1. An Italian multicentre study of perampanel in progressive myoclonus epilepsies
- Author
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Carlo Avolio, Francesca Ragona, Giuseppina Barbella, Elena Freri, Patrizia Riguzzi, Chiara Sueri, Edoardo Ferlazzo, Paolo Tinuper, Loretta Giuliano, Davide Rossi Sebastiano, Cinzia Costa, Carlo Di Bonaventura, Elena Nardi Cesarini, Tommaso Martino, Silvana Franceschetti, Francesca Bisulli, Adriana Magaudda, Giuseppe d'Orsi, Vito Sofia, Federica Zibordi, Laura Licchetta, Laura Canafoglia, Francesca Beccaria, Martina Fanella, Antonio Gambardella, Tiziana Granata, Pasquale Striano, Umberto Aguglia, Roberto Michelucci, Elisa Visani, 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., Cesarini E.N., Fanella M., Sebastiano D.R., Riguzzi P., Gambardella A., Bonaventura C.D., Michelucci R., Granata T., Bisulli F., Licchetta L., Tinuper P., Beccaria F., Visani E., and Franceschetti S.
- Subjects
Adult ,Male ,Myoclonus ,0301 basic medicine ,Pediatrics ,medicine.medical_specialty ,Pyridones ,Progressive myoclonus epilepsy ,EPM1 ,EPM2 ,Irritability ,Perampanel ,Young Adult ,03 medical and health sciences ,chemistry.chemical_compound ,Epilepsy ,0302 clinical medicine ,Seizures ,Rating scale ,Nitriles ,medicine ,Humans ,Kufs disease ,Myoclonus scale ,Perampanel, Progressive myoclonus epilepsy, EPM1, EPM2, Irritability, Myoclonus scale ,Aged ,business.industry ,Middle Aged ,Myoclonic Epilepsies, Progressive ,medicine.disease ,Treatment Outcome ,030104 developmental biology ,Neurology ,chemistry ,Etiology ,Anticonvulsants ,Female ,Neurology (clinical) ,medicine.symptom ,business ,030217 neurology & neurosurgery - 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.
- Published
- 2019