Prediction model for the differential diagnosis of MOG-IgG associated disease and multiple sclerosis at first central nervous system demyelinating episode in paediatric patients

• Ano de defesa: 2020
• Autor(a) principal: Costa, Bruna Klein da
• Tipo de documento: Tese
• Tipo de acesso: Acesso aberto
• Idioma: eng
• Instituição de defesa: Pontifícia Universidade Católica do Rio Grande do Sul; Escola de Medicina; Brasil; PUCRS; Programa de Pós-Graduação em Medicina e Ciências da Saúde
• Palavras-chave em Português: Predictive Model; Paediatric CNS Demyelinating Disease; Multiple Sclerosis; MOGIgG Associated Disease; Modelo Preditivo; Doença Inflamatória Desmielinizante do SNC em Pediatria; Esclerose Múltipla; Doença Associada ao MOG-IgG; CIENCIAS DA SAUDE::MEDICINA
• Link de acesso: http://tede2.pucrs.br/tede2/handle/tede/9525

Multiple Sclerosis (MS) is the most known chronic inflammatory demyelinating disease of the Central Nervous System (CNS). However, in paediatric patients there is a high frequency of acquired demyelinating events with a monophasic course. More recently, other demyelinating conditions have been increasingly identified in this age group. The anti-myelin oligodendrocyte glycoprotein immunoglobulin-G (MOG-IgG) associated disease (MOGAD) is more frequent in children and adolescents than in adults. The diagnosis of MOGAD relies on the detection of MOG-IgG using cell-based assays. However, it is not yet widely available worldwide. Its recognition and differential diagnosis with MS have prognostic and therapeutic implications. Therefore, it is critical to define the likelihood of MOGAD over MS at the first demyelinating attack. Here, we propose the first predictive score exclusively based on the clinical characteristics at first clinical attack for the differential diagnosis between MOGAD and MS patients. This is a nested case-control study of patients ≤ 18 years of a Brazilian paediatric multicentric prospective cohort (EMOCEMP – “Estudo multicêntrico observacional para caracterização da esclerose múltipla pediátrica no Brasil” – NCT03087136). We selected the MOGAD and MS patients and compared their clinical characteristics at first presentation identifying those more strongly associated with the risk of MOGAD. We found that younger age at presentation, male sex, bilateral optic neuritis and either isolated optic neuritis or multifocal presentation with encephalopathy were associated with MOGAD. Two or more points in our proposed clinical composite score has 80% sensibility and 66% specificity for the diagnosis of MOGAD. Combined clinical and demographic characteristics at first attack may guide diagnostic serologic testing for MOG-IgG and help to differentiate MS from MOGAD, support treatment decisions and optimize the use of health resources.