Pediatric Multiple Sclerosis: Distinguishing Clinical and MR Imaging Features

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Key points

  • Overall, 3% to 10% of patients with multiple sclerosis (MS) show their first clinical event during childhood.

  • Children with MS have higher relapse rates compared with adult-onset MS.

  • As in adult patients, pediatric MS diagnosis requires dissemination in space and time, clinically or by MR imaging findings.

  • Alternative diagnoses such as acute disseminating encephalomyelitis and neuromyelitis optica spectrum disorder must be differentiated from MS.

  • Revised MR imaging criteria are a useful tool to

Diagnostic criteria of pediatric multiple sclerosis

In 2007, the International Pediatric Multiple Sclerosis Study Group (IPMSSG) met to propose consensus definitions for pediatric-onset acquired inflammatory demyelinating disorders of the central nervous system (CNS), including acute disseminated encephalomyelitis (ADEM), neuromyelitis optica (NMO), clinically isolated syndromes (CIS), and MS.6 These definitions were developed to improve consistency in terminology, avoid misclassification, and facilitate epidemiologic studies and clinical

Distinguishing clinical features

The presenting phenotype of patients with pediatric-onset MS may correspond to the following categories: a clinically monofocal event, with all the symptoms and neurologic findings responding to a single CNS location; a clinically polyfocal event without encephalopathy, when more than one CNS site is required to explain the neurologic findings; or an ADEM-like event, when polyfocal deficits are identified in the context of an acute encephalopathy. These presenting phenotypes are highly variable

Conventional MR imaging

MR imaging probably represents the single most important diagnostic tool to support the diagnosis of MS in both children and adults. Typical MS lesions appear as well-defined, high-signal ovoid-shaped areas on T2-weighted and T2-fluid-attenuated inversion recovery (FLAIR) images spread throughout the white matter (WM) in different regions, such as the juxtacortical and periventricular areas, corpus callosum, brainstem, and cerebellum (Figs. 1A, B and 2).

Applicability of conventional MR imaging

Differential diagnosis

After initial presentation with a CNS demyelinating event, children can meet diagnostic criteria for MS if serial changes are documented on MR imaging and other disorders are excluded. However, there is a broad spectrum of pediatric disorders showing WM abnormalities on MR imaging. As an example, in a large prospective cohort of children meeting criteria for acquired demyelinating syndromes, 6% were identified with other causes after performing appropriate diagnostic testing.64 Because the

Summary

Pediatric-onset acquired inflammatory demyelinating disorders are increasingly recognized and continue to expand. Nevertheless, MS continues being a challenging diagnosis in children and adolescents. Advances in conventional MR imaging have contributed to improve the identification of distinguishing features of pediatric MS and provided imaging criteria to differentiate between children who will not develop a relapsing disease and children at risk for MS diagnosis. In addition, MR imaging has a

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    Funding Sources: Dr S.N. Tenembaum: no funding reported.

    Conflict of Interest: Dr S.N. Tenembaum served as an advisory board member and speaker for Merck Serono. She serves on the clinical trial advisory board for Genzyme-Sanofi. Professional travel and accommodation expenses have been awarded to Dr S.N. Tenembaum by Merck-Serono.

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