Acquired Demyelinating Syndromes in Children

Collectively, pediatric acquired demyelinating syndromes encompass a diverse clinical spectrum and include disorders such as multiple sclerosis (MS), acute disseminated encephalomyelitis, acute transverse myelitis, optic neuritis, and neuromyelitis optica spectrum disorders (NMOSDs). Acquired demyelination may occur as a monophasic illness or represent chronic demyelinating disease (such as MS or NMOSD) and thus prompt identification and classification is essential for proper treatment and guidance. As such, approximately 3% of all patients affected by MS experience their first attack during childhood. In general, one quarter of children diagnosed with an acute demyelinating syndrome will be ultimately confirmed to have MS. The likelihood that an incident demyelinating attack represents the first attack of MS is increased in adolescents, females, patients with positive cerebrospinal fluid oligoclonal bands, and those with clinically silent T2 bright and T1 hypointense lesions on brain magnetic resonance imaging at onset. Early therapeutic intervention for MS and NMOSD is recommended to reduce relapses, modify relapse severity, and, it is hoped, limit future disability and reduce demyelination-related central nervous system injury. Of particular clinical relevance in children, there are many mimics of demyelinating disease that must be considered.