TY - JOUR
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 - Moshé, Solomon L.
AU - Clark, Peggy
AU - Glauser, Tracy A.
N1 - Funding Information:
S. Kessler has been funded by NIH grants K12-NS049453 and 2U01-NS045911, Patient-Centered Outcomes Research Institute (Cognitive AED Outcomes in Pediatric Localization Related Epilepsy [COPE] Study), Simons Foundation, Foerderer Fund, Physical Therapy Foundation, Friedreich Ataxia Research Alliance, and the Epilepsy Study Consortium. She has also given expert testimony in medico-legal cases. S. Shinnar is funded by NIH grants 2R37-NS043209, 2U01-NS045911, U10NS077308, and 1U01NS088034. A. Cnaan is funded by NIH grants 2U01-NS045911, UL1RR031988, P30HD040677, P50AR060836, R01AR061875, and R01HD058567; Department of Defense grant; and Department of Education grant H133B090001. D. Dlugos is funded by NIH grants 1R01NS053998, 2U01NS045911, 1R01LM011124, and U01NS077276; by the Epilepsy Study Consortium; and by prestudy protocol development agreements with Insys Therapeutics and Bio-Pharm Solutions. J. Conry is funded by the Patient-Centered Outcomes Research Institute (COPE Study) and participates in clinical trials sponsored by SAGE Pharmaceuticals, Pfizer, and Accorda. She serves as chair of the Carbaglu Data Safety and Monitoring Board. She serves on the Scientific Advisory Board for Upsher-Smith. F. Hu is funded by NIH grants 2U01-NS045911, P30HD040677, P50AR060836, R01AR061875, and R01HD058567; Department of Defense grant W81XWH-09-1-0592; and Department of Education grant H133B090001. C. Liu reports no disclosures relevant to the manuscript. E. Mizrahi is funded by NIH grant U01-NS045911, Department of Defense grant W81XWH-08-2-0149, and NeuroPace, Inc (Responsive Neurostimulator System Long-Term Treatment Clinical Investigation). S. Moshé is the Charles Frost Chair in Neurosurgery and Neurology and is funded by NIH grants NS43209 and NS45911, Citizens United for Research in Epilepsy, US Department of Defense, and the Heffer Family and the Siegel Family foundations. P. Clark is funded by NIH grants 2U01-NS045911 and U10-NS077311. T. Glauser is funded by NIH grants 2U01-NS045911, U10-NS077311, R01-NS053998, R01-NS062756, R01-NS043209, R01-LM011124, and R01-NS065840.
Publisher Copyright:
© 2017 American Academy of Neurology.
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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U2 - 10.1212/WNL.0000000000004226
DO - 10.1212/WNL.0000000000004226
M3 - Article
C2 - 28724582
AN - SCOPUS:85027530759
SN - 0028-3878
VL - 89
SP - 673
EP - 679
JO - Neurology
JF - Neurology
IS - 7
ER -