Treatment/Management
Children and Adults with Dravet syndrome experience multiple seizure types that are resistant to most anti-epileptic medications. Currently, the evidence supports the use of “rational polytherapy” which consists of a step-wise introduction of medications that have been shown to improve seizure control in patients with Dravet syndrome until the patient either responds favorably or experiences unacceptable side effects. It must be emphasized that significant differences exist between countries with regard to drug dose preferences and availability of anti-epileptic medications.
The following medications have been shown to benefit patients with Dravet syndrome:
- divalproex sodium and derivatives (Depakote, Depakene,Epilim, Epival, Micropakine)
- topiramate (Topamax)
- stiripentol (Diacomit)
- clobazam (Frisium, Urbanyl)
- clonazepam (Klonipin, Rivotril)
- levetiracetam (Keppra)
- bromides
The following medications may aggravate seizures in Dravet syndrome:
- lamotrigine (Lamictal)
- phenytoin (Dilantin, Epanutin)
- fosphenytoin (Cerebyx, Prodilantin)
- carbamazepine (Tegretol, Calepsin, Cargagen, Barbatrol, Epitol, Finlepsin, Sirtal, Stazepine)
- oxcarbazepine (Trileptal)
- vigabatrin (Sabril, Sabrilan, Sabrilex)
Non-pharmacologic therapy with the ketogenic diet has been shown to improve seizure control in a significant percentage of children with Dravet syndrome.
Focal resective surgery is usually not helpful as SMEI is a systemic disorder without identifiable focal pathology.
Read more about this topic: Generalized Epilepsy With Febrile Seizures Plus
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