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Key Features

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  • Pediatric cases account for 5–10% of all cases

  • Exciting discoveries have highlighted the importance of genetic and environmental factors alone and in combination; for example,

    • HLA subtypes

    • Viral exposures

    • Smoking exposure

    • Vitamin D deficiency

  • Diagnostic criteria, including clinical, MRI, and laboratory studies are different among prepubertal patients compared with postpubertal patients

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Clinical Findings

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  • Although there are many similarities between pediatric-onset and adult-onset MS, an earlier age at disease presentation seems to be associated with specific features such as encephalopathy, seizures, and brainstem and cerebellar symptoms

  • A diagnosis of pediatric MS may be made after one episode of demyelination if the MRI scan meets criteria for dissemination in time and space

  • If these criteria are not met, a clinically isolated syndrome is diagnosed; for example,

    • Optic neuritis

    • Transverse myelitis

    • Brainstem, cerebellar, or hemispheric dysfunction

  • Atypical clinical features of pediatric MS include

    • Fever

    • Involvement of the peripheral nervous system or other organ systems

    • Elevated erythrocyte sedimentation rate

    • Marked cerebrospinal fluid pleocytosis

  • Encephalopathy is more commonly associated with acute disseminated encephalomyelitis (ADEM)

  • However, in young children, MS exacerbations may present with encephalopathy, making differentiation of the two disorders difficult

  • Differential diagnosis

    • ADEM

    • Idiopathic transverse myelitis

    • Optic neuritis

    • Rheumatologic disorders like systemic lupus erythematosus, Behçet and Sjögren syndrome and neuromyelitis optica

    • Many other infections, metabolic disorders, and degenerative diseases

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Diagnosis

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  • Initial brain MRI scan of younger patients shows more frequent involvement of the posterior fossa and higher numbers of ovoid, ill-defined T2-bright foci that often partially resolve on the follow-up scan

  • There are several sophisticated MRI criteria to separate pediatric MS diagnosis from alternative diagnoses (eg, ADEM)

  • Spinal fluid in younger patients may fail to reveal oligoclonal bands or elevated IgG index at disease onset

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Treatment

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  • There is no FDA-approved therapy for MS in children

  • Current practice is to use disease-modifying therapies that are approved for adults

  • Retrospective data has shown them to be effective in children

  • Immunomodulatory treatment to prevent relapses in children include

    • Interferon-β 1a or glatiramer acetate (injections)

    • Oral agents fingolimod, teriflunomide, and dimethyl fumarate

    • Natalizumab, rituximab, or cyclophosphamide may be used in refractory cases

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