Sudha Kilaru Kessler1, Shlomo Shinnar2, Avital Cnaan2, Dennis Dlugos2, Joan Conry2, Deborah G Hirtz2, Fengming Hu2, Chunyan Liu2, Eli M Mizrahi2, Solomon L Moshé2, Peggy Clark2, Tracy A Glauser2. 1. From the Children's Hospital of Philadelphia (S.K.K., D.D.), Perelman School of Medicine, University of Pennsylvania; Montefiore Medical Center (S.S., S.L.M.), Albert Einstein College of Medicine, Bronx, NY; Children's National Health System (A.C., J.C., F.H.), Washington, DC; National Institute of Neurological Disorders and Stroke (D.G.H.), Bethesda, MD; Comprehensive Epilepsy Center (C.L., P.C., T.A.G.), Division of Neurology, Cincinnati Children's Hospital Medical Center, OH; and Baylor College of Medicine (E.M.M.), Houston, TX. kesslers@email.chop.edu. 2. From the Children's Hospital of Philadelphia (S.K.K., D.D.), Perelman School of Medicine, University of Pennsylvania; Montefiore Medical Center (S.S., S.L.M.), Albert Einstein College of Medicine, Bronx, NY; Children's National Health System (A.C., J.C., F.H.), Washington, DC; National Institute of Neurological Disorders and Stroke (D.G.H.), Bethesda, MD; Comprehensive Epilepsy Center (C.L., P.C., T.A.G.), Division of Neurology, Cincinnati Children's Hospital Medical Center, OH; and Baylor College of Medicine (E.M.M.), Houston, TX.
Abstract
OBJECTIVE: To determine seizure semiology in children with newly diagnosed childhood absence epilepsy and to evaluate associations with short-term treatment outcomes. METHODS: For participants enrolled in a multicenter, randomized, double-blind, comparative-effectiveness trial, semiologic features of pretreatment seizures were analyzed as predictors of treatment outcome at the week 16 to 20 visit. RESULTS: Video of 1,932 electrographic absence seizures from 416 participants was evaluated. Median seizure duration was 10.2 seconds; median time between electrographic seizure onset and clinical manifestation onset was 1.5 seconds. For individual seizures and by participant, the most common semiology features were pause/stare (seizure 95.5%, participant 99.3%), motor automatisms (60.6%, 86.1%), and eye involvement (54.9%, 76.5%). The interrater agreement for motor automatisms and eye involvement was good (72%-84%). Variability of semiology features between seizures even within participants was high. Clustering analyses revealed 4 patterns (involving the presence/absence of eye involvement and motor automatisms superimposed on the nearly ubiquitous pause/stare). Most participants experienced more than one seizure cluster pattern. No individual semiologic feature was individually predictive of short-term outcome. Seizure freedom was half as likely in participants with one or more seizure having the pattern of eye involvement without motor automatisms than in participants without this pattern. CONCLUSIONS: Almost all absence seizures are characterized by a pause in activity or staring, but rarely is this the only feature. Semiologic features tend to cluster, resulting in identifiable absence seizure subtypes with significant intraparticipant seizure phenomenologic heterogeneity. One seizure subtype, pause/stare and eye involvement but no motor automatisms, is specifically associated with a worse treatment outcome.
RCT Entities:
OBJECTIVE: To determine seizure semiology in children with newly diagnosed childhood absence epilepsy and to evaluate associations with short-term treatment outcomes. METHODS: For participants enrolled in a multicenter, randomized, double-blind, comparative-effectiveness trial, semiologic features of pretreatment seizures were analyzed as predictors of treatment outcome at the week 16 to 20 visit. RESULTS: Video of 1,932 electrographic absence seizures from 416 participants was evaluated. Median seizure duration was 10.2 seconds; median time between electrographic seizure onset and clinical manifestation onset was 1.5 seconds. For individual seizures and by participant, the most common semiology features were pause/stare (seizure 95.5%, participant 99.3%), motor automatisms (60.6%, 86.1%), and eye involvement (54.9%, 76.5%). The interrater agreement for motor automatisms and eye involvement was good (72%-84%). Variability of semiology features between seizures even within participants was high. Clustering analyses revealed 4 patterns (involving the presence/absence of eye involvement and motor automatisms superimposed on the nearly ubiquitous pause/stare). Most participants experienced more than one seizure cluster pattern. No individual semiologic feature was individually predictive of short-term outcome. Seizure freedom was half as likely in participants with one or more seizure having the pattern of eye involvement without motor automatisms than in participants without this pattern. CONCLUSIONS: Almost all absence seizures are characterized by a pause in activity or staring, but rarely is this the only feature. Semiologic features tend to cluster, resulting in identifiable absence seizure subtypes with significant intraparticipant seizure phenomenologic heterogeneity. One seizure subtype, pause/stare and eye involvement but no motor automatisms, is specifically associated with a worse treatment outcome.
Authors: Tracy A Glauser; Avital Cnaan; Shlomo Shinnar; Deborah G Hirtz; Dennis Dlugos; David Masur; Peggy O Clark; Peter C Adamson Journal: Epilepsia Date: 2012-11-21 Impact factor: 5.864
Authors: Tracy A Glauser; Avital Cnaan; Shlomo Shinnar; Deborah G Hirtz; Dennis Dlugos; David Masur; Peggy O Clark; Edmund V Capparelli; Peter C Adamson Journal: N Engl J Med Date: 2010-03-04 Impact factor: 91.245
Authors: Tracy A Glauser; Katherine Holland; Valerie P O'Brien; Mehdi Keddache; Lisa J Martin; Peggy O Clark; Avital Cnaan; Dennis Dlugos; Deborah G Hirtz; Shlomo Shinnar; Gregory Grabowski Journal: Ann Neurol Date: 2017-03 Impact factor: 10.422
Authors: Dennis Dlugos; Shlomo Shinnar; Avital Cnaan; Fengming Hu; Solomon Moshé; Eli Mizrahi; David Masur; Yoshi Sogawa; J B Le Pichon; Calley Levine; Deborah Hirtz; Peggy Clark; Peter C Adamson; Tracy Glauser Journal: Neurology Date: 2013-05-29 Impact factor: 9.910
Authors: Gerhard S Drenthen; Floor Fasen; Eric L A Fonseca Wald; Walter H Backes; Albert P Aldenkamp; R Jeroen Vermeulen; Mariette Debeij-van Hall; Jos Hendriksen; Sylvia Klinkenberg; Jacobus F A Jansen Journal: Neuroimage Clin Date: 2020-04-23 Impact factor: 4.881
Authors: Simone Mattozzi; Caterina Cerminara; Maria A Sotgiu; Alessandra Carta; Antonella Coniglio; Denis Roberto; Delia M Simula; Gian Luca Pruneddu; Silvia Dell'Avvento; S Sonia Muzzu; Maria Fadda; Giovanni M Luzzu; Stefano Sotgiu; Susanna Casellato Journal: Clin Neurophysiol Pract Date: 2021-06-11
Authors: Vincenzo Crunelli; Magor L Lőrincz; Cian McCafferty; Régis C Lambert; Nathalie Leresche; Giuseppe Di Giovanni; François David Journal: Brain Date: 2020-08-01 Impact factor: 13.501