title: Ketogenic diet treatment for pediatric super-refractory status epilepticus.
abstract: OBJECTIVE
  We aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm.
  
  
  METHODS
  A retrospective review was performed at Phoenix Children's Hospital on patients with super-refractory status epilepticus undergoing ketogenic diet therapy from 2011 to 2015.
  
  
  RESULTS
  Ten children with super-refractory status epilepticus, ages 2-16 years, were identified. 4/10 patients had immune mediated encephalitis, including Rasmussen encephalitis, anti-N-methyl-d-aspartate receptor encephalitis, and post-infectious mycoplasma encephalitis. Other etiologies included Lennox Gastaut Syndrome, non-ketotic hyperglycinemia, PCDH19 and GABRG2 genetic epilepsy, New Onset Refractory Status Epilepticus, and Febrile Infection-Related Epilepsy Syndrome. 4/10 patients' EEG features suggested focal with status epilepticus, and 6/10 suggested generalized with status epilepticus. Median hospital length was 61days and median ICU length was 27days. The median number of antiepileptic medications prior to diet initiation was 3.0 drugs, and the median after ketogenic diet treatment was 3.5 drugs. Median duration of status epilepticus prior to KD was 18days. 9/10 patients had resolution of super-refractory status epilepticus in a median of 7days after diet initiation. 8/9 patients were weaned off anesthesia within 15days of diet initiation, and within 1day of achieving ketonuria. 1/10 patients experienced side effects on the diet requiring supplementation.
  
  
  CONCLUSIONS
  Most patients achieved resolution of status epilepticus on KD therapy, suggesting it could be an effective therapy that can be utilized early in the treatment of children with super refractory status epilepticus.
authors.affiliation: Barrow Neurological Institute at Phoenix Children's Hospital, 1919 E. Thomas Road, Ambulatory Building, 3rd Floor, Phoenix, AZ 85016, United States. Electronic address: bappavu@phoenixchildrens.com.
authors.forename: Brian
authors.initials: B
authors.lastname: Appavu
authors.affiliation: Barrow Neurological Institute at Phoenix Children's Hospital, 1919 E. Thomas Road, Ambulatory Building, 3rd Floor, Phoenix, AZ 85016, United States. Electronic address: lvanatta@phoenixchildrens.com.
authors.forename: Lisa
authors.initials: L
authors.lastname: Vanatta
authors.affiliation: Barrow Neurological Institute at Phoenix Children's Hospital, 1919 E. Thomas Road, Ambulatory Building, 3rd Floor, Phoenix, AZ 85016, United States. Electronic address: jcondie@phoenixchildrens.com.
authors.forename: John
authors.initials: J
authors.lastname: Condie
authors.affiliation: Barrow Neurological Institute at Phoenix Children's Hospital, 1919 E. Thomas Road, Ambulatory Building, 3rd Floor, Phoenix, AZ 85016, United States. Electronic address: jkerrigan@phoenixchildrens.com.
authors.forename: John F
authors.initials: JF
authors.lastname: Kerrigan
authors.affiliation: Barrow Neurological Institute at Phoenix Children's Hospital, 1919 E. Thomas Road, Ambulatory Building, 3rd Floor, Phoenix, AZ 85016, United States. Electronic address: rjarrar@phoenixchildrens.com.
authors.forename: Randa
authors.initials: R
authors.lastname: Jarrar
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D055423:Diet, Ketogenic / Q000379:methods*; D005260:Female; D006801:Humans; D008297:Male; D010372:Pediatrics; D012189:Retrospective Studies; D013226:Status Epilepticus / Q000178:diet therapy*; D016896:Treatment Outcome
keywords: Encephalitis; Epilepsy; Ketogenic diet; Pediatrics; Super refractory status epilepticus