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. 2021 Apr 14;12:664664. doi: 10.3389/fneur.2021.664664

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Copyright © 2021 Bhagavati.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Autoimmmune diseases of the CNS: (A) Excitatory synapses: NMDAR encephalitis is caused by anti–NMDA receptor (NMDAR) antibodies binding and cross-linking GluN1 subunits, disrupting interaction of NMDAR with ephrin type B receptor 2 (EPHB2) causing internalization of NMDAR and impaired glutamergic transmission. LGI1 encephalitis is caused by auto-antibodies to leucine rich glioma inactivated protein 1 (LGI1), a neuronal glycoprotein secreted into the synapse. LGI1 interacts with presynaptic disintegrin and metalloproteinase domain-containing protein 2 (ADAM23) and postsynaptic ADAM22, modulates AMPA receptor trafficking and presynaptic Kv1, voltage gated potassium channel function. PSD95, post-synaptic density protein 95. Other receptors targeted: AMPAR; mGluR1/5. Inhibitory synapses: Autoantibodies to GABA type A receptor (GABA_AR) on inhibitory synapses may lead to encephalitis with intractable seizures. Autoantibodies to the glycine receptor (GlyR) cause painful spasms; progressive encephalitis with rigidity and myoclonus (PERM). Stiff person syndrome can also be caused by auto-antibodies to amphiphysin, a presynaptic protein expressed in all synapses, important in clathrin mediated endocytosis leading to a decreased number of presynaptic vesicles filled with neurotransmitter available for exocytosis. Higher tonic activity may make inhibitory synapses (release of GABA and glycine) especially vulnerable. GABARAP, GABA associated receptor protein. GAD, decarboxylates glutamate to GABA (not shown). (B) Neuromyelitis optica (NMO) is caused by auto-antibodies to Aquaporin 4 (AQP4) water channel expressed widely in the body including on astrocytic foot processes abutting capillary endothelial cells and ependymal cells. Predominant pathology is in spinal cord, optic nerve and brain peri-ependymal regions.