학술논문

Characterizing pediatric-onset neuromyelitis optica spectrum disorder in British Columbia.
Document Type
Article
Source
British Columbia Medical Journal. Jan/Feb2016, Vol. 58 Issue 1, p25-29. 5p.
Subject
*NEUROMYELITIS optica
*MULTIPLE sclerosis
Language
ISSN
0007-0556
Abstract
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 immuno-globulin or AQP4-IgG (also known as NMO-IgG). Differentiating NMOSD from multiple sclerosis has important prognostic and treatment implications. the features of pediatric-onset multiple sclerosis have been reported previously and are known to overlap considerably with adult-onset 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 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. [ABSTRACT FROM AUTHOR]