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Comprehensive Autopsy Program for Individuals with Multiple Sclerosis
Published on: July 19, 2019
Long-term prognostic features of pediatric-onset multiple sclerosis: A large cohort from Türkiye
Canan Duman İlki1, Ahmed Serkan Emekli2, Tuncay Gündüz2
1Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey; Department of Neurology, Sakarya University Training and Research Hospital, Sakarya, Turkey.
Insights
Pediatric-onset multiple sclerosis (POMS) patients with onset after age 14, brainstem involvement, or poor initial recovery face earlier disability milestones. Early characteristics are key for optimizing POMS treatment strategies.
Area of Science:
- Neurology
- Pediatrics
- Immunology
Background:
- Pediatric-onset multiple sclerosis (POMS) is a rare condition with distinct characteristics from adult-onset MS.
- Understanding POMS demographics, clinical course, and long-term outcomes is crucial for effective management.
Purpose of the Study:
- To characterize the demographic features, clinical presentations, and long-term outcomes of POMS patients in a Turkish tertiary MS center.
- To identify predictors of disability progression and conversion to secondary progressive MS (SPMS) in POMS.
Main Methods:
- Retrospective analysis of 143 POMS patients diagnosed before age 16, monitored between 1981 and 2018.
- Data collected included age at onset, initial symptoms, relapse history, and Expanded Disability Status Scale (EDSS) scores.
- Kaplan-Meier analysis and Cox regression were used to examine disability milestones and SPMS conversion.
Main Results:
- The cohort (69.2% female) had a median follow-up of 11 years, with a median onset age of 14.6 years.
- 22.4% reached EDSS 4 and 16.8% converted to SPMS.
- Onset after age 14, male sex, higher baseline EDSS, and incomplete initial recovery predicted disability progression. Delayed treatment initiation also increased risk.
Conclusions:
- In this Turkish POMS cohort, later onset, brainstem involvement, and poor initial recovery correlated with earlier disability milestones.
- Early disease characteristics significantly influence prognosis in POMS.
- Optimizing treatment strategies based on early prognostic factors is essential for improving long-term outcomes in pediatric MS.
Introduction:
This study aimed to characterize demographic features, clinical presentations, and long-term outcomes of pediatric-onset multiple sclerosis (POMS) patients in a Turkish tertiary MS center.
Methods:
We retrospectively analyzed 143 patients diagnosed with multiple sclerosis (MS) before age 16, based on the 2017 McDonald criteria, and clinically monitored between 1981 and 2018. Demographic and clinical data, including age at onset, initial symptoms, relapse history, and Expanded Disability Status Scale (EDSS) scores, were collected. Disability milestones (EDSS 4 and 6) and conversion to secondary progressive MS (SPMS) were examined using Kaplan-Meier survival analysis and Cox regression.
Results:
We followed 143 POMS patients for a median of 11 years (69.2% female; median onset age: 14.6 years). By study completion, 22.4% reached EDSS 4 and 16.8% converted to SPMS. Onset before age 14 was associated with a longer time to EDSS 4 (p < 0.001) and SPMS-free survival (p = 0.03). In multivariate analysis, male sex, onset after age 14, higher baseline EDSS, and incomplete recovery from the first attack independently predicted disability progression. Delayed disease modifying therapy initiation (≥12 months) was also associated with increased risk of reaching EDSS 4. SPMS conversion was predicted by onset after age 14, higher baseline EDSS, brainstem onset, and incomplete recovery from the first attack.
Conclusion:
In this large Turkish POMS cohort, onset after age 14, brainstem involvement, and poor recovery from the first attack were associated with earlier disability milestones. These findings highlight the prognostic role of early disease characteristics and emphasize the importance of optimizing treatment strategies in pediatric MS.
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