Author Of 2 Presentations
P0732 - Neuromyelitis Optica Spectrum Disorder in the U.S. Active-Duty Military (ID 1203)
Neuromyelitis Optica Spectrum Disorder (NMOSD) is a devastating neurological disorder of particular importance to the U.S. Military because it is highly disabling, often rapidly causing complete or partial blindness and/or paralysis. NMOSD can permanently disqualify a service-member from continued duty, leading not only to personal disability for the member, but degraded capability for the Force.
The objective of this study is to define the epidemiology of NMOSD among U.S. Military Active Duty Service Members (ADSM). Specifically, we aimed to understand the prevalence, demographics, disease subtypes (seropositive (SP), seronegative (SN), anti-MOG), and disease associations among ADSM diagnosed with NMOSD between 2004 and 2019.
A U.S. Military Health Systems database (MHS Mart, M2) was searched for outpatient clinic encounters that listed ICD9 (341.0) and ICD10 (G36.0) codes for NMOSD between 2004 and 2019. ADSM with at least one encounter listing an ICD9 or ICD10 code for NMOSD were included in the study. Relevant information was then abstracted systematically from clinical electronic medical records systems (AHLTA, HAIMS, JLV). A clinical database was generated. Data was analyzed using standard statistical methods. This project was reviewed and approved by the Walter Reed National Military Medical Center IRB and the University of Utah/SLC VA IRB.
118 patients were identified. Forty Seven (48) met 2015 criteria for NMOSD. Of these, 28 were women. There were 34 SP, 10 SN, and 4 anti-MOG patients. The average age at time of disease onset was 36 years for women, and 40 years for men. Age of onset by serostatus was 40 years for SP, and 32 years for SN patients. Distribution by race was as follows: Black: 25, Caucasian: 16, Other or Unknown: 4, Hispanic: 2, Asian / Pacific Islander: 1. SP patients presented with longitudinally extensive transverse myelitis (LETM) (15), optic neuritis (ON) (11), area postrema syndrome (4), or multifocal symptoms (3). SN patients presented with LETM (5), ON (2), or multifocal symptoms (2). Co-existing autoimmunity was seen in 19 SP patients and 1 SN patient. The most common co-existing autoimmune disease was Sjogren Syndrome (7). The incidence of NMOSD in the US Military ranged from 0 – 0.7 cases per 100,000 ADSM for the period in question.
This study clarifies the epidemiology of NMOSD within the US Military during the years 2004-2019. It demonstrates disease burden based on race and gender and confirms an association of co-existing autoimmunity among patients with SP NMOSD. Further research assessing pre-symptomatic sera for AQP4-IgG from the Department of Defense Serum Repository in this cohort will hopefully shed further light on this disease.
P0848 - Characterization of Retinal Nerve Fiber Layer Thickness in a Cohort with Glutamic Acid Decarboxylase and Glycine Receptor Autoimmunity (ID 1154)
Glutamic acid decarboxylase (GAD-65) and glycine receptor (GlyR) autoimmunity includes a wide range of clinical phenomena including stiff-person syndrome and epilepsy. Both GAD and GlyR interact with the retina. Optical coherence tomography (OCT) has previously been used in a variety of other neurological disorders to establish baseline characteristics and monitor disease course. This presents a noninvasive opportunity to evaluate for a biomarker that may assist with treatment of these rare but debilitating disorders.
To provide a description of the retinal nerve fiber layer (RNFL) in patients with GAD-65 and GlyR neurological autoimmunity.
OCT measures of RNFL were studied in patients with GAD-65 and GlyR neurological autoimmunity and compared to that of 148 healthy control eyes and 472 eyes from patients with multiple sclerosis.
The 15 patients were mostly female, in keeping with reported female preponderance in these conditions. We report initial post-diagnosis mean RNFL thickness and standard deviations, by sector, for the whole group, as compared with respective controls and patients with multiple sclerosis. Multiple sectors showed RNFL thinning, most apparent in the anti-GAD-65 group.
This study provides insight into baseline post-diagnosis RNFL thickness in a group with GAD-65 and GlyR autoimmunity, two conditions which may produce varied symptoms. While limited by sample size, RNFL thinning was most evident in the anti-GAD-65 group, and to a lesser extent, in the anti-GlyR group. This provides a baseline characterization and suggests that future studies should be done to determine the utility of OCT as a biomarker for these conditions.