Doença de Erdheim Chester com envolvimento isolado do SNC
Erdheim Chester disease with isolated CNS involvement
DOI:
https://doi.org/10.34119/bjhrv5n5-103Keywords:
Doença de Erdheim-Chester, células não-Langerhans, manifestações, tratamentosAbstract
Introdução: A Doença de Erdheim-Chester é uma forma de histiocitose de células não-Langerhans que ocorre mais frequentemente após os 40 anos de idade, com leve predomínio no sexo masculino. Apresentação do caso: Paciente, sexo masculino, branco, X anos de idade, admitido no Hospital das Clínicas de Goiânia, com sintomas de cefaléia crônica, de caráter progressivo de inicio há 3 meses, associados a sintomas neurológicos de ataxia e diplopia. Referiu febre baixa diária, não aferida, com perda de 10kg no período descrito, não associado a dieta ou mudança de hábitos de vida. Nega comorbidades ou uso de medicamentos. Ao exame físico, apresenta quadro de Nistagmo e Romberg positivo e alteração da úvula para direita. Paciente não apresentava outros sinais dignos de nota. Discussão: Foram selecionados para a revisão, os artigos de relato de caso ou séries de caso relacionados a manifestações clínicas da doença de Erdheim-Chester. Os principais tipos manifestações clínicas relatadas na literatura são: neurológicas, cardiovasculares, oftálmicas, endócrinas, urinárias, hematológicas, ósseas, cutâneas, respiratórias, gastrointestinais, envolvimento de múltiplos sistemas e outras. Entre esses tipos de manifestações, as mais frequentes foram neurológicas, cardiovasculares e oftálmicas, correspondendo a 48% das manifestações relatadas. Conclusão: É fundamental ter uma equipe multidisciplinar acompanhando o paciente, para que sejam evitadas complicações e se possa proporcionar qualidade de vida ao paciente.
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