Characterizing pediatric-onset neuromyelitis optica spectrum disorder in British Columbia

Background: Neuromyelitis optica spectrum disorder (NMOSD) has emerged as a disorder distinct from multiple sclerosis, largely due to the discovery in 2004 of a novel disease marker, aquaporin-4 immunoglobulin or AQP4-IgG (also known as NMO-IgG). Differentiating NMOSD from multiple sclerosis has important prognostic and treatment implications. The features of pediatriconset multiple sclerosis have been reported previously and are known to overlap considerably with adultonset multiple sclerosis. Less is known about the presentation of pediatric-onset NMOSD. Methods: Demographic and clinical characteristics of pediatric-onset NMOSD patients in British Columbia were identified using UBC Hospital records. Data from 10 cases were collected and analyzed over 5 years. All cases were diagnosed by a neurologist with expertise in NMOSD. Results: Cases of NMOSD with AQP4-IgG (30.0%) and without AQP4-IgG (40.0%) were identified, along with cases of longitudinally extensive transverse myelitis (30.0%). A diverse ethnic population was involved in the study, which included Characterizing pediatric-onset neuromyelitis optica spectrum disorder in British Columbia A review of patient records at UBC Hospital indicates that more females than males are affected by a rare and debilitating disease that can be mistaken for multiple sclerosis, and that many patients are of Asian descent. Ms Lee is an undergraduate research student at the University of British Columbia. Dr Katrina McMullen is a clinical researcher in the Department of Medicine (Neurology) at the University of British Columbia. Dr Robert Carruthers is a clinical assistant professor in the Department of Medicine (Neurology) and the associate director of multiple sclerosis and neuromyelitis optica clinical trials at the University of British Columbia. Dr Traboulsee is an associate professor in the Department of Medicine (Neurology) at the University of British Columbia. He is also MS Society of Canada Research Chair and director of the MS Clinic and NMO Clinic and Research Program at UBC Hospital. patients of Chinese (50.0%), Korean (20.0%), East Indian (10.0%), and Caucasian (20.0%) descent. More females (70.0%) than males (30.0%) were identified, and more patients experienced optic neuritis (60.0%) than opticospinal attacks (30.0%) or spinal attacks (10.0%) as the first event. Average age of onset was 10.0 years. Conclusions: In common with findings from studies in Germany, Brazil, and South Korea, female patients outnumbered male patients in BC and a preponderance of patients were of Asian descent. In contrast to findings from France, where similar percentages of patients experienced optic neuritis (50.0%) and spinal attacks (41.7%), more patients in BC were affected by optic neuritis (60.0%) than spinal attacks (10.0%). As well, a lower percentage of patients in BC tested positive for AQP4-IgG (30.0%) when compared with patients in South Korea (66.7%) and France (66.7%). It is essential to raise awareness of pediatric-onset NMOSD and its presenting features in the medical community and to find out more about the disorder so that we can expand on the limited treatment options available. Background Neuromyelitis optica spectrum disorder (NMOSD), previously known as neuromyelitis optica (NMO) and Devic disease, is a rare neuroinflammatory disease of the central nervous system. This debilitating disease is clinically and pathophysiologically distinct from multiple sclerosis (MS), and primarily affects the optic nerve and spinal cord, resulting in severe optic neuritis (ON) and transverse myelitis (TM). In NMOSD, spinal cord lesions can be seen on MRI to extend over three or more spinal segments. Defined as longitudinally extensive transverse myelitis This article has been peer reviewed.