Overview
Dravet syndrome, also known as Severe Myoclonic Epilepsy of Infancy (SMEI), is a rare and serious form of epilepsy that begins in the first year of life. It is caused by changes (mutations) in a gene that helps brain cells communicate properly. When this gene does not work as it should, the brain becomes overly excitable, leading to frequent and hard-to-control seizures. Children with Dravet syndrome typically start having seizures between 5 and 8 months of age. These early seizures are often triggered by fever or a rise in body temperature. Over time, different types of seizures appear, and children may also experience delays in development, problems with balance and walking, and difficulties with learning, behavior, and communication. The condition affects not just the brain but also daily life for the whole family. Treatment focuses on reducing seizures and improving quality of life, since there is currently no cure. Several medications are used, including valproate, clobazam, stiripentol, and cannabidiol (Epidiolex), which is FDA-approved specifically for Dravet syndrome. Fenfluramine (Fintepla) is another FDA-approved option. While seizures often remain difficult to fully control, early diagnosis and the right combination of treatments can make a meaningful difference in a child's development and safety.
Key symptoms:
Frequent seizures that are hard to control with medicationSeizures triggered by fever or overheatingMultiple types of seizures (including full-body convulsions and staring spells)Developmental delays or regression in skillsIntellectual disability ranging from mild to severeProblems with balance, coordination, and walking (often a crouched gait)Behavioral challenges such as hyperactivity, impulsivity, or autism-like traitsDifficulty with speech and languageSleep problemsSensitivity to light (photosensitivity)Increased risk of sudden unexpected death in epilepsy (SUDEP)
Clinical phenotype terms (46)— hover any for plain English
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Infantile
Begins in infancy, roughly 1 month to 2 years old
FDA & Trial Timeline
10 eventsElizabeth Donner — PHASE4
Byteflies — NA
Assistance Publique - Hôpitaux de Paris
Stoke Therapeutics, Inc — PHASE3
University of Colorado, Denver — PHASE4
Longboard Pharmaceuticals — PHASE3
UCB BIOSCIENCES, Inc. — PHASE3
Encoded Therapeutics — PHASE1, PHASE2
Encoded Therapeutics — PHASE1, PHASE2
Encoded Therapeutics — PHASE1, PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
3 availableFintepla
FINTEPLA is indicated for the treatment of seizures associated with Dravet syndrome in patients 2 years of age and older
Epidiolex
EPIDIOLEX is indicated for the treatment of seizures associated with Dravet syndrome (DS) in patients 1 year of age and older
Diacomit
Treatment of seizures associated with Dravet syndrome (DS) in patients taking clobazam who are 6 months of age and older and weighing 7 kg or more
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Financial Resources
3 resourcesTravel Grants
No travel grants are currently matched to Dravet syndrome.
Community
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Start the conversation →Latest news about Dravet syndrome
1 articlesCaregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Which medications are safest for my child's type of Dravet syndrome, and which ones should we avoid?,Should we have rescue medication at home, and can you train us on how to use it?,Is genetic testing recommended for other family members, and what does a positive SCN1A result mean for future pregnancies?,What is the risk of SUDEP for my child, and what steps can we take to reduce it?,Would a ketogenic diet be appropriate for my child, and how do we get started?,What developmental therapies and educational supports should we be pursuing right now?,Are there any clinical trials or new treatments we should consider?
Common questions about Dravet syndrome
What is Dravet syndrome?
Dravet syndrome, also known as Severe Myoclonic Epilepsy of Infancy (SMEI), is a rare and serious form of epilepsy that begins in the first year of life. It is caused by changes (mutations) in a gene that helps brain cells communicate properly. When this gene does not work as it should, the brain becomes overly excitable, leading to frequent and hard-to-control seizures. Children with Dravet syndrome typically start having seizures between 5 and 8 months of age. These early seizures are often triggered by fever or a rise in body temperature. Over time, different types of seizures appear, and
How is Dravet syndrome inherited?
Dravet syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Dravet syndrome typically begin?
Typical onset of Dravet syndrome is infantile. Age of onset can vary across affected individuals.
Are there clinical trials for Dravet syndrome?
Yes — 12 recruiting clinical trials are currently listed for Dravet syndrome on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Dravet syndrome?
25 specialists and care centers treating Dravet syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Dravet syndrome?
1 patient support program are currently tracked on UniteRare for Dravet syndrome. See the treatments and support programs sections for copay assistance, eligibility, and contact details.