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Objective: To detect determinants for photoparoxysmal EEG response (PPR) in SCN1A -related Dravet syndrome (DS). Methods: Data were studied from nationwide medical histories and EEGs of DS-patients ( n =53; 31 males, age 2–19years). Detailed questionnaires on visual stimuli were completed by parents ( n =49). Results: PPR was found in 22 patients (42%; median age 1.25yr), and repeatedly in 17%. PPR (17% of 249 intermittent photic stimulation (IPS)-EEGs) occurred more often with optimal IPS protocols (OR 2.11 [95%CI 1.09–4.13]) and in EEGs showing spontaneous epileptiform abnormalities (OR 5.08 [95%CI 2.05–12.55]). PPR-positive patients tended to be younger at first ( p =0.072) and second seizure ( p =0.049), showed severe intellectual disability ( p =0.042), and had more often spontaneous occipital epileptiform abnormalities ( p Clinical sensitivity was reported in medical files in 22% of patients and by parents in 43% (self-induction 24%). Clinical or EEG proven visual sensitivity was detected in 65% of cases. Conclusions: Sensitivity to visual stimuli is very common in DS and more often noticed by parents than confirmed by EEG. Detection of PPR improves with repetitive tests using accurate IPS protocols. Significance: Photosensitivity is an important feature in DS and seems to be a marker of the severity of the disorder. Therefore repeated standardized IPS should be encouraged.

Purpose Accurate classification of epileptic seizures, epilepsies, and epilepsy syndromes is mandatory in both clinical practice and epilepsy research. In 2010, the International League Against Epilepsy (ILAE) proposed a new classification scheme. The aim of this study is to determine whether application of this new classification for epileptic seizures and epilepsies has improved interobserver agreement compared to the classification schemes used previously. Methods Three pediatric neurologists working in different university hospitals retrospectively classified seizures and epilepsies of 80 children (165 seizures) referred to the University Center Utrecht, based on anonymized data, according to the newly proposed (2010) as well as the old (1981/1989) ILAE classification schemes. We determined interobserver agreement of the application of both ILAE classifications with kappa statistics. Key Findings Interobserver agreement of the new classification for seizures and epilepsies is comparable to that of previous classifications. There is substantial agreement on the newly introduced etiologic axis. Significance Introduction of the new epilepsy classification has not substantially improved interobserver agreement. This study shows which items cause considerable interobserver disagreement and therefore need specification.

Summary Purpose: To evaluate the effect of stress on seizure frequency in childhood epilepsy, and to assess possible differences between children in whom seizures are precipitated by stress and those in whom they are not. Methods: Parents or caregivers of children with active epilepsy (aged 2–16 years) were sent questionnaires on developmental and epilepsy characteristics, life-time stress exposure, and the effect of stressful periods and moments of acute stress on seizure frequency in their child. Further information was extracted from patient files. Key Findings: Parents or caregivers of 153 children with a median age of 8.8 years responded to the questionnaires. Thirty-nine percent reported an increase in seizure frequency during periods of stress, with a median increase of 2.5 times the frequency compared to nonstressful periods. Thirty-seven percent reported that seizures were precipitated by acute stress, with stress being a precipitating factor in 33% (median value) of the seizures. Overall, 51% of the patients reported stress sensitivity of seizures. A higher number of negative life events experienced in total life was related to an increase in seizure frequency in stressful periods (odds ratio [OR] 1.3, p = 0.01) as well as to the precipitation of seizures by acute stress (OR 1.3, p = 0.02). Significance: Stress sensitivity is reported in half of the children with epilepsy. Results of this study suggest a relation between experienced negative life events and stress sensitivity of childhood epilepsy. One possible explanation could be that experiencing negative life events may cause a larger response to daily stressors, thereby increasing the likelihood to induce epileptic activity.

Stress is the most often reported seizure-precipitant in epilepsy. As most evidence for the relation between stress and epilepsy is derived from human self-reports, observational studies including a larger part of the population could provide additional proof. A stressor often reported to increase seizure frequency in children with epilepsy in the Netherlands is the national celebration of Saint Nicholas' eve (December 5) and the weeks before; this is the main period of festivities for children in this country. To study the relation between stress and epilepsy, we analyzed epilepsy information-seeking behavior on the Internet, an indirect measure of seizure frequency, around this national children's celebration.Google Trends was used to extract relative search percentages for 'epilepsy' on Google in the Netherlands, the United States, and the United Kingdom between 2004 and 2013. Relative search percentages during the Saint Nicholas period were compared with baseline.Epilepsy searches increased by 14% in the Saint Nicholas period compared with baseline (p0.001). This effect was not found for searches performed in the same period in the United States or the United Kingdom, countries where this holiday is not celebrated.The increase in epilepsy information-seeking behavior in the Saint Nicholas period is possibly caused by an increased occurrence of epileptic seizures. This underscores the potential of health information-seeking behavior on the Internet to answer clinically relevant research questions and provides circumstantial evidence for a relation between stress and the occurrence of epileptic seizures.

To evaluate the effect of stress on seizure frequency in childhood epilepsy, and to assess possible differences between children in whom seizures are precipitated by stress and those in whom they are not.Parents or caregivers of children with active epilepsy (aged 2-16 years) were sent questionnaires on developmental and epilepsy characteristics, life-time stress exposure, and the effect of stressful periods and moments of acute stress on seizure frequency in their child. Further information was extracted from patient files.Parents or caregivers of 153 children with a median age of 8.8 years responded to the questionnaires. Thirty-nine percent reported an increase in seizure frequency during periods of stress, with a median increase of 2.5 times the frequency compared to nonstressful periods. Thirty-seven percent reported that seizures were precipitated by acute stress, with stress being a precipitating factor in 33% (median value) of the seizures. Overall, 51% of the patients reported stress sensitivity of seizures. A higher number of negative life events experienced in total life was related to an increase in seizure frequency in stressful periods (odds ratio [OR] 1.3, p = 0.01) as well as to the precipitation of seizures by acute stress (OR 1.3, p = 0.02).Stress sensitivity is reported in half of the children with epilepsy. Results of this study suggest a relation between experienced negative life events and stress sensitivity of childhood epilepsy. One possible explanation could be that experiencing negative life events may cause a larger response to daily stressors, thereby increasing the likelihood to induce epileptic activity.