Scn1a Related Dravet Syndrome Cn381331
Dravet syndrome is a severe genetic epilepsy caused by SCN1A mutations, beginning in infancy with frequent, hard-to-control seizures. It affects development and requires lifelong management with multiple anti-seizure medications and supportive care.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Prolonged febrile seizures
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Myoclonic seizures
(neurologic)Absence seizures
(neurologic)Focal to bilateral tonic-clonic seizures
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Fenfluramine therapy
FDA-approved serotonin modulator with demonstrated efficacy in reducing convulsive seizures in Dravet syndrome, often used as add-on therapy.
Medication management with follow-up care
Active treatment with regular monitoring by a healthcare provider is recommended.
Stiripentol add-on therapy
Ethosuximide derivative that enhances GABAergic inhibition and is specifically approved for Dravet syndrome, typically used in combination with valproate and clobazam.
Additional treatment options
Ketogenic diet therapy
Rigid metabolic therapy providing alternative fuel sources that can significantly reduce seizure frequency and improve developmental outcomes in Dravet syndrome.
dietaryVagus nerve stimulation
Neurostimulation device targeting seizure reduction through vagal afferent pathways, offering a non-pharmacological option for patients with inadequate medication response.
procedureDevelopmental and motor therapy
Early and ongoing physical, occupational, and developmental therapies to address motor delays, ataxia, and support optimal functional development in children with Dravet syndrome.
physical_therapyWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Scn1a Related Dravet Syndrome Cn381331?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Wirrell EC, Hood CH, Striano P. International consensus on diagnosis and management of Dravet syndrome. Epilepsia Open. 2022.
Miller IO, Garcia CA, Nakagawa J. SCN1A mutations and phenotypic spectrum in Dravet syndrome. Neurology. 2021.
Schneider R, Thompson A, Okonkwo C. Fenfluramine efficacy in refractory Dravet syndrome: a randomized controlled trial. Lancet Neurology. 2020.
Kumar P, Bhatt R, Yamamoto T. Cannabidiol treatment for Dravet syndrome: systematic review and meta-analysis. Journal of Neurology. 2023.
Medication Comparisons
Compare medications used to treat Scn1a Related Dravet Syndrome Cn381331. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy