Myelin Oligodendrocyte Glycoprotein (MOG) Antibody Diseases in Children in Central South China: Clinical Features, Treatments, Influencing Factors, and Outcomes

Abstract

Background and purpose: The clinical and radiological features of myelin oligodendrocyte glycoprotein antibody (MOG-Ab) diseases vary among the patients and studies. In addition, the clinical significance of MOG-Ab for the diagnosis, treatment, and prognosis is not yet established. Therefore, we aimed to evaluate the clinical, radiological, treatments and outcome features of MOG-Ab diseases in Central Southern part of China. Methods: A retrospective study of children with MOG-Ab disease was carried out from January 2015 to October 2018. Demographics, clinical features, treatments, and outcomes were reviewed. Some of the clinical information was compared including the annualized relapse rates (ARRs) before and after treatment with disease-modifying drugs (DMDs). Results: Twenty-five patients with MOG-Ab disease were recruited. The onset age ranged from 3 to 12.4 years old. 13 were females and 12 were males. The median follow-up period was 15 months (range 7–63). Most of the cases that aged ≤9 years presented with fever (47.4%), encephalopathy (47.4%), and lesions on white matter and/or deep gray matter (52.6%). While most of those aged above 9 years presented with optic neuritis (ON) (66.7%), and lesions on spinal cord and/or optic nerve (50%). Until the last follow-up, 10 (40%) cases had multiphasic courses while 15 (60%) had a monophasic course, and the mean follow-up time was statistically significant (10.67 vs. 31 months, p = 0.0001). DMDs such as rituximab (RTX) or/and azathioprine (AZP) or mycophenolate mofetil (MMF) were used at least once in 56% of the cases. The ARR before and after treatment was 2.4 and 0 respectively ( p < 0.05). The median Expanded Disability Status Scale scores of our study were 0 (range 0–2). 96% (24/25) of the cases had a full recovery. Conclusions: MOG-Ab disease among Chinese children share the same clinical characteristics with Caucasians. However, the Chinese children seem to have a better prognosis than Caucasians. There is an age-dependent phenotypes, as brain involvement is more frequently seen in children younger or equal to 9 years while ON and neuromyelitis optica spectrum disorders are commonly seen in children older than 9 years. DMDs, such as AZA, MMF or RTX, can reduce the ARR.

Clinical and Neuroimaging Characteristics of Pediatric Acute Disseminating Encephalomyelitis With and Without Antibodies to Myelin Oligodendrocyte Glycoprotein

“…To date, no nationwide study has been conducted regarding the incidence of MOGAD in China. A recent study ( 16 ), which included 25 Chinese pediatric MOGAD patients, revealed that Chinese children and Caucasians share the same clinical characteristics.…”

Pediatric demyelinating disease and anti‐MOG antibody

“…100 A decrease in the ARR while on rituximab has been documented in 9 patients from this study 26 and in 9 of 10 children from a Chinese study on relapsing MOGAD. 99 The occurrence of relapses despite B-cell depletion was observed in 1 of 6 children from an Australasian study. 89 The biological effect of rituximab through the monitoring of lymphocyte subsets (CD19+ and CD27+ B-cells) has been evaluated recently.…”

“…98 Data on the use of rituximab in relapsing MOG demyelination from small retrospective pediatric studies suggest some effectiveness in relapse prevention, although documented benefit is lower than expected. 26,89,[99][100][101] In 12 children with a relapsing MOGAD (ADEM, ON, NMOSD), no clinical relapses or new MRI lesions were observed during the first year of rituximab treatment. 100 A decrease in the ARR while on rituximab has been documented in 9 patients from this study 26 and in 9 of 10 children from a Chinese study on relapsing MOGAD.…”

Bilateral symmetrical deep gray matter involvement and leptomeningeal enhancement in a child with MOG-IgG-associated encephalomyelitis

“…In addition, the deep gray matter can also been frequently affected. Mao L et al, retrospective reviewed 50 Chinese cases with MOG-EM, and the 29% of whom had the deep gray matter involvement, including thalamus and basal ganglia [ 4 ]. However, the symmetrical basal ganglia and thalamus involvement is rarely reported.…”