title: Ketogenic diet in 3 cases of childhood refractory status epilepticus.
abstract: BACKGROUND
  Refractory status epilepticus (RSE) in children is associated with a significant risk of death or neurological morbidity. Recently attention has been drawn to the ketogenic diet (KD) as an acute treatment, as it has shown promise in controlling seizures in otherwise refractory status epilepticus in several cases. We have listed these and reviewed all cases of KD used in RSE at our centre. KD was given as 4:1 fat:carbohydrate-protein solution.
  
  
  RESULTS
  A 3-year-old girl with RSE due to Hemiconvulsion-Hemiplegia Epilepsy syndrome. KD was instigated on day 6. Seizures stopped with ketosis on day 7. A 10-year-old boy rapidly developing RSE. After months a mitochondrial disorder was discovered. KD was tried twice with severe side-effects but no seizure control. 11-year-old healthy boy with RSE as FIRES. On KD seizures stopped for 24 h one day after reaching ketosis. He improved over 3-4 weeks.
  
  
  CONCLUSIONS
  KD was efficient in two of three cases of RSE. The non-responder had severe side-effects and proved to have a mitochondrial disorder which is arguably a contraindication for KD. More studies are needed to prove efficacy of KD in RSE, to define optimal timing of KD and possible contraindications for KD in RSE.
authors.affiliation: Department of Paediatrics, Hillerød Hospital, 3400 Hillerød, Denmark. Electronic address: sort@dadlnet.dk.
authors.forename: Rune
authors.initials: R
authors.lastname: Sort
authors.forename: Alfred P
authors.initials: AP
authors.lastname: Born
authors.forename: Karen N
authors.initials: KN
authors.lastname: Pedersen
authors.forename: Lise
authors.initials: L
authors.lastname: Fonsmark
authors.forename: Peter
authors.initials: P
authors.lastname: Uldall
mesh_terms: D002648:Child; D002675:Child, Preschool; D055423:Diet, Ketogenic / Q000592:standards*; D005260:Female; D006801:Humans; D008297:Male; D013226:Status Epilepticus / Q000178:diet therapy*
keywords: Ketogenic diet; Non-pharmacological treatment in status epilepticus; Pharmacoresistent status epilepticus; Refractory status epilepticus; Status epilepticus