What is the life expectancy of a person with Dravet syndrome?

What is the life expectancy of a person with Dravet syndrome?

The average life expectancy of people with Dravet syndrome is not clear, but estimates suggest that 10–20% of individuals with Dravet syndrome do not survive beyond the age of 10. Sudden unexpected death in epilepsy (SUDEP) is the most common cause.

Is Dravet syndrome autism?

Many children with Dravet syndrome show signs of autism, such as mild social deficits and repetitive behaviors. In a 2011 study, researchers found that roughly one in four children with Dravet syndrome have autism.

What causes Dravet?

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.

Does Dravet syndrome get worse?

The seizures experienced by people with Dravet syndrome become worse as the patient ages, as the disease is not very observable when symptoms first appear.

Can you grow out of Dravet syndrome?

Lifespan. An estimated 10-20 percent of Dravet syndrome patients pass away before reaching adulthood.

What is the most severe form of epilepsy?

Lennox-Gastaut syndrome (LGS) is a severe form of epilepsy that typically becomes apparent during infancy or early childhood. Affected children experience several different types of seizures most commonly atonic, tonic and atypical absence seizures.

Can adults have Dravet syndrome?

Adult patients suffer multiple co-mordities in addition to the seizures, including but not limited to cognitive disabilities, behavioral challenges, decreased mobility, sleep issues, and gastrointestinal issues. Characteristics of Dravet syndrome often change in adulthood.

Who is at risk for Dravet syndrome?

Reports suggest 1 in 20,000 to 1 in 40,000 people have Dravet syndrome. Three to 8% of children who have their first seizure by 12 months old may have Dravet syndrome.

What are the symptoms of Dravet syndrome?

Dravet syndrome (DS) is a severe form of epilepsy characterized by frequent, prolonged seizures often triggered by high body temperature (hyperthermia), developmental delay, speech impairment, ataxia, hypotonia, sleep disturbances, and other health problems.

What is the most severe type of epilepsy?

Lennox-Gastaut syndrome (LGS) is a severe form of epilepsy that typically becomes apparent during infancy or early childhood. Affected children experience several different types of seizures most commonly atonic, tonic and atypical absence seizures.

What is the life expectancy of someone with Dravet syndrome?

• Dravet syndrome patients tend to have a much lower than normal life expectancy. They may require medical care for the rest of their lives. Effective management of the condition can prolong their life expectancy. The average life expectancy of a Dravet Syndrome child is seven or eight years.

What is the cause of Dravet syndrome?

• The condition usually isn’t inherited from parents. Most cases are caused by a mutated gene called SCN1A. About 80% of people with Dravet syndrome have the altered SCN1A gene. Dravet syndrome can be hard to diagnose, partly because it’s so rare.

What is the history of Dravet syndrome?

• Dravet syndrome is a rare and severely disabling type of epilepsy presenting in infancy, sometimes called a catastrophic epilepsy. It was first described in 1978 by Charlotte Dravet, a paediatric epilepsy specialist working in Marseille.

How does Dravet syndrome affect development?

• Dravet syndrome is a rare form of epilepsy that begins during infancy and can result in moderate to severe developmental delays.
• Many people diagnosed with the condition have a gene mutation in the SCN1A gene.
• Medications,therapies,and lifestyle changes can help manage the symptoms of Dravet syndrome or reduce the length and frequency of seizures.