Other names: Severe Myoclonic Epilepsy of Infancy; SMEI
Dravet syndrome is a rare type of epilepsy that begins in the first year of life in an otherwise healthy child. A syndrome is a combination of specific signs and symptoms that when occur together are diagnosed as a medical condition.
Dravet syndrome patients have a particular type and pattern of epileptic seizures and as the syndrome progresses other issues become apparent including developmental delay or regression, sleep disturbance issues, behaviour problems and mobility concerns such as unsteady gait. The first seizure usually occurs in the first 12 months of life and this often occurs while the child has fever due to illness or vaccination. Following the first seizure more seizures follow with increasing severity, changes in seizure type and often the seizures become unpredictable, frequent and difficult to control.
The seizures often start out as jerky movements (clonic seizures), that are prolonged and more likely to affect one side of the body. As the syndrome progresses the seizures types can include myoclonic seizures (affecting whole or half of the body), atypical absence seizures, focal seizures, serial seizures or status epilepticus. Dravet syndrome patients are more prone status epilepticus, which is when there is a long lasting seizure or when seizures occur close together without recovery between them. With Dravet syndrome there is an increased risk of sudden unexplained death in epilepsy (SUDEP) compared to other types of epilepsy.
Most patients with Dravet syndrome have a change or mutation in the SCN1a gene which causes a faulty protein to be made. This faulty protein reduces the brain’s ability to control electrical messages within the brain and causes seizures.
Drugs used to treat Dravet Syndrome
The medications listed below are related to or used in the treatment of this condition.
For ratings, users were asked how effective they found the medicine while considering positive/adverse effects and ease of use (1 = not effective, 10 = most effective).
Activity
Activity is based on recent site visitor activity relative to other medications in the list.
Rx
Prescription only.
OTC
Over-the-counter.
Rx/OTC
Prescription or Over-the-counter.
Off-label
This medication may not be approved by the FDA for the treatment of this condition.
EUA
An Emergency Use Authorization (EUA) allows the FDA to authorize unapproved medical products or unapproved uses of approved medical products to be used in a declared public health emergency when there are no adequate, approved, and available alternatives.
Expanded Access
Expanded Access is a potential pathway for a patient with a serious or immediately life-threatening disease or condition to gain access to an investigational medical product (drug, biologic, or medical device) for treatment outside of clinical trials when no comparable or satisfactory alternative therapy options are available.
Pregnancy Category
A
Adequate and well-controlled studies have failed to demonstrate a risk to the fetus in the first trimester of pregnancy (and there is no evidence of risk in later trimesters).
B
Animal reproduction studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women.
C
Animal reproduction studies have shown an adverse effect on the fetus and there are no adequate and well-controlled studies in humans, but potential benefits may warrant use in pregnant women despite potential risks.
D
There is positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience or studies in humans, but potential benefits may warrant use in pregnant women despite potential risks.
X
Studies in animals or humans have demonstrated fetal abnormalities and/or there is positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience, and the risks involved in use in pregnant women clearly outweigh potential benefits.
N
FDA has not classified the drug.
Controlled Substances Act (CSA) Schedule
M
The drug has multiple schedules. The schedule may depend on the exact dosage form or strength of the medication.
U
CSA Schedule is unknown.
N
Is not subject to the Controlled Substances Act.
1
Has a high potential for abuse. Has no currently accepted medical use in treatment in the United States. There is a lack of accepted safety for use under medical supervision.
2
Has a high potential for abuse. Has a currently accepted medical use in treatment in the United States or a currently accepted medical use with severe restrictions. Abuse may lead to severe psychological or physical dependence.
3
Has a potential for abuse less than those in schedules 1 and 2. Has a currently accepted medical use in treatment in the United States. Abuse may lead to moderate or low physical dependence or high psychological dependence.
4
Has a low potential for abuse relative to those in schedule 3. It has a currently accepted medical use in treatment in the United States. Abuse may lead to limited physical dependence or psychological dependence relative to those in schedule 3.
5
Has a low potential for abuse relative to those in schedule 4. Has a currently accepted medical use in treatment in the United States. Abuse may lead to limited physical dependence or psychological dependence relative to those in schedule 4.
Alcohol
X
Interacts with Alcohol.
Further information
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