title: Vigabatrin as First-Line Treatment for Infantile Spasms Not Related to Tuberous Sclerosis Complex.
abstract: BACKGROUND
  Infantile spasms are a rare, catastrophic, age-specific seizure disorder of infancy. Adrenocorticotropic hormone or vigabatrin have been recommended for the short-term treatment of infantile spasms by the Child Neurology Society/American Academy of Neurology practice parameter.
  
  
  OBJECTIVE
  This retrospective study reviewed the Hospital for Sick Children's experience with the short-term efficacy of vigabatrin as first-line treatment for infantile spasms not related to tuberous sclerosis complex.
  
  
  METHODS
  We performed a single-center, retrospective analysis of all cases of newly diagnosed infantile spasms between January 2010 and September 2013. Duration of follow-up was at least 6 months from treatment initiation.
  
  
  RESULTS
  Eighteen of the 61 infants (30%) had a clinical and electrographic response to vigabatrin therapy within 4 weeks of treatment. Of the vigabatrin responders, 2/18 (11%) relapsed. At final follow-up after initiation of vigabatrin therapy, 17/61 (27%) of the vigabatrin responders were free of all clinical seizure types. Normal development at the time of infantile spasms diagnosis was statistically associated with vigabatrin response.
  
  
  CONCLUSIONS
  These findings do not support our hypothesis that vigabatrin is effective as first-line, short-term treatment of infantile spasms in non-tuberous sclerosis complex patients. However, when used in this setting, vigabatrin is most effective in children with normal development at the time of diagnosis. These findings may assist clinicians in the optimal treatment choice for children at the first presentation of infantile spasms not related to tuberous sclerosis complex.
authors.affiliation: The Division of Neurology, Department of Pediatrics, McMaster Children's Hospital, Hamilton, Ontario, Canada. Electronic address: joneskc@mcmaster.ca.
authors.forename: Kevin
authors.initials: K
authors.lastname: Jones
authors.affiliation: The Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Cristina
authors.initials: C
authors.lastname: Go
authors.affiliation: The Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Boyd
authors.affiliation: The Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Ayako
authors.initials: A
authors.lastname: Ochi
authors.affiliation: The Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Blathnaid
authors.initials: B
authors.lastname: McCoy
authors.affiliation: The Division of Psychology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Klajdi
authors.initials: K
authors.lastname: Puka
authors.affiliation: The Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: O Carter
authors.initials: OC
authors.lastname: Snead
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D004569:Electroencephalography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000188:drug therapy*; D013997:Time Factors; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000503:physiopathology*; D020888:Vigabatrin / Q000627:therapeutic use*
chemical_list: D000927:Anticonvulsants; D020888:Vigabatrin
keywords: efficacy; epileptic encephalopathy; infantile spasms; nontuberous sclerosis complex; predictors; vigabatrin