title: Effect of ACTH therapy for epileptic spasms without hypsarrhythmia.
abstract: OBJECTIVE
  We analyzed the short- and long-term effects of adrenocorticotropic hormone (ACTH) therapy for patients with epileptic spasms (ESs) who did not meet the criteria of West syndrome (WS).
  
  
  METHODS
  The subjects were 30 patients, including 13 boys and 17 girls, who had received ACTH therapy between 1970 and 2003. We excluded patients with WS, but included those with a history of WS who no longer showed hypsarrhythmia at the period of ACTH therapy. The age at onset of ESs and at ACTH therapy ranged from 2 to 82 months with a median of 18 months, and from 11 to 86 months with a median of 29 months, respectively.
  
  
  RESULTS
  Excellent and poor responses were obtained in 19 (63%) and 11 (37%) patients, respectively, as a short-term effect. Although the patients could be subclassified into five subgroups according to the previous reports, no difference was seen in short- term response to ACTH. Among 17 of the 19 patients with excellent short-term outcomes and a follow-up of >1 year after the ACTH therapy, eight patients have continued to be seizure free (29%; excellent long-term effect), whereas the remaining nine patients had a recurrence of seizures (complex partial seizures, four; generalized tonic seizures, three; ESs, two) at 9 months to 198 months (median, 49 months) after ACTH therapy. In addition, nine of the 17 patients demonstrated a localized frontal EEG focus after the ACTH therapy, although most of these had previously shown diffuse epileptic EEG abnormality.
  
  
  CONCLUSIONS
  ACTH therapy is worth trying for patients with resistant ESs, even without features of WS. However, the long-term effect is uncertain because recurrences of various types of seizures, including focal, were frequently observed.
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, Shinjuku-ku, Japan. hoguni@ped.twmu.ac.jp
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Oguni
authors.forename: Makoto
authors.initials: M
authors.lastname: Funatsuka
authors.forename: Kaori
authors.initials: K
authors.lastname: Sasaki
authors.forename: Tae
authors.initials: T
authors.lastname: Nakajima
authors.forename: Keisuke
authors.initials: K
authors.lastname: Yoshii
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Nishimura
authors.forename: Makiko
authors.initials: M
authors.lastname: Osawa
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D002648:Child; D003366:Cosyntropin / Q000008:administration & dosage / Q000627:therapeutic use*; D004334:Drug Administration Schedule; D004569:Electroencephalography / Q000706:statistics & numerical data; D017034:Epilepsy, Frontal Lobe / Q000145:classification / Q000175:diagnosis; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy*; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D003366:Cosyntropin