Modeling new therapies for infantile spasms.
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.
Chudomelova
Lenka
L
Saul R Korey Department of Neurology and Montefiore/Einstein Epilepsy Management Center, Albert Einstein College of Medicine, Bronx, New York 10461, USA.
Scantlebury
Morris H
MH
Raffo
Emmanuel
E
Coppola
Antonietta
A
Betancourth
David
D
Galanopoulou
Aristea S
AS
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D000818:Animals; D000927:Anticonvulsants / Q000627:therapeutic use; D004195:Disease Models, Animal; D006801:Humans; D007223:Infant; D051379:Mice; D051381:Rats; D018079:Receptors, GABA / Q000187:drug effects; D020123:Sirolimus / Q000627:therapeutic use; D013036:Spasms, Infantile / Q000188:drug therapy / Q000209:etiology*; D020888:Vigabatrin / Q000627:therapeutic use
D000927:Anticonvulsants; D018079:Receptors, GABA; D000324:Adrenocorticotropic Hormone; D020888:Vigabatrin; D020123:Sirolimus