Pediatr. praxi. 2017;18(2):100-102 | DOI: 10.36290/ped.2017.017

Dravet syndrome in paediatric practice

MUDr. Štefánia Aulická, Ph.D.¹, MUDr. Ondřej Horák¹, MUDr. Katarína Brunová¹, doc. RNDr. Lenka Fajkusová, CSc.², Mgr. Jana Pavloušková², doc. MUDr. Hana Ošlejšková, Ph.D.²

1Klinika dětské neurologie LF MU a FN Brno, Centrum pro epilepsie, Brno, 2Centrum molekulární biologie a genové terapie Interní hematoonkologické kliniky LF MU a FN, Brno

Dravet syndrome is classified as a rare progressive genetically determined epilepsy. The onset of epilepsy is typically during the

first year of life in normal developed children and it´s characterized by frequently prolonged unilateral or generalized clonic seizures,

often associated with fever. Differential diagnosis between classical febrile seizures and initial phase of Dravet syndrome is

fundamental. In the next period, other types of seizures can occur (atypical absences, myoclonic seizures, partial complex seizures)

accompanied by significant deterioration of psychomotor development. Genetic examination is essential for confirmation of the



diagnosis: In the treatment asserts valproate, clobazame and stiripentol. Pharmacoresistance is often. In non-pharmacological

treatment implantation of vagal stimulator and ketogenic diet can be used. The prognosis is serious.

Keywords: Dravet syndrome, febrile seizures, psychomotoric deterioration, pharmacoresistance

Published: May 1, 2017  Show citation