Syndrom Dravetové v pediatrické praxi

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Title in English Dravet syndrome in paediatric practise
Authors

AULICKÁ Štefánia HORÁK Ondřej BRUNOVÁ Katarína FAJKUSOVÁ Lenka PAVLOUŠKOVÁ Jana OŠLEJŠKOVÁ Hana

Year of publication 2017
Type Article in Periodical
Magazine / Source Pediatrie pro praxi
MU Faculty or unit

Faculty of Medicine

Citation
Field Neurology, neurosurgery, neurosciences
Keywords Dravet syndrome; Febrile seizures; Pharmacoresistance; Psychomotoric deterioration
Description 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.
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