Bilateral Intracranial Optic Nerve and Chiasmal Involvement in IgG-4 Related Disease

"IgG-4 related disease (IgG4-RD) is a disease that can affect many organs and is characterised by lymphoplasmocytic infiltration of IgG4-positive cells, obliterative phlebitis and storiform fibrosis. Orbital and ocular adnexal involvement in IgG4-RD has been reported with reports of optic nerve involvement. Systemic steroids are the primary treatment used in IgG4-RD. However, the visual outcome is not frequently reported. We present a case of severe loss of vision due to biopsy-proven IgG4-RD bilateral intracranial optic nerve and chiasmal involvement with pachymeningitis.