Fact Sheet: Pediatric Multiple Sclerosis and Demyelinating Disorder

Multiple sclerosis (MS) is an autoimmune disease affecting the central nervous system (CNS) with a clinical course involving repeated episodes of inflammation, demyelination, and blood-brain barrier breakdown. These episodes result in varied and often unpredictable neurological symptoms including dyspraxia, incontinence, visual changes, fatigue, as well as changes in cognition and mood. Pediatric MS refers to onset of symptoms prior to age 18 years. MS is one of several demyelinating diseases including acute disseminated encephalomyelitis (ADEM), clinically isolated syndrome (CIS) and neuromyelitis optica (NMO). 

Prevalence and Course

A single episode of acute demyelination occurs in approximately one-two per 100,000 children.  About 20 percent go on to have a diagnosis of MS (defined as a second demyelinating event involving new areas of the CNS determined by symptoms and/or by MRI). As many as 5 percent of adult MS patients may have had onset in childhood. Most pediatric patients experience a relapsing-remitting course of MS. Major symptoms usually resolve with treatment (steroids and disease modifying drugs). However, many patients continue to experience fatigue, sensitivity to temperature, and other more minor symptoms. There is also increasing evidence that pediatric MS may have subtle but long-term effects on cognition. There is typically less involvement of the spinal cord compared to adult onset MS.

Health and Psychosocial Consequences

Because of the varied symptoms of MS, effects on children are wide-ranging. School achievement, involvement in extracurricular activities and spots may be impacted. Similar to adults with MS, there is an elevated rate of depression, stress and anxiety among pediatric patients, and decreased health related quality of life. Treatment adherence to injections declines in adolescence. While frequencies are wide ranging, 14-73 percent of patients experience fatigue, 0-48 percent experience psychiatric complications, and 0-77 percent experience cognitive impairment (particularly deficits in executive functions, processing speed and language processing).

Evidence-based Assessment

Relevant domains of assessment include physical disability status, cognition, fatigue, pain, academic, social, emotional and family functioning. Study groups are beginning to agree upon common assessments.

  • Physical Disability: The Expanded Disability Status Scale (EDSS)

  • Cognition and academic functioning: Comprehensive neuropsychological assessment

  • Fatigue: Pediatric Multidimensional Fatigue Scale

  • Social, emotional and family functioning: Peds-QL, CDI, BASC-2

Culture, Dversity, Demographic and Developmental Factors

Incidences seem to be higher in Northern Europe and North America, lower in Southern Europe and very low in African and Asian countries. There seems to be greater ethnic diversity in pediatric than in adult MS. The incidence of MS is equal in males and females in childhood, but females outnumber males in adolescence.

Evidence-based Interventions

There are few evidence based psychological interventions for pediatric MS to date. Cognitive rehabilitation can be effective for children experiencing cognitive effects of MS. Empirically supported interventions are used to treat symptoms (e.g. CBT for depression and anxiety). Multidisciplinary care is important.

References

Chitnis, T., Krupp, L., Yeh, A., Rubin, J., et al. (2011). Pediatric Multiple Sclerosis. Neurologic Clinics 29, 481-505.

Langer-Gould, A., Zhang, J.L., Chung, J., Yeung, Y., Waubant, E., Yao, J. (2011). Incidence of acquired CNS demyelinating syndromes in a multiethnic cohort of children. Neurology 77, 1143-1148.

Mowry, E.M., Julian, L.J., Im-Wang, S., Chabas, D., Galvin, A.J., Strober, J.B., Waubant, E. (2010). Health-related quality of life is reduced in early pediatric multiple sclerosis. Pediatric Neurology 43, 97-102.

Portaccio, E., Goretti, B., Zipoli, V., et al. (2010). Cognitive rehabilitation in children and adolescents with multiple sclerosis. Neurological Sciences 31(2), 275-278.

Thannhauser, J., Mah, J., & Metz, L. (2009). Adherence of adolescents to multiple sclerosis disease-modifying therapy. Pediatric Neurology 41, 119-123.