MOG-IgG- versus AQP4-IgG-positive neuromyelitis optica spectrum disorders in children: Clinical characteristics and prognosis

  Background  Neuromyelitis optica spectrum disorder (NMOSD) is one of inflammatory demyelinating diseases of the central nervous system that primarily involves the optic nerve and spinal cord. Its pathogeny is mainly associated with aquaporin-4 immunoglobulin-G antibodies (AQP4-IgG) and myelin oligodendrocyte glycoprotein immunoglobulin-G (MOG-IgG). The incidence of NMOSD in children is much lower than that in adults, and the clinical features also varies between them. There are few studies about NMOSD on children.

  Objective  To analyze and summarize the differences in clinical manifestations, imaging characteristics and prognosis in aquaporin-4 immunoglobulin-G (AQP4-IgG) positive and myelin oligodendrocyte glycoprotein immunoglobulin-G (MOG-IgG) positive pediatric NMOSD, so as to provide diagnosis and treatment ideas for pediatric NMOSD.

  Methods  From January 2008 to July 2021, clinical data including expanded disability status scale (EDSS), annualized relapse rate (ARR), imaging and laboratory tests about 18 AQP4-IgG-positive and 17 MOG-IgG-positive pediatric NMOSD patients in the First Medical Center of Chinese PLA General Hospital were retrospectively summarized and analyzed.

  Results  The female rate of AQP4-IgG-positive children was more than MOG-IgG-positive children (14/18 vs 8/17), but the difference was not statistically significant (P＞0.05). The MOG-IgG-positive group was more likely to have binocular vision loss than that of the AQP4-IgG group (14/17 vs 7/18, P=0.015), whereas children in the AQP4-IgG-positive group were more likely to have monocular or binocular absence of light perception (9/18 vs 0/17, P=0.01). AQP4-IgG group was more likely to have ANA positivity (5/18 vs 0, P=0.045). More children in the MOG-IgG-positive group had brain lesions than those in the AQP4-IgG-positive group (17/17 vs 8/18, P<0.001) There was no difference in the median EDSS 3.0 (4.0, 5.0) vs 3.0 (4.0, 5.0) between the two groups at the most severe stage of the disease, but the EDSS score was significantly lower in the MOG-IgG-positive children than that in the AQP4-IgG-positive group at the last follow-up 1.0 (1.0, 2.0) vs 5.0 (4.0, 5.0), P<0.001.

  Conclusion  Children with MOG-IgG-positive NMOSD are more likely to develop bilateral vision loss, brain lesions and brain syndromes, but have a low disability rate and a relatively good prognosis.