Crisis con fiebre en el primer año de vida: ¿epilepsia del espectro Dravet?

Dravet syndrome is a drug resistant epilepsy which starts in the first year of life with febrile seizures, followed by cognitive impairment and epilepsy with multiple seizure types. Diagnosis has been typically made at the age of three to four years, but earlier diagnosis is now possible as clinical...

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Detalhes bibliográficos
Autores: Sanchez-Carpintero-Abad, R. (Rocío)|||/items/5812a8c2-16ce-4a9f-b3d5-1cee50cedf2f, Nuñez, M.T. (M.T.)|||/items/022a6851-97fa-4dcd-a7b2-cc5036ddbb0b, Aznárez-Sanado, N. (Nerea)|||/items/b4dff4e2-b51f-4552-883e-2ee9ea37b3dd, Narbona, J. (Juan)|||/items/2be25859-b8a1-4525-b3e4-52cc1f4a705d
Tipo de documento: artigo
Data de publicação:2012
País:España
Recursos:Universidad de Navarra
Repositório:Dadun. Depósito Académico Digital de la Universidad de Navarra
Idioma:espanhol
OAI Identifier:oai:dadun.unav.edu:10171/22469
Acesso em linha:https://hdl.handle.net/10171/22469
Access Level:Acceso aberto
Palavra-chave:Dravet syndrome
Infant epilepsy
Severe myoclonic epilepsy
Descrição
Resumo:Dravet syndrome is a drug resistant epilepsy which starts in the first year of life with febrile seizures, followed by cognitive impairment and epilepsy with multiple seizure types. Diagnosis has been typically made at the age of three to four years, but earlier diagnosis is now possible as clinical features are better recognised and molecular diagnosis is available. PATIENTS AND METHODS: We studied a series of 14 children with Dravet syndrome or Dravet spectrum epilepsy. A screening test, developed by other authors to distinguish the febrile seizures in Dravet syndrome from febrile seizures from other origin, was applied to the clinical features of the seizures occurring during the first year of life in our patients. RESULTS: Clinical suspicion of Dravet spectrum epilepsy was possible in 100% of children in our series. Moreover, taking into consideration only the first seizure, 79% of patients scored sufficiently to detect Dravet syndrome. CONCLUSIONS: Dravet syndrome can be recognised during the first year of life. It is important that physicians are made aware of these clinical criteria capable to distinguish febrile seizures in Dravet syndrome from febrile seizures of other origin, and set up a protocol to collect appropriate data regarding febrile seizures occurring in the first year of life.