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Sustained seizure remission on perampanel in progressive myoclonic epilepsy (Lafora disease).

Schorlemmer K, Bauer S, Belke M, Hermsen A, Klein KM, Reif PS, Oertel WH, Kunz WS, Knake S, Rosenow F, Strzelczyk A - Epilepsy Behav Case Rep (2013)

Bottom Line: After dosage reduction to 6 mg/day, seizures recurred; however, on increasing the daily dose to 10 mg, seizures stopped for another three months.There is evidence for its effectiveness in generalized epilepsies, and phase III studies for this indication are on the way.Considering its impressive efficacy in this case, we suggest a prospective, multicenter study evaluating perampanel in PME.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology and Epilepsy Center Hessen, Philipps-University, Marburg, Germany.

ABSTRACT

Aim: The aim of this report is to provide initial evidence that add-on treatment with perampanel might be highly effective in progressive myoclonic epilepsy such as Lafora disease.

Case report: We report on a 21-year-old woman suffering from persistent myoclonus and generalized tonic-clonic seizures for more than seven years. Additionally, ataxia, a disturbance in speech and gait, as well as a cognitive decline were rapidly progressing. Subsequently, the diagnosis of Lafora disease was confirmed by the identification of a novel homozygous missense mutation in exon 3 of the EPM2A gene (c.538C>G; p.L180V). Adjunctive therapy with perampanel was started in this patient with advanced Lafora disease and was titrated up to 8 mg/day. A sustained and reproducible remission of myoclonus and GTCS could be achieved for a follow-up of three months. After dosage reduction to 6 mg/day, seizures recurred; however, on increasing the daily dose to 10 mg, seizures stopped for another three months. The patient also regained her ability to walk with help and the aid of a walker.

Conclusions: Perampanel is a selective, noncompetitive antagonist of AMPA-type glutamate receptors and recently licensed as adjunctive therapy for the treatment of refractory focal onset seizures. There is evidence for its effectiveness in generalized epilepsies, and phase III studies for this indication are on the way. Our case illustrates the possibility that perampanel might be a valuable option for treatment in PME. Considering its impressive efficacy in this case, we suggest a prospective, multicenter study evaluating perampanel in PME.

No MeSH data available.


Related in: MedlinePlus

EEG with bifrontal polyspikes and generalized slowing on follow-up.
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f0015: EEG with bifrontal polyspikes and generalized slowing on follow-up.

Mentions: Subsequently, the patient was admitted in September 2012 because of seizure exacerbation caused by infection and fluoroquinolone antibiotic therapy. The EEG showed generalized epileptiform discharges and frequent myoclonus (Fig. 2). The patient was started on an adjunctive therapy with the newly licensed perampanel, which was quickly titrated up to 8 mg/day within one week. No side effects were observed. The other anticonvulsant agents, such as valproate (1350 mg/day), levetiracetam (4000 mg/day), zonisamide (600 mg/day), clonazepam (12 mg/day), piracetam (12,000 mg/day), and the ketogenic diet (4:1 ratio) were not changed at this point in time. The GTCS stopped, and the patient was discharged. On follow-up four months later, the parents reported sustained seizure remission without myoclonus and GTCS for more than 3 months on an unchanged therapeutic regimen (see Table 1). The EEG showed less epileptiform discharges (Fig. 3). The patient was able to walk with help and the aid of a walker and could climb a few stairs during physiotherapy. A few weeks later, the parents reported gait problems, and therefore, perampanel was decreased from 8 mg/day to 6 mg/day. Generalized tonic–clonic seizures recurred, but control was again achieved after increasing perampanel to 10 mg/day with a follow-up of another three months.


Sustained seizure remission on perampanel in progressive myoclonic epilepsy (Lafora disease).

Schorlemmer K, Bauer S, Belke M, Hermsen A, Klein KM, Reif PS, Oertel WH, Kunz WS, Knake S, Rosenow F, Strzelczyk A - Epilepsy Behav Case Rep (2013)

EEG with bifrontal polyspikes and generalized slowing on follow-up.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4150630&req=5

f0015: EEG with bifrontal polyspikes and generalized slowing on follow-up.
Mentions: Subsequently, the patient was admitted in September 2012 because of seizure exacerbation caused by infection and fluoroquinolone antibiotic therapy. The EEG showed generalized epileptiform discharges and frequent myoclonus (Fig. 2). The patient was started on an adjunctive therapy with the newly licensed perampanel, which was quickly titrated up to 8 mg/day within one week. No side effects were observed. The other anticonvulsant agents, such as valproate (1350 mg/day), levetiracetam (4000 mg/day), zonisamide (600 mg/day), clonazepam (12 mg/day), piracetam (12,000 mg/day), and the ketogenic diet (4:1 ratio) were not changed at this point in time. The GTCS stopped, and the patient was discharged. On follow-up four months later, the parents reported sustained seizure remission without myoclonus and GTCS for more than 3 months on an unchanged therapeutic regimen (see Table 1). The EEG showed less epileptiform discharges (Fig. 3). The patient was able to walk with help and the aid of a walker and could climb a few stairs during physiotherapy. A few weeks later, the parents reported gait problems, and therefore, perampanel was decreased from 8 mg/day to 6 mg/day. Generalized tonic–clonic seizures recurred, but control was again achieved after increasing perampanel to 10 mg/day with a follow-up of another three months.

Bottom Line: After dosage reduction to 6 mg/day, seizures recurred; however, on increasing the daily dose to 10 mg, seizures stopped for another three months.There is evidence for its effectiveness in generalized epilepsies, and phase III studies for this indication are on the way.Considering its impressive efficacy in this case, we suggest a prospective, multicenter study evaluating perampanel in PME.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology and Epilepsy Center Hessen, Philipps-University, Marburg, Germany.

ABSTRACT

Aim: The aim of this report is to provide initial evidence that add-on treatment with perampanel might be highly effective in progressive myoclonic epilepsy such as Lafora disease.

Case report: We report on a 21-year-old woman suffering from persistent myoclonus and generalized tonic-clonic seizures for more than seven years. Additionally, ataxia, a disturbance in speech and gait, as well as a cognitive decline were rapidly progressing. Subsequently, the diagnosis of Lafora disease was confirmed by the identification of a novel homozygous missense mutation in exon 3 of the EPM2A gene (c.538C>G; p.L180V). Adjunctive therapy with perampanel was started in this patient with advanced Lafora disease and was titrated up to 8 mg/day. A sustained and reproducible remission of myoclonus and GTCS could be achieved for a follow-up of three months. After dosage reduction to 6 mg/day, seizures recurred; however, on increasing the daily dose to 10 mg, seizures stopped for another three months. The patient also regained her ability to walk with help and the aid of a walker.

Conclusions: Perampanel is a selective, noncompetitive antagonist of AMPA-type glutamate receptors and recently licensed as adjunctive therapy for the treatment of refractory focal onset seizures. There is evidence for its effectiveness in generalized epilepsies, and phase III studies for this indication are on the way. Our case illustrates the possibility that perampanel might be a valuable option for treatment in PME. Considering its impressive efficacy in this case, we suggest a prospective, multicenter study evaluating perampanel in PME.

No MeSH data available.


Related in: MedlinePlus