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

Abstract

Aim: To investigate the clinical characteristics and prevalence of paediatric antimyelin oligodendrocyte glycoprotein (MOG) antibody-associated autoimmune encephalitis.Method: A total of 94 paediatric patients (46 males, 48 females, median age 9 years 5 months, range: 8 months-17 years 8 months) with autoimmune encephalitis were recruited at Seoul National University Children's Hospital. We evaluated autoantibody status and identified patients with anti-MOG antibody-associated autoimmune encephalitis. Retrospective reviews of medical records were performed to describe clinical presentations, laboratory findings, treatments, and outcomes.Results: Eight patients (five males, three females, median age 11 years 9 months) with anti-MOG antibody-associated encephalitis were identified (8.5% of those with autoimmune encephalitis), one of whom was copositive for anti-N-methyl-daspartate receptor (NMDAR) antibodies. Anti-NMDAR antibodies were identified in 23 patients (23 out of 94, 24.5%). Unilateral or bilateral cortical involvement was identified in five patients. Focal contrast enhancement was also identified in three of the five patients with cortical lesions. All patients showed favourable response to immunotherapy with a Modified Rankin Scale ≤2 at the last follow-up. Relapse was found in one patient and clinico-radiological remission was achieved with cyclic intravenous immunoglobulin therapy.Interpretation: Anti-MOG antibody-associated encephalitis accounts for a significant proportion of clinically defined paediatric patients with autoimmune encephalitis. Anti-MOG antibody-associated encephalitis should be included in the clinical spectrum of anti-MOG-associated diseases.

A Case of Recurrent Anti-MOG-Associated Cerebral Cortical Encephalitis Mimicking Viral Encephalitis and Hemiplegic Migraine

“…However, the clinical presentation of the patient was consistent with those of previously reported anti-MOG-associated encephalitis cases in children [7]. Fever, headache, seizures, focal neurologic deficits including motor weakness, and altered mental state are the main symptoms of anti-MOG-associated encephalitis; the patient had all these symptoms except fever, which was described as occurring in 50%-78% cases of anti-MOG-associated encephalitis [6,7]. The MRI findings of our patient were consistent with those of anti-MOG-associated unilateral CCE reported by Ogawa et al [2].…”

“…There may be disagreement on the diagnosis of anti-MOG-associated encephalitis because of the limited neuro-immunological work-up. However, the clinical presentation of the patient was consistent with those of previously reported anti-MOG-associated encephalitis cases in children [7]. Fever, headache, seizures, focal neurologic deficits including motor weakness, and altered mental state are the main symptoms of anti-MOG-associated encephalitis; the patient had all these symptoms except fever, which was described as occurring in 50%-78% cases of anti-MOG-associated encephalitis [6,7].…”

Brazilian consensus recommendations on the diagnosis and treatment of autoimmune encephalitis in the adult and pediatric populations

“…An interesting outcome of the present study was the recommendation of anti-MOG testing for children with AIE, based on results from Brazilian, Spanish, 54 Danish, and Chinese 56 pediatric cohorts, which identified anti-MOG as the second most common ab associated with AIE among children. 11 54 55 56 57 Although the literature 37 58 59 60 shows that anti-MOG titers may predict recurrent myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), its clinical significance in MOGAD encephalitis is not fully understood.…”

Autoimmune diseases and cancers overlapping with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD): A systematic review

“… 3 This finding implies a difference in the immunopathogenic mechanisms involved in the development of each entity, such that AQP4-IgG + NMOSD is more attributable to a defect in immune tolerance, while MOGAD is more likely to be an aberrant autoimmune response to certain triggers, such as infection, vaccination, or malignancy. 3 …”