PT  - JOURNAL ARTICLE
AU  - Amrutkar, Chaitanya
AU  - Burton, Erik
TI  - Chronic Relapsing Inflammatory Optic Neuropathy (P2.147)
DP  - 2018 Apr 10
TA  - Neurology
PG  - P2.147
VI  - 90
IP  - 15 Supplement
4099  - http://n.neurology.org/content/90/15_Supplement/P2.147.short
4100  - http://n.neurology.org/content/90/15_Supplement/P2.147.full
SO  - Neurology2018 Apr 10; 90
AB  - Objective: To highlight the diagnostic challenges and need for early recognition of Chronic Relapsing Inflammatory Optic Neuropathy (CRION)To describe a case of CRION successfully managed with of atumumab, a fully human CD20 monoclonal antibodyBackground: CRION is an exceedingly rare cause of optic neuritis, often overlooked, leading to poor visual outcomes.Design/Methods: Case presentationResults: 42 yo AAF with PMHx of asthma and migraine, with family history of ocular sarcoidosis and lupus in first degree relatives, presented with recurrent episodes of painful left eye vision loss. She was treated by a neuro-ophthalmologist with multiple pulse therapies of corticosteroids which helped regain some vision, but only temporarily. She had multiple attacks over next few years, eventually loosing vision in the left eye without right eye involvement. She never experienced weakness, numbness or b/b incontinence. MRI brain revealed post contrast enhancement of left optic nerve associated with recurrent optic neuritis. Neurosarcoidosis, white matter disease, rheumatological disorders were ruled out by extensive evaluation including imaging studies, CSF and blood tests. We evaluated her first in 2014 for new painful right vision loss. On examination, she had no light perception, very pale optic disc, and fixed pupil in left eye. Right eye vision was limited to hand movements, had reactive pupil, and pale optic disc. OCT showed severe thinning of retinal nerve fiber layer in both eyes. VEP had no measurable responses in either eye. She was diagnosed with CRION. She was treated with high dose methylprednisolone and plasma exchange with some recovery in right eye vision. Rituximab was initiated for long term immune-suppression but was discontinued due to allergy. She is now clinically stable on of atumumab.Conclusions: CRION is a diagnosis of exclusion that requires early diagnosis and treatment. Treatment by B-cell depletion appears to be effective.Disclosure: Dr. Amrutkar has nothing to disclose. Dr. Burton has nothing to disclose.