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Overall, 3% to 10% of patients with multiple sclerosis (MS) show their first clinical event during childhood.
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Children with MS have higher relapse rates compared with adult-onset MS.
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As in adult patients, pediatric MS diagnosis requires dissemination in space and time, clinically or by MR imaging findings.
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Alternative diagnoses such as acute disseminating encephalomyelitis and neuromyelitis optica spectrum disorder must be differentiated from MS.
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Revised MR imaging criteria are a useful tool to
Pediatric Multiple Sclerosis: Distinguishing Clinical and MR Imaging Features
Section snippets
Key points
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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Cited by (10)
Assessment of acute motor deficit in the pediatric emergency room
2017, Jornal de PediatriaCitation Excerpt :There is an ongoing controversy on whether ADEM and multiple sclerosis (MS) belong to the same disease spectrum or are completely distinct entities.16 Although ADEM usually follows a monophasic course, its recurring and multiphasic variants blurs the distinction from MS. Brain MRI may be the most valuable tool to accomplish such distinction, as ADEM cases exhibit large areas of increased signal intensity on T2 and FLAIR-weighted images, with ill-defined borders, distributed bilaterally in the cerebral white matter (WM) and often affecting the basal ganglia, brainstem, and cerebellar and cerebral cortex gray matter, while MS cases usually show well-defined lesions that are confined to the periventricular WM.17 Absence of encephalopathy, age above 10 years, presence of optic neuritis, and intrathecal oligoclonal bands during an acute CNS demyelination event increase the risk of MS development.15 CSF may be normal or may display mild pleocytosis, with or without elevated protein levels.15
Viral encephalitis: a practical review on diagnostic approach and treatment
2020, Jornal de PediatriaCitation Excerpt :ADEM patients have encephalopathy with or without focal symptoms, usually associated with previous history of vaccination or infection in the previous two months. The brain MRI may reveal diffuse, bilateral, poorly defined T2WI hyperintense lesions with involvement of white matter and deep grey matter.40 Lesions are usually formed at the same time, so all may (or may not) show contrast enhancement.
Diagnostic challenge in children with an acquired demyelinating syndrome: an illustrative case report
2023, Frontiers in NeuroscienceClinical approach to pediatric demyelinating disease
2021, Neuroimmunology: Multiple Sclerosis, Autoimmune Neurology and Related DiseasesImaging in Pediatric Multiple Sclerosis: An Iconographic Review
2021, Clinical NeuroradiologyDemyelinating conditions, myelitis and encephalitides
2020, Pediatric Rehabilitation: Principles and Practice
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.