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Published on: September 21, 2021
Natural history of multiple sclerosis with childhood onset
Christel Renoux1, Sandra Vukusic, Yann Mikaeloff
1Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, France.
Insights
Childhood-onset multiple sclerosis (MS) progresses slower but leads to disability at a younger age compared to adult-onset MS. This study highlights key differences in disease course and prognosis for pediatric MS patients.
Area of Science:
- Neurology
- Pediatric Neurology
- Autoimmune Diseases
Background:
- The clinical course and long-term prognosis of childhood-onset multiple sclerosis (MS) remain incompletely understood.
- Limited data exists on the progression patterns and disability milestones in pediatric MS patients.
Purpose of the Study:
- To describe the course and prognosis of multiple sclerosis (MS) with onset in childhood (age 16 or younger).
- To compare the disease progression and disability accumulation in childhood-onset MS versus adult-onset MS.
Main Methods:
- Utilized data from the European Database for Multiple Sclerosis (EDMUS) network.
- Identified and compared a cohort of 394 childhood-onset MS patients with 1775 adult-onset MS patients.
- Assessed clinical features, onset dates, relapse rates, conversion to secondary progression, and irreversible disability using the Kurtzke Disability Status Scale.
Main Results:
- Childhood-onset MS patients had a longer median time to secondary progression (28 years) and irreversible disability (20-37 years).
- Despite longer progression times, irreversible disability was reached at younger median ages (34.6-50.5 years) compared to adult-onset MS.
- Childhood-onset MS was more common in females (2.8:1 ratio) and presented with an exacerbating-remitting course (98%) more frequently than adult-onset MS (84%).
Conclusions:
- Patients with childhood-onset multiple sclerosis experience a prolonged disease course before reaching irreversible disability.
- However, these individuals attain disability milestones at a significantly younger chronological age than those with adult-onset multiple sclerosis.
- These findings underscore distinct disease trajectories and necessitate tailored management strategies for pediatric MS.
Background:
The course and prognosis of childhood-onset multiple sclerosis have not been well described.
Methods:
We used data from 13 adult neurology departments affiliated with the European Database for Multiple Sclerosis (EDMUS) network to identify a cohort of 394 patients who had multiple sclerosis with an onset at 16 years of age or younger and a comparison group of 1775 patients who had multiple sclerosis with an onset after 16 years of age. We determined the initial clinical features, the dates of disease onset, and the occurrence of outcomes, including relapse, conversion to secondary progression, and irreversible disability as measured by scores of 4 (limited walking ability but ability to walk more than 500 m without aid or rest), 6 (ability to walk with unilateral support no more than 100 m without rest), and 7 (ability to walk no more than 10 m without rest while using a wall or furniture for support) on the Kurtzke Disability Status Scale (range, 0 to 10; higher scores indicate more severe disability).
Results:
For patients with childhood-onset multiple sclerosis, the estimated median time from onset to secondary progression was 28 years, and the median age at conversion to secondary progression was 41 years. The median times from onset to disability scores of 4, 6, and 7 were 20.0, 28.9, and 37.0 years, respectively, and the corresponding median ages were 34.6, 42.2, and 50.5 years. In comparison with patients with adult-onset disease, those with childhood-onset disease were more likely to be female than male (female:male ratio, 2.8 vs. 1.8), were more likely to have an exacerbating-remitting initial course (98% vs. 84%), took approximately 10 years longer to reach secondary progression and irreversible disability, and reached these landmarks at an age approximately 10 years younger (P<0.001 for all comparisons).
Conclusions:
Patients with childhood-onset multiple sclerosis take longer to reach states of irreversible disability but do so at a younger age than patients with adult-onset multiple sclerosis.
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