Use of perampanel in children and adolescents with Lennox–Gastaut Syndrome

Auvin, Stéphane; Dozieres, Blandine; Iléa, Adina; Delanoë, Catherine

doi:10.1016/j.yebeh.2017.05.036

Cited by 39 publications

(26 citation statements)

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“…Perampanel efficacy has also been assessed in other defined epileptic syndromes. A recent study conducted in 13 Lennox-Gastaut syndrome patients reported that nine patients (69.2%) were responders, and seven patients (53.8%) were improved in cognitive function and/ or behavior [30]. Other studies also indicated a responder of 40-66.6% in a small group of nine patients [28,29,31].…”

Section: Discussionmentioning

confidence: 96%

“…A few observational studies of the use of PER in children and adolescents have been reported in the last 3 years. Data from these real-world clinical experiences, although comprising heterogeneous groups of patients, also indicated that PER was reasonably effective in pediatric patients with refractory epilepsy or with severe epileptic encephalopathies and generally with a well-tolerability profile [26][27][28][29][30].…”

Section: Introductionmentioning

confidence: 99%

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Efficacy and tolerability of perampanel in children and adolescents with pharmacoresistant epilepsy: The first real-world evaluation in Asian pediatric neurology clinics

Lin

Chou

et al. 2018

Epilepsy & Behavior

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Section: Discussionmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%

Efficacy and tolerability of perampanel in children and adolescents with pharmacoresistant epilepsy: The first real-world evaluation in Asian pediatric neurology clinics

Lin

Chou

et al. 2018

Epilepsy & Behavior

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“…PER is a selective non-competitive antagonist of AMPA receptors [75], It was administrated as add-on therapy to 58 children and adolescents with refractory epilepsies, including five with LGS, showing ≥50% seizure frequency reduction in 31% and seizure freedom in 9.1% [70]. PER as adjunctive treatment was also assessed in a prospective study, involving a small sample-13 LGS patients-who received PER for a mean period of 10.8 months [71]. A ≥ 50% reduction in total seizure frequency was reported for 9 patients (69.2%), while 4 had nor improvement, neither seizure worsening, indicating a potential utility of PER in LGS.…”

Section: Other Aedsmentioning

confidence: 99%

The pharmacological management of Lennox-Gastaut syndrome and critical literature review

Verrotti

Striano

Iapadre

et al. 2018

Seizure

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A B S T R A C TLennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy with a prevalence of 1-2% of all patients with epilepsy. It is characterized by multiple pharmaco-resistant seizure types, including tonic, atypical absences and tonic or atonic drop attacks, and the presence of electroencephalographic abnormalities, such as slow-spike waves and paroxysmal fast rhythms. Intellectual disability, behavioural and psychiatric disorders are common comorbidities; these disturbances have a multi-factorial pathogenesis. The selection of the most appropriate drug must be tailored to each patient and guided by the prevalent seizure type. In this paper available pharmacological options are discussed and for each pharmacological agent, current evidence of efficacy and tolerability is provided. Valproic acid represents one of the first-line options in the treatment of LGS. Anyway, other antiepileptic drugs (AEDs) may be considered and added: lamotrigine, rufinamide, topiramate, clobazam can be efficacious. The use of felbamate must be carefully evaluated because of its adverse events. Perampanel, zonisamide, levetiracetam and fenfluramine have shown to be useful in the treatment of selected patients; nevertheless, the lack of RCTs does not allow to recommend their use in a systematic way. Recently, cannabidiol has provided high evidence of efficacy against LGS seizures; however, these data must be confirmed by long-term extensive studies and by trials comparing different AEDs, one to each other.

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“…While no results have been reported from the clinical trial to date, the efficacy and safety of perampanel in LGS patients has been demonstrated in retrospective, open-label, observational studies [113][114][115][116][117][118]. Response rates (≥ 50% seizure reduction) of 64.8% (46/71 adult patients [113]) and 69.2% (9/13 children/adolescents [117]) have been reported in LGS patients. In addition, in a large real-world study of 2396 individuals with treatment-resistant epilepsy who were treated with add-on perampanel, 9% were seizure free for ≥ 6 months, and the 1-year retention rate was 48% [118].…”

Section: Perampanelmentioning

confidence: 99%

Expanding the Treatment Landscape for Lennox-Gastaut Syndrome: Current and Future Strategies

Strzelczyk

Schubert‐Bast

2021

CNS Drugs

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Lennox-Gastaut syndrome (LGS), a childhood-onset severe developmental and epileptic encephalopathy (DEE), is an entity that encompasses a heterogenous group of aetiologies, with no single genetic cause. It is characterised by multiple seizure types, an abnormal EEG with generalised slow spike and wave discharges and cognitive impairment, associated with high morbidity and profound effects on the quality of life of patients and their families. Drug-refractory seizures are a hallmark and treatment is further complicated by its multiple morbidities, which evolve over the patient’s lifetime. This review provides a comprehensive overview of the current and future options for the treatment of seizures associated with LGS. Six treatments are specifically indicated as adjunct therapies for the treatment of seizures associated with LGS in the US: lamotrigine, clobazam, rufinamide, topiramate, felbamate and most recently cannabidiol. These therapies have demonstrated reductions in drop seizures in 15%–68% of patients across trials, with responder rates (≥ 50% reduction in drop seizures) of 37%–78%. Valproate is still the preferred first-line treatment, generally in combination with lamotrigine or clobazam. Other treatments frequently used off-label include the broad spectrum anti-epileptic drugs (AED) levetiracetam, zonisamide and perampanel, while recent evidence from observational studies has indicated that a newer AED, the levetiracetam analogue brivaracetam, may be effective and well tolerated in LGS patients. Other treatments in clinical development include fenfluramine in late phase III, perampanel, soticlestat–OV953/TAK-953, carisbamate and ganaxolone. Non-pharmacologic interventions include the ketogenic diet, vagus nerve stimulation and surgical interventions; these are also expanding, with the potential for less invasive techniques for corpus callosotomy that have promise for reducing complications. However, despite these advancements, patients continue to experience a significant burden. Because LGS is not a single entity, tailoring of treatment is needed as opposed to a ‘one size fits all’ approach. Further research is needed into the underlying aetiologies and pathophysiology of LGS, together with advancements in treatments that encompass the spectrum of seizures associated with this complex syndrome.

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Use of perampanel in children and adolescents with Lennox–Gastaut Syndrome

Cited by 39 publications

References 12 publications

Efficacy and tolerability of perampanel in children and adolescents with pharmacoresistant epilepsy: The first real-world evaluation in Asian pediatric neurology clinics

Efficacy and tolerability of perampanel in children and adolescents with pharmacoresistant epilepsy: The first real-world evaluation in Asian pediatric neurology clinics

The pharmacological management of Lennox-Gastaut syndrome and critical literature review

Expanding the Treatment Landscape for Lennox-Gastaut Syndrome: Current and Future Strategies

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