Author Of 1 Presentation
P0715 - Failure of Age-Expected Brain Growth in Children with ADEM is Independent of MOG-Antibody Status (ID 893)
Abstract
Background
Acute disseminated encephalomyelitis (ADEM) is an acquired demyelinating syndrome (ADS) presenting with polyfocal neurological symptoms, encephalopathy, and predominant white matter MRI changes that mainly occurs in children. Although ADEM is typically a monophasic disease, a negative impact on brain growth over time was previously reported. Recently, it was shown that a large proportion of ADEM patients is seropositive for autoantibodies against myelin oligodendrocyte glycoprotein (MOG-abs). Furthermore, MOG-abs were associated with an increased risk for relapsing disease. However, the effect of MOG-abs on brain volume development remains unclear.
Objectives
Analysis of whole brain and ventricular volumes in children with ADEM with and without MOG-abs at disease onset and over time.
Methods
Twenty-four ADEM patients (median age 4.5 years; range 0-15; 13 females) from 12 different centers were included. MOG-abs were detected in 16 patients. All patients had a cerebral MRI scan at disease onset before steroid treatment. A total of 58 MRI scans (including 34 follow-up MRIs from 16 patients) were analyzed using FSL SIENAX for whole brain and ventricular volume. Patient brain volumes were compared to age- and gender-matched healthy controls by longitudinal mixed-effect models and group comparison (1:5; n=290) using healthy controls from the NIH Pediatric MRI Data Repository as well as an additional matched local control cohort (n=24).
Results
ADEM patients showed significantly increased ventricular volume (median volume [IQR] 34.25cm3 [17.07] vs. 22.29cm3 [9.88]; p=1.118e-05) and a trend of reduced whole brain volume (1741.9cm3 [160.1] vs. 1788.1cm3 [113.1]; p=0.055) at baseline compared to matched healthy NIH controls. Longitudinal-mixed-effect models showed failure of age-expected brain growth in all ADEM patients. Importantly, MOG-ab status was not a significant predictor of brain volume suggesting no difference in brain volume development between MOG-ab-positive and -negative patients. Patients with relapsing disease (n=6, all MOG-ab-positive) showed increased ventricular volume compared to monophasic patients at last visit (median ventricular volume z-score [IQR] 3.72 [3.58] vs. 0.38 [IQR 0.40]; p=0.04).
Conclusions
Children with ADEM exhibit significant brain volume loss and failure of age-expected brain growth compared to healthy controls. Importantly, this affects both MOG-ab-positive and -negative patients. Relapsing disease seems to be associated with more pronounced brain volume loss.