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Dravet syndrome

Dravet syndrome is a rare, catastrophic form of epilepsy associated with neurological development disorders. The onset is during the first year of life in a normal developing child.1‑3

Dravet syndrome was first described in 1978 as severe myoclonic epilepsy in infancy (SMEI) by Dr Charlotte Dravet.4

The first description of the clinical picture was:

In the first year of life, seizures appeared in an otherwise normally developing infant5:

  • Generalized or unilateral clonic seizures, with or without fever5
  • These seizures lasted, in some cases, more than 20 minutes and required emergency treatment (rectal or intravenous injection of an anti-convulsive drug)3,5

Between 2–3 years of age, other types of seizures developed6:

  • Myoclonic seizures, atypical absences, and partial (focal) seizures occurred, accompanied by developmental regression or stagnation with behavior disorders3,4
  • The electroencephalogram (EEG) remained normal4

As it was discovered that not all of the original clinical signs were present in every patient, the diagnostic criteria were expanded.7

In 2001, the International League Against Epilepsy changed the epilepsy syndrome name from SMEI to Dravet syndrome, which is how it is now known throughout the world.8,9

Dravet syndrome is estimated to appear in

1/15,700 births10

in the United States, with approximately 2,800 patients 2–18 years of age.11

Worldwide, Dravet syndrome seems to affect an equal number of boys and girls, and prevalence does not differ drastically across geographic regions.1,10,13‑16


1. Villas N, Meskis MA, Goodliffe S. Dravet syndrome: characteristics, comorbidities, and caregiver concerns. Epilepsy Behav. 2017;74:81‑86. 2. Schmuely S, Sisodiya SM, Gunning WB, Sander JW, Thijs RD. Mortality in Dravet syndrome: a review. Epilepsy Behav. 2016;64(pt A):69‑74. 3. Dravet C. The core Dravet syndrome phenotype. Epilepsia. 2011;52(suppl 2):3‑9. 4. Dravet C. Les épilepsies graves de l’enfant [Severe epilepsy in children]. Vie Médicale. 1978;8:543‑548. 5. Dravet C, Oguni H. Dravet syndrome (severe myoclonic epilepsy in infancy). In: Dulac O, Lassonde M, Sarnat HB, eds. Handbook of Clinical Neurology. Vol 111. Amsterdam, Netherlands: Elsevier; 2013:627‑631. 6. Ohki T, Watanabe K, Negoro T, et al. Severe myoclonic epilepsy in infancy: evolution of seizures. Seizure. 1997;6(3):219‑224. 7. Dravet C. Dravet syndrome history. Dev Med Child Neurol. 2011;53(suppl 2):1‑6. 8. European Medicines Agency. EPAR – Scientific Discussion: Diacomit. Published 2007. Accessed March 12, 2019. 9. Millichap JJ, Koh S, Laux LC, Nordli DR Jr. Child neurology: Dravet syndrome: when to suspect the diagnosis. Neurology. 2009;73(13):e59‑e62. 10. Wu YW, Sullivan J, McDaniel SS, et al. Incidence of Dravet syndrome in a US population. Pediatrics. 2015;136(5):e1310‑e1315. 11. European Medicines Agency. Relevant sources for orphan disease prevalence data. Published December 16, 2014; valid through January 9, 2018. Accessed March 12, 2019. 12. United States Census Bureau. Annual estimates of the resident population by single year of age and sex for the United States, states, and Puerto Rico Commonwealth: April 1, 2010 to July 1, 2017. Accessed March 7, 2019. 13. Skluzacek JV, Watts KP, Parsy O, Wical B, Camfield P. Dravet syndrome and parent associations: the IDEA League experience with comorbid conditions, mortality, management, adaptation, and grief. Epilepsia. 2011;52(suppl 2):95‑101. 14. Brunklaus A, Ellis R, Reavey E, Forbes GH, Zuberi SM. Prognostic, clinical and demographic features in SCN1A mutation-positive Dravet syndrome. Brain. 2012;135(pt 8):2329‑2336. 15. Rosander C, Hallböök T. Dravet syndrome in Sweden: a population-based study. Dev Med Child Neurol. 2015;57(7):628‑633. 16. Bayat A, Hjalgrim H, Møller RS. The incidence of SCN1A‑related Dravet syndrome in Denmark is 1:22,000: a population-based study from 2004 to 2009. Epilepsia. 2015;56(4):e36‑e39. doi:10.1111/epi.12927.


DIACOMIT (stiripentol) capsules for oral use or powder for oral suspension are indicated for the treatment of seizures associated with Dravet syndrome in patients 2 years of age and older taking clobazam.

There are no clinical data to support the use of DIACOMIT as monotherapy in Dravet syndrome.


No contraindications are listed.


DIACOMIT can cause somnolence. Monitor patients for somnolence, particularly when DIACOMIT is used concomitantly with other CNS depressants or clobazam, which is also known to cause somnolence.

Decreased Appetite and Decreased Weight

DIACOMIT can cause decreases in appetite and weight. The growth and weight of pediatric patients treated with DIACOMIT should be carefully monitored.

Neutropenia and Thrombocytopenia

DIACOMIT can cause significant declines in neutrophil and platelet counts. Hematologic testing should be obtained prior to starting treatment with DIACOMIT and then every 6 months.

Withdrawal Symptoms

As with most antiepileptic drugs (AEDs), DIACOMIT should be gradually withdrawn to minimize the risk of increased seizure frequency and status epilepticus.

Risks in Patients with Phenylketonuria (PKU)

DIACOMIT powder for suspension contains phenylalanine, which can be harmful to patients with PKU. Before prescribing DIACOMIT powder for suspension to a patient with PKU, consider the total daily intake of phenylalanine from all sources, including DIACOMIT powder for suspension. DIACOMIT capsules do not contain phenylalanine.

Suicidal Behavior and Ideation

AEDs, including DIACOMIT, increase the risk of suicidal thoughts or behavior. Patients treated with any AED for any indication should be monitored for the emergence or worsening of depression, suicidal thoughts or behavior, and/or any unusual changes in mood or behavior.


The most common adverse reactions that occurred in at least 10% of DIACOMIT-treated patients and more frequently than on placebo were somnolence, decreased appetite, agitation, ataxia, decreased weight, hypotonia, nausea, tremor, dysarthria, and insomnia.


There are no adequate data on the developmental risks associated with the use of DIACOMIT in pregnant women. Based on animal data, DIACOMIT may cause fetal harm.

There is a pregnancy exposure registry that monitors pregnancy outcomes in women exposed to AEDs, such as DIACOMIT, during pregnancy. Physicians are advised to recommend that pregnant patients taking DIACOMIT enroll in the North American Antiepileptic Drug (NAAED) Pregnancy Registry (information at This can be done by calling the toll free number 1‑888‑233‑23341‑888‑233‑2334, and must be done by patients themselves or their caregiver.

To report suspected adverse reactions, contact BIOCODEX at 1‑866‑330‑30501‑866‑330‑3050 or FDA at 1‑800‑FDA‑10881‑800‑FDA‑1088 or

Please see full prescribing information for DIACOMIT®.