title: ACTH therapy on intractable epilepsy in Hemiconvulsion-Hemiplegia-Epilepsy syndrome.
abstract: BACKGROUND
  In the chronic phase of Hemiconvulsion-Hemiplegia-Epilepsy (HHE) syndrome, developing epilepsy may be intractable. Herein, we report a case where adrenocorticotropic hormone (ACTH) ceased an intractable habitual partial seizure in a patient with HHE syndrome.
  
  
  METHODS
  A developmentally normal one-year-old girl presented with left focal motor status epilepticus in the clinical course of rotavirus infection. She was diagnosed with HH syndrome. At 4 months after status epilepticus, she developed partial seizures that occurred daily, and which resulted in a stooped posture, head rotation to the right, and contraction of both upper limbs predominantly in the left arm. At this time, she was diagnosed with idiopathic HHE syndrome. Her seizures were not reduced by sodium valproate, clonazepam, clobazam, zonisamide, phenytoin, phenobarbital, topiramate, lamotrigine, or liposteroid. At the age of 7, ACTH therapy was performed. On the 10th day of ACTH therapy, the habitual seizure was ceased. However, partial seizures characterized by left arm contraction then developed. Treatment with 350 mg/day lamotrigine prevented this emerging seizure. She has been free of both seizure types for more than one year, with no serious adverse effects of ACTH therapy.
  
  
  CONCLUSIONS
  We suggest that ACTH therapy may be useful for patients with HHE, although further studies are required.
authors.affiliation: Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan. Electronic address: ZVQ10523@nifty.com.
authors.forename: Shuichi
authors.initials: S
authors.lastname: Shimakawa
authors.affiliation: Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Shohei
authors.initials: S
authors.lastname: Nomura
authors.affiliation: Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Motoko
authors.initials: M
authors.lastname: Ogino
authors.affiliation: Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Miho
authors.initials: M
authors.lastname: Fukui
authors.affiliation: Department of Pediatrics, Hirakata Municipal Hospital, Hirakata, Osaka, Japan.
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Kashiwagi
authors.affiliation: Department of Pediatric Neurology, Tanabe Children's Clinic, Hirakata, Osaka, Japan.
authors.forename: Takuya
authors.initials: T
authors.lastname: Tanabe
authors.affiliation: Department of Pediatrics, Osaka Medical College, Takatsuki, Osaka, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Tamai
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000009:adverse effects / Q000627:therapeutic use*; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D002675:Child, Preschool; D000069279:Drug Resistant Epilepsy / Q000150:complications / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D004569:Electroencephalography; D004828:Epilepsies, Partial / Q000150:complications / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006429:Hemiplegia / Q000150:complications*; D006801:Humans; D008279:Magnetic Resonance Imaging
chemical_list: D000324:Adrenocorticotropic Hormone
keywords: Adrenocorticotropic hormone therapy; Hemiconvulsion–Hemiplegia–Epilepsy syndrome; Lamotrigine; Treatment