Abstract Details

Title Surveying the Clinical and Radiographic Heterogeneity of Pediatric Anti-MOG-Associated Disease

Topic Autoimmune Neurology

Presentation(s) S3 - Autoimmune Neurology 1: Mechanisms of Disease, Clinical Practice (2:00 PM-2:12 PM)

Poster/Presentation
Number 006

Background Pediatric anti-MOG-associated disease has been traditionally described as monophasic encephalitis in younger children or a relapsing, demyelinating opticospinal phenotype in older children. Yet the full heterogeneity is newly appreciated, as novel presentations are reported.

Objective To survey symptoms, relapses, biomarkers, and imaging patterns associated with pediatric anti-myelin oligodendrocyte glycoprotein (MOG)-associated disease in a large pediatric cohort.

Design/Methods We conducted retrospective chart and imaging review on 62 patients with anti-MOG-associated disease, focusing on their initial presentation and up to two relapses.

Results

Median age at presentation was 7.5 years (IQR = 4 to 11 years). Mean follow up was 8 years (IQR = 3 to 10 years). Optic neuritis was the most common symptom (58%), followed by headache (32%), altered mental status (19%), and fever (18%). 52% of patients (32/62) presented with multiple symptoms, though patients with isolated symptoms largely exhibited optic neuritis (22/30, 73%).

26% of patients had monophasic illness (16/62), 19% (12/62) had a single relapse, and 55% (34/62) had multiple relapses. The mean interval between onset and relapse, or between relapses, was one year. Total relapses did not correlate with age or initial symptoms.

Neither MOG titers during acute episodes nor asymptomatic MOG titers correlated with symptomatology and did not predict relapse.

CSF oligoclonal bands were present in few patients (16%, 5/32). The magnitude of CSF pleocytosis correlated with myelitis (p< 0.01) but not other symptoms.

Of patients with available imaging, 65% (26/40) had a different radiographic phenotype by first relapse compared to presenting phenotype, and 58% (15/26) had a different radiographic phenotype by second relapse compared to first.

Conclusions Pediatric anti-MOG-associated disease presents with heterogeneous symptoms, though isolated optic neuritis was most common. Most patients experienced relapse within one year, often associated with phenotypic change. MOG titers did not correlate with symptomatology or risk of relapse.

Authors/Disclosures
Jeffrey Russ, MD, PhD (Duke Pediatric Neurology) PRESENTER	An immediate family member of Dr. Russ has received personal compensation for serving as an employee of Pivot Bio. The institution of Dr. Russ has received research support from NINDS.
Elizabeth George	No disclosure on file
Alexandria Valdrighi, MD (University of California, San Francisco)	Dr. Valdrighi has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Emmanuelle Waubant, MD, PhD, FAAN (USCF MS Center)	Dr. Waubant has received personal compensation in the range of $500-$4,999 for serving as a Consultant for emerald pharmaceuticals. Dr. Waubant 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. The institution of Dr. Waubant has received research support from NIH. The institution of Dr. Waubant has received research support from NMSS. The institution of Dr. Waubant has received research support from PCORI. The institution of Dr. Waubant has received research support from Race to Erase MS. The institution of Dr. Waubant has received research support from Roche. The institution of Dr. Waubant has received research support from Biogen. The institution of Dr. Waubant has received research support from Department of Defense. Dr. Waubant has received publishing royalties from a publication relating to health care.
Carla M. Francisco, MD (University of California, San Francisco)	Dr. Francisco has nothing to disclose.
	No disclosure on file