title: Lennox-Gastaut syndrome symptomatic to hypothalamic hamartoma: evolution and long-term outcome following surgery.
abstract: BACKGROUND
  Lennox-Gastaut syndrome is a catastrophic childhood cryptogenic or symptomatic epilepsy. Hypothalamic hamartomas cause refractory epilepsy often consistent with Lennox-Gastaut syndrome.
  
  
  METHODS
  Children with Lennox-Gastaut syndrome were defined by a triad of multiple generalized seizure types, slow spike-and-wave on EEG, and mental retardation.
  
  
  RESULTS
  Twenty-one of 159 hypothalamic hamartoma patients (14%) met the diagnostic criteria of Lennox-Gastaut syndrome. The median age of patients at epilepsy onset was 0.9 years (range, birth to 9 years). Six of the 21 patients (28%) had preceding infantile spasms. All patients underwent different surgical approaches, including endoscopic, transcallosal, orbitozygomatic resections, and radiosurgery treatment. Five of the 21 (24%) were seizure free with an additional 9 (42%) having at least >90% seizure reduction. Only 1 patient was not effectively treated (<50% seizure reduction). Eighty-eight percent of parents reported improvement in behavioral functioning. Shorter duration of epilepsy prior to surgery was a significant predictor of surgical outcome.
  
  
  CONCLUSIONS
  Patients with Lennox-Gastaut syndrome symptomatic to hypothalamic hamartomas have better postsurgical outcome due to other etiologies compared with cryptogenic and symptomatic Lennox-Gastaut syndrome patients. However, compared with overall hypothalamic hamartomas postsurgical outcomes, this cohort was less favorable. Earlier surgery may lead to better outcomes.
authors.affiliation: Department of Neurology, Barrow Neurological Institute, Phoenix, AZ, USA.
authors.forename: Sandipan
authors.initials: S
authors.lastname: Pati
authors.forename: Aman
authors.initials: A
authors.lastname: Deep
authors.forename: Matthew M
authors.initials: MM
authors.lastname: Troester
authors.forename: Eric H
authors.initials: EH
authors.lastname: Kossoff
authors.forename: Yu-Tze
authors.initials: YT
authors.lastname: Ng
mesh_terms: D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D016208:Databases, Factual / Q000639:trends; D005260:Female; D005500:Follow-Up Studies; D006222:Hamartoma / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery*; D006801:Humans; D007027:Hypothalamic Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery*; D065768:Lennox Gastaut Syndrome; D008297:Male; D011446:Prospective Studies; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome