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Mult Scler. 2017 Oct 1:1352458517735191. doi: 10.1177/1352458517735191. [Epub ahead of print]

Epidemiology of NMOSD in Catalonia: Influence of the new 2015 criteria in incidence and prevalence estimates.

Author information

1
Center of Neuroimmunology, Laboratory of Advanced Imaging in Neuroimmunological Diseases, Hospital Clinic of Barcelona and Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
2
Department of Preventive Medicine and Epidemiology, Hospital Clinic of Barcelona, University of Barcelona, Barcelona, Spain.
3
Servei de Neurologia-Neuroimmunologia, Centre d'Esclerosi Múltiple de Catalunya (Cemcat), Vall d'Hebron Institut de Recerca, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
4
Servei de Neurologia-Neuroimmunologia, Centre d'Esclerosi Múltiple de Catalunya (Cemcat), Vall d'Hebron Institut de Recerca, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain; Department of Preventive Medicine and Epidemiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
5
Multiple Sclerosis Unit, Department of Neurology, Hospital Universitari de Bellvitge, Barcelona, Spain.
6
Center of Neuroimmunology, Laboratory of Advanced Imaging in Neuroimmunological Diseases, Hospital Clinic of Barcelona and Institut d'Investigacions Biomediques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain/Pediatric Neuroimmunology and Neuroinfections Unit, Hospital Sant Joan de Déu Barcelona, University of Barcelona, Spain/Centre for Biomedical Network Research on Rare Diseases (CIBERER), Madrid, Spain.
7
Department of Neurology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
8
Neuroimmunology and Multiple Sclerosis Unit, Neurology Department, Doctor Josep Trueta University Hospital, Girona, Spain/ Santa Caterina Hospital, Girona, Spain/Biomedical Research Institute (IDIBGI), Girona, Spain/Medical Sciences Department, University of Girona, Girona, Spain.
9
Multiple Sclerosis Unit, Neuroscience Department, Hospital Universitari Germans Trias i Pujol, Barcelona, Spain.
10
Multiple Sclerosis Unit, Neurology Department, Parc de Salut Mar, Barcelona, Spain.
11
Institut Guttmann, Barcelona, Spain.
12
Multiple Sclerosis Unit, Neurology Department, Hospital de Sant Joan Despí Moisès Broggi, Barcelona, Spain.
13
Multiple Sclerosis Unit, Neurology Department, Hospital Universitari Arnau de Vilanova, Lleida, Spain.
14
Neurology Department, Hospital Universitari Sant Joan de Reus, Tarragona, Spain.
15
Multiple Sclerosis Unit, Neurology Department, Hospital Parc Taulí de Sabadell, Barcelona, Spain.
16
Neurology Department, Hospital Universitari de Tarragona Joan XXIII, Tarragona, Spain.
17
Neurology Department, Hospital de Mataró, Barcelona, Spain.
18
Unit of Information and Knowledge, Catalan Health Service, Barcelona, Spain.
19
Servei de Neurologia-Neuroimmunologia, Centre d'Esclerosi Múltiple de Catalunya (Cemcat), Vall d'Hebron Institut de Recerca, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain; Division of Neurology, St. Michael's Hospital, University of Toronto, Toronto, ON, Canada.

Abstract

BACKGROUND:

Population-based studies on neuromyelitis optica spectrum disorders (NMOSD) are limited, and it is unclear whether the rates have changed with the implementation of the new 2015 criteria.

OBJECTIVES:

To estimate the incidence and prevalence of NMOSD in Catalonia (Spain), using both the 2006 and the 2015 criteria.

METHODS:

In this clinic-based retrospective study, patients diagnosed with NMOSD between 2006 and 2015 were identified using multiple sources, including direct contact to all Catalan hospitals, identification of cases through the Catalan Health Surveillance System, and registry of antibodies to aquaporin-4 (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG) in a reference laboratory. The incidence rate was calculated for the period 1 January 2006-1 January 2016 and prevalence for the date 1 January 2016.

RESULTS:

We identified 74 patients (by the 2015 criteria). Most patients were Caucasian (81%), and female (76%) with a median age at disease onset of 42 years (range, 10-76 years). In total, 54 (73%) patients were positive for AQP4-IgG, 11 (15%) double-seronegative, and 9 (12%) MOG-IgG-positive. Rates of incidence and prevalence (0.63/1,000,000 person-years and 0.89/100,000, respectively) were 1.5-fold higher than those reported by the 2006 criteria. Lowest rates were seen in children and elder people and highest in women and middle-aged people (40-59 years). The female predominance was lost in incident AQP4-IgG-seronegative children and AQP4-IgG-positive elder people. MOG-IgG and double-seronegativity contributed similarly but did not influence the long-term outcome.

CONCLUSION:

The new criteria increase the estimates, but NMOSD remains as a rare disease. The differences in age- and sex-specific estimates highlight the importance of the serologic classification.

KEYWORDS:

AQP4-antibodies; MOG-antibodies; Neuromyelitis optica spectrum disorders; incidence; prevalence

PMID:
28984163
DOI:
10.1177/1352458517735191

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