Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatment
Table of ContentsView All
View All
Table of Contents
Symptoms
Causes
Diagnosis
Treatment
Unfortunately, you can’t outgrow Dravet syndrome. It’s a lifelong condition due to a genetic defect in the SCN1A gene. The life expectancy of a child with Dravet syndrome is not the average lifespan. However, most individuals with Dravet syndrome do reach adulthood. Symptoms can be managed with various forms of therapy.
This article explains the symptoms and causes of Dravet syndrome. It also discusses how the condition is diagnosed and treated.
Dravet Syndrome Symptoms
Seizures are the earliest symptom of Dravet syndrome. Developmental problems, as well as seizures, generally worsen as a child gets older.
Symptoms of Dravet syndrome include:
Prognosis for Dravet Syndrome
Dravet syndrome is a lifelong condition and the symptoms do not improve. There is an increased risk of early death in about 15 percent to 20 percent of people with Drave syndrome due to sudden unexpected death in epilepsy (SUDEP), long-lasting seizures, seizure-related accidents like drowning, and infections.
Most people with Dravet syndrome (80% or more) live into adulthood. Around 92% of them will still experience seizures often.
Still, the life expectancy is reduced, and adulthood is short-lived. About 93% of people with Dravet syndrome die before the age of 20 years, mostly due to SUDEP.
What Triggers Dravet Syndrome?
Genetics
The disease is associated with a genetic defect in the SCN1A gene, which affects the sodium ion channel. More than 80 percent of people with Dravet syndrome have a mutation in that gene, but not all SCN1A mutations result in Dravet syndrome.
These genetic mutations appear to be new in about 90 percent of children. They are not inherited from a parent. A number of other genes have also been associated with Dravet syndrome but may produce slightly different symptoms.
Diagnosing Dravet Syndrome
Dravet syndrome is diagnosed based on a physician’s clinical evaluation. Diagnostic studies can support the diagnosis, but they do not confirm or exclude it. Clinical characteristics of Dravet syndrome include at least four of the following five characteristics:
Diagnostic Tests
Is There Treatment for Dravet Syndrome?
Dravet syndrome is a lifelong condition, so it can’t be cured. However, the symptoms can be treated. Treatment for the physical, cognitive, and behavioral problems of Dravet syndrome is individualized and may include physical therapy, speech therapy, and behavioral therapy.
Cannabidiol
In June 2018, the United States Food and Drug Administration (FDA) approved Epidiolex (cannabidiol) for treatment of Dravet syndrome as well as another epilepsy syndrome,Lennox Gastaut syndrome. Earlier studies showed that children with Dravet syndrome experienced a decrease in seizure frequency with Epidiolex and were able to tolerate the medication.
Medications That Worsen Dravet Syndrome
Standard anticonvulsants believed to have an effect on sodium channels are not recommended in the treatment of Dravet syndrome. These may worsen, rather than improve, seizures in Dravet syndrome:
Summary
Many of the symptoms of Dravet syndrome can partially improve with proper treatment. Treatment strategies may need to change as your child grows physically and as the condition changes with age.
7 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Dravet Syndrome Foundation.Dravet syndrome in adults.Dravet Syndrome Foundation.Dravet syndrome in adults.Shmuely S, Sisodiya SM, Gunning WB, Sander JS, Thijs RD.Mortality in Dravet syndrome: a review.Epilepsy Behav. 2016;64:69-74. doi:10.1016/j.yebeh.2016.09.007National Organization for Rare Disorders.Dravet syndrome: causes.Dravet Syndrome Foundation.What is Dravet syndrome?Dravet Syndrome Foundation.The diagnosis and treatment of Dravet syndrome.U.S. Food & Drug Administration.FDA approves first drug comprised of an active ingredient derived from marijuana to treat rare, severe forms of epilepsy.Additional ReadingDevinsky O, Cross JH, Laux L, et al. Trial of cannabidiol for drug-resistant seizures in the Dravet syndrome.N Engl J Med. 2017;376(21):2011-2020. doi:10.1056/NEJMoa1611618Wirrell EC. Treatment of Dravet syndrome.Can J Neurol Sci. 2016;43 Suppl 3:S13-8. doi:10.1017/cjn.2016.249.
7 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Dravet Syndrome Foundation.Dravet syndrome in adults.Dravet Syndrome Foundation.Dravet syndrome in adults.Shmuely S, Sisodiya SM, Gunning WB, Sander JS, Thijs RD.Mortality in Dravet syndrome: a review.Epilepsy Behav. 2016;64:69-74. doi:10.1016/j.yebeh.2016.09.007National Organization for Rare Disorders.Dravet syndrome: causes.Dravet Syndrome Foundation.What is Dravet syndrome?Dravet Syndrome Foundation.The diagnosis and treatment of Dravet syndrome.U.S. Food & Drug Administration.FDA approves first drug comprised of an active ingredient derived from marijuana to treat rare, severe forms of epilepsy.Additional ReadingDevinsky O, Cross JH, Laux L, et al. Trial of cannabidiol for drug-resistant seizures in the Dravet syndrome.N Engl J Med. 2017;376(21):2011-2020. doi:10.1056/NEJMoa1611618Wirrell EC. Treatment of Dravet syndrome.Can J Neurol Sci. 2016;43 Suppl 3:S13-8. doi:10.1017/cjn.2016.249.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Dravet Syndrome Foundation.Dravet syndrome in adults.Dravet Syndrome Foundation.Dravet syndrome in adults.Shmuely S, Sisodiya SM, Gunning WB, Sander JS, Thijs RD.Mortality in Dravet syndrome: a review.Epilepsy Behav. 2016;64:69-74. doi:10.1016/j.yebeh.2016.09.007National Organization for Rare Disorders.Dravet syndrome: causes.Dravet Syndrome Foundation.What is Dravet syndrome?Dravet Syndrome Foundation.The diagnosis and treatment of Dravet syndrome.U.S. Food & Drug Administration.FDA approves first drug comprised of an active ingredient derived from marijuana to treat rare, severe forms of epilepsy.
Dravet Syndrome Foundation.Dravet syndrome in adults.
Shmuely S, Sisodiya SM, Gunning WB, Sander JS, Thijs RD.Mortality in Dravet syndrome: a review.Epilepsy Behav. 2016;64:69-74. doi:10.1016/j.yebeh.2016.09.007
National Organization for Rare Disorders.Dravet syndrome: causes.
Dravet Syndrome Foundation.What is Dravet syndrome?
Dravet Syndrome Foundation.The diagnosis and treatment of Dravet syndrome.
U.S. Food & Drug Administration.FDA approves first drug comprised of an active ingredient derived from marijuana to treat rare, severe forms of epilepsy.
Devinsky O, Cross JH, Laux L, et al. Trial of cannabidiol for drug-resistant seizures in the Dravet syndrome.N Engl J Med. 2017;376(21):2011-2020. doi:10.1056/NEJMoa1611618Wirrell EC. Treatment of Dravet syndrome.Can J Neurol Sci. 2016;43 Suppl 3:S13-8. doi:10.1017/cjn.2016.249.
Devinsky O, Cross JH, Laux L, et al. Trial of cannabidiol for drug-resistant seizures in the Dravet syndrome.N Engl J Med. 2017;376(21):2011-2020. doi:10.1056/NEJMoa1611618
Wirrell EC. Treatment of Dravet syndrome.Can J Neurol Sci. 2016;43 Suppl 3:S13-8. doi:10.1017/cjn.2016.249.
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