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 language | English |
|---|---|
| Article number | 106191 |
| Journal | Epilepsy Research |
| Volume | 156 |
| DOIs | |
| Publication status | Published - Oct 1 2019 |
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Keywords
- EPM1
- EPM2
- Irritability
- Myoclonus scale
- Perampanel
- Progressive myoclonus epilepsy
ASJC Scopus subject areas
- Neurology
- Clinical Neurology
Cite this
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 journal › Article
}
TY - JOUR
T1 - An Italian multicentre study of perampanel in progressive myoclonus epilepsies
AU - Canafoglia, Laura
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.
PY - 2019/10/1
Y1 - 2019/10/1
N2 - 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.
AB - 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.
KW - EPM1
KW - EPM2
KW - Irritability
KW - Myoclonus scale
KW - Perampanel
KW - Progressive myoclonus epilepsy
UR - http://www.scopus.com/inward/record.url?scp=85070899276&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85070899276&partnerID=8YFLogxK
U2 - 10.1016/j.eplepsyres.2019.106191
DO - 10.1016/j.eplepsyres.2019.106191
M3 - Article
C2 - 31446282
AN - SCOPUS:85070899276
VL - 156
JO - Epilepsy Research
JF - Epilepsy Research
SN - 0920-1211
M1 - 106191
ER -