title: The ketogenic diet in two paediatric patients with refractory myoclonic status epilepticus.
abstract: OBJECTIVE
  We describe two patients with refractory myoclonic status epilepticus treated with the ketogenic diet.
  
  
  METHODS
  Between May 1, 2014 and January 1, 2015, two patients who met the diagnostic criteria for refractory myoclonic status epilepticus, seen at our department, were placed on the ketogenic diet and followed for a minimum of six months.
  
  
  RESULTS
  One patient with myoclonic epilepsy of unknown aetiology had a 75-90% seizure reduction, and the other with progressive encephalopathy associated with myoclonic epilepsy had a 50% seizure reduction. Both patients retained good tolerability for the diet. At the last control, one patient had isolated myoclonias and EEG showed occasional generalized spike-and-polyspike waves; the patient is now successfully attending kindergarten. The quality of life of the second patient improved significantly. In both cases, the number of antiepileptic drugs was reduced.
  
  
  CONCLUSIONS
  The ketogenic diet is an effective and well-tolerated treatment option for patients with refractory myoclonic status epilepticus and should be considered earlier in the course of treatment.
authors.affiliation: Servicio de Neurología, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Roberto Horacio
authors.initials: RH
authors.lastname: Caraballo
authors.affiliation: Servicio de Neurología, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Gabriela Reyes
authors.initials: GR
authors.lastname: Valenzuela
authors.affiliation: Servicio de Neurología, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Marisa
authors.initials: M
authors.lastname: Armeno
authors.affiliation: Servicio de Neurología, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Sebastian
authors.initials: S
authors.lastname: Fortini
authors.affiliation: Servicio de Neurología, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Graciela
authors.initials: G
authors.lastname: Mestre
authors.affiliation: Servicio de Neurología, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Araceli
authors.initials: A
authors.lastname: Cresta
mesh_terms: D001921:Brain / Q000503:physiopathology; D002675:Child, Preschool; D055423:Diet, Ketogenic; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000178:diet therapy* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008297:Male; D013226:Status Epilepticus / Q000178:diet therapy* / Q000503:physiopathology; D016896:Treatment Outcome
keywords: ketogenic diet; myoclonias; seizures; status epilepticus; super-refractory