Author Of 3 Presentations
LB1231 - Demographic and Clinical Profile of Pediatric patients with Multiple Sclerosis infected with SARS-Cov2 (ID 2111)
Abstract
Background
COVID-19, the disease caused by SARS CoV2, causes severe respiratory disease, and rarely multisystem inflammatory syndrome, in some pediatric patients. Little is known about the disease course among patients with pediatric-onset multiple sclerosis.
Objectives
To describe the demographic and clinical characteristics of a subgroup of pediatric-onset multiple sclerosis (POMS) patients infected with SARS CoV2.
Methods
The Network of Pediatric Multiple Sclerosis Centers (NPMSC), a consortium of 10 US pediatric multiple sclerosis (MS) centers contributes clinical information about POMS patients and demyelinating disorders to a centralized database, the Pediatric Demyelinating Disease Database (PeMSDD), to facilitate research for this rare disorder. In addition to collecting clinical data on clinical course, comorbidities, disease modifying therapy use, and functional status, the NPMSC developed a screening questionnaire to administer to patients during standard of care visits to further evaluate their COVID- 19 status. Additionally POMS patients with confirmed or highly suspected COVID-19, will be assessed for risk factors including smoking use, recent glucocorticoid use, comorbidities; clinical presentation, including symptoms, radiological and laboratory data; COVID-19 treatments and outcomes. POMS patients will also complete the COViMS (COVID-19 Infections in MS & Related Diseases) database, a joint effort of the US National MS Society and the Consortium of MS Centers to capture information on outcomes of people with MS and other central nervous system (CNS) demyelinating diseases (Neuromyelitis Optica Spectrum Disease, or MOG antibody disease) who have developed COVID-19. Together with data collected from the PeMSDD, we will present comprehensive data on the POMS patient experience with COVID-19 and compare it to POMS patients without known or suspected COVID-19.
Results
Data collection continues. Results available by the meeting due date will describe the demographics, risk factors, treatments and outcomes of POMS with COVID-19.
Conclusions
Conclusions will be drawn pending results of data analysis. We anticipate reporting on demographic data, risk factors, outcomes and any associations with disease modifying therapy.
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.
P1082 - Therapeutic Response in Pediatric Neuromyelitis Optics Spectrum Disorder (ID 1820)
Abstract
Background
Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune condition which can led to significant disability. Approximately 4% of the NMOSD cases are pediatric onset. At present, there are limited studies that aim at guiding physicians in their treatment choices for NMOSD in children.
Objectives
To evaluate the effect of different disease modifying therapies (DMT) with respect to attack prevention in children with NMOSD.
Methods
Cohort study that included 12 clinical centers participating in the US Network of Pediatric MS Centers. Cases were validated for NMOSD diagnostic criteria and classified via serostatus as AQP4+, MOG+, or double-seronegative (DS). Clinical data, including demographics, attack details, type of initial DMT (rituximab, mycophenolate mofetil, azathioprine, IVIg) and neurological visit data were extracted from charts, centrally collected in a database, and analyzed. Treatment response in the three serostatus subgroups was evaluated. Effect of DMTs on annualized relapse rate (ARR) was assessed by negative binomial regression.
Results
111 pediatric patients with NMOSD were identified: 80 AQP4+, 10 MOG+, 14 double seronegative (DS), and 7 with unknown serostatus (94 females and 17 males; 48 white, 47 African American, 13 other races). Mean follow-up duration was 1.9 years (SD±2.2). About 6% of patients were treatment-naive. First-line DMTs varied by serostatus: in the AQP4+ subgroup 42% used rituximab, 16% mycophenolate mofetil, 16% azathioprine, and 8% IVIg. Among MOG+ patients, 13% received rituximab, 13% azathioprine, 13% mycophenolate, and 38% IVIg. Within the DS group, rituximab was used in 21% of cases, azathioprine in 7%, mycophenolate in 21%, and IVIg in 21%. In the unknown serogroup, 33% received rituximab, 17% azathioprine, 0% mycophenolate, and 33% IVIg. The ARR calculated in all the serogroups was 0.25 (95% CI 0.13-0.46) for rituximab, 0.73 (95% CI 0.27-2.00) for azathioprine, 0.40 (95% CI 0.18-0.89) for mycophenolate, and 0.56 (95% CI 0.26-1.20) for IVIg. In the AQP4+ subgroup, the patients started on rituximab showed an ARR of 0.25 (95% CI 0.13-0.48), those on azathioprine an ARR of 0.76 (95% CI 0.24-2.39), those on mycophenolate an ARR 0.43 (95% CI 0.17-1.07), and those on IVIg an ARR of 0.63 (95% CI 0.26-1.55).
Conclusions
This retrospective study showed that rituximab is associated with a lowered annual relapse rate in pediatric NMOSD and in particular in the AQP4+ subgroup.