Suspect-and-test checklist
Dravet syndrome
Developmental and epileptic encephalopathy, SCN1A in more than 85%. Normal EEG and MRI early do not exclude it.
ICD-10-CMG40.833, G40.834Trial Friend for Clinicians
1Red flags present
First seizures, age 1 to 20 months
Triggers
After the first year
Medicines and family
2Already labeled as
3Family to test
4Testing, in the order the sources describe
- Apply the consensus testing criteriaNormal infant 2 to 15 months, first prolonged hemiclonic seizure or status, with fever or vaccination, no other cause.Result
- Brain MRIRecommended to exclude other causes; usually normal in Dravet syndrome.Result
- EEGOften normal before 12 months; discharges appear in most by age 5.Result
- Epilepsy multigene panel with deletion/duplicationPreferred over SCN1A alone. Sequencing misses whole-exon deletions.Result
- Hattori score if febrile seizures before age 1Score of 6 or more prompted SCN1A analysis.Result
- Parental testing after a positive resultVariant de novo in 76 of 80 children.Result
- No variant foundAn ILAE alert, not an exclusion. Review the phenotype and other genes.Result
5Results the sources say do not rule it out
- Normal MRI or a normal first-year EEG. Both are expected early in Dravet syndrome.
- Negative SCN1A sequencing alone. Deletions need deletion/duplication analysis.
- Seizures blamed on a vaccine. Vaccination may only bring SCN1A Dravet onset earlier.
- No family history. Most SCN1A variants in Dravet syndrome are de novo.
6Specialists the sources name
Diagnosis codes: G40.833 Dravet syndrome, intractable, with status epilepticus; G40.834 Dravet syndrome, intractable, without status epilepticusCodes patients often carry before diagnosis are listed in the online guide.
Full guide and sourcesEvery point here is sourced in the online guide, which also has a patient note to print.
Trial Friend, trialfriend.com/for-clinicians. Key sources: 2022 international consensus; ILAE 2022 position statement; ILAE diagnostic manual (2024); ILAE diagnostic criteria (2024); ILAE differential diagnoses (2024); GeneReviews (2022); all 18 with links in the online guide. Checked November 10, 2026. A reference checklist for licensed clinicians that summarizes published sources. It is not a diagnostic tool, a score or a protocol, and it does not replace clinical judgment, local practice or the prescribing information. Not coding advice. Trial Friend never receives anything written or typed on this sheet.