Pediatric Acquired Demyelinating Disorders

Continuum (Minneap Minn). 2022 Aug 1;28(4):1104-1130. doi: 10.1212/CON.0000000000001128.

Abstract

Purpose of review: This article reviews the clinical presentation, diagnostic evaluation, treatment, and prognosis of the most common monophasic and relapsing acquired demyelinating disorders presenting in childhood.

Recent findings: Our understanding of neuroimmune disorders of the central nervous system is rapidly expanding. Several clinical and paraclinical factors help to inform the diagnosis and ultimately the suspicion for a monophasic versus relapsing course, including the age of the patient (prepubertal versus postpubertal), presence or absence of clinical encephalopathy, identification of serum autoantibodies (eg, myelin oligodendrocyte glycoprotein [MOG] and aquaporin-4), presence of intrathecally unique oligoclonal bands, and location/extent of radiologic abnormalities. Collaborative international research efforts have facilitated understanding of the safety and efficacy of currently available immunotherapies in children with acquired demyelinating disorders, particularly multiple sclerosis.

Summary: Although many of the demyelinating disorders presented in this article can affect children and adults across the age spectrum, the clinical and radiologic phenotypes, treatment considerations, and long-term prognoses are often distinct in children.

Publication types

  • Review

MeSH terms

  • Aquaporin 4
  • Autoantibodies*
  • Humans
  • Multiple Sclerosis*
  • Myelin-Oligodendrocyte Glycoprotein

Substances

  • Aquaporin 4
  • Autoantibodies
  • Myelin-Oligodendrocyte Glycoprotein