How common is Dravet Syndrome?

Dravet syndrome affects an estimated 1:15,700 individuals in the U.S., or 0.0064% of the population (Wu 2015). Approximately 80-90% of those, or 1:20,900 individuals, have both an SCN1A mutation and a clinical diagnosis of DS.

Can you grow out of Dravet syndrome?

While there is no cure for Dravet syndrome, early diagnosis and treatment may minimize seizure frequency and intensity. Without treatment, individuals will continue to have frequent seizures and can suffer from seizure-related accidents or even mortality.

Is Dravet syndrome life limiting?

Dravet syndrome is a rare, severe, and lifelong form of epilepsy (seizure disorder). Most people affected by this condition have a good life expectancy. The disease typically starts in the first year of life, and around 80-85% of the children survive into adulthood.

What is the rarest form of epilepsy?

Dravet syndrome is a rare, catastrophic, lifelong form of epilepsy that begins in the first year of life with frequent and/or prolonged seizures. Previously known as Severe Myoclonic Epilepsy of Infancy (SMEI) 80% of people have a mutation in their SCN1A gene.

What triggers Dravet syndrome?

In at least 80 percent of cases, Dravet syndrome is caused by defects in a gene required for the proper function of brain cells. Mutations in the SCN1A gene (a gene that encodes as a sodium channel, a part of the cell membrane involved in nervous system function) are the primary causes of Dravet syndrome.

Dravet Syndrome Overview

At what age is Dravet syndrome diagnosed?

Dravet syndrome is a clinical diagnosis. Presentation is uniquely characteristic and, according to the 2017 consensus of North American neurologists with expertise in DS, includes: Typical onset between 1 and 18 months, most often <12 months, average 5.2 (Cetica 2017, Wirrell 2017)

Are you born with Dravet syndrome?

No. In most children (80%), Dravet syndrome is caused by a genetic mutation in the SCN1A gene.

Is Dravet syndrome progressive?

Dravet syndrome is a severe and progressive genetic epilepsy characterized by frequent, prolonged and refractory seizures that usually begin within the first year of life.

How is Dravet syndrome inherited?

Most cases of Dravet syndrome occur when the SCN1A gene is not working correctly. It can be inherited in an autosomal dominant pattern, but most people with Dravet syndrome do not have a family history of the condition. Diagnosis is based on a clinical exam, medical history, and the results of genetic testing.

What does Dravet syndrome look like?

Seizures lasting more than 10 minutes, seizures occurring on one side of the body, and seizures triggered by a warm water bath in children under 12 months old are clues for a Dravet syndrome diagnosis.

Does Dravet syndrome cause brain damage?

Dravet syndrome is a disease characterized by prolonged and frequent seizures that begin in the first year of life and are difficult to control with medication. These seizures are harmful to the developing brain and can cause cognitive and behavioral impairment in patients.

What is the life expectancy of a child with epilepsy?

Ninety-six percent of these children reached the age of 10 years, 89% the age of 20 years and 80% the age of 40 years. In the same study, 87% of children with idiopathic seizures reached 40 years of age, compared with 93% of those with cryptogenic seizures and 73% of those with remote symptomatic seizures.

Can Dravet syndrome be caused by vaccines?

Fever is the most common trigger of seizures in Dravet patients. Because vaccination frequently results in vaccine-induced fever, it becomes the trigger in about 50% of these patients' first seizures. However, vaccines do not cause Dravet syndrome and they do not change the course of the disease.

Does CBD help Dravet syndrome?

CBD resulted in a significant decrease of convulsive seizures and seizures of all types in Dravet syndrome, a pharmacoresistant epilepsy known to be associated with high mortality rates.

How do you test for Dravet syndrome?

Doctors use a blood test to screen for the SCN1A gene and other genetic changes linked to Dravet syndrome. Your child might need a genetic test if they: Had two or more seizures by age 1 that lasted more than 10 minutes each. Had one long seizure and one seizure that caused jerking on one side of their body by age 1.

Is frontal lobe epilepsy rare?

Frequency. ADNFLE appears to be an uncommon form of epilepsy; its prevalence is unknown. This condition has been reported in more than 100 families worldwide.

Is Jeavons syndrome rare?

Jeavons syndrome is very rare and accounts for only 1-2% of people seen at specialty epilepsy centers. Jeavons syndrome is a rare form of generalized epilepsy that can have several possible seizure types ³⁾: Eyelid myoclonia with or without absence seizures: Eyelid myoclonia is the most common seizure type.

Can a febrile seizure cause autism?

Dr. Capal: There is no scientific evidence linking febrile seizures to autism.

What foods should epileptics avoid?

white bread; non-wholegrain cereals; biscuits and cakes; honey; high-sugar drinks and foods; fruit juices; chips; mashed potatoes; parsnips; dates and watermelon. In general, processed or overcooked foods and over-ripe fruits.

Which famous person has epilepsy?

Vincent van Gogh suffered from seizures doctors believed were cause by temporal lobe epilepsy. Composer George Gershwin was diagnosed with epilepsy. Actress Margaux Hemingway had from epilepsy from the age of seven. Korn front man Jonathan Davis believes his epilepsy was induced by years of drug use.

Do seizures shorten your life?

Epilepsy can shorten life, but most of the time it does not. Be aware of risks and take sensible precautions, but do not let the risks put you into a state of constant worry. Consult with your doctor for specific information about your risk factors for injury due to seizures.

Do seizures damage your brain?

Most types of seizures do not cause damage to the brain. However, having a prolonged, uncontrolled seizure can cause harm. Because of this, treat any seizure lasting over 5 minutes as a medical emergency.

Can I fly with epilepsy?

While most people with seizures can travel safely by airplanes, guidelines of the Aerospace Medical Association do recommend that people with uncontrolled seizures not travel by commercial airliner. An airline may ask for a letter from your doctor that you are okay to fly.

How likely is a second seizure?

The chance of another seizure can range from 16% to 61%, depending on the circumstances surrounding the seizure and results of a neurological exam or other tests.

Can I marry a girl with epilepsy?

A: From a medical point of view, persons with epilepsy can marry. However, in some countries there have been laws and in others even now there are laws that prevent persons with epilepsy to marry. It is important that the would be spouse of a person with epilepsy should be aware of the situation.