Abstract Details

Title Microcystic Macular Edema in Neuromyelitis Optica Is Associated with Visual Disability and Retinal Axonal and Neuronal Loss

Topic MS and Related Diseases

Presentation(s) IN4 - (-)

Poster/Presentation
Number 009

Objective

Background NMO-associated optic neuritis (ON) is characterized by poor visual outcomes, often resulting in blindness. Studies utilizing OCT have demonstrated severe retinal axonal and neuronal loss following NMO-associated ON, primarily thought to represent sequelae of optic nerve injury. However, abnormalities of the retinal vasculature have also been identified in NMO-ON eyes, suggesting that direct retinal injury may also be operative in NMO. Interestingly, aquaporin-4 (the target of NMO-IgG) is highly expressed in the retina.

Design/Methods In this cross-sectional study, 39 patients with NMO-spectrum disorders and 39 age and sex-matched healthy-controls underwent spectral-domain OCT and visual function testing.

Results Microcystic macular edema (MME) of the inner nuclear layer (INL) was identified in 10 of 39 patients (26%) and was exclusively found in eyes with a history of ON. MME eyes had significantly lower high and low-contrast letter-acuity scores (100%:p=0.002; 2.5%:p=0.002; 1.25%:p=0.004), lower peri-papillary retinal nerve fiber layer (RNFL) thickness (p=0.04), lower macular-RNFL thickness (p=0.004), lower ganglion cell layer+inner plexiform layer (GCIP) thickness (p=0.007), higher INL thickness (p<0.001), and a greater number of ON episodes (p=0.008), relative to non-MME eyes with a history of ON. NMO-spectrum eyes without ON history had lower macular-RNFL thickness (p=0.003), GCIP thickness (p=0.002), ONL thickness (p=0.02) and low-contrast letter-acuity scores (2.5%:p=0.03; 1.25%:p=0.002) compared to healthy-controls.

Conclusions MME of the INL in NMO is associated with severe retinal axonal and neuronal loss and corresponding visual disability, and may contribute to poor visual outcomes following NMO-associated ON. Additionally, involvement of the anterior visual pathway may occur in NMO-spectrum disorders in the absence of symptomatic ON.

Authors/Disclosures
Elias S. Sotirchos, MD (Johns Hopkins University) PRESENTER	Dr. Sotirchos 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. Sotirchos 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. Dr. Sotirchos has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Sotirchos 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. The institution of Dr. Sotirchos has received research support from National Institutes of Health. The institution of Dr. Sotirchos has received research support from National Multiple Sclerosis Society. The institution of Dr. Sotirchos has received research support from Sumaira Foundation. The institution of Dr. Sotirchos has received research support from Genentech. The institution of Dr. Sotirchos has received research support from UCB. The institution of Dr. Sotirchos has received research support from Astoria Biologica. The institution of Dr. Sotirchos has received research support from Ad Scientiam. The institution of Dr. Sotirchos has received research support from Alexion. The institution of Dr. Sotirchos has received research support from Corevitas. Dr. Sotirchos has received personal compensation in the range of $500-$4,999 for serving as a Ad Hoc Reviewer with National Institutes of Health.
Makoto Akimoto	No disclosure on file
Shiv Saidha, MD (Johns Hopkins)	Dr. Saidha has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ReWind Therapeutics. Dr. Saidha has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Saidha 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. Saidha has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Clene Pharmaceuticals. Dr. Saidha has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Saidha has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Saidha 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. Saidha has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Multiple Sclerosis Journal ETC. Dr. Saidha has stock in June Brain. Dr. Saidha has stock in Lapix Therapeutics. The institution of Dr. Saidha has received research support from Biogen. The institution of Dr. Saidha has received research support from Genentech. The institution of Dr. Saidha has received research support from Novartis. The institution of Dr. Saidha has received research support from Lapix Therapeutics. The institution of Dr. Saidha has received research support from Novartis.
Gita Byraiah, MHS	No disclosure on file
Maureen A. Mealy, PhD, RN	No disclosure on file
	No disclosure on file
	No disclosure on file
Scott D. Newsome, DO, FAAN (Johns Hopkins Hospital)	Dr. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Newsome has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Newsome 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. Newsome has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myers Squibb. Dr. Newsome 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. The institution of Dr. Newsome has received research support from Biogen. The institution of Dr. Newsome has received research support from Genentech/Roche. The institution of Dr. Newsome has received research support from Department of Defense. The institution of Dr. Newsome has received research support from Patient Centered Outcomes Research Institute. The institution of Dr. Newsome has received research support from National MS Society. The institution of Dr. Newsome has received research support from The Stiff Person Syndrome Research Foundation. The institution of Dr. Newsome has received research support from Lundbeck. The institution of Dr. Newsome has received research support from Sanofi. Dr. Newsome has received personal compensation in the range of $10,000-$49,999 for serving as a Lead PI for Clinical Trial with Roche.
John Ratchford, MD (Genentech)	Dr. Ratchford has received personal compensation for serving as an employee of Genentech. Dr. Ratchford has stock in F. Hoffman La-Roche.
Elliot M. Frohman, MD, FAAN	Dr. Frohman has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Janssen. Dr. Frohman has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Janssen. Dr. Frohman 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 Up To Date. Dr. Frohman has received publishing royalties from a publication relating to health care.
Laura J. Balcer, MD, MSCE, FAAN (NYU Grossman School of Medicine)	Dr. Balcer has received personal compensation for serving as an employee of North American Neuro-Ophthalmology Society. An immediate family member of Dr. Balcer has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Children's Hospital of Philadelphia.
	No disclosure on file
Michael Levy, MD, PhD, FAAN (Massachusetts General Hospital/Harvard Medical School)	Dr. Levy has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi Pharma. Dr. Levy has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB Pharma. Dr. Levy has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Levy 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. Dr. Levy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Levy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Levy has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. Dr. Levy has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Various law firms. The institution of Dr. Levy has received research support from National Institutes Health.
Peter A. Calabresi, MD, FAAN (Johns Hopkins University)	Dr. Calabresi has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis. Dr. Calabresi has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Lilly. Dr. Calabresi has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Idorsia. An immediate family member of Dr. Calabresi has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for MyMD. Dr. Calabresi has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Myelin Repair Foundation. The institution of Dr. Calabresi has received research support from Genentech. Dr. Calabresi has received publishing royalties from a publication relating to health care. Dr. Calabresi has received personal compensation in the range of $500-$4,999 for serving as a Study Section Member with NIH. Dr. Calabresi has received personal compensation in the range of $500-$4,999 for serving as a Grant reveiwer with Myelin Repair Foundation. Dr. Calabresi has received personal compensation in the range of $500-$4,999 for serving as a Speaker for CME with NYAS. Dr. Calabresi has received personal compensation in the range of $500-$4,999 for serving as a Speaker with Academic CME.

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