Paediatric multiple sclerosis and other acute demyelinating diseases

Neuroimmunological diseases encompass a wide spectrum of diseases in children. Apart from the discovery of autoantibodies affecting primarily grey matter structures and the improved clinical characterization of rare entities such as N-methyl D-aspartate receptor-R- encephalitis, important strides have also been made in autoimmune-mediated white matter diseases, including paediatric multiple sclerosis (pedMS) and other acute demyelinating syndromes (ADS) often associated with antibodies (abs) against myelin-oligodendrocyte-glycoprotein (MOG). This review will cover findings of recent studies in pedMS, in the emerging field of non-MS acute demyelinating episodes associated with MOG abs and lastly from new imaging techniques such as diffusion tensor imaging (DTI) revealing new insights in the pathogenesis of ADS in children.

The first prospective randomized clinical pedMS trial assessing the clinical and radiological efficacy of fingolimod versus a standard disease-modifying agent has shown clear superiority of fingolimod. The clinical spectrum of MOG-associated diseases has been characterized in more detail revealing clinical subtypes distinct from pedMS. A recent large European study further showed that MOG-associated diseases do not respond to first-line disease-modifying treatment (DMT) in MS but fare better with B-cell modulating therapies including regular intravenous immunoglobulin (IVIG).

Recent findings strongly indicate that in particular in highly active pedMS characterized by new relapses or accrual of new MRI lesions despite first-line DMT treatment should be escalated. Secondly, several studies have shown that MOG-spectrum diseases include children with monophasic and recurrent subtypes other than MS with different clinical, radiological characteristics and treatment challenges.