title: Model of cryptogenic infantile spasms after prenatal corticosteroid priming.
abstract: Infantile spasms (IS) is a devastating epilepsy syndrome of childhood. IS occurs in 3-12-month-old infants and is characterized by spasms, interictal electroencephalography (EEG) hypsarrhythmia, and profound mental retardation. Hormonal therapy [adrenocorticotropic hormone (ACTH), corticosteroids] is frequently used, but its efficacy is tainted by severe side effects. For research of novel therapies, a validated animal model of IS is required. We propose the model of spastic seizures triggered by N-methyl-d-aspartate (NMDA) in infant rats prenatally exposed to betamethasone. The spasms have remarkable similarity to human IS, including motor flexion spasms, ictal EEG electrodecrement, and responsiveness to ACTH. Interestingly, the spasms do not involve the hippocampus. Autoradiographic metabolic mapping as well as tagging of the areas of neuronal excitation with c-fos indicates a strong involvement of hypothalamic structures such as the arcuate nucleus, which has significant bilateral connections with other hypothalamic nuclei as well as with the brainstem.
authors.affiliation: The Saul R Korey Department of Neurology, Albert Einstein College of Medicine, Bronx, New York 10461, USA. libor.velisek@einstein.yu.edu
authors.forename: Libor
authors.initials: L
authors.lastname: Velísek
authors.forename: Tamar
authors.initials: T
authors.lastname: Chachua
authors.forename: Mi-Sun
authors.initials: MS
authors.lastname: Yum
authors.forename: Ka-Lai
authors.initials: KL
authors.lastname: Poon
authors.forename: Jana
authors.initials: J
authors.lastname: Velísková
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000494:pharmacology; D000818:Animals; D000831:Animals, Newborn; D001623:Betamethasone / Q000494:pharmacology*; D001933:Brain Stem / Q000503:physiopathology; D004195:Disease Models, Animal; D004569:Electroencephalography; D006624:Hippocampus / Q000503:physiopathology; D006801:Humans; D007031:Hypothalamus / Q000503:physiopathology; D007223:Infant; D016202:N-Methylaspartate / Q000494:pharmacology*; D051381:Rats; D013036:Spasms, Infantile / Q000139:chemically induced / Q000209:etiology* / Q000503:physiopathology
chemical_list: D016202:N-Methylaspartate; D000324:Adrenocorticotropic Hormone; D001623:Betamethasone