Retinal pathology in spontaneous opticospinal experimental autoimmune encephalitis mice.

Retinal ganglion cells (RGC) are lost as a sequela of optic nerve inflammation in myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD), but the mechanisms of injury remain incompletely understood and there remains a need to characterize the murine model of MOGAD. Several studies have shown that RGC loss occurs in association with optic neuritis in MOG35-55 experimental autoimmune encephalomyelitis (EAE), but retinal pathology has not been studied in the double transgenic opticospinal EAE (OSE) model, in which animals develop spontaneous disease associated with MOG35-55 peptide specific T cells and B cells producing MOG-specific antibodies. Herein, we show that at 8-weeks OSE mice develop optic nerve inflammation, reactive astrogliosis, and RGC loss. By 10-weeks of age, affected mice have a 50% reduction in RGCs as compared to age matched wild type mice without EAE. The retinal pathology that ensues from spontaneous optic neuritis in OSE mice mirrors that seen following human optic neuritis and may be a useful model for screening neuroprotective compounds for MOGAD and other diseases with optic neuritis.