Attack phenotypes and disease course in pediatric MOGAD

Abstract

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune demyelinating condition that affects children differently than adults. We performed a literature review to assess the presentation and clinical course of pediatric MOGAD. The most common initial phenotype is acute disseminated encephalomyelitis, especially among children younger than five years, followed by optic neuritis (ON) and/or transverse myelitis. Approximately one-quarter of children with MOGAD have at least one relapse that typically occurs within three years of disease onset and often includes ON, even if ON was not present at onset. Clinical risk factors for a relapsing course have not been elucidated.

Figure 1

Flow diagram of article inclusion.

Figure 2

Initial clinical phenotype of first attack in pediatric MOGAD according to large inception cohorts for all pediatric ages (based upon ¹¹ , ¹² , ¹³ , ¹⁴ , ¹⁵ , ¹⁶ ).

Figure 3

Initial clinical phenotype of first attack in pediatric MOGAD according to large inception cohorts for patients age 0–10 years (based upon ¹² , ¹⁵ ).

Figure 4

Initial clinical phenotype of first attack in pediatric MOGAD according to large inception cohorts for patients age ≥ 11 years (based upon ¹² , ¹⁵ ).

Figure 5

MOGAD initial phenotype and subsequent phenotype at most recent data collection. The initial phenotypes are shown on the left, and the most recent phenotypes are shown on the right. All patients with only one episode of MOGAD are categorized as “monophasic” for the most recent phenotype. Lines connecting the initial and most recent phenotypes represent individual patients' disease courses. The width of the lines is proportional to the number of patients observed with each disease course (based upon ¹¹ , ¹² , ¹⁶ , ⁴⁵ ).