Clinical Spectrum of Myelin Oligodendrocyte Glycoprotein-Immunoglobulin G-Associated Disease in Korean Children

Abstract

Background and Purpose The myelin oligodendrocyte glycoprotein (MOG) antibody is detected at a high rate in childhood acquired demyelinating syndrome (ADS). This study aimed to determine the diagnostic value of the MOG antibody in ADS and the spectrum of MOGantibody-positive demyelinating diseases in children. Methods This study included 128 patients diagnosed with ADS (n=94) or unexplained encephalitis (n=34). The MOG antibody in serum was tested using an in-house live-cell-based immunofluorescence assay. Results The MOG antibody was detected in 48 patients (46 ADS patients and 2 encephalitis patients, comprising 23 males and 25 females). Acute disseminated encephalomyelitis (ADEM) (35.4%) was the most-common diagnosis, followed by the unclassified form (17.4%), isolated optic neuritis (ON) (15.2%), neuromyelitis optica spectrum disorder (13.0%), multiple sclerosis (MS) (10.8%), other clinically isolated syndromes [monophasic event except ADEM, isolated ON, or transverse myelitis (TM)] (8.7%), and unexplained encephalitis (4.3%). At the initial presentation, 35 out of the 46 patients with ADS had brain lesions detected in magnetic resonance imaging, and 54% of these 35 patients had encephalopathy. Nine of the 11 patients without brain lesions exhibited only ON. Thirty-nine percent of the patients experienced a multiphasic event during the mean follow-up period of 34.9 months (range 1.4-169.0 months). Encephalopathy at the initial presentation was frequently confirmed in the monophasic group (p= 0.011). Conclusions MOG antibodies were identified in all pediatric ADS phenotypes except for monophasic TM. Therefore, the MOG antibody test is recommended for all pediatric patients with ADS, especially before a diagnosis of MS and for patients without a clear diagnosis.

Clinico‐radiological characteristics of anti‐myelin oligodendrocyte glycoprotein antibody‐associated autoimmune encephalitis in children

“…MOG and anti-synaptic antibody testing were performed as described previously. 19,20 Medical records were reviewed retrospectively for age at onset, sex, clinical presentation, laboratory results including CSF profile, follow-up duration, relapse, treatment regimen, and outcome. Autoimmune encephalitis relapse was defined as any new or worsening neurological symptoms after at least 4 weeks of improvement or stabilization.…”

“…Among the patients included in the study, we investigated neuronal and glial antibodies including anti‐MOG, anti‐NMDAR, anti‐α‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazolepropionic acid receptor, anti‐dipeptidyl peptidase‐like protein 6, anti‐leucine‐rich glioma‐inactivated 1, anti‐contactin‐associated protein‐like 2, anti‐γ‐aminobutyric acid type B receptor, and anti‐glutamic acid decarboxylase antibodies. MOG and anti‐synaptic antibody testing were performed as described previously 19,20 . Medical records were reviewed retrospectively for age at onset, sex, clinical presentation, laboratory results including CSF profile, follow‐up duration, relapse, treatment regimen, and outcome.…”

Attack phenotypes and disease course in pediatric MOGAD

“…This general distribution of MOGAD phenotypes was also observed when assessing all identified studies (Table 1). Overall, 39 identified articles (including the six aforementioned incident cohort studies) reported the phenotype at initial presentation for 1,686 pediatric MOGAD patients 4–6,8,11–45 . Most of these studies were limited by the potential for selection bias in patient identification (e.g., patients with stored serum available for retrospective MOG‐Ab testing, relapsing disease course, etc.).…”

Clinical and Magnetic Resonance Imaging Characteristics of Pediatric Acute Disseminating Encephalomyelitis With and Without Antibodies to Myelin Oligodendrocyte Glycoprotein

“…Previous studies have indicated that approximately 40% of children with ADEM and nearly 100% of children with multiphasic disseminated encephalomyelitis (MDEM) were seropositive for MOG-IgG ( 10 ). Previous studies have reported the clinical and neuroradiological characteristics of pediatric ADEM with MOG-IgG ( 11 – 14 ). The clinical and MRI characteristics of patients with ADEM with or without MOG-IgG in northern China have not been reported.…”