Renata Barbosa Paolilo1, José Albino da Paz2, Samira Luisa Apóstolos-Pereira3, Carolina de Medeiros Rimkus4, Dagoberto Callegaro3, Douglas Kazutoshi Sato5. 1. Department of Neurology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), Rua dr Eneias de Carvalho Aguiar, 255, São Paulo 05403-000, Brazil. Electronic address: renatabpaolilo@gmail.com. 2. Department of Neurology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), Rua dr Eneias de Carvalho Aguiar, 255, São Paulo 05403-000, Brazil. Electronic address: jose.paz@hc.fm.usp.br. 3. Department of Neurology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), Rua dr Eneias de Carvalho Aguiar, 255, São Paulo 05403-000, Brazil. 4. Department of Radiology (INRAD), Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, Brazil. 5. Instituto do Cérebro do Rio Grande do Sul (InsCer), Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS), Porto Alegre, Brazil. Electronic address: douglas.sato@pucrs.br.
Abstract
BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) is a severe condition associated with high disability and low quality of life (QoL) in adults. Since this evaluation had been rarely perfomed in children, this study aimed to describe QoL in pediatric-onset NMOSD with positive aquaporin4 antibody (AQP4-IgG) patients. METHODS: This was a cross-section evaluation of patients and parents' proxy QoL from individuals enrolled in a longitudinal cohort of AQP4-IgG positive NMOSD with onset ≤ 18 years of age. RESULTS: Eighteen patients were included, sixteen girls. The mean (SD) age at disease onset was 11.5 (3.6) years. Eleven of patients experienced disability during a mean (SD) of 8.3 (5.3) years of follow-up. NMOSD had impact in QoL in 10 patients, being associated with higher EDSS and poor academic performance at last follow-up. Results from the PedsQL inventory for 13 patients and 10 parents disclosed low QoL specially in emotional functioning. CONCLUSION: This study indicates impaired quality of life, high disability and high impact of the disease in daily life of adolescents and young adults with pediatric onset NMOSD.
BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) is a severe condition associated with high disability and low quality of life (QoL) in adults. Since this evaluation had been rarely perfomed in children, this study aimed to describe QoL in pediatric-onset NMOSD with positive aquaporin4 antibody (AQP4-IgG) patients. METHODS: This was a cross-section evaluation of patients and parents' proxy QoL from individuals enrolled in a longitudinal cohort of AQP4-IgG positive NMOSD with onset ≤ 18 years of age. RESULTS: Eighteen patients were included, sixteen girls. The mean (SD) age at disease onset was 11.5 (3.6) years. Eleven of patients experienced disability during a mean (SD) of 8.3 (5.3) years of follow-up. NMOSD had impact in QoL in 10 patients, being associated with higher EDSS and poor academic performance at last follow-up. Results from the PedsQL inventory for 13 patients and 10 parents disclosed low QoL specially in emotional functioning. CONCLUSION: This study indicates impaired quality of life, high disability and high impact of the disease in daily life of adolescents and young adults with pediatric onset NMOSD.