Clinical analysis of myelin oligodendrocyte glycoprotein antibody-associated demyelination in children: A single-center cohort study in China.

BACKGROUND AND PURPOSE: Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are detected at a high rate in childhood of acquired demyelinating syndrome (ADS), but the spectrum and characteristics of MOG-Abs-associated disorders (MOGAD) in children are to be determined. This study aimed to identify clinical features in Chinese children with MOGAD.

METHODS

Of 48 children in whom MOGAD were diagnosed in our hospital, we analyzed the manifestations, laboratory test results, imaging characteristics, autoimmune antibodies in cerebrospinal fluid and serum, and response to treatment. We used a cell transfection immunofluorescence assay to test for MOG-Abs in serum.

RESULTS

Of the 48 children, the most common phenotypes were acute disseminated encephalomyelitis (ADEM) (20/48, 41.7%) and optic neuritis (ON) (13/48, 27.1%). The onset ages of ON were significantly higher than those of ADEM (8.68±2.86 & 4.80±2.77, P<0.01). Cerebral lesions manifested as ADEM-like, leukodystrophy-like and other patterns. All children received first-line immunomodulatory therapy and some of them received second-line drugs, whose acute clinical symptoms were alleviated to some extent. 34 patients (34/48,70.8%) experienced one episode, the main phenotypes were ADEM (19/34,55.9%) and encephalitis (9/34,26.5%), and 14 children (14/48,29.2%) had two or more episodes, the primary expressions were ADEM-ON (8/14,57.1%) and recurrent ON (3/14,21.4%). During our follow-up, 8 patients suffered relapsed, but the MOG-Ab titers were not increase during acute stages. 4 patients (4/9,44.4%) of ADEM with ON were developed cognitive impairment, epilepsy and other sequelae, and 2 patients (2/3, 66.7%) of repeated ON suffered visual impairment.

CONCLUSION

The clinical phenotypes of MOGAD are age-dependent, the onset ages of ADEM are significantly younger than those of the ON children, and leukodystrophy-like pattern could occur in infancy. Cerebral lesions of MRI were extensive and various, manifested as ADEM-like, leukodystrophy-like, ON and other patterns. The titers of MOG-Ab should not be used as the only basis for recurrence and long-term immunoregulatory treatment. Most children had a good prognosis, however, the phenotype of ADEM-ON at onset was tend to relapse, sometimes with cognitive impairment, epilepsy, and other sequelae. Repeated ON could cause visual impairment.