What is the pathophysiology of pediatric multiple sclerosis (MS)?

Updated: Jan 30, 2019
  • Author: Alice K Rutatangwa, DO, MSc; Chief Editor: Amy Kao, MD  more...
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Answer

Data concerning the pathophysiology of pediatric MS and how it may differ from adult MS are limited. Pathologic studies have offered some insights; however, brain biopsy is undertaken only in the most severe cases, so results may be biased toward a more severe phenotype. Most cases involve tumefactive demyelination. Cases with detailed pathology report a dense accumulation of lymphocytes and macrophages in a prominent perivascular distribution, with rare B cells. Axonal damage is typically limited. [7]

As in adults, identification of a self-antigen as an immunologic trigger is elusive, but T cells are believed to play a major role in CNS inflammation. [8] Several groups have studied T-cell responses to various antigenic stimuli in pediatric MS. A 2008 study of a large cohort of children with CNS inflammatory demyelination, type 1 diabetes, or CNS injury demonstrated that children with these conditions exhibited heightened peripheral T-cell responses to a wide array of self-antigens. [9] Anti–myelin oligodendrocyte glycoprotein (MOG) and anti–myelin basic protein (MBP) have been studied in both adults and children. In children, anti-myelin antibodies have been seen in patients with different demyelinating disorders, including ccute disseminated encephalomyelitis (ADEM).

CSF studies have demonstrated that children younger than 11 years exhibit a distinct cellular profile when compared with older children. Younger children with their first MS event were more likely to lack evidence of intrathecal antibody production (OCBs or an elevated IgG index) and had a higher percentage of neutrophils in their CSF, suggesting prominent activation of the innate immune response, as opposed to the typical activation of the adaptive response seen in older patients.


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