Seizure control and improvement of neurological dysfunction in Lafora disease with perampanel

Dirani, Maya; Nasreddine, Wassim; Abdulla, Fatema; Beydoun, Ahmad

doi:10.1016/j.ebcr.2014.09.003

Cited by 71 publications

(80 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Case reports are suggesting efficacy of PER in progressive myoclonus epilepsies [10,41]. In a 21-year-old woman with genetically proven Lafora disease, PER at a daily dose of 8 mg/day resulted in a sustained remission of myoclonus and generalized tonic-clonic seizures for more than 3 months despite previously continuous daily seizures for more than 7 years.…”

Section: Use In Progressive Myoclonus Epilepsiesmentioning

confidence: 99%

Perampanel in the treatment of focal and idiopathic generalized epilepsies and of status epilepticus

Strzelczyk

Willems

Willig

et al. 2015

Expert Review of Clinical Pharmacology

View full text Add to dashboard Cite

Perampanel is the latest approved antiepileptic drug in focal and generalized epilepsies and has a distinct and selective mode of action on AMPA-receptors. Several thousand patients have received perampanel within randomized placebo-controlled trials, open-label extension trials and post-marketing observational studies. Significant median partial-onset seizure reduction rates of 23% for 4 mg/day, 26-31% for 8 mg/day and 18-35% for 12 mg/day were reported. Likewise 50 percent responder rates were 29% for 4 mg/day, 33-38% for 8 mg/day and 34-36% for 12 mg/day. Primary generalized tonic-clonic seizures were reduced by 76.5% (8 mg) vs 38.4% (placebo) in a recent controlled trial. Overall, perampanel is well tolerated and the main adverse events are dizziness, somnolence and fatigue. There are also anecdotal reports on use in progressive myoclonic epilepsies and status epilepticus. Perampanel will likely remain an important, possibly broad-spectrum AED with a significant market share, especially in patients with drug-refractory epilepsies.

show abstract

Section: Use In Progressive Myoclonus Epilepsiesmentioning

confidence: 99%

Perampanel in the treatment of focal and idiopathic generalized epilepsies and of status epilepticus

Strzelczyk

Willems

Willig

et al. 2015

Expert Review of Clinical Pharmacology

View full text Add to dashboard Cite

show abstract

“…It has been approved by FDA and EMA as an add-on treatment of partial seizures and of primary GTCS (in the setting of idiopathic generalized epilepsies) in patients older than 12 years. Two single case reports [49,50] and 2 open-label studies [51, 52] suggest its potential use in PMEs. Schorlemmer et al [49] described a 21-year- old bedridden patient with LD on VPA LEV, ZNS, CNZ, PIR, and ketogenic diet, who experienced a drastic improvement of myoclonus and seizures with PER at 3-month-follow-up.…”

Section: Resultsmentioning

confidence: 99%

“…Schorlemmer et al [49] described a 21-year- old bedridden patient with LD on VPA LEV, ZNS, CNZ, PIR, and ketogenic diet, who experienced a drastic improvement of myoclonus and seizures with PER at 3-month-follow-up. Dirani et al [50] prescribed PER to a 15-year-old LD girl in whom previous treatment with VPA, LTG, TPM, LEV and CNZ were abruptly stopped a few days before. PER at 10 mg/day was started with improvement of myoclonus and seizures at 1-month-follow-up.…”

Section: Resultsmentioning

confidence: 99%

Update on Pharmacological Treatment of Progressive Myoclonus Epilepsies

Ferlazzo

Trenité²,

Haan

et al. 2018

CPD

View full text Add to dashboard Cite

Background Progressive myoclonus epilepsies (PMEs) are a group of rare inherited diseases featuring a combination of myoclonus, seizures and variable degree of cognitive impairment. Despite extensive investigations, a large number of PMEs remain undiagnosed. In this review, we focus on the current pharmacological approach to PMEs. Methods References were mainly identified through PubMed search until February 2017 and backtracking of references in pertinent studies. Results The majority of available data on the efficacy of antiepileptic medications in PMEs are primarily anecdotal or observational, based on individual responses in small series. Valproic acid is the drug of choice, except for PMEs due to mitochondrial diseases. Levetiracetam and clonazepam should be considered as the first add-on treatment. Zonisamide and perampanel represent promising alternatives. Phenobarbital and primidone should be reserved to patients with resistant disabling myoclonus or seizures. Lamotrigine should be used with caution due to its unpredictable effect on myoclonus. Avoidance of drugs known to aggravate myoclonus and seizures, such as carbamazepine and phenytoin, is paramount. Psychiatric (in particular depression) and other comorbidities need to be adequately managed. Although a 3- to 4-drug regimen is often necessary to control seizures and myoclonus, particular care should be paid to avoid excessive pharmacological load and neurotoxic side effects. Target therapy is possible only for a minority of PMEs. Conclusions Overall, the treatment of PMEs remains symptomatic (i.e. pharmacological treatment of seizures and myoclonus). Further dissection of the genetic background of the different PMEs might hopefully help in the future with individualised treatment options.

show abstract

“…Selektif, non-kompetetif AMPA tipi glutamat reseptör antagonisti olan perampanelin kullanıldığı sınırlı sayıdaki olguda, ilacın etkinliği sadece nöbet sıklığında azalma ile değil, nörokognitif ve serebellar disfonksiyondaki iyileşme şeklinde tanımlanmıştır. [20,21] Ancak bu verilerin doğrulana-bilmesi için daha fazla sayıda çalışma yapılması gereklidir.…”

Section: Discussionunclassified