HCPs for Children
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Could it be Dravet Syndrome in Children?

Dravet syndrome is a rare genetic condition whose course is variable from patient to patient. Most individuals with Dravet syndrome have prolonged seizures, often febrile, including status epilepticus before age one, along with multiple seizure types that are resistant to treatment; developmental delays; lowered immunity; orthopedic concerns; and hyperactivity. Some, but not all patients have a family history of febrile seizures or seizure disorders.

A recent U.S. epidemiology study concluded that Dravet syndrome is twice as common as previously recognized, affecting 1:15,700 infants, 80% of whom have an SCN1A mutation [1]. Tragically, a large number of children are not receiving an early diagnosis which could have a dramatic impact on long term development and quality of life. The study concluded that genetic testing via an epilepsy panel should be considered in children with 2 or more prolonged seizures by 1 year of age.

Genetic testing should be considered for any of the following:

  • 2 or more prolonged seizures by 1 year of age
  • 1 prolonged seizure and any hemi-clonic seizure by 1 year of age
  • 2 seizures of any length that seem to affect alternating sides of the body
  • History of seizures prior to 18 months of age and later emergence of myoclonic and/or absence seizures

A child may have Dravet syndrome without meeting the above criteria, as there are many additional symptoms and presentations that could indicate Dravet syndrome. Individuals with Dravet syndrome are often misdiagnosed with another seizure disorder (such as Lennox-Gastaut syndrome) or given a broad diagnosis of intractable epilepsy because infants with Dravet syndrome are initially developmentally on track with no delays until the second year of life. A correct and early diagnosis can have positive, long-lasting effects on the overall development of an individual with Dravet syndrome. A diagnosis at ANY AGE can benefit the patient and often improve his or her quality of life.

It is with the help of our dedicated neurologists, epileptologists, neurosurgeons, and neuroscientists that our children have been diagnosed quicker and more efficiently to provide them with access to information on potential treatment options.

If you would like to order a box of complimentary patient educational materials for your office, please contact us.

ICD-10 Codes for Dravet Syndrome

Having the appropriate coding in a patient’s medical record may also make it easier to secure coverage for indicated medications and medical testing required for recognized co-morbidities of the disease. And, without a specific ICD-10 code, it is difficult to track how many people have the disease and where they are located. If patients are not being properly coded, we might not be accurately tracking all of the characteristics of the disease, as well as assuring that patients are receiving appropriate care.

The codes are:                   

  • G40.83    Dravet syndrome

    Polymorphic epilepsy in infancy (PMEI)
    Severe myoclonic epilepsy in infancy (SMEI)

  • G40.833 Dravet syndrome, intractable, with status epilepticus
  • G40.834 Dravet syndrome, intractable, without status epilepticus

Professional Resources

Transition Document

Preparing the transition between child and adult HCPs

Clinical Trials

Clinical trials & registries available for your patients

Physician Directory

Join a directory of physicians for Dravet syndrome

Research Grants

Funding for research directly related to Dravet syndrome

DSF Family Network

Connect your patients with the DSF Family Network

Treatment Consensus

View the International Treatment Consensus

Educational Webinars

Presentations on relevant topics for Dravet syndrome

Surgery in DS Patients

Considerations for surgery and other procedures

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