Natural History of Multiple Sclerosis with Childhood Onset

ABSTRACT

Background

The course and prognosis of childhood-onset multiplesclerosis have not been well described.

Methods

We used data from 13 adult neurology departments affiliatedwith the European Database for Multiple Sclerosis (EDMUS) networkto identify a cohort of 394 patients who had multiple sclerosiswith an onset at 16 years of age or younger and a comparisongroup of 1775 patients who had multiple sclerosis with an onsetafter 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, andirreversible disability as measured by scores of 4 (limitedwalking ability but ability to walk more than 500 m withoutaid or rest), 6 (ability to walk with unilateral support nomore than 100 m without rest), and 7 (ability to walk no morethan 10 m without rest while using a wall or furniture for support)on the Kurtzke Disability Status Scale (range, 0 to 10; higherscores indicate more severe disability).

Results

For patients with childhood-onset multiple sclerosis,the estimated median time from onset to secondary progressionwas 28 years, and the median age at conversion to secondaryprogression was 41 years. The median times from onset to disabilityscores 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 50.5years.  In comparison with patients with adult-onset disease,those with childhood-onset disease were more likely to be femalethan male (female:male ratio, 2.8 vs. 1.8), were more likelyto have an exacerbating–remitting initial course (98%vs. 84%), took approximately 10 years longer to reach secondaryprogression and irreversible disability, and reached these landmarksat an age approximately 10 years younger (P<0.001 for allcomparisons).

Conclusions

Patients with childhood-onset multiple sclerosistake longer to reach states of irreversible disability but doso at a younger age than patients with adult-onset multiplesclerosis.