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
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Data
2018-04-26
Autores
Fragomeni, Manuela de Oliveira [UNIFESP]
Orientadores
Oliveira, Enedina Maria Lobato de [UNIFESP]
Tipo
Dissertação de mestrado
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Introdução: Esclerose múltipla (EM) e neuromielite óptica (NMO) são doenças inflamatórias desmielinizantes do sistema nervoso central (SNC), que ocorrem com maior frequência em adultos jovens, sendo raros os casos com início na população pediátrica, abaixo de 18 anos de idade. São doenças de etiologia autoimune com fisiopatologia, quadro clínico, tratamento e prognósticos distintos. Na faixa etária pediátrica, muitas vezes estas condições apresentam sintomas clínicos semelhantes e pode haver dificuldade em distingui-las entre si e entre outros diagnósticos diferenciais. Objetivos: Descrever as características clínicas e epidemiológicas, avaliar a resposta ao tratamento e comparar as principais características clínicas dos pacientes com EM e NMO pediátricas acompanhados na Universidade Federal de São Paulo (UNIFESP). Métodos: Estudo retrospectivo dos pacientes acompanhados por EM e NMO com início antes dos 18 anos na UNIFESP. Foram selecionados para análise aqueles que preenchessem os critérios diagnósticos de McDonald 2010 para EM e os critérios de 2006 para NMO. Para análise de tratamento, foram selecionados os pacientes com acompanhamento superior a 6 meses. Resultados: Sessenta e oito pacientes preencheram os critérios de inclusão para EM e foram selecionados para análise. A idade média de início de sintomas foi de 15 anos, 73,5% dos pacientes eram do sexo feminino e a média de tempo de acompanhamento foi de 6,7 anos. A taxa anualizada de surtos (TAS) média foi de 0,82 surto/ano e o índice de progressão (IP) médio foi de 0,31 ponto EDSS/ano. O tratamento com betainterferona (b-IFN) e acetato de glatiramer (AG) foi seguro, com redução estatisticamente significativa do IP com uso de b-IFN de alta dose e AG. Onze pacientes preencheram os critérios de inclusão para NMO: a idade média de início de sintomas foi de 14 anos, 72,7% dos pacientes eram do sexo feminino e a média de tempo de acompanhamento foi de 6,3 anos. A TAS média foi de 1,5 surto/ano e o IP médio foi de 2,2 ponto EDSS/ano. O tratamento com azatioprina foi seguro, com redução estatisticamente significativa do IP. Pacientes com NMO atingiram EDSS 6 significativamente mais cedo do que os pacientes com EM. Conclusões: As características clínicas dos pacientes com EM e NMO pediátricas avaliadas são semelhantes às descritas previamente. Os tratamentos oferecidos são seguros e efetivos nesta população. NMO é uma doença mais agressiva e incapacitante do que a EM na população pediátrica.
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.
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.
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Citação
FRAGOMENI, Manuela de Oliveira. 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. 2018. 88 f. Dissertação (Mestrado em Neurologia/Neurociências) - Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, 2018.