Animal models of autoimmune encephalitis

Area of Science:

• Neuroimmunology
• Autoimmune Diseases
• Neurology

Background:

• Autoimmune encephalitides targeting neuronal surface antigens are severe neurological disorders.
• These conditions affect patients of all ages and are often treatable with immunotherapy.
• They offer insights into immune-mediated disruptions of neuronal function, leading to cognitive, behavioral, and motor impairments.

Purpose of the Study:

• To review animal models and mechanisms of autoimmune encephalitides.
• To explore autoantibodies targeting neuronal surface antigens.
• To understand immune disruptions of neuronal proteins.

Main Methods:

• Review of existing literature on animal models for autoimmune encephalitides.
• Analysis of passive transfer models using patient-derived samples (CSF, serum IgG, monoclonal antibodies).
• Evaluation of active immunization models, particularly for anti-NMDAR encephalitis.

Main Results:

• 18 known encephalitides involve cell-surface autoantibodies, with 16 targeting neuronal proteins.
• Passive transfer models demonstrate autoantibody pathogenicity but have limitations in studying neuro-immunobiology and long-term disease.
• Active immunization models, like those for anti-NMDAR encephalitis, overcome limitations, enabling study of chronic disease and novel therapies.

Conclusions:

• Animal models, despite imperfections, provide significant neurobiological and immunological insights.
• These models are instrumental in translating experimental findings into clinical applications for autoimmune encephalitides.
• Further development of animal models is essential for advancing treatment strategies.