Article ; Online: Modeling new therapies for infantile spasms.
2010 Volume 51 Suppl 3, Page(s) 27–33
Abstract: Infantile spasms are the classical seizure type of West syndrome. Infantile spasms often herald a dismal prognosis, due to the high probability to evolve into intractable forms of epilepsies with significant cognitive deficits, especially if not ... ...
Abstract | Infantile spasms are the classical seizure type of West syndrome. Infantile spasms often herald a dismal prognosis, due to the high probability to evolve into intractable forms of epilepsies with significant cognitive deficits, especially if not adequately treated. The current therapies-high doses of adrenocorticotropic hormone, steroids, or the gamma-aminobutyric acid (GABA) transaminase inhibitor vigabatrin--are often toxic and may not always be effective. The need to identify new therapies for spasms has led to the generation of a number of rodent models of infantile spasms. These include acute and chronic models of infantile spasms, with cryptogenic or symptomatic origin, many of which are based on specific etiologies. In this review, we summarize the clinical experience with treating infantile spasms and the main features of the new animal models of infantile spasms and discuss their utility in the preclinical development of new therapies for infantile spasms. |
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MeSH term(s) | Adrenocorticotropic Hormone/therapeutic use ; Animals ; Anticonvulsants/therapeutic use ; Disease Models, Animal ; Humans ; Infant ; Mice ; Rats ; Receptors, GABA/drug effects ; Sirolimus/therapeutic use ; Spasms, Infantile/drug therapy ; Spasms, Infantile/etiology ; Vigabatrin/therapeutic use |
Chemical Substances | Anticonvulsants ; Receptors, GABA ; Adrenocorticotropic Hormone (9002-60-2) ; Vigabatrin (GR120KRT6K) ; Sirolimus (W36ZG6FT64) |
Language | English |
Publishing date | 2010-07-04 |
Publishing country | United States |
Document type | Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review |
ZDB-ID | 216382-2 |
ISSN | 1528-1167 ; 0013-9580 |
ISSN (online) | 1528-1167 |
ISSN | 0013-9580 |
DOI | 10.1111/j.1528-1167.2010.02605.x |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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