Abstract Details

Title Retinal Pathology Occurs in Stiff Person Syndrome

Topic General Neurology

Presentation(s) P16 - Poster Session 16 (5:30 PM-6:30 PM)

Poster/Presentation
Number 6-006

Objective To evaluate whether structural and functional changes occur in the afferent visual system of patients with stiff person syndrome (SPS), given the high concentration of γ-aminobutyric acid (GABA)ergic neurons in the retina, and whether these changes correlate with disease burden.

Background SPS is a rare neuroimmunological disorder associated with anti-glutamic acid decarboxylase (antiGAD65) antibodies purportedly targeting inhibitory GABAergic neurons, and for which there is a paucity of biomarkers of disease burden.

Design/Methods In this single-center, cross-sectional study, SPS patients and healthy controls (HCs) underwent optical coherence tomography (OCT), with a subset undergoing high- and low- contrast visual acuity (VA) assessments. Burden of disease was assessed via the number of body regions affected and serum antiGAD65 antibody titers. Individuals with uncontrolled hypertension, or co-morbid neurological or ophthalmological disorders were excluded. Statistical analyses were performed using mixed-effects linear regression models.

Results Thirty-five SPS patients and 40 age- and sex-matched HCs underwent OCT. A subset of 23 SPS patients and 28 HCs underwent VA assessments. Relative to HCs, SPS patients had lower ganglion cell+inner plexiform layer (GCIPL) thicknesses (SPS: 74.36µm [SD: 5.7]; HCs: 76.33µm [SD: 4.2]; p=0.005); inner nuclear layer thicknesses (SPS: 44.37µm [SD: 2.7]; HCs: 45.18µm [SD: 2.2]; p=0.042); 100% (SPS: 53 [SD: 9.6]; HCs: 57.5 [SD: 6.1]; p=0.005), 2.5% (SPS: 24.35 [SD: 10.1]; HCs: 30.16 [SD: 7.7], p=0.006), and 1.25% contrast (SPS: 16.41 [SD: 10.6]; HCs: 20.84 [SD: 8.6]; p=0.034) letter acuity scores. GCIPL thicknesses correlated with the number of body regions affected in SPS (decrease of 1.25µm [95% CI: -2.2µm to -0.3µm; p=0.008] per additional body region affected). Higher antiGAD65 levels were associated with lower thicknesses of the INL (R²=0.25; p=0.006).

Conclusions Retinal neuronal pathology can occur in SPS. OCT may have utility as a biomarker of disease burden in SPS.

Authors/Disclosures
Jeffrey Lambe, MD PRESENTER	Dr. Lambe has nothing to disclose.
Alissa Rothman	No disclosure on file
Jerry Prince	No disclosure on file
Shiv Saidha, MD (Johns Hopkins)	Dr. Saidha has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Setpoint Medical. Dr. Saidha has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. Dr. Saidha has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. 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 $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 $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. 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.
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.
Scott D. Newsome, DO, FAAN (Johns Hopkins Hospital)	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 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 Lundbeck. The institution of Dr. Newsome has received research support from Sanofi. The institution of Dr. Newsome has received research support from Kyverna Therapeutics. 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.

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