Efficacy and tolerability of fenfluramine in patients with Dravet syndrome: A systematic review and meta-analysis

Sharawat, Indar Kumar; Panda, Prateek Kumar; Kasinathan, Ananthanarayanan; Joshi, Kriti

doi:10.1016/j.seizure.2020.12.016

Cited by 23 publications

(20 citation statements)

References 23 publications

(46 reference statements)

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“…Historically, phenytoin (PHT), valproate, and carbamazepine are considered first‐line anti‐seizure medications (ASMs) for epilepsy patients on an outpatient basis or even in cases for acute seizure control 1,2 . However, in the last two decades, other medications like levetiracetam, lacosamide, topiramate, and lamotrigine have become increasingly popular, as the older ASMs are known to have more frequent and more severe treatment‐emergent adverse effects(TEAEs).…”

Section: Introductionmentioning

confidence: 99%

Efficacy of lacosamide and phenytoin in status epilepticus: A systematic review

Panda

Sharawat

2021

Acta Neurol Scand

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Objectives To compare the evidence on efficacy, safety, tolerability, and impact on short term/long functional outcome of lacosamide (LCM) and phenytoin (PHT) in patients with status epilepticus. Materials & Methods We conducted a systematic literature search of relevant electronic databases using a suitable search strategy to identify studies directly comparing PHT and LCM, irrespective of dose and duration in patients with convulsive and/or nonconvulsive status epilepticus (SE). We used a standardized assessment form to extract information on the study design, data sources, methodologic framework, efficacy, and adverse events attributed to PHT and LCM from included studies and compared the efficacy and safety outcomes, using a fixed/random effect model. Results Five studies were found to be eligible for inclusion out of 192 search items, enrolling a total of 115 and 166 participants (predominantly with SE) in LCM and PHT arm, respectively. Baseline characteristics were comparable between both arms. The proportion with seizure control was comparable between both arms (57.3% in LCM vs. 45.7% in PHT arm, p = 0.28) and even in the subgroup analysis separately for convulsive and non‐convulsive SE. Proportion with treatment‐emergent adverse events (TEAE) were comparable in both (17.6% vs. 12.2%, p = 0.20), but serious adverse events (SAE) were higher in PHT arm (5.1% vs. 0.8%, p = 0.049). The proportion with all‐cause mortality and survival with moderate‐severe disability were comparable between both arms (p = 0.23 and 0.37, respectively). Conclusion LCM has comparable efficacy with fewer SAEs as compared to PHT for achieving seizure control in patients with SE.

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Section: Introductionmentioning

confidence: 99%

Efficacy of lacosamide and phenytoin in status epilepticus: A systematic review

Panda

Sharawat

2021

Acta Neurol Scand

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“…A lag between completion/publication of clinical research and the incorporation of their key findings into disease specific guidelines is expected, and this is reflected in the absence of identified guidelines for DS that include recommendations for the drug fenfluramine, which is the latest treatment approved for this indication ( 58 , 59 ). However with the recent emergence of novel treatments for DS, LGS and CDD ( 3 , 59 – 61 ), and for other diseases in general, it is hoped that this lag will become as short as possible.…”

Section: Discussionmentioning

confidence: 99%

Treatment Guidelines for Rare, Early-Onset, Treatment-Resistant Epileptic Conditions: A Literature Review on Dravet Syndrome, Lennox-Gastaut Syndrome and CDKL5 Deficiency Disorder

et al. 2021

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Background: Dravet syndrome (DS), Lennox-Gastaut syndrome (LGS) and CDKL5 deficiency disorder (CDD) are rare epileptic conditions, characterised by drug-resistant seizures. Seizure management in these patients requires careful therapy selection. This targeted literature review (TLR) aimed to collate and synthesise information from country-specific and international treatment guidelines for DS, LGS and CDD.Methods: A TLR was performed between 25th January and 11th March 2021. Online rare diseases and guideline databases were manually searched in addition to websites of national health technology assessment bodies for the following countries: Australia, Canada, France, Germany, Israel, Italy, Japan, Spain, Switzerland, UK and US, as defined by pre-specified eligibility criteria. Search terms, developed for each condition, were translated into local languages where appropriate. Descriptive analyses were performed to examine the geographical distribution of included guidelines; methodologies used to develop guidelines; cross-referencing of treatment recommendations made within other guidelines; patterns of treatment recommendations. An author map was created using R version 3.5.1, to visualise the extent of collaboration between authors.Results: Forty total guidelines were included, of which 29, 34 and 0 contained recommendations for DS, LGS and CDD, respectively (some provided recommendations for ≥1 condition). Most were country-specific, with guideline authors predominantly publishing in regional groups. Five guidelines were classified as “International” and displayed connections between author groups in the US, UK, France and Italy. Reported guideline development processes were lacking [43% (17 guidelines) had unclear/absent literature review methodologies] and those reported were variable, including both systematic and targeted literature reviews. Use of expert consultation was also variable. A high degree of heterogeneity was observed in the availability of treatment recommendations across disorders, with 271 and 190 recommendations for LGS and DS, respectively, and contradictory positive and negative treatment recommendations for several drugs in each indication [35% (11/31) and 22% (6/27) in LGS and DS, respectively].Conclusions: This review highlights the need for further high-quality international consensus-based treatment guidelines for LGS, DS, and particularly for CDD (for which no treatment guidelines were identified). Supra-national consensus guidance based on findings from a wider geographical range may improve resource allocation and establish an improved world-wide standard of care.

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“…La información disponible hasta la fecha deberá ser revisada por la EMA con objeto de determinar si el medicamento puede mantener la designación de huérfano. 1,[14][15][16][17][18][19] El síndrome de Dravet conocido también como epilepsia mioclónica severa de la infancia, es una forma rara y catastrófica de epilepsia intratable (farmacorresistente) que comienza en el primer año de vida, con una incidencia estimada de 1 cada 16.000 nacimientos.…”

Section: Cultivo Celular Autólogo De Cd34+ Enriquecido Con Células Madre Y Células Progenitoras Hematopoyéticas Transducidas Ex Vivo Con unclassified

Evaluación positiva de medicamentos: octubre 2020

Aparicio-Hernández¹,

García-Luque²,

Acuña-Vega³

et al. 2021

Sanid. Mil.

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Efficacy and tolerability of fenfluramine in patients with Dravet syndrome: A systematic review and meta-analysis

Cited by 23 publications

References 23 publications

Efficacy of lacosamide and phenytoin in status epilepticus: A systematic review

Efficacy of lacosamide and phenytoin in status epilepticus: A systematic review

Treatment Guidelines for Rare, Early-Onset, Treatment-Resistant Epileptic Conditions: A Literature Review on Dravet Syndrome, Lennox-Gastaut Syndrome and CDKL5 Deficiency Disorder

Evaluación positiva de medicamentos: octubre 2020

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