Abstract Details

Title A Multi-center Case Series of Sarcoid Optic Neuropathy

Topic Autoimmune Neurology

Presentation(s) Autoimmune Neurology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 040

Objective The diagnosis of sarcoid optic neuropathy is time-sensitive, as delayed treatment risks irreversible vision loss. We sought to analyze its characteristics and outcomes.

Background The optic nerve is one of the most common cranial nerves to be involved by neurosarcoidosis. Sarcoid optic neuropathy mimics other autoimmune, infectious, and neoplastic causes of optic neuropathy. Current diagnostic criteria for definitive neurosarcoidosis require a central nervous system biopsy demonstrating non-caseating granulomas. Although optic nerve biopsy may confirm the diagnosis, it risks iatrogenic vision loss.

Design/Methods We performed a multi-center retrospective study of sarcoid optic neuropathy among 5 USA medical centers. Inclusion criteria were: 1) clinical optic neuropathy; 2) optic nerve enhancement on neuroimaging; 3) pathological confirmation of systemic or nervous system sarcoidosis.

Results Fifty-one patients were included. The median onset age was 50 years (range, 17-70 years) and 71% were female. The median visual acuity at nadir in the most affected eye was 20/80 (range, 20/20 to no-light-perception). Thirty-four of 51 (67%) patients had radiologic evidence of other nervous system involvement and 20 (39%) patients had symptoms/signs of other cranial nerve dysfunction. Cerebrospinal fluid analysis revealed an elevated white blood cell count in 22 of 31 (71%) patients (median: 14/µL; range: 1-643/µL). Pathologic confirmation of sarcoidosis was by biopsy of systemic/pulmonary site, 34 (67%); optic nerve/sheath, 9 (18%); or other nervous system region, 8 (16%). Despite improvement with treatment (corticosteroids, 98%; steroid-sparing immunosuppressants, 65%) in 40 patients (78%), 11 of 46 (24%) with details available had severe deficits in visual acuity (<20/200) at last follow-up.

Conclusions Sarcoid optic neuropathy rarely occurs in isolation and diagnostic confirmation occasionally requires optic nerve/sheath biopsy. Improvement with treatment is common but most patients have some residual visual disability. Improved recognition and a more expeditious diagnosis and treatment may spare patients from permanent vision loss.

Authors/Disclosures
Lauren M. Webb, MD PRESENTER	Dr. Webb has nothing to disclose.
John Chen	John Chen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB. John Chen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. John Chen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB.
Allen J. Aksamit, Jr., MD, FAAN (Mayo Clinic)	Dr. Aksamit has nothing to disclose.
Shamik Bhattacharyya, MD, FAAN (Brigham and Women's Hospital)	Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NeuroLambda. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Continuum. Dr. Bhattacharyya has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wiley. Dr. Bhattacharyya has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Merck. The institution of Dr. Bhattacharyya has received research support from Alexion Pharmaceuticals. The institution of Dr. Bhattacharyya has received research support from National Institute of Health. The institution of Dr. Bhattacharyya has received research support from UCB. The institution of Dr. Bhattacharyya has received research support from Genentech. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care.
Bart Chwalisz, MD (Massachusetts General Hospital, Department of Neurology)	Dr. Chwalisz has nothing to disclose.
Denis T. Balaban, MD (Massachusetts General Hospital)	Dr. Balaban has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Infucare. The institution of Dr. Balaban has received research support from ArgenX.
Giovanna Manzano, MD	Dr. Manzano has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Gilead Sciences. Dr. Manzano has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for InfuCare Rx.
	No disclosure on file
Jennifer Lord, MD (Novant Health)	Dr. Lord has nothing to disclose.
Stacey Clardy, MD, PhD, FAAN (University of Utah)	Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca/Alexion. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen/Horizon. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. Dr. Clardy has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology/AAN Publications. The institution of Dr. Clardy has received research support from NIH/NINDS. The institution of Dr. Clardy has received research support from SRNA. The institution of Dr. Clardy has received research support from Alexion/AstraZeneca. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with U of Iowa, Miami, Stanford, Barrow, Beaumont Health, CCF, Emory, Penn State, Mayo Clinic, Walter Reed.
Rohini D. Samudralwar, MD (The University of Pennsylvania)	Dr. Samudralwar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Samudralwar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Samudralwar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Samudralwar has received personal compensation in the range of $500-$4,999 for serving as a Speaker with Multiple Sclerosis Association of America.
Yang Mao-Draayer, MD (University of Michigan, Department of Neurology)	Dr. Mao-Draayer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen Idec. Dr. Mao-Draayer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genzyme-Sanofi. Dr. Mao-Draayer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. Dr. Mao-Draayer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genzyme-Sanofi. Dr. Mao-Draayer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Mao-Draayer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizone. Dr. Mao-Draayer has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen Idec. The institution of Dr. Mao-Draayer has received research support from NIH .
	No disclosure on file
M. Tariq Bhatti, MD (Kaiser Permanente, Northern California)	Dr. Bhatti has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Receptos . Dr. Bhatti has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for NIH LHON gene therapy study .
	No disclosure on file
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic)	The institution of Dr. Flanagan has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Merck. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology that is relevant to AAN interests or activities.

��ɫ��

��ɫ��