H. Abboud
University Hospitals Cleveland Medical Center
Neurology

Author Of 2 Presentations

 On-Demand Program
Imaging Late Breaking Abstracts

LB1177 - PAMRINO: International MRI and clinical data repository for neuromyelitis optica spectrum disorder (ID 469)

Speakers
Authors
Presentation Number
LB1177
Presentation Topic
Imaging

Abstract

Background

Neuromyelitis optica spectrum disorders (NMOSD) encompasses a group of rare inflammatory diseases which primarily target the optic nerves, spinal cord, and brain. Typically, magnetic resonance imaging (MRI) data from single-center studies comprise 20-50 patients, limiting statistical power for outcomes research. Using retrospective data from the PArallel MRI in NmOsd (PAMRINO) study, a novel prospective NMOSD image repository (NMOsDIR) representing multiple international sites was coordinated by Charité-Universitätsmedizin Berlin and the Medical Image Analysis Center (Basel).

Objectives

The PAMRINO study aimed to investigate and analyze retrospective MRIs collected from NMOSD-specialized centers, potentially for the evaluation of disease-related brain and spinal cord changes. NMOsDIR serves as an international imaging research resource (comprising standardized retinal optical coherence tomography and MRI scans) and clinical data hub for prospective studies in NMOSD. Linking imaging and clinical data, as well as enabling analysis pipelines for each modality, will facilitate multi-centered studies using sufficient data and statistical power to advance outcomes research in this rare disease.

Methods

For clinical data collection in PAMRINO, a Research Electronic Data Capture (REDCap) platform was used, where participating centers entered data relevant for NMOSD patient monitoring. An image database (XNAT) was established for image uploads. This large collection of MRI data is currently being analyzed in a joint international effort of NMOSD clinical neuroradiologists and scientists.

Results

Brain, spinal cord, and optic nerve MRI scans with associated clinical data were collected from 514 NMOSD patients and 56 healthy controls from 17 international centers. Roughly 20,000 individual MRI scans from patients and healthy controls were collected. Of these, 78% had T1-weighted cerebral MRIs (55% with 3D scans), 80% had T2-weighted cerebral MRIs (54% with 3D scans), 86% had T2-weighted spinal cord MRIs (55% with 3D scans), and 35% had optic nerve MRIs.

Conclusions

We successfully established PAMRINO, an international collaborative retrospective MRI and clinical data repository. The knowledge gained during this process provided important new insights, where the initial analysis of the dataset has underscored the large degree of heterogeneity in image and clinical data collection in NMOSD-specialized centers. Thus, calling for more standardized methods of data acquisition and imaging analysis, as not to limit research opportunities. The new longitudinal, prospective NMOsDIR will help us to answer many pressing - yet open - questions regarding patients seropositive for aquaporin-4-IgG+, myelin oligodendrocyte glycoprotein-IgG+ and other autoimmune-related diseases. In turn, such a strategy will strengthen future capabilities in research, diagnosis, monitoring and improving NMOSD patient care.

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Neuromyelitis Optica and Anti-MOG Disease Poster Presentation

P0745 - Predictors of Diagnosis With A First Presenting Symptom Of Optic Neuritis (ID 1574)

Speakers
Authors
Presentation Number
P0745
Presentation Topic
Neuromyelitis Optica and Anti-MOG Disease

Abstract

Background

Optic neuritis (ON) is a shared symptom between Multiple Sclerosis (MS), Neuromyelitis Optica Spectrum Disorder(NMOSD) with aquaporin-4 antibody (AQP4-IgG), Myelin Oligodendrocyte Glycoprotein antibody (MOG-IgG) associated disease (MOGAD), double seronegative NMOSD, and several other inflammatory conditions. With the initial ON attack, the clinical phenotype may not be apparent at the time of initial evaluation and management. Predicting the clinical phenotype at the time of presentation can have important implications on acute immunotherapy, bridging treatment, selection of disease modifying therapy, and prognosis. Identifying characteristics of the ON presentation that predict the final phenotype can inform treatment decisions and expedite appropriate management.

Objectives

To evaluate the frequency and predictors of the final clinical phenotype following a first diagnosed attack of optic neuritis.

Methods

We performed a retrospective analysis of patients presenting to our hospital system with a first ON attack to determine eventual outcome. Only patients with an evaluation by an ophthalmologist within 3 weeks of symptom onset and at least 6-month follow-up following the initial attack were included. Patients with prior neurological attacks or known clinical phenotype prior to the ON presentation were excluded.

Results

Forty-three patients met inclusion criteria. Of those, 20 (47%) were eventually diagnosed with MS, 8 (19%) with idiopathic monophasic ON, 6 (14%) with MOGAD, 3 (7%) with NMOSD with AQP4-IgG, 1 (2%) with double seronegative NMOSD, 1 (2%) with chronic relapsing inflammatory optic neuritis (CRION), and 4 (9%) with optic neuritis secondary to a systemic disorder (Sarcoidosis, Scleroderma, or Mixed Connective Tissue Disease). Of those patients with mildly diminished visual acuity (20/40 or better), 85% were eventually diagnosed with MS. Of those patients with severely diminished visual acuity (light perception, counting fingers, or hand movement vision), only 40% were eventually diagnosed with MS, and 25% were diagnosed with MOGAD or NMOSD. All patients with bilateral optic nerve involvement at onset developed NMOSD, MOGAD, or idiopathic disease, and none developed.

Conclusions

Based on our data, MS is the most common final phenotype in patients whose first neurological presentation is optic neuritis followed by idiopathic monophasic disease. MOGAD was the third most common final phenotype and was more common than NMOSD with AQP4, double seronegative NMOSD, and seronegative CRION. Patients with a more severe visual acuity insult on presentation tended to develop MOGAD or NMOSD, while those with milder presentations were more likely to develop MS. Bilateral optic nerve involvement seemed to exclusively occur in patients who developed an MOGAD or NMOSD.

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