Introduction

Dravet syndrome is a rare, lifelong condition that usually starts in the first year of life. It affects a child’s brain and how nerve cells send signals.

One of the main features of Dravet syndrome is repeated, long seizures.

Over time, children may also develop:

• developmental delay or learning difficulties
• delayed speech and language development
• behavioural challenges
• difficulty with movement and coordination
• sensitivities to temperature changes in the body or environment
• sleep disturbances.

How Dravet syndrome affects each child can be different. It is hard to know exactly how the condition will progress.

Dravet syndrome affects about 1 in 16,000 children. In around 80% of cases, it is caused by a change in the SCN1A gene which helps to make and send signals in the brain. Less commonly, Dravet syndrome can also be caused by changes in other genes.

There is currently no cure for Dravet syndrome. Early diagnosis and careful management can help improve a child’s quality of life.

The main symptom of Dravet syndrome is seizures. Seizures are uncontrolled changes in your child’s movement and behaviour. They are caused by a disruption in the brain's normal electrical activity, and may cause your child to lose consciousness or pass out.

Most children with Dravet syndrome start having seizures between 4 and 8 months old. In some cases, seizures can start as late as 20 months.

Seizures can be triggered if your child:

has a fever or high temperature

• is in a hot environment, like a hot bath
• is sick, like with a cold or gastroenteritis
• has just had a vaccination
• sees flashing lights - in older children.

It is important to diagnose Dravet Syndrome early so your child can get the right treatment.

Your child’s doctor will diagnose Dravet syndrome by looking at your child’s general health and development, and their seizure history. This includes:

• types of seizures
• when they happen
• how long they last for.

Your child’s doctor will also do a physical check and ask about their medical history. 

They may also order some tests, including:

• electroencephalogram (EEG)- a test that records electrical activity in the brain using small sensors on the head
• genetic testing – to look for changes in SCN1A gene and other genes that can cause similar conditions. 

Treatment of Dravet syndrome focuses on 

• reducing the number and length of seizures
• supporting your child’s overall development and quality of life.

Treatment may include: 

• anti-seizure medication – the right type of anti-seizure medication is important. Some anti-seizure medications need to be avoided in Dravet Syndrome.
• avoiding or limiting triggers - things like hot baths, flashing lights, fever and illness which can make seizures more likely
• therapies - like physiotherapy, occupational therapy and speech therapy to help with movement, learning and daily skills.

It is important to work with your child’s doctor to develop a clear management plan.

This can include:

• what to do if a seizure happens
• when and how to use emergency medication for long seizures
• a “sick day plan” to lower the risk of seizures when your child is unwell or has a fever.

Research is ongoing, and new treatments are being developed that may improve life for children and adults with Dravet syndrome in the future.