A population-based study of neuromyelitis optica in Caucasians
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Abstract
Background: Epidemiologic studies have suggested different prevalence of neuromyelitis optica (NMO) in different ethnic groups. However, data on the incidence and prevalence of NMO in Caucasians are scarce.
Objective: To estimate the incidence and prevalence of NMO in a predominantly Caucasian population based on the Wingerchuk 2006 criteria.
Methods: The study was a population-based retrospective case series with longitudinal follow-up. Patients with multiple sclerosis (MS), optic neuritis (ON), acute transverse myelitis (TM), and NMO from the 4 neurology and 3 ophthalmology departments in the Region of Southern Denmark having been diagnosed between 1998 and 2008 were investigated. Patients were included based on 1) episodes of ON or TM and 2) an initial brain MRI not diagnostic for MS. An immunofluorescence assay was used to determine aquaporin-4 (AQP-4) antibodies.
Results: A total of 477 patients with MS, TM, or ON were evaluated: 163 fulfilled the inclusion criteria, 42 (26%) qualified for the diagnosis of NMO, 26 (62.0%) of these were AQP4 antibody positive. All except one were Caucasian, the female:male ratio was 2.8:1, and mean age at onset was 35.6 years (range 15–64 years). The clinical presentation was heterogeneous including TM, longitudinally extensive TM, ON, and brainstem syndromes. The yearly incidence rate of NMO in the population was estimated to be 0.4 per 105 person-years (95% confidence interval [CI] 0.30–0.54) and the prevalence was 4.4 per 105 (95% CI 3.1–5.7).
Conclusions: Despite being a rare disease, NMO is more common in a Caucasian population than earlier believed.
Footnotes
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Study funding: Supported by the Danish Foundation for Neurological Research, the Ole Jacobsen Commemoration Foundation, the Sonderborg Hospital Research Fund, the Vejle Hospital Research Fund, the Esbjerg, Sønderborg and Vejle Hospitals of the Region of Southern Denmark, and the University of Southern Denmark.
-
- AQP4=
- aquaporin-4;
- CI=
- confidence interval;
- DNPR=
- Danish National Patient Registry;
- EDSS=
- Expanded Disability Status Scale;
- IDD=
- inflammatory demyelinating disease;
- IgG=
- immunoglobulin G;
- LETM=
- longitudinally extensive transverse myelitis;
- MS=
- multiple sclerosis;
- NMO=
- neuromyelitis optica;
- NMSS=
- National Multiple Sclerosis Society;
- ON=
- optic neuritis;
- TM=
- acute transverse myelitis;
- VEP=
- visual evoked potential
- Received November 16, 2010.
- Accepted January 19, 2011.
- Copyright © 2011 by AAN Enterprises, Inc.
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