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Dravet Syndrome

Severe genetic epilepsy starting with prolonged febrile seizures in early childhood.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Dravet Syndrome?

Dravet syndrome is a severe epileptic encephalopathy that begins in infancy (usually within the first year) with prolonged febrile seizures, progressing later with multiple seizure types, developmental delay and gait disturbance.

In the great majority of cases, de novo mutations in the SCN1A gene are responsible, with impairment of voltage-gated sodium channel function.

The disease continues into adulthood; since diagnosis is often delayed, it should be considered when febrile seizures recur.

Symptoms

Prolonged clonic seizures triggered by fever
Myoclonic, atypical absence and focal seizures
Sensitivity to triggers such as heat and bathing
Developmental delay after age 2
Ataxic, stiff gait
Behavioral problems and autistic features
Risk of SUDEP (sudden unexpected death)

Risk Factors

De novo mutation in the SCN1A gene
Family history of epilepsy (rarely)
Early-onset history of febrile seizures

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Febrile seizure lasting more than 15 minutes in infants
  • Coexistence of multiple seizure types
  • Resistant and recurrent seizures
  • Plateauing of developmental milestones
  • Seizures triggered by heat exposure

Treatment Methods

01
Triplet of valproic acid, clobazam, stiripentol
02
Cannabidiol (CBD) treatment
03
Fenfluramine approved treatment option
04
Ketogenic diet
05
Avoidance of sodium channel blockers (carbamazepine, lamotrigine)
06
Genetic counseling and SUDEP prevention education

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.