Monophasic Acquired Demyelinating Syndromes of the Central Nervous System in Children

Abstract

Acquired demyelinating syndromes of the central nervous system are immune-mediated demyelinating disorders that can affect the brain, optic nerves, and spinal cord. These disorders have become increasingly recognized in children due to advances in imaging techniques, improvements in diagnostic testing, extensive research into understanding the pathophysiology underlying these disorders, and collaborative multi-institutional efforts to raise awareness of these disorders in children. Moreover, developments in the field of neuroimmunology have allowed us to identify autoantibodies that have presumed causal roles in acquired demyelinating syndromes. Identification of these autoantibodies helps determine clinical course (ie, monophasic vs relapsing course), prognosis, and treatment approach. Acquired demyelinating disorders can affect both children and adults. However, the clinical features, disease course, and treatments are often unique in the pediatric population. Thus, it is important to understand the spectrum of these disorders in children to help provide a timely diagnosis and prompt treatment to achieve optimal outcomes. In this article, the epidemiology, clinical features, diagnosis, treatment, and outcomes of the most common monophasic acquired demyelinating syndromes in children will be reviewed.