| Title: |
Electroclinical Features and Long-term Seizure Outcome in Patients With Eyelid Myoclonia With Absences |
| Authors: |
Cerulli Irelli, Emanuele; Cocchi, Enrico; Ramantani, Georgia; Caraballo, Roberto H; Giuliano, Loretta; Yilmaz, Tulay; Morano, Alessandra; Panagiotakaki, Eleni; Operto, Francesca F; Gonzalez Giraldez, Beatriz; Silvennoinen, Katri; Casciato, Sara; Comajuan, Marion; Balestrini, Simona; Fortunato, Francesco; Coppola, Antonietta; Di Gennaro, Giancarlo; Labate, Angelo; Sofia, Vito; Kluger, Gerhard J; Kasteleijn-Nolst Trenité, Dorothée G A; Gambardella, Antonio; Baykan, Betul; Sisodiya, Sanjay M; Arzimanoglou, Alexis; Striano, Pasquale; Di Bonaventura, Carlo |
| Contributors: |
Cerulli Irelli, Emanuele; Cocchi, Enrico; Ramantani, Georgia; Caraballo, Roberto H; Giuliano, Loretta; Yilmaz, Tulay; Morano, Alessandra; Panagiotakaki, Eleni; Operto, Francesca F; Gonzalez Giraldez, Beatriz; Silvennoinen, Katri; Casciato, Sara; Comajuan, Marion; Balestrini, Simona; Fortunato, Francesco; Coppola, Antonietta; Di Gennaro, Giancarlo; Labate, Angelo; Sofia, Vito; Kluger, Gerhard J; Kasteleijn-Nolst Trenité, Dorothée G A; Gambardella, Antonio; Baykan, Betul; Sisodiya, Sanjay M; Arzimanoglou, Alexi; Striano, Pasquale; Di Bonaventura, Carlo |
| Publisher Information: |
LIPPINCOTT WILLIAMS & WILKINS; TWO COMMERCE SQ, 2001 MARKET ST, PHILADELPHIA, PA 19103 USA |
| Publication Year: |
2022 |
| Collection: |
Sapienza Università di Roma: CINECA IRIS |
| Subject Terms: |
Anticonvulsant; Child; Preschool; Electroencephalography; Eyelid; Human; Recurrence; Retrospective Studie; Seizure; Epilepsy; Myoclonu; Seizures; Febrile; Status Epilepticu; Substance Withdrawal Syndrome |
| Description: |
Background and ObjectivesEyelid myoclonia (EM) with absences (EMA) is a generalized epilepsy syndrome with a prognosis and clinical characteristics that are still partially undefined. We investigated electrodinical endophenotypes and long-term seizure outcome in a large cohort of patients with EMA.MethodsIn this multicenter retrospective study, patients with EMA with >= 5 years of follow-up were included. We investigated prognostic patterns and sustained terminal remission (STR), along with their prognostic factors. Moreover, a 2-step cluster analysis was used to investigate the presence of distinct EMA endophenotypes.ResultsWe included 172 patients with a median age at onset of 7 years (interquartile range [IQR] 5-10 years) and a median follow-up duration of 14 years (IQR 8.25-23.75 years). Sixty-six patients (38.4%) displayed a nonremission pattern, whereas remission and relapse patterns were encountered in 56 (32.6%) and 50 (29.1%) participants. Early epilepsy onset, history of febrile seizures (FS), and EM status epilepticus significantly predicted a nonremission pattern according to multinomial logistic regression analysis. STR was achieved by 68 (39.5%) patients with a mean latency of 14.05 years (SD +/- 12.47 years). Early epilepsy onset, psychiatric comorbid conditions, and a history of FS and generalized tonic-clone seizures were associated with a lower probability of achieving STR according to a Cox regression proportional hazards model. Antiseizure medication (ASM) withdrawal was attempted in 62 of 172 patients, and seizures recurred in 74.2%. Cluster analysis revealed 2 distinct clusters with 86 patients each. Cluster 2, which we defined as EMA-plus, was characterized by an earlier age at epilepsy onset, higher rate of intellectual disability, EM status epilepticus, generalized paroxysmal fast activity, self-induced seizures, FS, and poor ASM response, whereas cluster 1, the EMA-only cluster, was characterized by a higher rate of seizure remission and more favorable neuropsychiatric ... |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| Relation: |
info:eu-repo/semantics/altIdentifier/pmid/35292555; info:eu-repo/semantics/altIdentifier/wos/WOS:000840305700008; volume:98; issue:18; journal:NEUROLOGY; https://hdl.handle.net/11573/1659039 |
| DOI: |
10.1212/WNL.0000000000200165 |
| Availability: |
https://hdl.handle.net/11573/1659039; https://doi.org/10.1212/WNL.0000000000200165 |
| Rights: |
info:eu-repo/semantics/openAccess |
| Accession Number: |
edsbas.F3CC0BA0 |
| Database: |
BASE |