Document Detail


Model of cryptogenic infantile spasms after prenatal corticosteroid priming.
MedLine Citation:
PMID:  20618421     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
Libor Velísek; Tamar Chachua; Mi-Sun Yum; Ka-Lai Poon; Jana Velísková
Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Epilepsia     Volume:  51 Suppl 3     ISSN:  1528-1167     ISO Abbreviation:  Epilepsia     Publication Date:  2010 Jul 
Date Detail:
Created Date:  2010-07-12     Completed Date:  2010-08-03     Revised Date:  2014-09-21    
Medline Journal Info:
Nlm Unique ID:  2983306R     Medline TA:  Epilepsia     Country:  United States    
Other Details:
Languages:  eng     Pagination:  145-9     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Adrenocorticotropic Hormone / pharmacology
Animals
Animals, Newborn
Betamethasone / pharmacology*
Brain Stem / physiopathology
Disease Models, Animal*
Electroencephalography
Hippocampus / physiopathology
Humans
Hypothalamus / physiopathology
Infant
N-Methylaspartate / pharmacology*
Rats
Spasms, Infantile / chemically induced,  etiology*,  physiopathology
Grant Support
ID/Acronym/Agency:
NS-041366/NS/NINDS NIH HHS; NS-056093/NS/NINDS NIH HHS; NS-059504/NS/NINDS NIH HHS; R01 NS056093/NS/NINDS NIH HHS; R01 NS056093-03/NS/NINDS NIH HHS; R21 NS041366/NS/NINDS NIH HHS; R21 NS041366-03/NS/NINDS NIH HHS; R21 NS059504/NS/NINDS NIH HHS; R21 NS059504-02/NS/NINDS NIH HHS
Chemical
Reg. No./Substance:
6384-92-5/N-Methylaspartate; 9002-60-2/Adrenocorticotropic Hormone; 9842X06Q6M/Betamethasone
Comments/Corrections

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