Impact of Seizures
More than 90% of children with Dravet syndrome are not able to achieve freedom from their persistent seizures.1
HIGH SEIZURE FREQUENCY
Many patients experience multiple seizures on a weekly or even daily basis.1-3
of patients experience 4 or more tonic-clonic seizures per month4
of patients experience 4 or more myoclonic seizures per month4
High seizure frequency has been associated with:1,5
Higher incidence of cognitive and behavioral impairment
More emergency admissions and ambulance calls
Lower patient health-related quality of life
PREMATURE MORTALITY
Dravet syndrome carries a high risk of premature mortality* due to sudden unexpected death in epilepsy (SUDEP), fatal status epilepticus, and accidents.2,6-8 The main SUDEP risk factors, including high frequency of convulsions and antiepileptic drug polytherapy, relate to epilepsy severity.7,9
The amount by which the overall risk of death is increased compared with the general population of patients with epilepsy8,10
The percentage of deaths due to SUDEP8
The percentage of SUDEP deaths in Dravet syndrome that occur before the age of 11 years7
of deaths due to SUDEP are sleep-related events. Most often, SUDEP occurs when the patient has a seizure while lying prone in bed during the night or early morning hours11
of deaths due to SUDEP occur unwitnessed, suggesting that nocturnal monitoring devices or having someone else sleep in the same room might decrease the risk11,12
*The mortality rate in Dravet syndrome was 15.84 per 1000 person-years (calculated over a total of 1073 person-years).8
There is an urgent need for continued research to help provide:
Meaningful, consistent, and sustained seizure reduction
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References
- Lagae L, Brambilla I, Mingorance A, Gibson E, Battersby A. Quality of life and comorbidities associated with Dravet syndrome severity: a multinational cohort survey. Dev Med Child Neurol. 2018;60(1):63-72. https://www.ncbi.nlm.nih.gov/pubmed/28984349
- Dravet C. Dravet syndrome history. Dev Med Child Neurol. 2011;53(suppl 2):1-6. https://www.ncbi.nlm.nih.gov/pubmed/21504424
- Granata T. Comprehensive care of children with Dravet syndrome. Epilepsia. 2011;52(suppl 2):90-94. https://www.ncbi.nlm.nih.gov/pubmed/21463289
- Aras LM, Isla J, Mingorance-Le Meur A. The European patient with Dravet syndrome: results from a parent-reported survey on antiepileptic drug use in the European population with Dravet syndrome. Epilepsy Behav. 2015;44:104-109. https://www.ncbi.nlm.nih.gov/pubmed/25666511
- Wolff M, Cassé-Perrot C, Dravet C. Severe myoclonic epilepsy of infants (Dravet syndrome): natural history and neuropsychological findings. Epilepsia. 2006;47(suppl 2):45-48. https://www.ncbi.nlm.nih.gov/pubmed/17105460
- Skluzacek JV, Watts KP, Parsy O, Wical B, Camfield P. Dravet syndrome and parent associations: the IDEA League experience with comorbid conditions, mortality, management, adaptation, and grief. Epilepsia. 2011;52(suppl 2):95-101. https://www.ncbi.nlm.nih.gov/pubmed/21463290
- Shmuely S, Sisodiya SM, Gunning WB, Sander JW, Thijs RD. Mortality in Dravet syndrome: a review. Epilepsy Behav. 2016;64(pt A):69-74. https://www.ncbi.nlm.nih.gov/pubmed/27732919
- Cooper MS, Mcintosh A, Crompton DE, et al. Mortality in Dravet syndrome. Epilepsy Res. 2016;128:43-47. https://www.ncbi.nlm.nih.gov/pubmed/27810515
- Jones LA, Thomas RH. Sudden death in epilepsy: insights from the last 25 years. Seizure. 2017;44:232-236. https://www.ncbi.nlm.nih.gov/pubmed/27773556
- Berg AT, Nickels K, Wirrell EC, et al. Mortality risks in new-onset childhood epilepsy. Pediatrics. 2013;132(1):124-131. https://www.ncbi.nlm.nih.gov/pubmed/23753097
- Lamberts RJ, Thijs RD, Laffan A, Langan Y, Sander JW. Sudden unexpected death in epilepsy: people with nocturnal seizures may be at highest risk. Epilepsia. 2012;53(2):253-257. https://www.ncbi.nlm.nih.gov/pubmed/22192074
- Purnell BS, Thijs RD, Buchanan GF. Dead in the night: sleep-wake and time-of-day influences on sudden unexpected death in epilepsy. Front Neurol. 2018;9:1079. https://www.ncbi.nlm.nih.gov/pubmed/30619039