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Antiepileptic drugs for the treatment of infants with severe myoclonic epilepsy.
Cochrane Database Syst Rev 2017; 5:CD010483CD

Abstract

BACKGROUND

This is an updated version of the original Cochrane review published in 2015, Issue 10.Severe myoclonic epilepsy in infants (SMEI), also known as Dravet syndrome, is a rare, refractory form of epilepsy, for which stiripentol (STP) has been recently licensed as add-on therapy.

OBJECTIVES

To evaluate the efficacy and tolerability of STP and other antiepileptic drug treatments (including ketogenic diet) for patients with SMEI.

SEARCH METHODS

For the latest update we searched the Cochrane Epilepsy Group Specialized Register (20 December 2016), the Cochrane Central Register of Controlled Trials (CENTRAL) via the Cochrane Register of Studies Online (CRSO, 20 December 2016), MEDLINE (Ovid, 1946 to 20 December 2016) and ClinicalTrials.gov (20 December 2016). Previously we searched the World Health Organization (WHO) International Clinical Trials Registry Platform ICTRP, but this was not usable at the time of this update. We also searched the bibliographies of identified studies for additional references. We handsearched selected journals and conference proceedings and imposed no language restrictions.

SELECTION CRITERIA

Randomised controlled trials (RCTs) or quasi-randomised controlled trials; double- or single-blinded or unblinded trials; and parallel-group studies. Administration of at least one antiepileptic drug therapy given singly (monotherapy) or in combination (add-on therapy) compared with add-on placebo or no add-on treatment.

DATA COLLECTION AND ANALYSIS

Review authors independently selected trials for inclusion according to predefined criteria, extracted relevant data and evaluated the methodological quality of trials. We assessed the following outcomes: 50% or greater seizure reduction, seizure freedom, adverse effects, proportion of dropouts and quality of life. We assessed outcomes by using a Mantel-Haenszel meta-analysis to calculate risk ratios (RRs) with 95% confidence intervals (95% CIs).

MAIN RESULTS

Since the last version of this review no new studies have been found. Specifically, we found no RCTs assessing drugs other than STP. The review includes two RCTs evaluating use of STP (total of 64 children). Both studies were generally at unclear risk of bias. A significantly higher proportion of participants had 50% or greater reduction in seizure frequency in the STP group compared with the placebo group (22/33 versus 2/31; RR 10.40, 95% CI 2.64 to 40.87). A significantly higher proportion of participants achieved seizure freedom in the STP group compared with the placebo group (12/33 versus 1/31; RR 7.93, 95% CI 1.52 to 41.21). Investigators found no significant differences in proportions of dropouts from the STP group compared with the placebo group (2/33 versus 8/31; RR 0.24, 95% CI 0.06 to 1.03). Only one study explicitly reported the occurrence of side effects, noting that higher proportions of participants in the STP group experienced side effects than in the placebo group (100% versus 25%; RR 3.73, 95% CI 1.81 to 7.67). We rated the quality of the evidence as low to moderate according to GRADE criteria, as most information is from studies judged to be at an unclear risk of bias.

AUTHORS' CONCLUSIONS

Data derived from two small RCTs indicate that STP is significantly better than placebo with regards to 50% or greater reduction in seizure frequency and seizure freedom. Adverse effects occurred more frequently with STP. Additional adequately powered studies with long-term follow-up should be conducted to unequivocally establish the long-term efficacy and tolerability of STP in the treatment of patients with SMEI.

Authors+Show Affiliations

Department of Neuroscience, Biomedicine and Movement, University of Verona, P.le L.A. Scuro, 10, Verona, Verona, Italy, 37134.Department of Neuropsychiatry, Federal Teaching Hospital, Abakaliki, Ebonyi State, Nigeria, 48000.Department of Health Sciences, Postgraduate School of Public Health, University of Genoa, Genoa, Italy.

Pub Type(s)

Journal Article
Meta-Analysis
Review
Systematic Review

Language

eng

PubMed ID

28521067

Citation

Brigo, Francesco, et al. "Antiepileptic Drugs for the Treatment of Infants With Severe Myoclonic Epilepsy." The Cochrane Database of Systematic Reviews, vol. 5, 2017, p. CD010483.
Brigo F, Igwe SC, Bragazzi NL. Antiepileptic drugs for the treatment of infants with severe myoclonic epilepsy. Cochrane Database Syst Rev. 2017;5:CD010483.
Brigo, F., Igwe, S. C., & Bragazzi, N. L. (2017). Antiepileptic drugs for the treatment of infants with severe myoclonic epilepsy. The Cochrane Database of Systematic Reviews, 5, p. CD010483. doi:10.1002/14651858.CD010483.pub4.
Brigo F, Igwe SC, Bragazzi NL. Antiepileptic Drugs for the Treatment of Infants With Severe Myoclonic Epilepsy. Cochrane Database Syst Rev. 2017 05 18;5:CD010483. PubMed PMID: 28521067.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Antiepileptic drugs for the treatment of infants with severe myoclonic epilepsy. AU - Brigo,Francesco, AU - Igwe,Stanley C, AU - Bragazzi,Nicola Luigi, Y1 - 2017/05/18/ PY - 2017/5/19/pubmed PY - 2017/8/10/medline PY - 2017/5/19/entrez SP - CD010483 EP - CD010483 JF - The Cochrane database of systematic reviews JO - Cochrane Database Syst Rev VL - 5 N2 - BACKGROUND: This is an updated version of the original Cochrane review published in 2015, Issue 10.Severe myoclonic epilepsy in infants (SMEI), also known as Dravet syndrome, is a rare, refractory form of epilepsy, for which stiripentol (STP) has been recently licensed as add-on therapy. OBJECTIVES: To evaluate the efficacy and tolerability of STP and other antiepileptic drug treatments (including ketogenic diet) for patients with SMEI. SEARCH METHODS: For the latest update we searched the Cochrane Epilepsy Group Specialized Register (20 December 2016), the Cochrane Central Register of Controlled Trials (CENTRAL) via the Cochrane Register of Studies Online (CRSO, 20 December 2016), MEDLINE (Ovid, 1946 to 20 December 2016) and ClinicalTrials.gov (20 December 2016). Previously we searched the World Health Organization (WHO) International Clinical Trials Registry Platform ICTRP, but this was not usable at the time of this update. We also searched the bibliographies of identified studies for additional references. We handsearched selected journals and conference proceedings and imposed no language restrictions. SELECTION CRITERIA: Randomised controlled trials (RCTs) or quasi-randomised controlled trials; double- or single-blinded or unblinded trials; and parallel-group studies. Administration of at least one antiepileptic drug therapy given singly (monotherapy) or in combination (add-on therapy) compared with add-on placebo or no add-on treatment. DATA COLLECTION AND ANALYSIS: Review authors independently selected trials for inclusion according to predefined criteria, extracted relevant data and evaluated the methodological quality of trials. We assessed the following outcomes: 50% or greater seizure reduction, seizure freedom, adverse effects, proportion of dropouts and quality of life. We assessed outcomes by using a Mantel-Haenszel meta-analysis to calculate risk ratios (RRs) with 95% confidence intervals (95% CIs). MAIN RESULTS: Since the last version of this review no new studies have been found. Specifically, we found no RCTs assessing drugs other than STP. The review includes two RCTs evaluating use of STP (total of 64 children). Both studies were generally at unclear risk of bias. A significantly higher proportion of participants had 50% or greater reduction in seizure frequency in the STP group compared with the placebo group (22/33 versus 2/31; RR 10.40, 95% CI 2.64 to 40.87). A significantly higher proportion of participants achieved seizure freedom in the STP group compared with the placebo group (12/33 versus 1/31; RR 7.93, 95% CI 1.52 to 41.21). Investigators found no significant differences in proportions of dropouts from the STP group compared with the placebo group (2/33 versus 8/31; RR 0.24, 95% CI 0.06 to 1.03). Only one study explicitly reported the occurrence of side effects, noting that higher proportions of participants in the STP group experienced side effects than in the placebo group (100% versus 25%; RR 3.73, 95% CI 1.81 to 7.67). We rated the quality of the evidence as low to moderate according to GRADE criteria, as most information is from studies judged to be at an unclear risk of bias. AUTHORS' CONCLUSIONS: Data derived from two small RCTs indicate that STP is significantly better than placebo with regards to 50% or greater reduction in seizure frequency and seizure freedom. Adverse effects occurred more frequently with STP. Additional adequately powered studies with long-term follow-up should be conducted to unequivocally establish the long-term efficacy and tolerability of STP in the treatment of patients with SMEI. SN - 1469-493X UR - https://www.unboundmedicine.com/medline/citation/28521067/Antiepileptic_drugs_for_the_treatment_of_infants_with_severe_myoclonic_epilepsy_ L2 - https://doi.org/10.1002/14651858.CD010483.pub4 DB - PRIME DP - Unbound Medicine ER -