Extending the use of stiripentol to SLC13A5-related epileptic encephalopathy.
Brain Dev
; 40(9): 827-829, 2018 Oct.
Article
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| MEDLINE
| ID: mdl-29895383
ABSTRACT
INTRODUCTION:
SLC13A5-related epileptic encephalopathy is a recently described autosomal recessive disorder that is characterized by infantile epilepsy and developmental delay. Seizures are markedly drug resistant and often induced by fever; they mainly occur in clusters, sometimes evolving into status epilepticus. METHODS ANDRESULTS:
We report the use of stiripentol as an adjunctive therapy in three siblings with drug-resistant SLC13A5-related epilepsy. The three patients showed remarkable improvement in the severity and frequency of seizures, status epilepticus, emergency department visits, and alertness.CONCLUSION:
These observations extend the use of stiripentol beyond the classical Dravet syndrome, and further studies on the use of this drug in drug-resistant epilepsy, mainly of genetic origin, are warranted.Palabras clave
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Base de datos:
MEDLINE
Asunto principal:
Simportadores
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Dioxolanos
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Epilepsia Refractaria
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Anticonvulsivantes
Idioma:
En
Revista:
Brain Dev
Año:
2018
Tipo del documento:
Article