Towards new therapeutic strategies based on cannabinoids for Dravet syndrome

Dravet syndrome (DS) is a rare genetic epileptic encephalopathy affecting children which, in approximately 70-80% of patients, is caused by loss-of-function mutations in the Scn1a gene, which encodes the α1 subunit of the voltage-gated sodium channel (NaV1.1). Clinically, these patients present diff...

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Detalles Bibliográficos
Autor: Alonso Gómez, Cristina
Tipo de recurso: tesis doctoral
Fecha de publicación:2022
País:España
Institución:Universidad Complutense de Madrid (UCM)
Repositorio:Docta Complutense
Idioma:inglés
OAI Identifier:oai:docta.ucm.es:20.500.14352/3567
Acceso en línea:https://hdl.handle.net/20.500.14352/3567
Access Level:acceso abierto
Palabra clave:616.853(043.2)
Epilepsy
Epilepsia
Neurociencias (Medicina)
2490 Neurociencias
Descripción
Sumario:Dravet syndrome (DS) is a rare genetic epileptic encephalopathy affecting children which, in approximately 70-80% of patients, is caused by loss-of-function mutations in the Scn1a gene, which encodes the α1 subunit of the voltage-gated sodium channel (NaV1.1). Clinically, these patients present different types of epileptic seizures, which are frequently accompanied by some comorbidities such as developmental delay, cognitive impairment, hyperactivity, autistic traits and a rate of premature mortality of around 20%. Therapeutic strategies typically involve a complex polytherapy, with antiepileptic drugs whose action mechanisms are focused on correcting hyperexcitability, i.e., the imbalance between excitation and inhibition occurring in epilepsy. Current treatment algorithms often lead to tolerance issues as well as adverse effects, and around 30% of patients remain refractory. Therefore, there is an urgent need for new and effective therapeutic approaches...