PT  - JOURNAL ARTICLE
AU  - Manzano, Giovanna
AU  - Salky, Rebecca
AU  - Mateen, Farrah
AU  - Klawiter, Eric
AU  - Chitnis, Tanuja
AU  - Levy, Michael
AU  - Matiello, Marcelo
TI  - Positive Predictive Value of MOG-IgG for Clinically Defined MOG-AD within a Real-world Large Institution Cohort (S41.003)
DP  - 2022 May 03
TA  - Neurology
PG  - 49
VI  - 98
IP  - 18 Supplement
4099  - http://n.neurology.org/content/98/18_Supplement/49.short
4100  - http://n.neurology.org/content/98/18_Supplement/49.full
SO  - Neurology2022 May 03; 98
AB  - Objective: To determine the positive predictive value of serum MOG-IgG for clinically defined MOG-AD in a large institutional cohort study.Background: Myelin oligodendrocyte glycoprotein-associated disease (MOG-AD) is a monophasic or relapsing CNS demyelinating disease, typically presenting as optic neuritis, transverse myelitis, and/or ADEM-like syndromes. The positive predictive value (PPV) of serum MOG-IgG has been reported to range from 82–100%, dependent upon assays and study design (Waters et al. (2019)).Design/Methods: The Massachusetts General Brigham Research Patient Data Registry database was queried for patients with positive serum MOG-IgG detection, at least once, between January 1, 2017 and March 25, 2021. All were tested via the Mayo Clinic MOG-IgG1 fluorescence-activated cell sorting assay. MOG-IgG positive cases were reviewed for fulfillment of typical MOG-AD clinical features, determined by treating neurologists and study authors.Results: Of 80 patients who tested positive for MOG-IgG with titer ≥1:20 (44F; 36M; ages 5–81, median 37 years), 67 had validated MOG-AD, yielding PPV of 83.3%. Using a ≥1:40 titer cutoff, PPV was 93.8%. Three MOG positive cases had a prototypical multiple sclerosis diagnosis (RRMS n=2, 1:20 and 1:40; PPMS n=1; 1:100). The treating diagnosis for one RRMS patient with a 1:40 titer was subsequently modified to MOG-AD by treating neurologists. Validated diagnoses of the remaining positive patients without MOG-AD included: migraine (n=2, 1:20, 1:100), inclusion body myositis (n=1; 1:100), autoimmune encephalitis (n=2; 1:20, 1:20), hypoxic ischemic brain injury (n=1; 1:20), IgG4-related disease (n=1, 1:20), idiopathic hypertrophic pachymeningitis (n=1, 1:20) and cases of reported prior monophasic vision loss and sensory changes without supportive objective data (n=2; 1:20, 1:40).Conclusions: In our cohort, PPV for MOG-IgG was 83.8% for titers ≥1:20 and 93.8% for titers ≥1:40, comparable to prior reports. The presence of positive cases with and without demyelinating features, emphasizes a need for appropriate ordering, analysis of titer value and appropriate clinical interpretation.Disclosure: Dr. Manzano has nothing to disclose. Ms. Salky has nothing to disclose. Dr. Mateen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Dr. Mateen 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. Mateen 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. Mateen 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. Mateen 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. The institution of Dr. Mateen has received research support from Genentech. The institution of Dr. Mateen has received research support from NIH. The institution of Dr. Mateen has received research support from National MS Society. The institution of Dr. Mateen has received research support from Sumaira Foundation. The institution of Dr. Mateen has received research support from EMD Serono. Dr. Mateen has received research support from Biogen. Dr. Klawiter has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Galen/Atlantica. Dr. Klawiter 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. Klawiter has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Banner Life Sciences. Dr. Klawiter has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Greenwich Biosciences. The institution of Dr. Klawiter has received research support from Biogen. The institution of Dr. Klawiter has received research support from Abbvie. The institution of Dr. Klawiter has received research support from Genzyme. The institution of Dr. Klawiter has received research support from Genentech. Dr. Chitnis has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. Dr. Chitnis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Chitnis has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Roche-Genentech. Dr. Chitnis has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Academic CME. The institution of Dr. Chitnis has received research support from Novartis. The institution of Dr. Chitnis has received research support from Sanofi. The institution of Dr. Chitnis has received research support from Octave. 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 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 Viela Bio. 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. Dr. Matiello has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for VielaBio.