title: Epileptic spasms - 175 years on: Trying to teach an old dog new tricks.
abstract: OBJECTIVE
  This text provides an overview of how the condition "infantile spasms" has evolved in the last 175 years.
  
  
  METHODS
  Key references are summarised to assimilate this review.
  
  
  RESULTS
  Infantile spasms, first described by Dr West in 1841, has undergone extensive investigation to understand the pathogenesis, aetiologies, optimal intervention and most likely prognosis for the affected child. The terminology has recently evolved such that the preferred term for the condition is now "epileptic spasms" in recognition of the fact that cases can present outside infancy. The aetiologies are diverse and can be structural, genetic, metabolic or acquired. Increasing numbers of presumed causative genetic mutations are now being identified. The condition is an epileptic encephalopathy such that without adequate control of the clinical seizures and correction of the abnormal EEG, ongoing neurological damage occurs. In some cases neuroregression is inevitable despite intervention. First-line treatments are either hormonal therapies, adrenocortcotrophic hormone or prednisolone, or vigabatrin. In the sub-group of patients with tuberous sclerosis complex, vigabatrin is the preferred treatment. High dose prednisolone may be a more viable option in resource limited settings. Recent research has suggested that combining hormonal therapies with vigabatrin will result in more patients achieving spasm cessation.
  
  
  CONCLUSIONS
  Despite extensive study, the pathogenic mechanisms remain an area of debate and in need of further exploration. The enigma, however, may be explained as the role of resting state and dysfunctional brain networks are elucidated further.
authors.affiliation: Department of Paediatric Neurology, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, University of Cape Town, South Africa. Electronic address: jo.wilmshurst@uct.ac.za.
authors.forename: Jo M
authors.initials: JM
authors.lastname: Wilmshurst
authors.affiliation: Department of Paediatric Neurology, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, University of Cape Town, South Africa.
authors.forename: Roland C
authors.initials: RC
authors.lastname: Ibekwe
authors.affiliation: Section of Clinical Neurosciences, UCL Institute of Child Health, London, United Kingdom.
authors.forename: Finbar J K
authors.initials: FJK
authors.lastname: O'Callaghan
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use; D019468:Disease Management; D004569:Electroencephalography; D049672:History, 19th Century; D049673:History, 20th Century; D049674:History, 21st Century; D006801:Humans; D007231:Infant, Newborn; D013036:Spasms, Infantile / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000266:history*; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants
keywords: ACTH; Infantile spasms; Late-onset epileptic spasms; Oral corticosteroids; Prednisolone; Vigabatrin