Pretreatment seizure semiology in childhood absence epilepsy

For the Childhood Absence Epilepsy Study Group

Research output: Contribution to journalArticle

5 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)673-679
Number of pages7
JournalNeurology
Volume89
Issue number7
DOIs
StatePublished - Aug 15 2017

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Absence Epilepsy
Seizures
Automatism
Cluster Analysis

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Pretreatment seizure semiology in childhood absence epilepsy. / For the Childhood Absence Epilepsy Study Group.

In: Neurology, Vol. 89, No. 7, 15.08.2017, p. 673-679.

Research output: Contribution to journalArticle

For the Childhood Absence Epilepsy Study Group 2017, 'Pretreatment seizure semiology in childhood absence epilepsy', Neurology, vol. 89, no. 7, pp. 673-679. https://doi.org/10.1212/WNL.0000000000004226
For the Childhood Absence Epilepsy Study Group. Pretreatment seizure semiology in childhood absence epilepsy. Neurology. 2017 Aug 15;89(7):673-679. https://doi.org/10.1212/WNL.0000000000004226
For the Childhood Absence Epilepsy Study Group. / Pretreatment seizure semiology in childhood absence epilepsy. In: Neurology. 2017 ; Vol. 89, No. 7. pp. 673-679.
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title = "Pretreatment seizure semiology in childhood absence epilepsy",
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.",
author = "{For the Childhood Absence Epilepsy Study Group} and Kessler, {Sudha Kilaru} and Shlomo Shinnar and Avital Cnaan and Dennis Dlugos and Joan Conry and Hirtz, {Deborah G.} and Fengming Hu and Chunyan Liu and Mizrahi, {Eli M.} and Moshe, {Solomon L.} and Peggy Clark and Glauser, {Tracy A.}",
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T1 - Pretreatment seizure semiology in childhood absence epilepsy

AU - For the Childhood Absence Epilepsy Study Group

AU - Kessler, Sudha Kilaru

AU - Shinnar, Shlomo

AU - Cnaan, Avital

AU - Dlugos, Dennis

AU - Conry, Joan

AU - Hirtz, Deborah G.

AU - Hu, Fengming

AU - Liu, Chunyan

AU - Mizrahi, Eli M.

AU - Moshe, Solomon L.

AU - Clark, Peggy

AU - Glauser, Tracy A.

PY - 2017/8/15

Y1 - 2017/8/15

N2 - 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.

AB - 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.

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