title: Efficacy of long term weekly ACTH therapy for intractable epilepsy.
abstract: BACKGROUND
  Adrenocorticotropic hormone (ACTH) therapy is the first-line therapy for infantile spasms, and is effective for many other intractable epilepsies. While spasms may respond to ACTH for weeks, a substantial proportion of patients develop recurrent seizures over a yearly period. To maintain efficacy, we treated two children with intractable epilepsy with weekly ACTH therapy for 1 year and described the changes in clinical seizures, electroencephalograms, developmental assessments and side effects.
  
  
  METHODS
  A girl with infantile spasms due to lissencephaly and a boy with atypical absence seizures were studied. In both cases, seizures were frequent and resistant to antiepileptic drugs; electroencephalograms showed continuous epileptiform activities, and the patients' development was delayed and stagnant prior to ACTH treatment. The initial ACTH therapy (daily 0.015 mg/kg for 2 weeks, 0.015 mg/kg every 2 days for 1 week, 0.0075 mg/kg every 2 days for 1 week), was transiently effective in both cases. The second-round ACTH therapy consisted of the initial ACTH therapy protocol followed by weekly ACTH injections (0.015 mg/kg or 0.0075 mg/kg) for 1 year. Both cases were followed for at least 1 year after therapy.
  
  
  RESULTS
  In both patients, clinical seizures were completely controlled during and 1 year after the second-round AHCH therapy. Continuous epileptiform discharges disappeared, while intermittent interictal epileptiform discharges remained. Both patients showed some developmental gains after achieving seizure control. No serious side effects were recorded.
  
  
  CONCLUSIONS
  Further studies are warranted to determine if a long-term weekly ACTH is a safe and effective treatment for intractable epilepsy.
authors.affiliation: Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, 20 Shishioto, Akiu Yumoto, Taihaku-ku, Sendai-shi, Miyagi 982-0241, Japan.
authors.forename: Takehiko
authors.initials: T
authors.lastname: Inui
authors.affiliation: Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: Tomoko
authors.initials: T
authors.lastname: Kobayashi
authors.affiliation: Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, 20 Shishioto, Akiu Yumoto, Taihaku-ku, Sendai-shi, Miyagi 982-0241, Japan; Department of Pediatrics, Nagoya City West Medical Center, Nagoya, Japan.
authors.forename: Satoru
authors.initials: S
authors.lastname: Kobayashi
authors.affiliation: Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, 20 Shishioto, Akiu Yumoto, Taihaku-ku, Sendai-shi, Miyagi 982-0241, Japan; Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Sato
authors.affiliation: Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, 20 Shishioto, Akiu Yumoto, Taihaku-ku, Sendai-shi, Miyagi 982-0241, Japan; Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: Wakaba
authors.initials: W
authors.lastname: Endo
authors.affiliation: Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: Atsuo
authors.initials: A
authors.lastname: Kikuchi
authors.affiliation: Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: Tojo
authors.initials: T
authors.lastname: Nakayama
authors.affiliation: Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: Mitsugu
authors.initials: M
authors.lastname: Uematsu
authors.affiliation: Department of Pediatrics, Sendai City Hospital, Sendai, Japan.
authors.forename: Masaru
authors.initials: M
authors.lastname: Takayanagi
authors.affiliation: Department of Pediatrics, Yamagata University Faculty of Medicine, Yamagata, Japan.
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Kato
authors.affiliation: Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
authors.forename: Hirotomo
authors.initials: H
authors.lastname: Saitsu
authors.affiliation: Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
authors.forename: Naomichi
authors.initials: N
authors.lastname: Matsumoto
authors.affiliation: Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: Shigeo
authors.initials: S
authors.lastname: Kure
authors.affiliation: Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, 20 Shishioto, Akiu Yumoto, Taihaku-ku, Sendai-shi, Miyagi 982-0241, Japan; Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan. Electronic address: khaginoya@kha.biglobe.ne.jp.
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Haginoya
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000008:administration & dosage* / Q000009:adverse effects; D000927:Anticonvulsants / Q000008:administration & dosage* / Q000009:adverse effects; D001921:Brain / Q000187:drug effects / Q000473:pathology / Q000503:physiopathology; D002675:Child, Preschool; D004569:Electroencephalography; D004827:Epilepsy / Q000188:drug therapy* / Q000209:etiology / Q000235:genetics / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D054082:Lissencephaly / Q000150:complications / Q000235:genetics / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D012008:Recurrence; D019233:Retreatment / Q000379:methods; D012640:Seizures / Q000188:drug therapy* / Q000209:etiology / Q000235:genetics / Q000503:physiopathology
chemical_list: D000927:Anticonvulsants; D000324:Adrenocorticotropic Hormone
keywords: ACTH; Atypical absence seizure; Infantile spasms; Intractable epilepsy; LIS1; Lissencephaly