Clinical characteristics, disease course, and outcomes of paediatric patients with myelin oligodendrocyte glycoprotein-Ab associated disease: A retrospective clinical study

Objective: To delineate the outcomes of paediatric patients with myelin oligodendrocyte glycoprotein antibody disease (MOGAD).

Methods: We retrospectively analyzed the clinical characteristics, treatment, and outcomes of 34 paediatric patients with MOGAD from July 2015 to January 2020.

Results: The median age at disease onset was 75.5 months (range: 19-170 months). The female-to-male ratio was 1:1.1. The median follow-up duration was 34.5 months (range: 14-63 months). Acute disseminated encephalomyelitis (ADEM) was the most common initial phenotype (52.9%), followed by optic neuritis (ON) (20.6%). Children with ADEM were younger than those with ON (P = 0.045). Twenty-eight (82.4%) and 18 (56.3%) children had abnormal brain and spinal magnetic resonance imaging, respectively, during the first acute attack. MOG-abs titers in children with ON were statistically higher than those in children with ADEM (P = 0.04). Thirty-two children accepted glucocorticoid treatment, while 33 (97%) children demonstrated clinical improvement within 1 week, 21 children (61.8%) achieved clinical recovery within 1 month. Eight children (23.5%) suffered a relapse, the median interval between the initial attack and recurrence was 13 (range: 3-36) months. We detected neurological sequelae in seven (20.6%) children, with visual dysfunction being the most common sequela (85.7%).

Conclusion: ADEM was the most common phenotype in both monophasic and relapsed paediatric MOGAD, followed by ON. Majority of pediatric MOGAD patients were highly responsive to glucocorticoid. Despite a benign prognosis in most patients, some patients endure neurological sequelae, mainly visual impairment. Patients with initial visual impairment should be carefully evaluated and administered individualized immunotherapy.