Dravet Syndrome in Babies – Causes, Symptoms, and Treatment

Even with medical attention Dravet syndrome does not resolve entirely. Babies having Dravet syndrome can show a variety of seizure types and velocities with multiple developmental drawbacks.

The previous names of Dravet syndrome were epilepsy with polymorphic seizures, severe myoclonic epilepsy of infancy, and polymorphic epilepsy in infancy. It is then named after Charlotte Dravet, an epilepsy specialist, who describes this condition first in 1978.

How Common is Dravet Syndrome in Babies?

Dravet syndrome is a serious but fortunately rare genetic condition. As per a systemic review published by the journal Frontiers in Neurology, Dravet syndrome occurs in about 1 in every 20,000 to 1 in 40,000 babies. And about 3 to 8% of babies who experience their first seizure within 12 months may have Dravet syndrome.

The initial seizures will be prolonged and can involve one-half of the body. The impact side of the body may change with each episode of seizures. As an abnormal genetic mutation is a primary cause behind most Dravet syndrome cases, the condition becomes unpredictable and unpreventable.

Causes of Dravet Syndrome in Babies

There is no outward reason behind Dravet syndrome that you can prevent or control.

Dravet syndrome is a rare condition that generally occurs due to the mutation of the SCN1A gene. Studies show that almost 80% of babies suffering from Dravet syndrome have a mutated SCN1A non-sex gene. One may or may inherit Dravet syndrome from their family. So it is impossible to detect and control Dravet syndrome before one shows its symptoms.

Signs and Symptoms of Dravet Syndrome in Babies

There are some significant symptoms of Dravet syndrome in babies. If you timely notice these symptoms and seek a diagnosis then proper medical care can subdue the problem to some extent. Here are the symptoms of Dravet syndrome in babies

• A sudden seizure or epileptic fit usually occurs with a fever in babies below one year. This is known as a febrile seizure. But febrile seizures can occur in many babies who may not develop Dravet syndrome in the future. So this symptom alone cannot confirm Dravet syndrome.
• In the case of babies suffering from Dravet syndrome, the seizures will get frequent with time(once every few weeks). They can have a seizure that lasts for minutes or a series of seizures at a time. Some of these seizures can only affect one side of the body.
• A hindrance in the growth and development of the baby can happen after the occurrence of the febrile seizure.
• Difficulty in movement and balancing which cause problems in gross as well as fine motor skills is also seen.
• Some babies can show speech and behavioral disorders.
• Though fever is mostly associated with the seizures of Dravet syndrome, any reason for a rise in body temperature can induce them. So a hot water bath, exposure to hits in the summer, bright light, and too much excitement or stress can trigger fits of Dravet syndrome.

[Read : Epilepsy in Babies]

How is Dravet Syndrome Diagnosed?

If your baby experiences any sort of fits or seizures you need to immediately consult the doctor for a diagnosis.

• The doctor will ask you in detail about the fit, its time, and its impact.
• If the doctor suspects Dravet syndrome then your baby will have to go through a blood test for the SCN1A gene test. If they cannot find a mutated gene in the test then other diagnostic tests such as MRI, CT scans, and electroencephalograms can diagnose the condition.

How to Treat Dravet Syndrome in Babies?

There is no cure for Dravet syndrome for now. Scientists are working hard to find out a cure but up until that day, medicines can only shorten the length and severity of the seizures. The treatment of Dravet syndrome includes

1. Medications

Anti-seizure drugs such as Cannabidiol, Stiripentol, and Fenfluramine HCI for babies of two years or more. They are helpful to control long seizures that often lead to medical emergencies.

Sometimes one medicine alone will not be sufficient to subdue the fits. The doctor will try combinations of medicines to check what best suits your baby’s condition. According to the recommendation of the North American Consensus Panel doctors should follow the patterns of first-line medicine, then second-line treatment and then vouch for third-line treatment.

2. Diet Modifications

Following a Ketogenic diet or a diet rich in protein and fat but low in carbohydrates is beneficial for infants having Dravet syndrome. The doctor can especially design this diet according to the condition of your baby. This is a second-line treatment that you can try if the medicines do not prove to be effective.

3. Nerve Stimulation

According to research study reports published by Science Direct, Vagus nerve stimulation treatment can reduce seizures by 50 to 63%. This is a third-line treatment. In this treatment, the doctor inserts a VNS or Vagus nerve stimulator under the neck skin of the baby to stimulate the vagus nerve on time through electrical impulses. This electrical impulse combats the abnormal impulse of the brain that is causing seizures.

4. Immunity Boosting

As poor immunity can also induce epilepsy, the doctor can provide your baby with intravenous immunoglobulin to boost immunity.

You will learn and observe with time that certain factors induce seizures in your baby. By avoiding such factors you can avoid seizures. Parents having children with Dravet syndrome often dress them with light breathable clothes and maintain a normal and stress-free environment around them.

Babies having Dravet syndrome show normal growth until they are 1 or 2 years old or experience the first seizure. Dravet syndrome delayed their physical and mental development. Development speed may get quicker after they cross 6 years, but this will impact their entire life. According to a report, almost 10 to 20% of infants with Dravet syndrome die before their adulthood. The rate of accidental deaths or drowning due to sudden seizure attacks is quite common in the case of Dravet syndrome. So parents need to be excessively protective with a baby having Dravet syndrome.

FAQ’s

1. Does Dravet Syndrome Cause Brain Damage?

Yes, as seizures in babies having Dravet syndrome occurs within the first 12 months, the underdeveloped brain of the baby gets severely affected. That is why babies show abnormality in physical, cognitive as well as behavioral development after the first seizure of Dravet syndrome.

2. Is Dravet Genetic?

Yes, Dravet syndrome is a rare genetic problem. Dravet is an autosomal dominant genetic disorder which means only one of the 22 nonsex chromosomes can cause this disease if it has an abnormal mutation. Dravet syndrome may or may not be inherited from the parents which means it may or may not run in families but is something that comes at birth.

Read Also: Hypotonia in Babies – Signs, Causes, Diagnosis and Treatment