Doenças desmielinizantes recorrentes na população pediátrica: estudo retrospectivo dos pacientes acompanhados no ambulatório de doenças desmielinizantes da Universidade Federal de São Paulo - Escola Paulista de Medicina

• Autor(a) principal: Fragomeni, Manuela De Oliveira [UNIFESP]
• Data de Publicação: 2018
• Tipo de documento: Dissertação
• Idioma: por
• Título da fonte: Repositório Institucional da UNIFESP
• Texto Completo: https://sucupira.capes.gov.br/sucupira/public/consultas/coleta/trabalhoConclusao/viewTrabalhoConclusao.jsf?popup=true&id_trabalho=6722193; https://repositorio.unifesp.br/handle/11600/52163
• topic: Esclerose múltipla; Neuromioelite óptica; Pediatria; Terapêutica; Prognóstico; Epidemiologia; Multiple sclerosis; Optic neuromyelitis; Pediatrics; Epidemiology; Therapeutics; Prognosis

Introduction: Multiple Sclerosis (MS) and neuromyelitis optica (NMO) are inflammatory and demyelinating diseases of the central nervous system more frequently in young adults and their beginning before 18 years of age is rare. They are autoimmune diseases with distinct pathophysiology, clinical presentation, treatment and prognoses. During childhood these conditions often, present similar clinical features and sometimes it is difficult to distinguish them from each other and among other differential diagnoses. Objectives: To describe the epidemiologic and clinical characteristics, to evaluate the response to treatment and to compare the mains characteristics between the patients with MS and NMO who had the first event prior to 18 years of age followed at the Universidade Federal de São Paulo (UNIFESP). Methods: Retrospective analysis of patients with MS and NMO who started the disease before 18 years of age followed for at UNIFESP. All patients fulfilled the McDonald 2010 criteria for MS and the 2006 diagnostic criteria for NMO. For treatment analysis, we select patients with a follow-up of more than 6 months. Results: Sixty-eight patients fulfilled the inclusion criteria for MS and were selected for analysis. Mean age of onset was 15 years, 73,5% were female and the mean follow-up was 6,7 years. Mean annualized relapse rate (ARR) observed was 0,82 relapse/year and mean progression index (PI) was 0,31 EDSS points/year. The treatment with interferon-beta (b-IFN) and glatiramer acetate (GA) was safe and patients treated with high dose IFN and GA had a statistically significant reduction in disability progression. Eleven patients fulfilled the inclusion criteria for NMO: mean age of onset was 14 years, 72,7% were female and the mean follow-up was 6,3 years. Mean annualized relapse rate (ARR) observed was 1,5 relapse/year and mean progression index (PI) was 2,2 EDSS points/year. The treatment with azathioprine was safe and significant halts disability progression. Patients with NMO reached EDSS 6 prior than those with MS. Conclusions: In this series, the clinical features of pediatric patients with MS and NMO are similar to those described previously. The treatments offered were effective and safe in this population. NMO is more severe and disabling than MS in pediatric patients.