Table 1.
Summary of published rodent models for several autoimmune encephalitides.
| Disease modeled | Strain/species (sex) | Autoantibody source | Delivery | Immune response | Neural consequences | Reference |
|---|---|---|---|---|---|---|
| Sydenham’s chorea/pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections | SJL mouse (F) | Unknown | Intranasal infection with S. pyogenes | Microglial activation and infiltrating CD4+ T cells in olfactory bulb (OB) | Decreased excitatory synapse proteins in OB glomeruli; blood–brain barrier breakdown in OB, lateral hypothalamus, and amygdala | (55) |
| Lewis rat | Induced in model | Subcutaneous immunization with S. pyogenes emulsion | Autoantibodies detected against tubulin | Dopamine D2-dependent compulsive grooming; impaired motor coordination; IgG deposition in striatum, thalamus, and cortex; IgG-induced elevation of CaMKII signaling in cultured neurons | (56) | |
| SJL mouse | Induced in model | Subcutaneous immunization with S. pyogenes emulsion | Specific increase in IgG1 subclass; no change in IgG2 nor IgG3 pool | Increased rearing; decreased motor coordination; impaired olfactory discrimination; improved spatial memory performance; IgG deposition in striatum and cerebellum | (57) | |
| SJL mouse (M) | Adoptive transfer from immunized cohort | Intravenous injection paired with intraperitoneal lipopolysaccharide injection | Not analyzed | Increased rearing; IgG deposition in dentate gyrus | (57) | |
| Lewis rat (M) | Induced in model | Subcutaneous immunization with S. pyogenes emulsion | Autoantibodies against D1R, D2R, and serotonin receptors | Impaired motor coordination; compulsive grooming | (58) | |
| Lewis rat (M) | Adoptive transfer from immunized cohort | Intra-striatal infusion | Not analyzed | Impaired motor coordination; IgG deposition in striatum | (58) | |
| SJL mouse (M) | Induced in model | Subcutaneous immunization with S. pyogenes emulsion | Microglial activation in white matter tracts; infiltrating CD3+ T cells | Impaired motor coordination; repetitive behaviors; increased rearing; excessive lactate; blunted startle response (PPI) | (59) | |
| NMDA receptor encephalitis | Lewis rat | Patient cerebrospinal fluid (CSF) | Bath application to cultured neurons | Not analyzed | Autoantibody-mediated internalization of NMDAR from synapses; selective loss of NMDA-mediated currents | (60) |
| Lewis rat (F) | Patient CSF | Intrahippocampal infusion | Not analyzed | Decreased NMDAR density in hippocampus | (60) | |
| C57BL/6 mouse | Patient sera | Intraventricular injection | Not analyzed | IgG deposition in hippocampus; more seizures and higher seizure scores after pro-convulsant challenge; no change in total NMDAR number | (61) | |
| C57BL/6N (ApoE−/−) mouse (M) | Patient sera | Intravenous injection | All autoantibody isotypes affect behavioral assessments and endocytosis | Decreased spontaneous locomotion and increased MK-801-evoked locomotion in ApoE−/− mice, but not WT, treated with autoantibody; increased endocytosis by cultured neurons after autoantibody treatment | (62) | |
| C57BL/6J mouse | Patient CSF | Intraventricular infusion | Not analyzed | Reversible memory deficits, anhedonia, and depressive-like behavior without locomotor impairment; hippocampal IgG deposition; decreased NMDAR densityin hippocampus | (63) | |
| Stiff person syndrome/cerebellar ataxia | Wistar rat (M) | Patient sera | Intracerebellar infusion | Not analyzed | Decreased potentiation from excitatory stimulus trains; decreased NMDA-mediated NO synthesis | (64) |
| Wistar rat (M) | Patient sera | Lumbar paraspinal injection | Not analyzed | Abnormal high baseline activity; increased excitability of anterior horn neurons | (64) | |
| Lewis rat (F) | Patient sera | Intrathecal infusion | Not analyzed | Recapitulation of paralysis; autoantibody-mediated internalization of amphiphysin on GABAergic neurons; decreased GABA release from cultured neurons; increased IPSC frequency and amplitude recorded in vivo from hippocampal granule cells | (65) | |
| Lewis rat (F) | Patient CSF | Intrahippocampal injection | Not analyzed | No changes in evoked and spontaneous GABAergic transmission in CA1 neurons | (66) | |
| Cultured mouse hippocampal neurons | Patient sera | Bath application | Not analyzed | No changes in evoked and spontaneous GABAergic transmission in cultured hippocampal networks | (67) | |