Purpose: Morvan syndrome (MoS) is a neurologic disorder belonging to a spectrum of autoimmune encephalitis, Contactin-associated protein-like 2 (Caspr2) antibody syndrome. We report a case of bilateral panuveitis associated with MoS.
Methods: Case report and review of the literature.
Results: A 57-year-old male with Morvan syndrome presented with painless vision loss and floaters. Initial visual acuities were 20/50 and 20/60. Hallmarks of this uveitis included persistently active vitritis, and nonhemorrhagic retinitis with nonperfusion and neovascularization. Uveitis consistently flared with attempted immunosuppressive tapers. Vision deteriorated to count fingers (2 ft) OU over 2.5 years despite corticosteroids, mycophenolate mofetil, intravenous immunoglobulin, adalimumab, and rituximab. Explanations for reduced final visual acuity included macular atrophy, disruption of retinal architecture, epiretinal membrane, vitritis, and cataract.
Conclusions: This case constitutes the first report of uveitis associated with MoS and Caspr2 antibody syndrome, raising the question of autoimmunity targeting the retinal inner and/or outer plexiform layers.
Keywords: Autoimmune; Caspr2; Morvan; Uveitis; encephalitis; vasculitis.