TY - JOUR
T1 - Modeling new therapies for infantile spasms
AU - Chudomelova, Lenka
AU - Scantlebury, Morris H.
AU - Raffo, Emmanuel
AU - Coppola, Antonietta
AU - Betancourth, David
AU - Galanopoulou, Aristea S.
PY - 2010/7
Y1 - 2010/7
N2 - 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 γ-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.
AB - 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 γ-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.
KW - Adrenocorticotropic hormone
KW - Infantile spasms
KW - Models
KW - Rapamycin
KW - Vigabatrin
KW - mTOR
UR - http://www.scopus.com/inward/record.url?scp=77954466566&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77954466566&partnerID=8YFLogxK
U2 - 10.1111/j.1528-1167.2010.02605.x
DO - 10.1111/j.1528-1167.2010.02605.x
M3 - Article
C2 - 20618396
AN - SCOPUS:77954466566
SN - 0013-9580
VL - 51
SP - 27
EP - 33
JO - Epilepsia
JF - Epilepsia
IS - SUPPL. 3
ER -