DR. AANCHAL SINGHAL

DR.(MAJ) SHANKAR SANDEEP, DR. ASHOK KUMAR, DR. CHAITANYA YV KRISHNA

Abstract

Optic neuritis (ON) typically presents unilaterally in young females frequently associated with MS. Bilateral or ‘Atypical’ ON is a rare occurrence associated with Neuromyelitis Optica (NMO) or systemic autoimmune disorders. Pemphigus foliaceus (PF) is an autoimmune disorder characterized by IgG auto-antibodies against glycoprotein desmoglein-1. A singular case of NMO in a patient with PF has been reported till date.

We present a case of bilateral acute ON with profound visual loss in a patient with PF in whom NMO was ruled out after thorough investigation. A miraculous response was seen following high dose systemic corticosteroids where significant improvement in vision from No Light Perception to 5/60 both eyes over a course of 7 days was seen which improved to 6/12 in right eye and 6/9 in Left eye after 1 month. To conclude, it is of utmost importance to recognize Atypical ON at the earliest, investigate thoroughly and manage aggressively for visual rehabilitation of the patient.