4 cases with incomplete medical records were excluded and total 65 cases were enrolled. Rufinamide was added on the existing antiepileptic drugs and the total seizure frequency at pre-medication, 3 months and 12 months were examined. Results: The mean age of 65 patients (44 male) were was 10 yrs (range, 1e24yrs). At 3 months after rufinamide initiation, 5 patients achieved freedom from seizures and 38 (58%) achieved a 50% seizure reduction. At 12 months, 8 of 55 patients achieved seizure freedom and 60% (23/55) achieved 50% seizure reduction. There was no significant difference of sex, age at seizure onset, duration of epilepsy, seizure types, and the diagnosis of Lennox-Gastaut syndrome (LGS) between responders and non-responders. The retention rate was hold up to 72% at 3 months, 64% at 12 months of study. Total 21 patients reported adverse events in order of somnolence, seizure aggravation, insomnia, common cold, nausea and vomiting and 3 cases of seizure aggravation, 1 case of somnolence, 1 case of visual hallucination had to stop the medication of rufinamide. Conclusion: In this study, rufinamide is effective and tolerable in patients with intractable epilepsy of childhood onset. The effectiveness is not significantly different according to their epileptic syndrome diagnosis of LGS. Further research is required to define the efficacy of rufinamide in intractable epilepsy other than LGS.
http://dx.doi.org/10.1016/j.ejpn.2017.04.816 P1-42 The effect of clobazam treatment in refractory epilepsies is reversible? Ali Cansu, Tu¨lay Kamas‚ak, Elif Acar Arslan, Sevim S‚ahin, Betu¨l Diler Durgut. Turkey Objective: Clobazam is a long-acting 1,5-benzodiazepine. It exhibits its anticonvulsant and anxiolytic effects via allosteric modulation of the inhibitory response of GABA, the most widespread neurotransmitter. Clobazam has begun being commonly used in refractory epilepsy. The aim of this study was to determine the changes clobazam produces in seizures and EEG, the types of seizure in which it is most effective, the side-effects occurring in patients and the drug's efficacy in seizure control. Methods: The records for 100 patients aged 2e17 under monitoring at the Karadeniz Technical University Pediatric Neurology clinics, started on Clobazam due to refractory epilepsy and using it for at least one year were investigated retrospectively. Seizure types and numbers, EEG findings before starting the drug, EEG changes after the drug, side- effects emerging during drug use, decreases in incidences of seizure
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and how long these decreases persisted were recorded. Results: A decrease in seizures was observed in 79% of patients, no change in incidence of seizures was determined in 21%. No EEG change was observed in 22% of patients, an improvement of less than 25% was observed in 20%, an improvement of 25e50% was observed in 29% and an improvement of more than 50% in 25%. A decrease of was observed in seizures, with 69% being reversible, 9% reversing after 1 to 3 month and 2% after 3 to 6 month, %11 reversing after 6 to 9 month, %12 reversing after 9 to 12 month and %15 reversing after one year later. The decrease in seizures persisted in 30% of patients. In terms of side-effects, drowsiness was seen in 34% of patients, and restlessness in 10%. No side effects were observed in 44% of patients. Conclusion: We conclude that clobazam therapy is well-tolerated and effective in the treatment of refractory epilepsy.
http://dx.doi.org/10.1016/j.ejpn.2017.04.817 P1-43 Efficacy of pulse methylprednisolone vs adrenocorticotrophic hormone in children with West Syndrome: An open-label pilot trial Pratibha Singhi, Anju Gupta, Madan Rajpurohit, Arushi Gahlot Saini, Vimlesh Soni. PGIMER, Chandigarh, India Objective: West syndrome is an age-dependent severe epileptic encephalopathy. According to guidelines Adrenocorticotrophic hormone (ACTH) is probably effective for short term management of infantile spasm but there is little uniformity in treatment due to variable response. We evaluated the efficacy of pulse methylprednisolone as compared to ACTH in children with west syndrome. Methods: Children between 3 months to 3 years with the diagnosis of west syndrome were included and treatment option of both ACTH and pulse methyprednisolone was explained with total cost for both. The parents who gave consent for ACTH were included to receive the same and others who did not take ACTH due to financial reasons were included to receive pulse methylprednisolone followed by oral prednisolone after taking consent. Primary outcome was to see the cessation of spasms after 6 weeks and secondary outcome was measured as reduction in number of spasms by at least 50% after 2 and 6 weeks and improvement in EEG score after 6 weeks. Results: Total 44 children were enrolled; 26 (59%) received ACTH and 18 (40%) received pulse methylprednisolone. After 2 weeks, both ACTH group and methylprednisolone group similarly showed >50% reduction in spasm (15/26 [57.6%] vs 10/ 18 [55.5%], p ¼ 0.88) and after 6 weeks also no significant difference was present in cessation of spasms in ACTH group as compared to methylprednisolone group (11/26 [42.3%] vs. 4/18 [22.2%], p ¼ 0.17). EEG Score did not show significant difference (p ¼ 0.57) in both the groups before and 6 weeks after the treatment. Conclusion: Pulse methylprednisolone followed by oral prednisolone for 6 weeks is as effective as ACTH for 6 weeks. Methylprednisolone therapy being more cost effective alternative to ACTH therapy in resource limited countries.
http://dx.doi.org/10.1016/j.ejpn.2017.04.818
P1-41 Rufinamide in patients with childhood onset intractable epilepsy Hyun-Ji Ahn, Young-Se Kwon, Mi-Sun Yum, Hye-Ryun Yeh, Tae-Sung Ko. Department of Pediatrics, Asan Medical Center Children's Hospital, Ulsan University College of Medicine, Seoul, Republic of Korea Objective: This study is aimed to evaluate the effectiveness and tolerability of rufinamide as add-on therapy in patients with intractable epilepsies. Methods: We retrospectively reviewed the medical records of 94 patients treated with rufinamide in Asan Medical Center, children's hospital. Twenty-five cases with less than 3 months of medication and 4 cases with incomplete medical records were excluded and total 65 cases were enrolled. Rufinamide was added on the existing antiepileptic drugs and the total seizure frequency at pre-medication, 3 months and 12 months were examined. Results: The mean age of 65 patients (44 male) were was 10 yrs (range, 1e24yrs). At 3 months after rufinamide initiation, 5 patients achieved freedom from seizures and 38 (58%) achieved a 50% seizure reduction. At 12 months, 8 of 55 patients achieved seizure freedom and 60% (23/55) achieved 50% seizure reduction. There was no significant difference of sex, age at seizure onset, duration of epilepsy, seizure types, and the diagnosis of Lennox-Gastaut syndrome (LGS) between responders and non-responders. The retention rate was hold up to 72% at 3 months, 64% at 12 months of study. Total 21 patients reported adverse events in order of somnolence, seizure aggravation, insomnia, common cold, nausea and vomiting and 3 cases of seizure aggravation, 1 case of somnolence, 1 case of visual hallucination had to stop the medication of rufinamide. Conclusion: In this study, rufinamide is effective and tolerable in patients with intractable epilepsy of childhood onset. The effectiveness is not significantly different according to their epileptic syndrome diagnosis of LGS. Further research is required to define the efficacy of rufinamide in intractable epilepsy other than LGS.
http://dx.doi.org/10.1016/j.ejpn.2017.04.816 P1-42 The effect of clobazam treatment in refractory epilepsies is reversible? Ali Cansu, Tu¨lay Kamas‚ak, Elif Acar Arslan, Sevim S‚ahin, Betu¨l Diler Durgut. Turkey Objective: Clobazam is a long-acting 1,5-benzodiazepine. It exhibits its anticonvulsant and anxiolytic effects via allosteric modulation of the inhibitory response of GABA, the most widespread neurotransmitter. Clobazam has begun being commonly used in refractory epilepsy. The aim of this study was to determine the changes clobazam produces in seizures and EEG, the types of seizure in which it is most effective, the side-effects occurring in patients and the drug's efficacy in seizure control. Methods: The records for 100 patients aged 2e17 under monitoring at the Karadeniz Technical University Pediatric Neurology clinics, started on Clobazam due to refractory epilepsy and using it for at least one year were investigated retrospectively. Seizure types and numbers, EEG findings before starting the drug, EEG changes after the drug, side- effects emerging during drug use, decreases in incidences of seizure
e39
and how long these decreases persisted were recorded. Results: A decrease in seizures was observed in 79% of patients, no change in incidence of seizures was determined in 21%. No EEG change was observed in 22% of patients, an improvement of less than 25% was observed in 20%, an improvement of 25e50% was observed in 29% and an improvement of more than 50% in 25%. A decrease of was observed in seizures, with 69% being reversible, 9% reversing after 1 to 3 month and 2% after 3 to 6 month, %11 reversing after 6 to 9 month, %12 reversing after 9 to 12 month and %15 reversing after one year later. The decrease in seizures persisted in 30% of patients. In terms of side-effects, drowsiness was seen in 34% of patients, and restlessness in 10%. No side effects were observed in 44% of patients. Conclusion: We conclude that clobazam therapy is well-tolerated and effective in the treatment of refractory epilepsy.
http://dx.doi.org/10.1016/j.ejpn.2017.04.817 P1-43 Efficacy of pulse methylprednisolone vs adrenocorticotrophic hormone in children with West Syndrome: An open-label pilot trial Pratibha Singhi, Anju Gupta, Madan Rajpurohit, Arushi Gahlot Saini, Vimlesh Soni. PGIMER, Chandigarh, India Objective: West syndrome is an age-dependent severe epileptic encephalopathy. According to guidelines Adrenocorticotrophic hormone (ACTH) is probably effective for short term management of infantile spasm but there is little uniformity in treatment due to variable response. We evaluated the efficacy of pulse methylprednisolone as compared to ACTH in children with west syndrome. Methods: Children between 3 months to 3 years with the diagnosis of west syndrome were included and treatment option of both ACTH and pulse methyprednisolone was explained with total cost for both. The parents who gave consent for ACTH were included to receive the same and others who did not take ACTH due to financial reasons were included to receive pulse methylprednisolone followed by oral prednisolone after taking consent. Primary outcome was to see the cessation of spasms after 6 weeks and secondary outcome was measured as reduction in number of spasms by at least 50% after 2 and 6 weeks and improvement in EEG score after 6 weeks. Results: Total 44 children were enrolled; 26 (59%) received ACTH and 18 (40%) received pulse methylprednisolone. After 2 weeks, both ACTH group and methylprednisolone group similarly showed >50% reduction in spasm (15/26 [57.6%] vs 10/ 18 [55.5%], p ¼ 0.88) and after 6 weeks also no significant difference was present in cessation of spasms in ACTH group as compared to methylprednisolone group (11/26 [42.3%] vs. 4/18 [22.2%], p ¼ 0.17). EEG Score did not show significant difference (p ¼ 0.57) in both the groups before and 6 weeks after the treatment. Conclusion: Pulse methylprednisolone followed by oral prednisolone for 6 weeks is as effective as ACTH for 6 weeks. Methylprednisolone therapy being more cost effective alternative to ACTH therapy in resource limited countries.
http://dx.doi.org/10.1016/j.ejpn.2017.04.818