Author Of 1 Presentation
P1071 - Central Vein Sign in Pediatric Multiple Sclerosis and MOG Antibody Associated Disease. (ID 859)
Abstract
Background
Multiple sclerosis (MS) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are demyelinating conditions of the central nervous system that have been difficult to distinguish based on clinical presentation or MRI findings. MOG-IgG titers are used to help differentiate between MS and MOGAD currently, but has been unreliable as MOG-IgG titers fluctuate, titer thresholds are not yet clear, and titers can become undetectable between relapses. MOGAD is detected in up to 30% of children with acute demyelination and treatment options and prognosis are different for MOGAD and MS. Thus, early and accurate diagnosis is essential. The central vein sign (CVS) on brain magnetic resonance imaging (MRI) is now a promising marker for adult MS and can differentiate MS from its mimics. The presence of this marker is not well established in pediatric patients.
Objectives
We aimed to evaluate the rate of CVS within demyelinating lesions of the brain in children with MS and MOGAD and determine its diagnostic value in distinguishing these diseases.
Methods
Patients with a diagnosis of pediatric onset MS (POMS) or MOGAD at last follow-up were identified in a pediatric demyelinating database at St. Louis Children’s Hospital, which was maintained for the US Network of Pediatric MS Centers. Two reviewers, blinded to the clinical diagnosis, retrospectively reviewed clinically obtained brain MRIs in each of these patients. Fluid attenuated inversion recovery (FLAIR) sequences were used to identify lesions. Susceptibility weighted imaging (SWI) fused to the FLAIR sequences were used to assess the prevalence of CVS. Differences in CVS between POMS and MOGAD were evaluated, and agreement in CVS number was reported using an intraclass correlation coefficient (ICC).
Results
A total of 20 pediatric patients, 10 with POMS and 10 MOGAD, were assessed. Mean (SD) age was 11.6(4.7) years in POMS and 7.1(3.3) years in MOGAD. 60% in POMS and 70% in MOGAD were female. The CVS was significantly more prevalent in POMS when compared to MOGAD, 53% of total lesions compared to 19%, respectively (p<0.001). Inter-rater reliability for identifying the total number of white matter lesions was good (ICC 0.88 [95%CI: 0.723, 0.950]). However, the inter-rater reliability for detecting the number of CVS lesions was poor (ICC 0.23 [95%CI: 0.221, 0.598]).
Conclusions
The CVS can be a useful diagnostic tool to help differentiate MS from MOGAD in pediatric patients, but poor inter-rater reliability using current clinical brain MRIs may limit the usefulness of CVS in individual cases. Application of CVS to individual cases of MS versus MOGAD could be improved with an MRI sequence optimized for detection of CVS, as well as a validated automated assessment to diminish the effects of variability between reviewers.