Soto de Castillo Ibis1, Molina Omaira2, Soto Arnoldo3, Armas Elizabeth4, Mendoza Sandra5, Castillo Maria Carlota2, Castro Elizabeth2, Vink Laura6, Leon Rosalba4, Valbuena Oscar7, Del Moral Luisa8, Acosta Nahir9, Freda Hernandez2. 1. Department of Neurology. Hospital Universitario de Maracaibo, Maracaibo, Venezuela. Electronic address: ibissotodecastillo@gmail.com. 2. Department of Neurology. Hospital Universitario de Maracaibo, Maracaibo, Venezuela. 3. Department of Neurology. Centro Medico Docente La Trinidad, Caracas, Venezuela. 4. Department of Neurology. Hospital Universitario de Caracas, Caracas, Venezuela. 5. Department of Neurology. Ciudad Hospitalaria "Dr. Enrique Tejera", Valencia, Venezuela. 6. Department of Neurology. Hospital Juan Daza Pereira, Barquisimeto, Venezuela. 7. Department of Pediatric Neurology, Hospital de especialidades pediátricas, Maracaibo, Venezuela. 8. Centro Medico de Cabimas, Cabimas, Venezuela. 9. Research Center, Instituto Cardiovascular del Cesar, Valledupar, Colombia.
Abstract
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.
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.
Authors: Fernando Gracia; Deyanira Ramírez; Alexander Parajeles-Vindas; Alejandro Díaz; Amado Díaz de la Fé; Nicia Eunice Ramírez Sánchez; Romy Castro Escobar; Luis Alberto García Valle; Roberto Weiser; Biany Santos; Awilda Candelario; Aron Benzadon; Pahola Araujo; Carlos Valderrama; Mario Larreategui; Gabriela Carrillo; Karla Gracia; Johana Vázquez-Céspedes; Priscilla Monterrey-Alvarez; Kenneth Carazo-Céspedes; Alfredo Sanabria-Castro; Gustavo Miranda-Loria; Andrea Balmaceda-Meza; Ligia Ibeth Portillo Rivera; Irma Olivera Leal; Luis Cesar Rodriguez Salinas; Arnold Thompson; Ericka López Torres; Daniel Enrique Pereira; Carolina Zepeda; César Abdón López; Ernesto Arturo Cornejo Valse; Karla Zinica Corea Urbina; Marco Antonio Urrutia; Ivonne Van Sijtveld; Blas Armien; Victor M Rivera Journal: Neurol Int Date: 2022-03-17