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1. Myoclonus: Differential diagnosis and current management

Author

Antonella Riva, Gianluca D'Onofrio, Edoardo Ferlazzo, Angelo Pascarella, Elena Pasini, Silvana Franceschetti, Ferruccio Panzica, Laura Canafoglia, Aglaia Vignoli, Antonietta Coppola, Valeria Badioni, Francesca Beccaria, Angelo Labate, Antonio Gambardella, Antonino Romeo, Giuseppe Capovilla, Roberto Michelucci, Pasquale Striano, and Vincenzo Belcastro

Subjects
electroclinical features, epilepsy, myoclonus, neurophysiology, nosology, Neurology. Diseases of the nervous system, RC346-429
Abstract

Abstract Myoclonus classically presents as a brief (10–50 ms duration), non‐rhythmic jerk movement. The etiology could vary considerably ranging from self‐limited to chronic or even progressive disorders, the latter falling into encephalopathic pictures that need a prompt diagnosis. Beyond the etiological classification, others evaluate myoclonus' body distribution (i.e., clinical classification) or the location of the generator (i.e., neurophysiological classification); particularly, knowing the anatomical source of myoclonus gives inputs on the observable clinical patterns, such as EMG bursts duration or EEG correlate, and guides the therapeutic choices. Among all the chronic disorders, myoclonus often presents itself as a manifestation of epilepsy. In this context, myoclonus has many facets. Myoclonus occurs as one, or the only, seizure manifestation while it can also present as a peculiar type of movement disorder; moreover, its electroclinical features within specific genetically determined epileptic syndromes have seldom been investigated. In this review, following a meeting of recognized experts, we provide an up‐to‐date overview of the neurophysiology and nosology surrounding myoclonus. Through the dedicated exploration of epileptic syndromes, coupled with pragmatic guidance, we aim to furnish clinicians and researchers alike with practical advice for heightened diagnostic management and refined treatment strategies. Plain Language Summary In this work, we described myoclonus, a movement characterized by brief, shock‐like jerks. Myoclonus could be present in different diseases and its correct diagnosis helps treatment.

Published
2024
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2. Adjunctive Brivaracetam in Older Patients with Focal Seizures: Evidence from the BRIVAracetam add‑on First Italian netwoRk Study (BRIVAFIRST)

Author

Simona Lattanzi, 1, Michele Ascoli, 2, Laura Canafoglia, 3, 4 5, Maria Paola Canevini, Sara Casciato, 6, Emanuele Cerulli Irelli, 7, Valentina Chiesa, 4, Filippo Dainese, 8, Giovanni De Maria, 9, Giuseppe Didato 10, Giancarlo Di Gennaro, 6, Giovanni Falcicchio 11, Martina Fanella, 7, Massimo Gangitano 12, Angela La Neve 11, Oriano Mecarelli, 7, Elisa Montalenti 13, Alessandra Morano, 7, Federico Piazza 14, Chiara Pizzanelli 15, Patrizia Pulitano, 7, Federica Ranzato 16, Eleonora Rosati 17, Laura Tassi 18, Carlo Di Bonaventura, 7, BRIVAFIRST Group Collaborators, Affiliations collapse Collaborators BRIVAFIRST Group: Angela Alicino, Giovanni, Assenza, Federica, Avorio, Valeria, Badioni, Paola, Banfi, Emanuele, Bartolini, Luca Manfredi Basili, Vincenzo, Belcastro, Simone, Beretta, Irene, Berto, Martina, Biggi, Giuseppe, Billo, Giovanni, Boero, Paolo, Bonanni, Jole, Bongiorno, Francesco, Brigo, Emanuele, Caggia, Claudia, Cagnetti, Carmen, Calvello, Edward, Cesnik, Gigliola, Chianale, Domenico, Ciampanelli, Roberta, Ciuffini, Dario, Cocito, Donato, Colella, Margerita, Contento, Cinzia, Costa, Eduardo, Cumbo, Alfredo, D'Aniello, Francesco, Deleo, Jacopo, C DiFrancesco, Roberta Di Giacomo, Alessandra Di Liberto, Elisabetta, Domina, Francesco, Donato, Fedele, Dono, Vania, Durante, Maurizio, Elia, Anna, Estraneo, Giacomo, Evangelista, Maria Teresa Faedda, Ylenia, Failli, Elisa, Fallica, Jinane, Fattouch, Alessandra, Ferrari, Ferreri, Florinda, Giacomo, Fisco, Davide, Fonti, Francesco, Fortunato, Nicoletta, Foschi, Teresa, Francavilla, Rosita, Galli, Sara, Gasparini, Stefano, Gazzina, Anna Teresa Giallonardo, Filippo Sean Giorgi, Loretta, Giuliano, Francesco, Habetswallner, Francesca, Izzi, Kassabian, Benedetta, Lorenzo, Kiferle, Angelo, Labate, Concetta, Luisi, Matteo, Magliani, Giulia, Maira, Luisa, Mari, Daniela, Marino, Addolorata, Mascia, Alessandra, Mazzeo, Stefano, Meletti, Chiara, Milano, Annacarmen, Nilo, Biagio, Orlando, Francesco, Paladin, Maria Grazia Pascarella, Chiara, Pastori, Giada, Pauletto, Alessia, Peretti, Gabriella, Perri, Marianna, Pezzella, Marta, Piccioli, Pietro, Pignatta, Nicola, Pilolli, Francesco, Pisani, Laura Rosa Pisani, Fabio, Placidi, Patrizia, Pollicino, Vittoria, Porcella, Monica, Puligheddu, Stefano, Quadri, Pier Paolo Quarato, Rui, Quintas, Rosaria, Renna, Adriana, Rum, Enrico Michele Salamone, Ersilia, Savastano, Maria, Sessa, David, Stokelj, Elena, Tartara, Mario, Tombini, Gemma, Tumminelli, Anna Elisabetta Vaudano, Ilaria, Viganò, Emanuela, Viglietta, Aglaia, Vignoli, Flavio, Villani, Elena, Zambrelli, Lelia, Zummo, Lattanzi S., Canafoglia L., Canevini M.P., Casciato S., Cerulli Irelli E., Chiesa V., Dainese F., De Maria G., Didato G., Falcicchio G., Fanella M., Ferlazzo E., Gangitano M., Giorgi F.S., La Neve A., Mecarelli O., Montalenti E., Morano A., Piazza F., Pulitano P., Quarato P.P., Ranzato F., Rosati E., Tassi L., and Di Bonaventura C.

Subjects
Antiseizure medication, Epilepsy, Settore MED/26, Pyrrolidinones, Focal seizures, Treatment Outcome, Drug Therapy, Double-Blind Method, Italy, Seizures, Brivaracetam, Combination, Aged, Drug Therapy, Combination, Humans, Retrospective Studies, Anticonvulsants, Pharmacology (medical), Settore MED/26 - Neurologia, Geriatrics and Gerontology
Abstract

BACKGROUND: The management of epilepsy in older adults has become part of daily practice because of an aging population. Older patients with epilepsy represent a distinct and more vulnerable clinical group as compared with younger patients, and they are generally under-represented in randomized placebo-controlled trials. Real-world studies can therefore be a useful complement to characterize the drug's profile. Brivaracetam is a rationally developed compound characterized by high-affinity binding to synaptic vesicle protein 2A and approved as adjunctive therapy for focal seizures in adults with epilepsy. OBJECTIVE: The aim of this study was to assess the 12-month effectiveness and tolerability of adjunctive brivaracetam in older patients (≥65 years of age) with epilepsy treated in a real-world setting. METHODS: The BRIVAFIRST (BRIVAracetam add-on First Italian netwoRk STudy) was a 12-month retrospective multicenter study including adult patients prescribed adjunctive brivaracetam. Effectiveness outcomes included the rates of seizure response (≥50% reduction in baseline seizure frequency), seizure freedom, and treatment discontinuation. Safety and tolerability outcomes included the rate of treatment discontinuation due to adverse events and the incidence of adverse events. Data were compared for patients aged ≥65 years of age ('older') vs those aged

Published
2022

3. Brivaracetam as add-on treatment in patients with post-stroke epilepsy: real-world data from the BRIVAracetam add-on First Italian netwoRk Study (BRIVAFIRST)

Author

Simona Lattanzi, Laura Canafoglia, Maria Paola Canevini, Sara Casciato, Emanuele Cerulli Irelli, Valentina Chiesa, Filippo Dainese, Giovanni De Maria, Giuseppe Didato, Giancarlo Di Gennaro, Giovanni Falcicchio, Martina Fanella, Edoardo Ferlazzo, Massimo Gangitano, Angela La Neve, Oriano Mecarelli, Elisa Montalenti, Alessandra Morano, Federico Piazza, Chiara Pizzanelli, Patrizia Pulitano, Federica Ranzato, Eleonora Rosati, Laura Tassi, Carlo Di Bonaventura, Angela Alicino, Michele Ascoli, Giovanni Assenza, Federica Avorio, Valeria Badioni, Paola Banfi, Emanuele Bartolini, Luca Manfredi Basili, Vincenzo Belcastro, Simone Beretta, Irene Berto, Martina Biggi, Giuseppe Billo, Giovanni Boero, Paolo Bonanni, Jole Bongorno, Francesco Brigo, Emanuele Caggia, Claudia Cagnetti, Carmen Calvello, Edward Cesnik, Gigliola Chianale, Domenico Ciampanelli, Roberta Ciuffini, Dario Cocito, Donato Colella, Margerita Contento, Cinzia Costa, Eduardo Cumbo, Alfredo D'Aniello, Francesco Deleo, Jacopo C DiFrancesco, Roberta Di Giacomo, Alessandra Di Liberto, Elisabetta Domina, Fedele Dono, Vania Durante, Maurizio Elia, Anna Estraneo, Giacomo Evangelista, Maria Teresa Faedda, Ylenia Failli, Elisa Fallica, Jinane Fattouch, Alessandra Ferrari, Florinda Ferreri, Giacomo Fisco, Davide Fonti, Francesco Fortunato, Nicoletta Foschi, Teresa Francavilla, Rosita Galli, Stefano Gazzina, Anna Teresa Giallonardo, Filippo Sean Giorgi, Loretta Giuliano, Francesco Habetswallner, Francesca Izzi, Benedetta Kassabian, Angelo Labate, Concetta Luisi, Matteo Magliani, Giulia Maira, Luisa Mari, Daniela Marino, Addolorata Mascia, Alessandra Mazzeo, Chiara Milano, Stefano Meletti, Annacarmen Nilo, Biagio Orlando, Francesco Paladin, Maria Grazia Pascarella, Chiara Pastori, Giada Pauletto, Alessia Peretti, Gabriella Perri, Marianna Pezzella, Marta Piccioli, Pietro Pignatta, Nicola Pilolli, Francesco Pisani, Laura Rosa Pisani, Fabio Placidi, Patrizia Pollicino, Vittoria Porcella, Silvia Pradella, Monica Puligheddu, Stefano Quadri, Pier Paolo Quarato, Rui Quintas, Rosaria Renna, Giada Ricciardo Rizzo, Adriana Rum, Enrico Michele Salamone, Ersilia Savastano, Maria Sessa, David Stokelj, Elena Tartara, Mario Tombini, Gemma Tumminelli, Anna Elisabetta Vaudano, Maria Ventura, Ilaria Viganò, Emanuela Viglietta, Aglaia Vignoli, Flavio Villani, Elena Zambrelli, Lelia Zummo, Lattanzi S., Canafoglia L., Canevini M.P., Casciato S., Cerulli Irelli E., Chiesa V., Dainese F., De Maria G., Didato G., Di Gennaro G., Falcicchio G., Fanella M., Ferlazzo E., Gangitano M., La Neve A., Mecarelli O., Montalenti E., Morano A., Piazza F., Pizzanelli C., Pulitano P., Ranzato F., Rosati E., Tassi L., Di Bonaventura C., Alicino A., Ascoli M., Assenza G., Avorio F., Badioni V., Banfi P., Bartolini E., Basili L.M., Belcastro V., Beretta S., Berto I., Biggi M., Billo G., Boero G., Bonanni P., Bongiorno J., Brigo F., Caggia E., Cagnetti C., Calvello C., Cesnik E., Chianale G., Ciampanelli D., Ciuffini R., Cocito D., Colella D., Contento M., Costa C., Cumbo E., D'Aniello A., Deleo F., DiFrancesco J.C., Di Giacomo R., Di Liberto A., Domina E., Dono F., Durante V., Elia M., Estraneo A., Evangelista G., Faedda M.T., Failli Y., Fallica E., Fattouch J., Ferrari A., Ferreri F., Fisco G., Fonti D., Fortunato F., Foschi N., Francavilla T., Galli R., Gazzina S., Giallonardo A.T., Giorgi F.S., Giuliano L., Habetswallner F., Izzi F., Kassabian B., Labate A., Luisi C., Magliani M., Maira G., Mari L., Marino D., Mascia A., Mazzeo A., Milano C., Meletti S., Nilo A., Orlando B., Paladin F., Pascarella M.G., Pastori C., Pauletto G., Peretti A., Perri G., Pezzella M., Piccioli M., Pignatta P., Pilolli N., Pisani F., Pisani L.R., Placidi F., Pollicino P., Porcella V., Pradella S., Puligheddu M., Quadri S., Quarato P.P., Quintas R., Renna R., Rizzo G.R., Rum A., Salamone E.M., Savastano E., Sessa M., Stokelj D., Tartara E., Tombini M., Tumminelli G., Vaudano A.E., Ventura M., Vigano I., Viglietta E., Vignoli A., Villani F., Zambrelli E., and Zummo L.

Subjects
Adult, Antiseizure medication, Brivaracetam, Cerebrovascular diseases, Focal seizures, Stroke, Settore MED/26, Antiseizure medication, Brivaracetam, Focal seizures, Stroke, Cerebrovascular diseases, Double-Blind Method, Drug Therapy, Seizures, Humans, Aged, Retrospective Studies, Epilepsy, General Medicine, Middle Aged, Pyrrolidinones, Treatment Outcome, Neurology, Italy, Combination, Anticonvulsants, Drug Therapy, Combination, Neurology (clinical)
Abstract

Objective: Post-stroke epilepsy (PSE) is one of the most common causes of acquired epilepsy and accounts for about 10-15% of all newly diagnosed epilepsy cases. However, evidence about the clinical profile of antiseizure medications in the PSE setting is currently limited. Brivaracetam (BRV) is a rationally developed compound characterized by high-affinity binding to synaptic vesicle protein 2A. The aim of this study was to assess the 12-month effectiveness and tolerability of adjunctive BRV in patients with PSE treated in a real-world setting. Methods: This was a subgroup analysis of patients with PSE included in the BRIVAracetam add-on First Italian netwoRk Study (BRIVAFIRST). The BRIVAFIRST was a 12-month retrospective, multicentre study including adult patients prescribed adjunctive BRV. Effectiveness outcomes included the rates of seizure response (≥50% reduction in baseline seizure frequency), seizure‐freedom, and treatment discontinuation. Safety and tolerability outcomes included the rate of treatment discontinuation due to adverse events (AEs) and the incidence of AEs. Results: Patients with PSE included in the BRIVAFIRST were 75 and had a median age of 57 (interquartile range, 42-66) years. The median daily doses of BRV at 3, 6, and 12 months from starting treatment were 100 (100-150) mg, 125 (100-200) mg and 100 (100-200) mg, respectively. At 12 months, 32 (42.7%) patients had a reduction in their baseline seizure frequency by at least 50%, and the seizure freedom rates was 26/75 (34.7%). During the 1-year study period, 10 (13.3%) patients discontinued BRV. The reasons of treatment withdrawal were insufficient efficacy in 6 (8.0%) patients and poor tolerability in 4 (5.3%) patients. Adverse events were reported by 13 (20.3%) patients and were rated as mild in 84.6% and moderate in 15.4% of cases. Significance: Adjunctive BRV was efficacious and generally well-tolerated when used in patients with PSE in clinical practice. Adjunctive BRV can be a suitable therapeutic option for patients with PSE.

Published
2022

4. Sustained seizure freedom with adjunctive brivaracetam in patients with focal onset seizures

Author

Simona Lattanzi, 1, Michele Ascoli, 2, Laura Canafoglia, 3, 4 5, Maria Paola Canevini, Sara Casciato, 6, Emanuele Cerulli Irelli, 7, Valentina Chiesa, 4, Filippo Dainese, 8, Giovanni De Maria, 9, Giuseppe Didato 10, Giancarlo Di Gennaro, 6, Giovanni Falcicchio 11, Martina Fanella, 7, Massimo Gangitano 12, Angela La Neve 11, Oriano Mecarelli, 7, Elisa Montalenti 13, Alessandra Morano, 7, Federico Piazza 14, Chiara Pizzanelli 15, Patrizia Pulitano, 7, Federica Ranzato 16, Eleonora Rosati 17, Laura Tassi 18, Carlo Di Bonaventura, 7, BRIVAFIRST Group Collaborators, Affiliations collapse Collaborators BRIVAFIRST Group: Angela Alicino, Giovanni, Assenza, Federica, Avorio, Valeria, Badioni, Paola, Banfi, Emanuele, Bartolini, Luca Manfredi Basili, Vincenzo, Belcastro, Simone, Beretta, Irene, Berto, Martina, Biggi, Giuseppe, Billo, Giovanni, Boero, Paolo, Bonanni, Jole, Bongiorno, Francesco, Brigo, Emanuele, Caggia, Claudia, Cagnetti, Carmen, Calvello, Edward, Cesnik, Gigliola, Chianale, Domenico, Ciampanelli, Roberta, Ciuffini, Dario, Cocito, Donato, Colella, Margerita, Contento, Cinzia, Costa, Eduardo, Cumbo, Alfredo, D'Aniello, Francesco, Deleo, Jacopo, C DiFrancesco, Roberta Di Giacomo, Alessandra Di Liberto, Elisabetta, Domina, Francesco, Donato, Fedele, Dono, Vania, Durante, Maurizio, Elia, Anna, Estraneo, Giacomo, Evangelista, Maria Teresa Faedda, Ylenia, Failli, Elisa, Fallica, Jinane, Fattouch, Alessandra, Ferrari, Ferreri, Florinda, Giacomo, Fisco, Davide, Fonti, Francesco, Fortunato, Nicoletta, Foschi, Teresa, Francavilla, Rosita, Galli, Sara, Gasparini, Stefano, Gazzina, Anna Teresa Giallonardo, Filippo Sean Giorgi, Loretta, Giuliano, Francesco, Habetswallner, Francesca, Izzi, Kassabian, Benedetta, Lorenzo, Kiferle, Angelo, Labate, Concetta, Luisi, Matteo, Magliani, Giulia, Maira, Luisa, Mari, Daniela, Marino, Addolorata, Mascia, Alessandra, Mazzeo, Stefano, Meletti, Chiara, Milano, Annacarmen, Nilo, Biagio, Orlando, Francesco, Paladin, Maria Grazia Pascarella, Chiara, Pastori, Giada, Pauletto, Alessia, Peretti, Gabriella, Perri, Marianna, Pezzella, Marta, Piccioli, Pietro, Pignatta, Nicola, Pilolli, Francesco, Pisani, Laura Rosa Pisani, Fabio, Placidi, Patrizia, Pollicino, Vittoria, Porcella, Monica, Puligheddu, Stefano, Quadri, Pier Paolo Quarato, Rui, Quintas, Rosaria, Renna, Adriana, Rum, Enrico Michele Salamone, Ersilia, Savastano, Maria, Sessa, David, Stokelj, Elena, Tartara, Mario, Tombini, Gemma, Tumminelli, Anna Elisabetta Vaudano, Ilaria, Viganò, Emanuela, Viglietta, Aglaia, Vignoli, Flavio, Villani, Elena, Zambrelli, Lelia, Zummo, Lattanzi S., Ascoli M., Canafoglia L., Paola Canevini M., Casciato S., Cerulli Irelli E., Chiesa V., Dainese F., De Maria G., Didato G., Di Gennaro G., Falcicchio G., Fanella M., Gangitano M., La Neve A., Mecarelli O., Montalenti E., Morano A., Piazza F., Pizzanelli C., Pulitano P., Ranzato F., Rosati E., Tassi L., and Di Bonaventura C.

Subjects
Adult, Freedom, focal seizures, Epilepsies, Settore MED/26, Double-Blind Method, Drug Therapy, antiseizure medication, brivaracetam, seizure freedom, sodium channel blockers, Drug Therapy, Combination, Humans, Middle Aged, Pyrrolidinones, Seizures, Treatment Outcome, Anticonvulsants, Epilepsies, Partial, anti-seizure medication, epilepsy, Neurology, Combination, Neurology (clinical), Partial
Abstract

The maintenance of seizure control over time is a clinical priority in patients with epilepsy. The aim of this study was to assess the sustained seizure frequency reduction with adjunctive brivaracetam (BRV) in real-world practice. Patients with focal epilepsy prescribed add-on BRV were identified. Study outcomes included sustained seizure freedom and sustained seizure response, defined as a 100% and a ≥50% reduction in baseline seizure frequency that continued without interruption and without BRV withdrawal through the 12-month follow-up. Nine hundred ninety-four patients with a median age of 45 (interquartile range = 32-56) years were included. During the 1-year study period, sustained seizure freedom was achieved by 142 (14.3%) patients, of whom 72 (50.7%) were seizure-free from Day 1 of BRV treatment. Sustained seizure freedom was maintained for ≥6, ≥9, and 12months by 14.3%, 11.9%, and 7.2% of patients from the study cohort. Sustained seizure response was reached by 383 (38.5%) patients; 236 of 383 (61.6%) achieved sustained ≥50% reduction in seizure frequency by Day 1, 94 of 383 (24.5%) by Month 4, and 53 of 383 (13.8%) by Month 7 up to Month 12. Adjunctive BRV was associated with sustained seizure frequency reduction from the first day of treatment in a subset of patients with uncontrolled focal epilepsy.

Published
2022

5. Long-term analysis of the effects of COVID-19 in people with epilepsy: Results from a multicenter on-line survey across the pandemic waves

Author

Chiara Parodi, Ilaria Viganò, Emerenziana Ottaviano, Valentina Massa, Elisa Borghi, Simone Beretta, Jacopo C. Di Francesco, Valeria Badioni, and Aglaia Vignoli

Subjects
Epilepsy, SARS-CoV-2, Antiseizure medications, COVID-19, Settore MED/07 - Microbiologia e Microbiologia Clinica, Settore MED/39 - Neuropsichiatria Infantile, Behavioral Neuroscience, COVID-19 Testing, Post-Acute COVID-19 Syndrome, Neurology, Surveys and Questionnaires, Communicable Disease Control, Humans, Settore MED/26 - Neurologia, Neurology (clinical), Pandemics
Abstract

The worldwide pandemic caused by SARS-CoV-2 virus posed many challenges to the scientific and medical communities, including the protection and management of fragile populations. People with epilepsy (PWE) are a heterogenous group of subjects, with different treatment regimens and severity of symptoms. During the National lockdown, in Italy many patients with chronic conditions lost their regular follow-up program. The aim of this study was to investigate the impact of COVID-19 on their health status, from the start of the pandemic (March 2020) to July 2021 and one year later.We proposed an online questionnaire to subjects followed up at different epilepsy centers located in Milano, MonzaLodi, three of Lombardy, Northern Italy, the most affected areas by the pandemic. Survey evaluated age, sex, characteristics of patients, type of epilepsy and therapies, COVID-19 diagnosis, vaccines, sleep quality, and anxiety status.Among 178 analyzed surveys, 37 individuals reported symptoms of COVID-19 in closed contacts, including 9 with molecular diagnosis and 16 PWE performing the nasopharyngeal swab with 3 positive cases. One year later, 35 individuals reported at least one symptom overlapping with those typical of COVID-19, 8 received COVID-19 diagnosis, among which 6 were positive for SARS-CoV-2 infection. According to the sleep quality scale assessment, most PWE (52.3%) had poor sleep quality. Assessing anxiety status, 32 (38.1%) had a pathological score.In this multicenter study, we observed that PWE do not appear to be at a higher risk of severe COVID-19. It will be fundamental monitoring this group to assess possible differences in long-COVID-19 and/or neuro-COVID-19 prevalence. On the other hand, our survey confirmed the impact of the pandemic on anxiety and quality of sleep in PWE. Thus, it is important to promptly recognize and treat psychological distress in PWE, because it could be a risk factor in seizure aggravation and quality-of-life deterioration. Telemedicine appears to be a useful tool to support patients with chronic diseases, such as epilepsy.

Published
2022
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