Add-on perampanel in Lance–Adams syndrome

Steinhoff, Bernhard J.; Bacher, Matthias; Kurth, Christoph; Staack, Anke M.; Kornmeier, Reinhold

doi:10.1016/j.ebcr.2016.05.001

Cited by 32 publications

(32 citation statements)

References 21 publications

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“…19 For one of these cases(alsopublishedasacasereport),perampanelwasthefinalAED administered prior to cessation of focal SE, 24 hours after its initial administration. 28 The case studies presented here provide further support for perampanel as a treatment for RSE. 28 The case studies presented here provide further support for perampanel as a treatment for RSE.…”

Section: Discussionsupporting

confidence: 56%

Perampanel for treatment of status epilepticus in Austria, Finland, Germany, and Spain

et al. 2019

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Objective Novel treatments are needed to control treatment‐resistant status epilepticus (SE). We report a summary of clinical cases where perampanel was used in established SE, refractory SE (RSE), or super‐refractory SE (SRSE). Methods Medical records were retrospectively reviewed for perampanel administration in SE at five European hospitals between 2011 and 2015. Results Of 1319 patients identified as experiencing SE, 52 (3.9%) received perampanel. Median latency from SE onset to perampanel initiation was 10 days. Patients with SE had previously failed benzodiazepines (when received) and a median of five other antiepileptic drugs (AEDs). Median initial perampanel dose was 6 mg/d, up‐titrated to a median maximum dose of 10 mg/d. Perampanel was the last drug added in 32/52 (61.5%) patients, with response attributed to perampanel in 19/52 (36.5%) patients. A greater proportion of perampanel non‐responders had SRSE (51.5%; 17/33) vs perampanel responders (31.6%; 6/19), and had failed a higher mean number of AEDs before initiating perampanel (5.9 vs 5.1, respectively). Most commonly reported adverse effects during perampanel treatment were dizziness (n = 1 [1.9%]) and somnolence (n = 1 [1.9%]). No serious adverse effects were documented, and none led to discontinuation of perampanel. Conclusions Perampanel was administered to patients with established SE, RSE, or SRSE at greater initial doses than those administered in clinical practice to patients with epilepsy. The SE cases reported here represent a refractory and heterogeneous population, and rate of seizure cessation attributed to perampanel treatment (36.5%) represents a notable response. These data should be confirmed in a larger patient population.

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Section: Discussionsupporting

confidence: 56%

Perampanel for treatment of status epilepticus in Austria, Finland, Germany, and Spain

et al. 2019

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“…Some case series have also been published on the use of perampanel in specific situations-seizure associated with glioma, 7,8 progressive myoclonus epilepsies, [9][10][11][12] other myoclonus, [13][14][15] and status epilepticus [16][17][18] -and case reports of adverse events (Table S2).…”

Section: Key Pointsmentioning

confidence: 99%

Perampanel in routine clinical use across Europe: Pooled, multicenter, observational data

et al. 2018

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Across a large, treatment-resistant population, add-on perampanel was retained for ≥1 year by 48% of individuals, and 9% were seizure-free for ≥6 months. TEAEs were in line with previous reports in routine clinical use, and less frequent than in the clinical trial setting. No new or unexpected TEAEs were seen. Despite the limitations of observational studies, our data indicate that some individuals may derive a marked benefit from the use of perampanel.

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“…Positive (often dramatic) responses of myoclonic and GTC seizures to perampanel have been reported in 12 patients with Unverricht‐Lundborg disease, 10 patients with Lafora disease, as well as individual case studies in Lance Adams syndrome, dentatorubral‐pallidoluysian atrophy (DRPLA), Lafora disease, and myoclonic status epilepticus …”

Section: Discussionmentioning

confidence: 99%

“…The median number of concomitant AEDs at baseline was 2 (range 0-4; mean 1.7), most commonly levetiracetam (79/ 149 patients, 53.0%), valproic acid (52, 34.9%), lamotrigine (35,23.5%), and zonisamide (34,22.8%). Exposure to concomitant AEDs significantly reduced over time from a mean (standard deviation, SD) of 1.7 (0.9) at baseline to 1.5 (0.9) at 12 months (P < 0.001; Friedman test).…”

Section: Concomitant Aedsmentioning

confidence: 99%

Perampanel in routine clinical use in idiopathic generalized epilepsy: The 12‐month GENERAL study

et al. 2018

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Summary Objective To analyze the effectiveness and tolerability of perampanel across different seizure types in routine clinical care of patients with idiopathic generalized epilepsy (IGE). Methods This multicenter, retrospective, 1‐year observational study collected data from patient records at 21 specialist epilepsy units in Spain. All patients who were aged ≥12 years, prescribed perampanel before December 2016, and had a confirmed diagnosis of IGE were included. Results The population comprised 149 patients with IGE (60 with juvenile myoclonic epilepsy, 51 generalized tonic–clonic seizures [GTCS] only, 21 juvenile absence epilepsy, 10 childhood absence epilepsy, 6 adulthood absence epilepsy, and one Jeavons syndrome). Mean age was 36 years. The retention rate at 12 months was 83% (124/149), and 4 mg was the most common dose. At 12 months, the seizure‐free rate was 59% for all seizures (88/149); 63% for GTCS (72/115), 65% for myoclonic seizures (31/48), and 51% for absence seizures (24/47). Seizure frequency was reduced significantly at 12 months relative to baseline for GTCS (78%), myoclonic (65%), and absence seizures (48%). Increase from baseline seizure frequency was seen in 5.2% of patients with GTCS seizures, 6.3% with myoclonic, and 4.3% with absence seizures. Perampanel was effective regardless of epilepsy syndrome, concomitant antiepileptic drugs (AEDs), and prior AEDs, but retention and seizure freedom were significantly higher when used as early add‐on (after ≤2 prior AEDs) than late (≥3 prior AEDs). Adverse events were reported in 50% of patients over 12 months, mostly mild or moderate, and irritability (23%), somnolence (15%), and dizziness (14%) were most frequent. Significance In routine clinical care of patients with IGE, perampanel improved seizure outcomes for GTCS, myoclonic seizures, and absence seizures, with few discontinuations due to adverse events. This is the first real‐world evidence with perampanel across different seizure types in IGE.

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Add-on perampanel in Lance–Adams syndrome

Cited by 32 publications

References 21 publications

Perampanel for treatment of status epilepticus in Austria, Finland, Germany, and Spain

Perampanel for treatment of status epilepticus in Austria, Finland, Germany, and Spain

Perampanel in routine clinical use across Europe: Pooled, multicenter, observational data

Perampanel in routine clinical use in idiopathic generalized epilepsy: The 12‐month GENERAL study

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