Nienke E Verbeek1, Merel Wassenaar2, Jolien S van Campen3, Anja Sonsma4, Boudewijn Gunning5, Nine Knoers6, Dick Lindhout7, Floor E Jansen8, Frans Leijten9, Eva H Brilstra10, Dorothée Kasteleijn-Nolst Trenité11. 1. Department of Medical Genetics, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address: n.verbeek@umcutrecht.nl. 2. 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. 3. 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. 4. Department of Medical Genetics, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address: a.c.m.sonsma@umcutrecht.nl. 5. Stichting Epilepsie Instellingen Nederland, Dr. Denekampweg 20, 8025 BV Zwolle, The Netherlands. Electronic address: bgunning@sein.nl. 6. Department of Medical Genetics, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address: v.v.a.knoers@umcutrecht.nl. 7. 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. 8. 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. 9. 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. 10. Department of Medical Genetics, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address: e.h.brilstra@umcutrecht.nl. 11. Department of Medical Genetics, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, The Netherlands. Electronic address: d.kasteleijn@umcutrecht.nl.
Abstract
OBJECTIVES: 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.
OBJECTIVES: 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.
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