Clinical characteristics and associated factors of pediatric acute disseminated encephalomyelitis patients with MOG antibodies: a retrospective study in Hangzhou, China

Abstract

Background To explore the clinical characteristics and related factors of children with acute disseminated encephalomyelitis (ADEM) with positive anti-myelin oligodendrocyte glycoprotein (MOG) antibody. Methods A retrospective study was conducted and enrolled pediatric ADEM patients who underwent serum MOG antibody detection from May 2017 to August 2020. The patients were divided into two groups: MOG- immunoglobulin G (IgG) positive (n = 35) and MOG-IgG negative (n = 50). We analyzed the clinical characteristics of MOG-IgG-positive ADEM pediatric patients and conducted a comparative analysis between the two groups. Results Thirty-five patients (21 males and 14 females) in the MOG-IgG-positive group with encephalopathy, multifocal neurological symptoms, and typical magnetic resonance imaging (MRI) abnormalities were enrolled. They usually had a favorable outcome, while some suffered from relapse. Compared to the MOG-IgG-negative group, MOG-IgG-positive ADEM patients had a longer disease duration (median: 10 vs. 6 days), more meningeal involvement (31.4% vs. 8%) and frontal lobe involvement (82.8% vs. 68%), higher relapse rates (14.3% vs. 2%), lower serum tumor necrosis factor (1–12.4 pg/ml, median 1.7 vs. 1–34 pg/ml, median 2.2) and interferon-gamma (1–9.4 pg/ml, median 1.3 vs. 1–64 pg/ml, median 3) (P < 0.05, respectively). Multivariate logistic regression analysis showed that the longer disease duration, meningeal involvement and frontal lobe involvement were the correlated factors of patients with ADEM with MOG antibody (P < 0.05). Conclusions Our findings provide clinical evidence that MOG-IgG positivity is associated with longer disease duration, meningeal involvement, and frontal lobe involvement.

Clinical, radiological, therapeutic and prognostic differences between MOG-seropositive and MOG-seronegative pediatric acute disseminated encephalomyelitis patients: a retrospective cohort study

“…With regard to MRI lesion distributions, Zhang et al (2020) reported greater involvement of the cerebellum (P = 0.045) in MOG-seropositive ADEM children, probably explaining why the children with MOG-abs in their study more frequently presented with ataxia clinically. In addition, for MOG-seropositive ADEM, Lei et al (2022) reported more lesions in the thalamus (P = 0.022) and Shen et al (2022) reported more lesions in the frontal lobe (P = 0.019) although both studies did not compare the breadth of lesion distributions. Some of the abovementioned variable conclusions explain the differences in clinical characteristics, but most of them were non-specific and made little sense for clinical practice.…”

“…Zhang et al (2020) reported that MOGseropositive children with ADEM tended to show more ataxia (P = 0.025), while MOG-seronegative children tended to show higher rates of movement disorders such as paralysis (P = 0.004) and rectal/bladder dysfunction (P = 0.035). Shen et al (2022) showed that the seropositive group had significantly more symptoms of meningeal involvement (P = 0.008). Nevertheless, two other studies showed no differences in the range of clinical symptoms between ADEM children with and without MOG-abs (Baumann et al, 2015;Lei et al, 2022).…”

“…The existing studies have paid little attention to ADEM patients without MOG-abs, and there is no consensus on the differences between them and MOG-seropositive children. Moreover, the existing retrospective comparative studies were characterized by insufficient sample sizes, inconsistent conclusions, and a lack of detailed descriptions and comparisons of treatments and prognosis (Baumann et al, 2015;Zhang et al, 2020;Lei et al, 2022;Shen et al, 2022). As a retrospective study with a relatively large sample size, our study provided information on the differences between MOG-seropositive and MOG-seronegative children in terms of clinical and imaging characteristics.…”

Treatment Options for Epstein-Barr Virus-Related Disorders of the Central Nervous System

“…Viral prodromal symptoms often precede an abrupt onset with multifocal neurological deficits that rapidly deteriorate over several days. 62 , 63 The disease is typically monophasic but recurrent forms of ADEM have been reported in case-series, typically in the context of persisting myelin oligodendrocyte glycoprotein (MOG) antibodies. 64 ADEM usually affects children and adolescents and may be difficult to distinguish from a first clinical event of MS.…”

“… 68 Most children and adolescents with monophasic ADEM eventually recover. 62 , 63 However, the risk of subsequently developing MS is substantially increased. 69 …”