Psychobehavioural and cognitive adverse events of anti-seizure medications for the treatment of developmental and epileptic encephalopathies

A Strzelczyk, S Schubert-Bast - CNS drugs, 2022 - Springer
The developmental and epileptic encephalopathies encompass a group of rare syndromes
characterised by severe drug-resistant epilepsy with onset in childhood and significant …

Dravet syndrome: a systematic literature review of the illness burden

A Strzelczyk, L Lagae, JM Wilmshurst… - Epilepsia …, 2023 - Wiley Online Library
We performed a systematic literature review and narrative synthesis according to a pre‐
registered protocol (Prospero: CRD42022376561) to identify the evidence associated with …

Incidence and phenotypes of childhood-onset genetic epilepsies: a prospective population-based national cohort

JD Symonds, SM Zuberi, K Stewart, A McLellan… - Brain, 2019 - academic.oup.com
Epilepsy is common in early childhood. In this age group it is associated with high rates of
therapy-resistance, and with cognitive, motor, and behavioural comorbidity. A large number …

Monogenic epilepsies: disease mechanisms, clinical phenotypes, and targeted therapies

R Guerrini, S Balestrini, EC Wirrell, MC Walker - Neurology, 2021 - neurology.org
A monogenic etiology can be identified in up to 40% of people with severe epilepsy. To
address earlier and more appropriate treatment strategies, clinicians are required to know …

Dravet syndrome: treatment options and management of prolonged seizures

JH Cross, RH Caraballo, R Nabbout, F Vigevano… - …, 2019 - Wiley Online Library
Over time, with careful delineation of Dravet syndrome, we have gained experience in
treatments most likely to lead to improvement in seizures, as well as those that should be …

Dravet syndrome: Advances in etiology, clinical presentation, and treatment

Z He, Y Li, X Zhao, B Li - Epilepsy Research, 2022 - Elsevier
Dravet syndrome (DS) is a form of genetic refractory epilepsy. More than 80% of DS patients
carry pathogenic SCN1A mutations, and this percentage is actually higher due to false …

Recent advances in the drug treatment of Dravet syndrome

EC Wirrell, R Nabbout - CNS drugs, 2019 - Springer
Dravet syndrome is a rare but severe epilepsy syndrome that begins in the first year of life
with recurrent seizures triggered by fever that are typically prolonged and hemiclonic. The …

[HTML][HTML] A systematic review of adults with Dravet syndrome

A Selvarajah, Q Zulfiqar-Ali, P Marques, M Rong… - Seizure, 2021 - Elsevier
Dravet Syndrome (DS) is a rare and severe infantile-onset epileptic encephalopathy. DS
research focuses mainly on children. We did a systematic review, completed on January 18 …

Fenfluramine HCl (Fintepla®) provides long‐term clinically meaningful reduction in seizure frequency: Analysis of an ongoing open‐label extension study

J Sullivan, IE Scheffer, L Lagae, R Nabbout… - …, 2020 - Wiley Online Library
Objective Fenfluramine has been shown to provide clinically meaningful and statistically
significant reductions in convulsive seizure frequency in children and adolescents (aged 2 …

Comparative efficacy and safety of stiripentol, cannabidiol and fenfluramine as first‐line add‐on therapies for seizures in Dravet syndrome: a network meta‐analysis

R Guerrini, C Chiron, D Vandame, W Linley… - Epilepsia …, 2024 - Wiley Online Library
Objectives Stiripentol, fenfluramine, and cannabidiol are licensed add‐on therapies to treat
seizures in Dravet Syndrome (DS). There are no direct or indirect comparisons assessing …