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Key Information About Dravet Syndrome

Updated on November 28, 2022
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Srikanth is passionate about helping people improve their quality of life.

Dravet syndrome is also known as Severe Myoclonic Epilepsy of Infancy (SMEI). A US epidemiology study concluded that it is twice as common as previously recognized, affecting 1:15,700 infants, 80 percent of whom have an SCN1A mutation.

“In the case of Dravet syndrome, the children develop completely normally after birth until they suffer their first epileptic seizure at the age of 3 to 9 months,” explains Dr. Ulrike Hedrich-Klimosch, biologist.


The syndrome was first described in 1982 by a French doctor named Charlotte Dravet and was initially named "severe myoclonic epilepsy of infancy." The name was later changed to honor the doctor who discovered it.


Dravet syndrome is believed to be caused by a defect in the function of the sodium channels and is described as a form of channelopathy.

This severe form of epilepsy is usually not inherited from parents. Most cases are caused by a mutated gene known as SCN1A. About 80 percent of Dravet syndrome patients have the altered SCN1A gene.

The SCN1A gene belongs to a family of genes which provide instructions to make sodium channels.

These channels, which transport positively charged sodium atoms into cells, play an important role in a cell's ability to generate and transmit electrical signals.

Scientists think that people with Dravet syndrome may have alterations in the serotonin signaling pathway, but exact problems are not known.

Research scientists from Children's Hospital of Philadelphia found that dysfunction in an important cell subtype in the brain's neuronal network contribute to chronic symptoms in Dravet syndrome.

The findings were published on March 29 2022 in the journal Cell Reports.


Dravet syndrome is characterized by seizures, cognitive deficits, and increased mortality.

This first seizure is usually febrile. These febrile seizures are usually so-called tonic-clonic seizures characterized by stiffness, unconsciousness, and jerking.

In infants with Dravet syndrome, febrile convulsions may be unusually long, sometimes lasting 15 to 30 minutes or longer.

Adult patients with Dravet syndrome may experience a decrease in seizures with age, but motor symptoms and gait become progressively worse, according to study findings published in the journal Neurology.

The study was conducted among patients who were identified at the Adult Epilepsy Genetic Clinic at Toronto Western Hospital in Toronto, Ontario, Canada, between 2010 and 2020.

Not only do young people with Dravet syndrome show cognitive, developmental and behavioral differences compared with their normally developing peers, but these differences tend to become more pronounced in adolescence, according to a study that was published in Epilepsy & Behavior in November 2022.



As children with Dravet syndrome get older, their decline in cognitive function stabilizes. The degree of intellectual disability varies widely from mild to profound, yet most teenages and adults with Dravet syndrome are dependent on caregivers.


Genetic blood tests can screen for SCN1A mutations and a few other mutations linked to Dravet syndrome.

"When my daughter Austen, now 6, started having seizures years ago due to Dravet syndrome, it took us over six months to receive a diagnosis," said Meagan Cheney, who lives in Colorado with her husband and three children.


Seizures in Dravet syndrome are difficult to control, but can be reduced by anticonvulsant drugs.

Stiripentol, sold under the brand name Diacomit, is an anticonvulsant medication used for the treatment of Dravet syndrome

Valproate, benzodiazepines, levetiracetam and sodium channel blockers are other commonly used medications to treat this disease.

A ketogenic diet, high in fats and low in carbohydrates, also may be beneficial.

Treatment with low-dose aripiprazole — a newer class of antipsychotic medication — in combination with psychotherapy, and behavioral and occupational therapy, was found to lessen behavior, attention, and educational problems in a 6-year-old girl with Dravet syndrome, according to a case study conducted in July 2022.

Fenfluramine, sold under the brand name Fintepla, is a serotonergic medication used for the treatment of seizures associated with Dravet syndrome.

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It is impossible to prevent Dravet syndrome because it is a genetic mutation, with no known causes.

However, if your child is diagnosed with this disease you can prevent potential triggers like fever, illness, or any change of temperature.

One important aspect is to control triggers enabling the child to lead a good quality of life without disabling their daily activities.

  • Dravet syndrome is also called SMEI.
  • It was first described by a French doctor named Charlotte Dravet.
  • It is described as a form of channelopathy.
  • About 80 percent of SMEI patients have the altered SCN1A gene.
  • In infants with SMEI, febrile convulsions sometimes last more than half an hour.
  • Genetic blood tests are used to diagnose Dravet syndrome.
  • Keto diet benefits SMEI patients.
  • It is impossible to prevent Dravet syndrome.

This content is accurate and true to the best of the author’s knowledge and does not substitute for diagnosis, prognosis, treatment, prescription, and/or dietary advice from a licensed health professional. Drugs, supplements, and natural remedies may have dangerous side effects. If pregnant or nursing, consult with a qualified provider on an individual basis. Seek immediate help if you are experiencing a medical emergency.

© 2019 Srikanth R


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