Objective: To investigate the electroclinical characteristics and the prognostic impact of generalized fast discharges in a large cohort of genetic generalized epilepsy (GGE) patients studied with 24-h prolonged ambulatory electroencephalography (paEEG). Methods: This retrospective multicenter cohort study included 202 GGE patients. The occurrence of generalized paroxysmal fast activity (GPFA) and generalized polyspike train (GPT) was reviewed. GGE patients were classified as having idiopathic generalized epilepsy (IGE) or another GGE syndrome (namely perioral myoclonia with absences, eyelid myoclonia with absences, epilepsy with myoclonic absences, generalized epilepsy with febrile seizures plus, or GGE without a specific epilepsy syndrome) according to recent classification proposals. Results: GPFA/GPT was found in overall 25 (12.4%) patients, though it was significantly less frequent in IGE compared with other GGE syndromes (9.3 vs. 25%, p = 0.007). GPFA/GPT was found independently of seizure type experienced during history, the presence of mild intellectual disability/borderline intellectual functioning, or EEG features. At multivariable analysis, GPFA/GPT was significantly associated with drug resistance (p = 0.04) and with a higher number of antiseizure medications (ASMs) at the time of paEEG (p < 0.001) and at the last medical observation (p < 0.001). Similarly, GPFA/GPT, frequent/abundant generalized spike-wave discharges during sleep, and a higher number of seizure types during history were the only factors independently associated with a lower chance of achieving 2-year seizure remission at the last medical observation. Additionally, a greater number of GPFA/GPT discharges significantly discriminated between patients who achieved 2-year seizure remission at the last medical observation and those who did not (area under the curve = 0.77, 95% confidence interval 0.57-0.97, p = 0.02). Conclusion: We found that generalized fast discharges were more common than expected in GGE patients when considering the entire GGE spectrum. In addition, our study highlighted that GPFA/GPT could be found along the entire GGE continuum, though their occurrence was more common in less benign GGE syndromes. Finally, we confirmed that GPFA/GPT was associated with difficult-to-treat GGE, as evidenced by the multivariable analysis and the higher ASM load during history. Keywords: 24-hour EEG; antiseizure medication (ASM); classification; generalized paroxysmal fast activity (GPFA); generalized polyspike train (GPT); idiopathic generalized epilepsy (IGE); prognosis; prolonged ambulatory EEG (paEEG).

Generalized Fast Discharges Along the Genetic Generalized Epilepsy Spectrum: Clinical and Prognostic Significance / CERULLI IRELLI, Emanuele; Barone, FRANCESCA ANTONIETTA; Mari, Luisa; Morano, Alessandra; Orlando, Biagio; Salamone, ENRICO MICHELE; Marchi, Angela; Fanella, Martina; Fattouch, Jinane; Placidi, Fabio; Giallonardo, Anna Teresa; Izzi, Francesca; DI BONAVENTURA, Carlo. - In: FRONTIERS IN NEUROLOGY. - ISSN 1664-2295. - (2022).

Generalized Fast Discharges Along the Genetic Generalized Epilepsy Spectrum: Clinical and Prognostic Significance

Emanuele Cerulli Irelli;Francesca Antonietta Barone;Alessandra Morano;Biagio Orlando;Enrico Michele Salamone;Angela Marchi;Jinane Fattouch;Anna Teresa Giallonardo;Carlo Di Bonaventura
2022

Abstract

Objective: To investigate the electroclinical characteristics and the prognostic impact of generalized fast discharges in a large cohort of genetic generalized epilepsy (GGE) patients studied with 24-h prolonged ambulatory electroencephalography (paEEG). Methods: This retrospective multicenter cohort study included 202 GGE patients. The occurrence of generalized paroxysmal fast activity (GPFA) and generalized polyspike train (GPT) was reviewed. GGE patients were classified as having idiopathic generalized epilepsy (IGE) or another GGE syndrome (namely perioral myoclonia with absences, eyelid myoclonia with absences, epilepsy with myoclonic absences, generalized epilepsy with febrile seizures plus, or GGE without a specific epilepsy syndrome) according to recent classification proposals. Results: GPFA/GPT was found in overall 25 (12.4%) patients, though it was significantly less frequent in IGE compared with other GGE syndromes (9.3 vs. 25%, p = 0.007). GPFA/GPT was found independently of seizure type experienced during history, the presence of mild intellectual disability/borderline intellectual functioning, or EEG features. At multivariable analysis, GPFA/GPT was significantly associated with drug resistance (p = 0.04) and with a higher number of antiseizure medications (ASMs) at the time of paEEG (p < 0.001) and at the last medical observation (p < 0.001). Similarly, GPFA/GPT, frequent/abundant generalized spike-wave discharges during sleep, and a higher number of seizure types during history were the only factors independently associated with a lower chance of achieving 2-year seizure remission at the last medical observation. Additionally, a greater number of GPFA/GPT discharges significantly discriminated between patients who achieved 2-year seizure remission at the last medical observation and those who did not (area under the curve = 0.77, 95% confidence interval 0.57-0.97, p = 0.02). Conclusion: We found that generalized fast discharges were more common than expected in GGE patients when considering the entire GGE spectrum. In addition, our study highlighted that GPFA/GPT could be found along the entire GGE continuum, though their occurrence was more common in less benign GGE syndromes. Finally, we confirmed that GPFA/GPT was associated with difficult-to-treat GGE, as evidenced by the multivariable analysis and the higher ASM load during history. Keywords: 24-hour EEG; antiseizure medication (ASM); classification; generalized paroxysmal fast activity (GPFA); generalized polyspike train (GPT); idiopathic generalized epilepsy (IGE); prognosis; prolonged ambulatory EEG (paEEG).
2022
generalized paroxysmal fast activity (GPFA); generalized polyspike train (GPT); idiopathic generalized epilepsy (IGE); prognosis; prolonged ambulatory EEG (paEEG); antiseizure medication (ASM); classification; 24-hour EEG
01 Pubblicazione su rivista::01a Articolo in rivista
Generalized Fast Discharges Along the Genetic Generalized Epilepsy Spectrum: Clinical and Prognostic Significance / CERULLI IRELLI, Emanuele; Barone, FRANCESCA ANTONIETTA; Mari, Luisa; Morano, Alessandra; Orlando, Biagio; Salamone, ENRICO MICHELE; Marchi, Angela; Fanella, Martina; Fattouch, Jinane; Placidi, Fabio; Giallonardo, Anna Teresa; Izzi, Francesca; DI BONAVENTURA, Carlo. - In: FRONTIERS IN NEUROLOGY. - ISSN 1664-2295. - (2022).
File allegati a questo prodotto
File Dimensione Formato  
Cerulli Irelli_Genralized Fast Discharges_2022.pdf

accesso aperto

Tipologia: Versione editoriale (versione pubblicata con il layout dell'editore)
Licenza: Creative commons
Dimensione 1.46 MB
Formato Adobe PDF
1.46 MB Adobe PDF

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1643898
Citazioni
  • ???jsp.display-item.citation.pmc??? 3
  • Scopus 8
  • ???jsp.display-item.citation.isi??? 8
social impact