title: Seizure precipitants in Dravet syndrome: What events and activities are specifically provocative compared with other epilepsies?
abstract: OBJECTIVE
  This study aimed to describe seizure precipitants in Dravet syndrome (DS) compared with other epilepsies.
  
  
  METHODS
  Seizure precipitants as reported in a Dutch cohort of patients with DS with pathogenic SCN1A mutations (n=71) were compared with those of a cohort with childhood epilepsy (n=149) and of a community-based cohort with epilepsy (n=248); for all three Dutch cohorts, the same type of questionnaire was used. Seizure precipitants were categorized as 'fever', 'visual stimuli', 'sleep deprivation', 'stress, including physical exercise', 'auditory stimuli', and 'other'.
  
  
  RESULTS
  For 70 (99%) of 71 patients with DS, at least one seizure precipitant was recalled by parents. Seizure precipitants that were reported in more than half of the cohort with DS were as follows: having a fever (97%), having a cold (68%), taking a bath (61%), having acute moments of stress (58%), and engaging in physical exercise (56%). Seizure precipitants freely recalled by parents were often related to ambient warmth or cold-warmth shifts (41%) and to various visual stimuli (18%). Patients with DS had more positive seizure precipitant categories (median 4) compared with the cohort with childhood epilepsy (median 2) and the community-based cohort with epilepsy (median 0) (p<0.001) and showed the highest percentage in each category (all p<0.001). Within the category 'stress, including physical exercise', physical exercise was more often reported to provoke seizures in stress-sensitive patients in the cohort with DS than in the cohort with childhood epilepsy (78% vs. 35%, p<0.001). In the cohort with childhood epilepsy, physical exercise was more often reported in fever-sensitive children than in other children (25% vs. 12%, p=0.042).
  
  
  CONCLUSIONS
  Our study shows a high prevalence of a range of seizure precipitants in DS. Our results underscore elevated body temperature as an important seizure precipitant, whether caused by fever, warm bath, ambient warmth, or physical exercise. Knowledge of these seizure precipitants may improve preventive strategies in the otherwise difficult treatment of DS.
authors.affiliation: Department of Medical Genetics, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address: n.verbeek@umcutrecht.nl.
authors.forename: Nienke E
authors.initials: NE
authors.lastname: Verbeek
authors.affiliation: Department of Neurology and Neurosurgery, Clinical Neurophysiology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands; Stichting Epilepsie Instellingen Nederland, Achterweg 5, 2103 SW Heemstede, The Netherlands. Electronic address: mwassenaar@sein.nl.
authors.forename: Merel
authors.initials: M
authors.lastname: Wassenaar
authors.affiliation: Department of Child Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands; Department of Translational Neuroscience, Brain Center Rudolf Magnus, University Medical Center Utrecht, Universiteitsweg 100, 3584 CG Utrecht, The Netherlands. Electronic address: j.s.vancampen-4@umcutrecht.nl.
authors.forename: Jolien S
authors.initials: JS
authors.lastname: van Campen
authors.affiliation: Department of Medical Genetics, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address: a.c.m.sonsma@umcutrecht.nl.
authors.forename: Anja
authors.initials: A
authors.lastname: Sonsma
authors.affiliation: Stichting Epilepsie Instellingen Nederland, Dr. Denekampweg 20, 8025 BV Zwolle, The Netherlands. Electronic address: bgunning@sein.nl.
authors.forename: Boudewijn
authors.initials: B
authors.lastname: Gunning
authors.affiliation: Department of Medical Genetics, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address: v.v.a.knoers@umcutrecht.nl.
authors.forename: Nine
authors.initials: N
authors.lastname: Knoers
authors.affiliation: Department of Medical Genetics, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands; Stichting Epilepsie Instellingen Nederland, Achterweg 5, 2103 SW Heemstede, The Netherlands. Electronic address: d.lindhout@umcutrecht.nl.
authors.forename: Dick
authors.initials: D
authors.lastname: Lindhout
authors.affiliation: Department of Child Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address: f.e.jansen-1@umcutrecht.nl.
authors.forename: Floor E
authors.initials: FE
authors.lastname: Jansen
authors.affiliation: Department of Neurology and Neurosurgery, Clinical Neurophysiology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands. Electronic address: f.s.s.leijten@umcutrecht.nl.
authors.forename: Frans
authors.initials: F
authors.lastname: Leijten
authors.affiliation: Department of Medical Genetics, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address: e.h.brilstra@umcutrecht.nl.
authors.forename: Eva H
authors.initials: EH
authors.lastname: Brilstra
authors.affiliation: Department of Medical Genetics, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address: d.kasteleijn@umcutrecht.nl.
authors.forename: Dorothée
authors.initials: D
authors.lastname: Kasteleijn-Nolst Trenité
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004831:Epilepsies, Myoclonic / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics*; D005260:Female; D005334:Fever / Q000150:complications / Q000453:epidemiology; D006358:Hot Temperature / Q000009:adverse effects; D006801:Humans; D008297:Male; D009154:Mutation; D062550:NAV1.1 Voltage-Gated Sodium Channel / Q000235:genetics*; D009426:Netherlands / Q000453:epidemiology; D010775:Photic Stimulation / Q000009:adverse effects; D015995:Prevalence; D012640:Seizures / Q000453:epidemiology* / Q000209:etiology / Q000503:physiopathology; D012892:Sleep Deprivation / Q000150:complications / Q000453:epidemiology; D013315:Stress, Psychological / Q000150:complications / Q000453:epidemiology; D011795:Surveys and Questionnaires
chemical_list: D062550:NAV1.1 Voltage-Gated Sodium Channel; C568242:SCN1A protein, human
keywords: Physical exercise; Provocation; SCN1A; SMEI; Severe myoclonic epilepsy of infancy; Triggers