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The Unexpected Triggers of Reflex Seizures in Dravet Syndrome: Warm baths, excitement, and even.. diaper changes?


Did you know there is such a thing as a seizure disorder that can cause seizures due to changes in non-infectious body temperature, excitement, or emotional stress? Neither did I, until recently.


As Registered EEG Technologists, we do a lot of research for taking our boards and see a lot of different seizure disorders while working in the field. One disorder I had never learned about until recently was Dravet Syndrome. It is one of the more severe types of epilepsy syndromes.


In this field, you will never stop learning!

 

Some background on Dravet Syndrome:

It is a rare, drug-resistant epilepsy that begins in the first year of life. The disease begins in infancy and is lifelong. Infants typically have a normal development when seizures begin, but as seizures continue, most children develop some level of developmental delay.

Dravet Syndrome is so rare, which makes it hard to diagnose. A physician will ask about the child’s seizure history during the exam, and if they think the child has Dravet Syndrome, they will be tested to see if the SCN1A gene is mutated. The physician will look for how often the patient has seizures, the motor and developmental skills of the patient before the first seizure if the patient has had two or more seizures lasting 10 minutes, and if there is a lack of improvement with anticonvulsants.



Seizures with Dravet Syndrome:

The first seizure with Dravet Syndrome usually occurs with a fever and it may be

a tonic-clonic seizure or a seizure including clonic movements on one side of the body.

These are often long, lasting more than 5 minutes, and can result in status epilepticus.

After the first seizure with a fever, seizures can happen again without a fever. These

children will be very sensitive to infections and frequently have seizures when they are

ill or have a fever.

Back to what I said in the beginning, the part that stood out to me the most, is that seizures can be triggered by a slight change in body temperature that is not caused by an infection. For example, a warm bath, intense exercise, or hot weather can be triggers for seizures. Even excitement or emotional stress may trigger seizures. Some children may have photosensitive seizures triggered by flashing lights. There is even research stating a young 9-month-old girl with Dravet Syndrome had a seizure from a diaper change. You can read the article here. These seizures are classified as reflex seizures. Reflex seizures happen when a certain trigger or stimulus brings on seizures.


 



This child's name is Frankie, he has been diagnosed with Dravet Syndrome since 11 months old. Frankie's seizures were so severe, that within 5 minutes he would seize being outside in the summer. Because of this, his parents had to take him to the park at night time. Frankie would even have to stay inside during the winter because the sun glaring off of the snow. This shows how severe Dravet Syndrome is.


Thankfully, Frankie is doing better after a trial of fenfluramine and is living his life to the fullest despite still having Dravet Syndrome.

You can read more about Frankie's story here.

 

Treatment for Dravet Syndrome:

There is currently no cure for Dravet Syndrome, and it is one of the epilepsy syndromes that are most resistant to epilepsy medicines. Treatment focuses on controlling or minimizing seizures. Medication cannot entirely prevent or control all Dravet Syndrome seizures, but it may help. Usually, 2 or more seizure medications are needed to treat seizures with this syndrome.

Dressing in cool vests on hot days, putting sunglasses on in bright light, a Vagus Nerve Stimulator and a ketogenic diet are some ways to reduce seizure activity in Dravet Syndrome that don't include medication.


You don't have to worry that if you go outside you or your child will have a seizure if it is hot outside. Dravet Syndrome is extremely rare. About 1 in 20,000 or 1 in 40,000 people have Dravet Syndrome, and about 80% have a SCN1A gene mutation.




Taylor Evans, R. EEG T.



References:

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