An Italian multicentre study of perampanel in progressive myoclonus epilepsies

Laura Canafoglia, Giuseppina Barbella, Edoardo Ferlazzo, Pasquale Striano, Adriana Magaudda, Giuseppe d'Orsi, Tommaso Martino, Carlo Avolio, Umberto Aguglia, Chiara Sueri, Loretta Giuliano, Vito Sofia, Federica Zibordi, Francesca Ragona, Elena Freri, Cinzia Costa, Elena Nardi Cesarini, Martina Fanella, Davide Rossi Sebastiano, Patrizia RiguzziAntonio Gambardella, Carlo Di Bonaventura, Roberto Michelucci, Tiziana Granata, Francesca Bisulli, Laura Licchetta, Paolo Tinuper, Francesca Beccaria, Elisa Visani, Silvana Franceschetti

Research output: Contribution to journalArticle

Abstract

Perampanel (PER) is a novel anti-seizure medication useful in different types of epilepsy. We intended to assess the effectiveness of PER on cortical myoclonus and seizure frequency in patients with progressive myoclonus epilepsy (PME), using quantitative validated scales. Forty-nine patients aged 36.6 ± 15.6 years with PME of various aetiology (18 EPM1, 12 EPM2, five with sialidosis, one with Kufs disease, one with EPM7, and 12 undetermined) were enrolled between January 2017 and June 2018. PER at the dose of 2–12 mg (5.3 ± 2.5) was added to existing therapy. Myoclonus severity was assessed using a minimal myoclonus scale (MMS) in all the patients before and after 4–6 months of steady PER dose, and by means of the Unified Myoclonus Rating Scale (UMRS) in 20 patients. Logistic regression analysis was used to identify the factors potentially predicting treatment efficacy. Four patients dropped out in the first two months due to psychiatric side effects. In the remaining patients, PER reduced myoclonus severity as assessed using MMS (Wilcoxon test: p < 0.001) and UMRS (p < 0.001), with the ‘Action myoclonus’ section of the UMRS showing the greatest improvement. The patients with EPM1 or EPM1-like phenotype were more likely to improve with PER (p = 0.011). Convulsive seizures which have recurred at least monthly in 17 patients were reduced by >50%. Side effects occurred in 22/49 (44.8%) patients, the most common being irritability followed by drowsiness. PER is effective in treating myoclonus and seizures in PME patients. The frequency of psychiatric side effects suggests the need for careful patient monitoring.

Original languageEnglish
Article number106191
JournalEpilepsy Research
Volume156
DOIs
Publication statusPublished - Oct 1 2019

Fingerprint

Progressive Myoclonic Epilepsy
Myoclonus
Multicenter Studies
Seizures
Psychiatry
Mucolipidoses
Neuronal Ceroid-Lipofuscinoses
perampanel
Sleep Stages
Physiologic Monitoring
Epilepsy
Logistic Models
Regression Analysis

Keywords

  • EPM1
  • EPM2
  • Irritability
  • Myoclonus scale
  • Perampanel
  • Progressive myoclonus epilepsy

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Canafoglia, L., Barbella, G., Ferlazzo, E., Striano, P., Magaudda, A., d'Orsi, G., ... Franceschetti, S. (2019). An Italian multicentre study of perampanel in progressive myoclonus epilepsies. Epilepsy Research, 156, [106191]. https://doi.org/10.1016/j.eplepsyres.2019.106191

An Italian multicentre study of perampanel in progressive myoclonus epilepsies. / Canafoglia, Laura; Barbella, Giuseppina; Ferlazzo, Edoardo; Striano, Pasquale; Magaudda, Adriana; d'Orsi, Giuseppe; Martino, Tommaso; Avolio, Carlo; Aguglia, Umberto; Sueri, Chiara; Giuliano, Loretta; Sofia, Vito; Zibordi, Federica; Ragona, Francesca; Freri, Elena; Costa, Cinzia; Nardi Cesarini, Elena; Fanella, Martina; Rossi Sebastiano, Davide; Riguzzi, Patrizia; Gambardella, Antonio; Di Bonaventura, Carlo; Michelucci, Roberto; Granata, Tiziana; Bisulli, Francesca; Licchetta, Laura; Tinuper, Paolo; Beccaria, Francesca; Visani, Elisa; Franceschetti, Silvana.

In: Epilepsy Research, Vol. 156, 106191, 01.10.2019.

Research output: Contribution to journalArticle

Canafoglia, L, Barbella, G, Ferlazzo, E, Striano, P, Magaudda, A, d'Orsi, G, Martino, T, Avolio, C, Aguglia, U, Sueri, C, Giuliano, L, Sofia, V, Zibordi, F, Ragona, F, Freri, E, Costa, C, Nardi Cesarini, E, Fanella, M, Rossi Sebastiano, D, Riguzzi, P, Gambardella, A, Di Bonaventura, C, Michelucci, R, Granata, T, Bisulli, F, Licchetta, L, Tinuper, P, Beccaria, F, Visani, E & Franceschetti, S 2019, 'An Italian multicentre study of perampanel in progressive myoclonus epilepsies', Epilepsy Research, vol. 156, 106191. https://doi.org/10.1016/j.eplepsyres.2019.106191
Canafoglia L, Barbella G, Ferlazzo E, Striano P, Magaudda A, d'Orsi G et al. An Italian multicentre study of perampanel in progressive myoclonus epilepsies. Epilepsy Research. 2019 Oct 1;156. 106191. https://doi.org/10.1016/j.eplepsyres.2019.106191
Canafoglia, Laura ; Barbella, Giuseppina ; Ferlazzo, Edoardo ; Striano, Pasquale ; Magaudda, Adriana ; d'Orsi, Giuseppe ; Martino, Tommaso ; Avolio, Carlo ; Aguglia, Umberto ; Sueri, Chiara ; Giuliano, Loretta ; Sofia, Vito ; Zibordi, Federica ; Ragona, Francesca ; Freri, Elena ; Costa, Cinzia ; Nardi Cesarini, Elena ; Fanella, Martina ; Rossi Sebastiano, Davide ; Riguzzi, Patrizia ; Gambardella, Antonio ; Di Bonaventura, Carlo ; Michelucci, Roberto ; Granata, Tiziana ; Bisulli, Francesca ; Licchetta, Laura ; Tinuper, Paolo ; Beccaria, Francesca ; Visani, Elisa ; Franceschetti, Silvana. / An Italian multicentre study of perampanel in progressive myoclonus epilepsies. In: Epilepsy Research. 2019 ; Vol. 156.
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AU - Barbella, Giuseppina

AU - Ferlazzo, Edoardo

AU - Striano, Pasquale

AU - Magaudda, Adriana

AU - d'Orsi, Giuseppe

AU - Martino, Tommaso

AU - Avolio, Carlo

AU - Aguglia, Umberto

AU - Sueri, Chiara

AU - Giuliano, Loretta

AU - Sofia, Vito

AU - Zibordi, Federica

AU - Ragona, Francesca

AU - Freri, Elena

AU - Costa, Cinzia

AU - Nardi Cesarini, Elena

AU - Fanella, Martina

AU - Rossi Sebastiano, Davide

AU - Riguzzi, Patrizia

AU - Gambardella, Antonio

AU - Di Bonaventura, Carlo

AU - Michelucci, Roberto

AU - Granata, Tiziana

AU - Bisulli, Francesca

AU - Licchetta, Laura

AU - Tinuper, Paolo

AU - Beccaria, Francesca

AU - Visani, Elisa

AU - Franceschetti, Silvana

N1 - Ricercatori distaccati presso IRCCS a seguito Convenzione esclusiva con Università di Bologna (Bisulli Francesca, Licchetta Laura, Tinuper Paolo). Richiesto CORRIGENDUM per modifica di affiliazione.

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KW - EPM2

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