Table 1.
Animal models of early-life epilepsies and epileptic encephalopathies
| Early-life epilepsy syndrome | Known etiologies | Animal models |
|---|---|---|
| Epileptic encephalopathies | ||
| West syndrome | Structural metabolic genetic (multiple) |
Acute NMDA rat model Prenatal betamethasone/stress, postnatal NMDA rat model GBL/Down syndrome mouse Chronic TTX rat model ARX CKO mouse (Arx−/Y CKO) ARX KI mouse (Arx(GCG)10+7) Multiple-hit rat model |
| Lennox–Gastaut syndrome | Structural metabolic genetic (multiple) |
No |
| Ohtahara syndrome | Structural metabolic genetic (ARX, CG1, STXBP1) |
No Mouse model STXBP1 KO: no seizure reported yet |
| EIEE with suppression burst | CG1/SLC25A22 STXBP1 |
No Mouse model STXBP1 KO: no seizure reported yet |
| Early myoclonic encephalopathy | No | |
| Dravet syndrome (severe myoclonic epilepsy) | SCN1A, PCDH19, GABRG2 | Models of SCN1A or SCN1B mutations or deletions |
| Myoclonic status in nonprogressive encephalopathy | No | |
| Landau–Kleffner syndrome | Structural (malformations, vascular) | No |
| Epilepsy with CSWS | Structural (malformations, vascular) | No Electrical stimulation or flurothyl models of spikes with functional impairment |
| Migrating partial epilepsy | No | |
| Myoclonic astatic epilepsy | Genetic (SCN1A, GABRG2) | No |
| Early-life epilepsies of genetic origin | ||
| Benign familial neonatal convulsions | KCNQ2/3 | KCNQ2 KI, A306T mouse, KCNQ3 KI, G311V mouse |
| Early-life epilepsies, structural/metabolic | ||
| Cortical dysplasias | Two-hit models In utero MAM In utero irradiation Focally induced dysplasias Tish rat |
|
| Tuberous sclerosis | Yes TSC1GFAPCKO (glial) TSC2GFAPCKO (glial) TSC2GFAP1CKO TSC1null-neuron TSC1interneuronalCKO TSC2+/− Eker rat TSC2+/− (neuronal) |
|
| TSC1Synapsin CKO TSC1DLX5/6 CKO |
||
| PTEN-related malformations | Yes NS-PTEN, NEX-PTEN, DG-PTEN |
|
| Pyridoxine-dependent epilepsy | TNAP-deficient mice | |
ARX, Aristaless-related homeobox X-linked; CKO, conditional knockout; CSWS, continuous spikes and waves during slow-wave sleep; EIEE, early-infantile epileptic encephalopathy; GABRG2, γ2 subunit of GABAA receptors; KCNQ, potassium channel; KO, knockout; MAM, methylazoxymethanol acetate; NMDA, N-methyl-d-aspartate; PTEN, phosphatase and tensin homolog; STXBP1, syntaxin binding protein 1; SCN, sodium channel; TSC, tuberous sclerosis complex; TTX, tetrodotoxin; KI, knockin; GBL, γ-butyrolactone; TNAP, tissue-nonspecific alkaline phosphatase; Tish, telencephalic internal structural heterotopia.