Clinical Features and Risk of Relapse in Children and Adults with Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease

Abstract

Objective The main objective was to compare clinical features, disease course, and myelin oligodendrocyte glycoprotein (MOG) antibody (Ab) dynamics between children and adults with MOG‐Ab–associated disease (MOGAD). Methods This retrospective multicentric, national study included 98 children and 268 adults with MOGAD between January 2014 and September 2019. Cox regression model for recurrent time‐to‐event data and Kaplan–Meier curves for time to antibody negativity were performed for the objectives. Results Isolated optic neuritis was the most frequent clinical presentation in both children (40.8%) and adults (55.9%, p = 0.013), and acute disseminated encephalomyelitis syndrome was more frequent in children (36.7% vs 5.6%, p < 0.001). Compared to adults, children displayed better recovery (Expanded Disability Status Scale ≥ 3.0 at last follow‐up reached only by 10 of 97 [10.3%] vs 66/247 [26.7%], p < 0.001). In the multivariate analysis, adults were at higher risk of relapse than children (hazard ratio = 1.41, 95% confidence interval [CI] = 1.12–1.78, p = 0.003). At 2 years, 64.2% (95% CI = 40.9–86.5) of nonrelapsing children became MOG‐Ab negative compared to 14.1% (95% CI = 4.7–38.3) of relapsing children (log‐rank p < 0.001), with no differences observed in adults (log‐rank p = 0.280). Interpretation MOGAD patients differ in the clinical presentation at onset, showing an age‐related shift in the clinical features across age groups. Compared to children, adults have a higher risk of relapse and worse functional recovery. Finally, children with monophasic disease become MOG‐Ab negative earlier than relapsing children, but this is not true in adults. Considering these differences, management and treatment guidelines should be considered independently in children and adults. ANN NEUROL 2021;89:30–41

COVID-19, HHV6 and MOG antibody: A perfect storm

“…In this case, the combined SARS-CoV-2 and HHV6 infection likely triggered immune-mediated myelitis. This was confirmed by the positivity of MOG-IgG, an antibody linked to monophasic or recurrent demyelinating disorders including acute disseminated encephalomyelitis, optic neuritis, and transverse myelitis ( Cobo-Calvo et al, 2020 ). MOG-IgG-related demyelination can be post-viral or idiopathic.…”

“…Demyelinating antibodies like MOG-IgG and AQP4-IgG should be tested in the setting of a suspicious clinical picture, such as longitudinally extensive myelitis or severe optic neuritis. MOG-IgG should then be repeated as persistent positivity may predict recurrent disease while transient positivity usually indicates a monophasic course ( Cobo-Calvo et al, 2020 ). This case expands the spectrum of autoimmune and infectious neurological complications of COVID-19.…”

<p>MOG-IgG- versus AQP4-IgG-Positive Optic Neuritis in Thailand: Clinical Characteristics and Long-Term Visual Outcomes Comparison</p>

“…This finding was supported by several multicenter studies showing that isolated ON is often the first presentation of MOGAD. 5 , 12 Moreover, in a large multicenter retrospective study comparing the clinical features and visual outcomes between Caucasian and Asian MOG-IgG + ON patients, the authors found that Asian patients were less likely to have non-ON demyelinating manifestations. 6 This finding was comparable to findings in our study, in which all patients in the MOG-IgG + ON group were Asian.…”

Treatment of myelin oligodendrocyte glycoprotein immunoglobulin G–associated disease

“…Cobo‐Calvo et al 92 recently reported on the relapse risk in 336 cases of children and adults with MOGAD. Adults were at higher risk of relapse and had worse functional outcomes compared to children.…”