Table 1.

Summary of current IS models

Model	Method	Result	Positives	Negatives	Reference
Developmental desynchronization	Intracortical or hippocampal infusion of TTX at P14	Spontaneous spasms at P24; abnormal EEG	Tests hypothesis of a ‘final common pathway’	Late age for spasms	Lee et al., 2008
Down’s inhibition	Down’s syndrome mouse model with IP injections of GABA	Provoked spasms from 1 week to 2 months of age; abnormal EEG	Down’s syndrome is a known genetic cause of IS; tests role of inhibitory networks in IS	Provoked spasms	Cortez et al., 2009
Remote symptomatic causes	P3 injection of LPS and doxorubicin into cortex, followed by IP injection of PCPA	Spontaneous spasms at P10; abnormal EEG	Models the most common group of causes of IS; correct age of spasms	Only available in abstract form; severe damage to the cortex	Scantlebury and Moshe, 2006
Directed therapies model	IP injection of NMDA	Provoked spasms at P15; abnormal EEG	Developed as a way to test therapies to stop spasms; correct age	Provoked spasms; does not test mechanism or hypothesis of spasm generation	Kabova et al., 1999; Velísek et al., 2007
Interneuronopathy	Conditional Arx knockout	Convulsive seizures that evolve to spasms in adult mice; abnormal EEG	Seizures that evolve; spasms present; abnormal EEG; tests the hypothesis of interneuronal dysfunction	Late age for spasms; Arx mutations rarely cause IS	Marsh et al., 2009

All five models described in the text are listed with the model, method and concise findings. The positive and negative features of each model are also listed. Abbreviations: P, postnatal day; TTX, tetrodotoxin; IP, intraperitoneal; IS, infantile spasms; LPS, lipopolysaccharide; PCPA, p-chlorophenylalanine; Arx, aristaless-related homeobox gene.