Author Of 1 Presentation
HT07.03 - Prevalence of MOG-Ab in a large cohort of neurological patients
Abstract
Background
Myelin oligodendrocyte glycoprotein (MOG) antibody disease is recognized as a distinct nosological entity. In adults, MOG IgG serum antibodies (MOG-Ab) are associated with neuromyelitis optica spectrum disorder (NMOSD) phenotype in particular isolated myelitis and recurrent often bilateral anterior optic neuritis. In children, MOG-Ab are primarily associated with acute disseminated encephalomyelitis. However, an increasing number of clinical phenotypes have been reported to be associated with MOG-Ab.
Objectives
To investigate the seroprevalence of MOG-Ab under consideration of demographics, disease entities and time course in a large cohort of neurological patients.
Methods
Blood samples of 2103 consecutive adult neurologic patients admitted to the neurological department of the Technical University of Munich between 2016-2019 were tested for MOG-IgG using a cell-based assay. In a subgroup of patients, MOG Ab-persistence was analyzed in follow-up samples. For validation selected positive samples were sent to two external laboratories.
Results
We found MOG-Ab in 28 of 2103 (1.33%) patients. High Ab-titers were rare but mostely associated with NMOSD phenotype (8/28), whereas low titers occurred in a wide range of neurological diseases, predominantly in other inflammatory CNS diseases (5/28) and stroke (6/28). Female gender and younger age at disease onset tended to be associated with higher MOG Ab-titers. Follow-up analyzes yielded Ab-persistence over time occurring not only in NMOSD but also in other neurological diseases. External validation confirmed high sensitivity and specificity of our cell-based assay for high titers but considerable variability for low MOG-Ab titer between laboratories.
Conclusions
The present study demonstrates of the occurrence of MOG-Ab in a wide range of neurological diseases. High Ab-titers seem to be specific for NMOSD in adults. Persistent low titer MOG-Ab are also found in non-inflammatory neurological diseases implying that their use for diagnostic purposes is highly limited.
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.