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The proportion of patients with multiple sclerosis (MS) who develop symptoms before 16 years of age has been variously estimated at between 0.4% and 10.5%. There have been few studies of the course of childhood‐onset MS and how it may differ from adult‐onset disease. Now data from the European Database for Multiple Sclerosis (EDMUS) network of adult neurology departments (Christel Renoux and colleagues. New England Journal of Medicine 2007;356:2603–13) have clarified the natural history of MS beginning in childhood.
Thirteen adult neurology departments in France and Belgium provided data on 394 patients with MS of childhood (age 16 years or younger) onset (COMS) and 1775 patients with MS of adult (>16 years) onset (AOMS). The proportion of the total cohort who had COMS was 2.2%. The female:male ratio was 2.8 in COMS and 1.8 in AOMS. In the COMS group, mean follow‐up was for 17 years and 49% received at least one disease‐modifying treatment (interferon β, azathioprine, mitoxantrone, cyclophosphamide, methotrexate, glatiramer acetate or mycophenolate mofetil) at some time. The mean age of onset of COMS was 13.7 years and 30 patients (7.6%) were aged 10 years or younger at disease onset. The first symptoms of COMS were isolated brain stem symptoms in 66 (17%), isolated long tract symptoms in 149 (38%), and other or combined symptoms in 87 (22%). The initial course was exacerbating‐remitting in 385 patients and progressive in nine. The median time to the second episode was 2 years. Among patients with an initial exacerbating‐remitting course, 110 (29%) converted to secondary progression during follow‐up (estimated median time to conversion to secondary progression, 28 years and estimated median age at conversion, 41 years). The estimated median times to scores on the Kurtzke Disability Status Scale of 4 (walking limited but can walk >500 m without aid), 6 (walking up to 100 m with support) or 7 (walking up to 10 m with support) were 20, 29 and 37 years, respectively. Walking was preserved for longer with younger age at onset. Patients with progressive disease at onset had shorter times to irreversible disability. Compared with AOMS, COMS was more likely to begin with isolated optic neuritis or isolated brain stem symptoms and less likely to begin with long tract symptoms. Encephalitic symptoms occurred in 7% of patients with COMS but were rare in AOMS. An exacerbating‐remitting initial course is more common in COMS (98% vs 84%) and the estimated times to conversion to secondary progression and to disability were 10 years longer in COMS than in AOMS. The estimated age at conversion or disability was about 10 years younger in COMS compared with AOMS.
Children with MS are more likely than adults to present with isolated optic neuritis or brain stem disorder and may present with an encephalopathic picture. They develop the disease about 20 years before adults and take about 10 years longer to progress to severe disability. They are therefore, on average, about 10 years younger than people with adult‐onset disease on reaching severe disability.