Infantile spasms: criteria for an animal model

Abstract

Infantile spasms is an epilepsy syndrome with several distinctive features, including age specificity during infancy, characteristic semiology (epileptic spasms), specific electroencephalographic patterns (interictal hypsarrhythmia and ictal voltage suppression), and responsiveness to the adrenocorticotropic hormone (ACTH). There is no adequate animal model of infantile spasms, perhaps due to these clinically unique features, that is specific for the developing human brain. An informative animal model would provide insights into the pathophysiology of this syndrome and form the basis for the development of innovative therapies. This chapter considers criteria for an "ideal" animal model of infantile spasms, as well as "minimal" criteria that we consider essential to yield useful information. Two animal models of infantile spasms have been described in rodents: seizures induced by corticotropin-releasing factor and N-methyl-D-aspartic acid. Neither of these models conforms exactly to the human analog, but each possesses intriguing similarities that provide testable hypotheses for future investigations.