Dravet syndrome is a genetically defined, paediatric-onset developmental and epileptic encephalopathy — a US incidence of 1 in 15,700 births, de novo SCN1A mutations in ~75% of clinical cases, and a SUDEP rate among the highest documented in epilepsy.
Dravet syndrome is a severe developmental and epileptic encephalopathy that begins in the first year of life, typically with prolonged, often fever-triggered seizures in a previously normally developing infant. A US population-based study at Kaiser Permanente Northern California estimated incidence at 1 per 15,700 births, roughly twice the earlier estimate of 1 in 40,000, with a likely-pathogenic de novo SCN1A mutation identified in six of eight clinical cases (about 1 in 20,900). All identified infants had febrile seizures, and most had prolonged seizures lasting more than 10 minutes by age one.
The burden extends well beyond seizure count. In a 100-patient Dravet cohort followed for a median of 17 years, 17 patients died at a median age of 7 years; the syndrome-specific mortality rate was 15.84 per 1,000 person-years and the SUDEP rate 9.32 per 1,000 person-years — the highest documented syndrome-specific SUDEP rate, and far above the ~5.1 per 1,000 reported for refractory epilepsy in adults. Near-universal developmental slowing and intellectual disability compound the clinical and caregiver burden, making Dravet a lifelong, multi-system condition rather than a seizure disorder alone.
Dravet syndrome epidemiology & disease burden — United States
| Parameter | Value | Source |
|---|---|---|
| US incidence | 1 per 15,700 births (~1 per 20,900 for SCN1A-confirmed cases) | Wu et al., Pediatrics 2015 (PMID 26438699) |
| Genetic basis | Likely-pathogenic de novo SCN1A mutation in ~75% of clinical cases (6 of 8) | Wu et al., Pediatrics 2015 (PMID 26438699) |
| Seizure onset | First year of life; ≥2 seizures before age 12 months; febrile seizures in all identified cases | Wu et al., Pediatrics 2015 (PMID 26438699) |
| Mortality rate | 15.84 per 1,000 person-years; 17 of 100 patients died over median 17-yr follow-up | Cooper et al., Epilepsy Res 2016 (PMID 27810515) |
| SUDEP rate | 9.32 per 1,000 person-years; median age at death 7 years | Cooper et al., Epilepsy Res 2016 (PMID 27810515) |
| Estimated US prevalence | ~6,000–8,000 patients (rare, paediatric-onset) | Dravet Syndrome Foundation |
Sources: Wu et al., Incidence of Dravet Syndrome in a US Population, Pediatrics 2015 (PMID 26438699); Cooper et al., Mortality in Dravet Syndrome, Epilepsy Res 2016 (PMID 27810515); Dravet Syndrome Foundation prevalence estimate.
What this assessment answers
Every section answers a named commercial question your team is asking, scoped to your asset.
Delivers
- US population-based incidence (1 in 15,700)
- the shift from the older 1-in-40,000 estimate
- de novo SCN1A fraction
- Dravet Syndrome Foundation prevalence framing for the addressable patient pool
Delivers
- First-year seizure onset and febrile-seizure trigger
- developmental slowing and intellectual disability trajectory
- mortality and SUDEP rates from long-term cohort follow-up
Delivers
- SCN1A testing as the diagnostic anchor
- clinical-versus-genetic diagnosis
- how confirmation gates access to Dravet-specific therapy and defines the paediatric-onset payer population
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Commission This AssessmentWhat's inside
- How a likely-pathogenic de novo SCN1A mutation was found in 6 of 8 clinical cases, about 75%.
- Why all identified infants had febrile seizures, most lasting more than 10 minutes by age one.
- How the Kaiser Permanente Northern California cohort study established the current 1-in-15,700 incidence figure.
- Why the SCN1A-confirmed incidence narrows to about 1 in 20,900 births when genetics alone are counted.
- How Dravet begins with prolonged, often fever-triggered seizures in a previously normally developing infant.
- Why at least two seizures before 12 months, most exceeding 10 minutes, defined onset in the Kaiser cohort.
- How near-universal developmental slowing and intellectual disability make Dravet a lifelong, multi-system condition.
- Why the 100-patient cohort followed for a median of 17 years shows burden extends well beyond seizures.
- How Dravet's 9.32-per-1,000-person-year SUDEP rate compares to roughly 5.1 for refractory epilepsy in adults.
- Why 17 of 100 patients died at a median age of 7, at a 15.84 mortality rate.
- How genetic SCN1A confirmation, not symptoms alone, anchors diagnosis and access to Dravet-specific therapy.
- Why an estimated 6,000 to 8,000 US patients define the treated population per Dravet Syndrome Foundation framing.
Included with every brief
How AXLRx builds this assessment
Prepared by MoatRx analysts.
Every AXLRx assessment is built from primary regulatory sources (FDA Drugs@FDA, ClinicalTrials.gov), peer-reviewed literature, and live payer and HTA documentation — not secondary summaries, market-research reports, or unverified estimates. Findings are independently verified before inclusion; if a figure cannot be sourced to a live record, it does not ship.
US Dravet Syndrome Disease Landscape sources: the US population-based incidence study (Wu et al., Pediatrics 2015, PMID 26438699); the Dravet mortality and SUDEP cohort (Cooper et al., Epilepsy Research 2016, PMID 27810515); and the Dravet Syndrome Foundation for US prevalence framing.
- US incidence (1 in 15,700) and de novo SCN1A fraction verified against Wu et al., Pediatrics 2015 (PMID 26438699)
- Mortality (15.84 / 1,000 person-years) and SUDEP (9.32 / 1,000 person-years) rates verified against Cooper et al., Epilepsy Research 2016 (PMID 27810515)
- Median age at death and cohort follow-up verified against Cooper et al., Epilepsy Research 2016 (PMID 27810515)
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