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.