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Published on: December 9, 2015
Comparing Natural History of Early and Late Onset Pediatric Multiple Sclerosis
Ermelinda De Meo1,2, Massimo Filippi1,2,3,4,5, Maria Trojano6
1Neuroimaging Research Unit, Division of Neuroscience, San Raffaele Scientific Institute, Scientific Institute for Research and Health Care, Milan, Italy.
Insights
Pediatric multiple sclerosis has different disease courses depending on onset age. Early-onset pediatric multiple sclerosis patients experience a slower disease progression and longer time to disability milestones compared to late-onset patients.
Area of Science:
- Neurology
- Pediatrics
- Immunology
Background:
- Pediatric multiple sclerosis (MS) is a rare autoimmune disorder affecting the central nervous system.
- Understanding the differences in disease course and prognosis between early-onset and late-onset pediatric MS is crucial for effective management.
Purpose of the Study:
- To describe and compare the disease course and prognosis of early-onset (before age 11) versus late-onset (after age 11) pediatric multiple sclerosis.
- To identify prognostic factors influencing long-term disability in different onset groups.
Main Methods:
- Analysis of prospectively collected data from 1993 pediatric MS patients in the Italian Multiple Sclerosis Register.
- Utilized Cox models to assess the risk of irreversible disability milestones and conversion to secondary progressive MS.
- Adjusted for covariates including sex, baseline disability, and disease-modifying treatments.
Main Results:
- Early-onset pediatric MS showed a higher proportion of males and isolated brainstem involvement.
- Early-onset patients had a longer time to reach disability milestones and convert to secondary progressive MS compared to late-onset.
- Key predictors for long-term disability varied between groups, with factors like recovery from initial events being crucial in early-onset MS.
Conclusions:
- Early and late-onset pediatric MS exhibit distinct natural histories.
- Prognostic factors differ significantly, necessitating tailored treatment and monitoring strategies for each group.
Objective:
This study was undertaken to describe and compare disease course and prognosis of early (ie, disease onset before age 11 years) and late (ie, disease onset after age 11 years) onset pediatric multiple sclerosis.
Methods:
Prospectively collected clinical information from Italian Multiple Sclerosis Register of 1993 pediatric multiple sclerosis patients, of whom 172 had early onset, was analyzed. Cox models adjusted for sex, baseline Expanded Disability Status Scale score, and disease-modifying treatments and stratified for diagnostic criteria adopted (Poser vs McDonald) were used to assess the risk of reaching irreversible Expanded Disability Status Scale scores of 3, 4, and 6, and conversion to secondary progressive phenotype in early versus late onset pediatric patients. Prognostic factors were also evaluated.
Results:
A greater proportion of males, isolated brainstem involvement, and longer time interval between first and second clinical episode were observed in early versus late onset pediatric patients. Compared to late onset, early onset pediatric patients took longer from disease onset to convert to secondary progressive phenotype and to reach all disability milestones. Recovery from first demyelinating event, time to first relapse, annualized relapse rate during the first 3 years of disease, and disease-modifying treatment exposure were independent predictors for long-term disability in early onset pediatric patients. In late onset pediatric patients, isolated optic neuritis, multifocal symptoms, and progressive course at disease onset were additional predictors for long-term disability.
Interpretation:
These findings point toward the existence of a different natural history in early versus late onset pediatric multiple sclerosis patients. ANN NEUROL 2022;91:483-495.

