Table 1.

Summary of current IS models

ModelMethodResultPositivesNegativesReference
Developmental desynchronizationIntracortical or hippocampal infusion of TTX at P14Spontaneous spasms at P24; abnormal EEGTests hypothesis of a ‘final common pathway’Late age for spasmsLee et al., 2008
Down’s inhibitionDown’s syndrome mouse model with IP injections of GABAProvoked spasms from 1 week to 2 months of age; abnormal EEGDown’s syndrome is a known genetic cause of IS; tests role of inhibitory networks in ISProvoked spasmsCortez et al., 2009
Remote symptomatic causesP3 injection of LPS and doxorubicin into cortex, followed by IP injection of PCPASpontaneous spasms at P10; abnormal EEGModels the most common group of causes of IS; correct age of spasmsOnly available in abstract form; severe damage to the cortexScantlebury and Moshe, 2006
Directed therapies modelIP injection of NMDAProvoked spasms at P15; abnormal EEGDeveloped as a way to test therapies to stop spasms; correct ageProvoked spasms; does not test mechanism or hypothesis of spasm generationKabova et al., 1999; Velísek et al., 2007
InterneuronopathyConditional Arx knockoutConvulsive seizures that evolve to spasms in adult mice; abnormal EEGSeizures that evolve; spasms present; abnormal EEG; tests the hypothesis of interneuronal dysfunctionLate age for spasms; Arx mutations rarely cause ISMarsh 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.