Epidemiological findings of neuromyelitis optica spectrum disorders in a Venezuelan study

Autor: Freda Hernandez, Valbuena Oscar, Soto Arnoldo, Acosta Nahir, Leon Rosalba, Vink Laura, Castro Elizabeth, Castillo Maria Carlota, Armas Elizabeth, Soto de Castillo Ibis, Mendoza Sandra, Molina Omaira, Del Moral Luisa
Rok vydání: 2021
Předmět:
Zdroj: Multiple Sclerosis and Related Disorders. 47:102652
ISSN: 2211-0348
Popis: Background Neuromyelitis optica spectrum disorders (NMOSD), is a rare autoimmune inflammatory disease of the central nervous system. Since the countries of Latin America (LATAM) show contrast in geographic, social, environmental factors, and genetic heterogeneity, the information about NMOSD epidemiology in the region allows a better understanding of the disease and its clinical outcome. Objectives To determine the prevalence, relative frequency (RF), and clinical characteristics of NMOSD in a multiethnic Venezuelan cohort of patients with demyelinating disorders. Methods We conducted a retrospective descriptive multicenter study of hospital case records of individuals with an established diagnosis of MS and NMOSD in the National Program for Multiple sclerosis (MS) from 2011 to 2018. We selected those NMOSD cases based on the 2006 Wingerchuck and the 2015 International panel for the diagnosis of Neuromyelitis optica (IPND) criteria. Results We identified 249 patients with NMOSD. The prevalence was 2.11 per100,000 individuals (95% confidence interval (CI)1.85 2.37), the RF was 23%, and the MS/NMOSD ratio was 3.2:1. The average disease onset occurred by the fourth decade of life (34±14.8 years of age); with a strong female predominance (female to male ratio: 4:1). Mestizos constituted 86,7% of this cohort. Most of the patients presented initially with simultaneous optic neuritis (ON) and acute transverse myelitis (ATM) and a recurrent course was registered in 82.3% of cases. The mean of the expanded disability status scale (EDSS) was 3.5 (IQR 2-7). Abnormal brain and spine MRI were present in 47.8% and 81.1% of patients, respectively. Antibodies against aquaporin-4 (AQP4) which were measured through a cell-based assay were positive in 55.3% of the individuals tested. The most used immunosuppressant agent was Azathioprine (57.4%). Conclusion NMOSD in Venezuela affects mainly young Mestizo women and shows one of the highest relative frequency in the region. Planning and developing healthcare programs for underserved populations as well as more comprehensive LATAM studies are required to identify the distribution and variations of its epidemiological picture.
Databáze: OpenAIRE
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