title: Adrenocorticotropic hormone versus pulsatile dexamethasone in the treatment of infantile epilepsy syndromes.
abstract: For treatment of intractable epilepsies, there are no data comparing conventional adrenocorticotropic hormone and pulsatile corticoid therapy with dexamethasone. A retrospective comparison of efficacy was therefore conducted for both forms of application. Between 1989 and 2001, a series of 11 children with West syndrome and 3 with Lennox-Gastaut syndrome were treated with adrenocorticotropic hormone (group 1); between 2003 and 2006, 7 children with West syndrome, 5 with electrical status epilepticus during slow sleep, and 2 with Lennox-Gastaut syndrome were treated with pulsatile corticoid therapy (group 2). In group 1 (n = 14), 9/11 West syndrome patients became seizure free, but none with Lennox-Gastaut syndrome (0/3). In group 2 (n = 14), 4/7 West syndrome patients became seizure-free, 1/2 with Lennox-Gastaut syndrome exhibited seizure-frequency reduction, and 2/5 patients with electrical status epilepticus during slow-wave sleep exhibited significant improvement according to electroencephalograms. In West syndrome, pulsatile corticoid therapy was an effective alternative treatment to adrenocorticotropic hormone, whereas in Lennox-Gastaut syndrome in general steroids did not lead to a significant seizure reduction. In electrical status epilepticus during slow-wave sleep, treatment with pulsatile corticoid therapy seems to be effective and should be investigated in a larger group of patients.
authors.affiliation: Department of Pediatrics, Division of Neuropediatrics and Inherited Metabolic Disorders, Medical University of Innsbruck, A-6020 Innsbruck, Austria. edda.haberlandt@uki.at
authors.forename: Edda
authors.initials: E
authors.lastname: Haberlandt
authors.forename: Christine
authors.initials: C
authors.lastname: Weger
authors.forename: Sara Baumgartner
authors.initials: SB
authors.lastname: Sigl
authors.forename: Markus
authors.initials: M
authors.lastname: Rauchenzauner
authors.forename: Sabine
authors.initials: S
authors.lastname: Scholl-Bürgi
authors.forename: Kevin
authors.initials: K
authors.lastname: Rostásy
authors.forename: Daniela
authors.initials: D
authors.lastname: Karall
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D000927:Anticonvulsants / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D001921:Brain / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003907:Dexamethasone / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D004569:Electroencephalography; D004827:Epilepsy / Q000188:drug therapy* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010507:Periodicity; D012189:Retrospective Studies; D012640:Seizures / Q000188:drug therapy / Q000503:physiopathology; D012894:Sleep Stages / Q000502:physiology; D013036:Spasms, Infantile / Q000188:drug therapy* / Q000503:physiopathology; D013226:Status Epilepticus / Q000188:drug therapy* / Q000503:physiopathology; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone