Adrenocorticotropic hormone versus pulsatile dexamethasone in the treatment of infantile epilepsy syndromes.
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.
Haberlandt
Edda
E
Department of Pediatrics, Division of Neuropediatrics and Inherited Metabolic Disorders, Medical University of Innsbruck, A-6020 Innsbruck, Austria. edda.haberlandt@uki.at
Weger
Christine
C
Sigl
Sara Baumgartner
SB
Rauchenzauner
Markus
M
Scholl-Bürgi
Sabine
S
Rostásy
Kevin
K
Karall
Daniela
D
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
D000927:Anticonvulsants; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone