Long-term follow-up of neuromyelitis optica with a pediatric onset

Background: Neuromyelitis optica (NMO) is a rare inflammatory disease. Average age at onset is 35 years. Few data exist on patients with pediatric-onset NMO (p-NMO), with disease onset before age 18 years. We report the clinical and paraclinical features and long-term outcome of patients with p-NMO and compare them with a large adult-onset NMO (a-NMO) cohort. Methods: We performed a retrospective, multicenter study of patients with p-NMO in pediatric and adult medical centers. We identified 125 patients with NMO (12 p-NMO; 113 a-NMO) fulfilling the 2006 criteria. Data were collected using hospital files and standardized assessment forms for NMO. Results: Patients with p-NMO were followed up during a mean 19.3 years. Median age at onset was 14.5 years (4.1-17.9) with a female: male ratio of 3:1. Three patients (25%) fulfilled Paty criteria for multiple sclerosis on first brain MRI, including one patient with acute disseminated encephalomyelitis. Median interval between onset and residual Expanded Disability Status Scale (EDSS) score 4 was 20.7 years, score 6 was 26 years, and score 7 was 28.7 years. Median interval between onset and residual visual loss <= 1/10 was 1.3 years. Compared with a-NMO, p-NMO showed a longer time to EDSS scores 4 and 6, largely explained by the severity of the first myelitis in the a-NMO group. Time to first treatment was longer in the p-NMO group (13.1 vs 3.4 years). Conclusion: Patients with p-NMO can present a diffuse inflammatory process on first brain MRI and have a longer time to disability than patients with a-NMO. Neurology (R) 2010;75:1084-1088