Autoantibodies against aquaporin-4 and myelin oligodendrocyte glycoprotein in paediatric CNS demyelination: Recent developments and future directions

Diagnosis and prognosis of acute inflammatory disorders of the central nervous system in both children and adults would be aided by the availability of serum biomarkers. Antibodies directed against the aquaporin-4 water channel has led to recognition of neuromyelitis optica (NMO) and NMO spectrum disorders in both children and adults and to the ability to predict a relapsing disease course in antibody-positive patients. Serum antibodies directed against myelin antigens are detectable in approximately one third of children with acute disseminated encephalomyelitis (ADEM) at the time of acute illness, and in approximately 25% of children with multiple sclerosis (MS) in whom seropositivity persists. Whether circulating antibodies contribute to disease pathogenesis or are secondary to cell damage is an area of active study. The contribution of T-cells, eosinophils, neutrophils, infection and blood brain barrier permeability are also receiving increasing attention in antibody-associated inflammatory demyelination. The present manuscript reviews the current clinical, laboratory and immunological features of acquired CNS inflammation in children.

► Antibody biomarkers are increasingly important and influence treatment decisions.
► Aquaporin-4 IgG defines children with NMO who are at risk of relapse and disability.
► MOG IgG is present in one third of children during an acute demyelinating syndrome.
► Persistence of MOG IgG appears to be associated with paediatric MS.
► AQP4 IgG is pathogenic, and MOG IgG may also have a pathogenic role.