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Comprehensive Autopsy Program for Individuals with Multiple Sclerosis
Published on: July 19, 2019
Pediatric acquired CNS demyelinating syndromes: Features associated with multiple sclerosis
Rogier Q Hintzen1, Russell C Dale2, Rinze F Neuteboom2
1From the Departments of Neurology (R.Q.H., R.F.N.) and Immunology (R.Q.H.), MS Centre ErasMS, Neurology, Erasmus MC, Rotterdam, the Netherlands; Institute for Neuroscience and Muscle Research (R.C.D.), The Kids Research Institute at the Children's Hospital at Westmead, Sydney Medical School, University of Sydney, Australia; Departments of Pediatric and Developmental Neurology (S.M.), Washington University School of Medicine, St. Louis, MO; and Division of Neurology (B.B.), The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania. r.hintzen@erasmusmc.nl.
About one-third of pediatric acquired demyelinating syndrome (ADS) cases develop multiple sclerosis (MS) within 2-4 years. Factors like adolescent age and polyfocal deficits increase MS risk, while acute disseminated encephalomyelitis suggests a monophasic outcome.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Acquired demyelinating syndrome (ADS) in children can be a precursor to multiple sclerosis (MS).
- Understanding the factors predicting MS development in pediatric ADS is crucial for early diagnosis and management.
- Distinguishing MS from other demyelinating conditions in children is clinically important.
Purpose of the Study:
- To review the likelihood of pediatric acquired demyelinating syndrome (ADS) progressing to multiple sclerosis (MS).
- To identify clinical and demographic factors associated with MS development in children with ADS.
- To discuss diagnostic markers, including antibody signatures, in pediatric demyelinating diseases.
Main Methods:
- Literature review of studies on pediatric acquired demyelinating syndrome and multiple sclerosis.
- Analysis of clinical presentation, MRI findings, and demographic data in pediatric demyelinating disease cohorts.
- Discussion of diagnostic criteria and relevant biomarkers, such as aquaporin-4 antibodies.
Main Results:
- Approximately one-third of children with ADS are diagnosed with MS within 2-4 years.
- Adolescent age, female sex, and polyfocal deficits are associated with a higher risk of MS.
- Acute disseminated encephalomyelitis, preceding infection, and younger age at onset are linked to a monophasic outcome.
Conclusions:
- Pediatric MS shares genetic risk factors with adult MS, but these are not individually diagnostic.
- Aquaporin-4 antibodies are key indicators for neuromyelitis optica in children with specific neurological syndromes.
- Further research is needed to identify other antibody signatures differentiating relapsing demyelination from MS.
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