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Perturbação neuropsiquiátrica auto-imune pediátrica associada a infecções por streptococcus (PANDAS)
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Resumo(s)
PANDAS (Pediatric Autoimmune Neuropsychiatric Disorder associated with Streptococcal Infections) trata-se de um síndrome controverso que pretende caracterizar um quadro clínico de um subgrupo de crianças que desenvolvem, de forma abrupta e disruptiva, perturbação obsessiva-compulsiva e/ou perturbação de tiques acompanhadas de outras manifestações neurológicas como movimentos coreiformes após uma infecção por Streptococcus B-Hemolítico do grupo A, apresentando um curso episódico com relapsos e remissões.
A pertinência deste diagnóstico prende-se não só ao impacto dos sintomas na qualidade de vida destas crianças mas sobretudo à hipótese etiofisiopatológica subjacente. Vários autores defendem tratar-se de um processo auto-imune pós-infeccioso resultante de mimetismo molecular entre antigénios estreptocócicos e antigénios neuronais, perspectiva que preconiza uma intervenção terapêutica dirigida à etiologia.
PANDAS tem suscitado controvérsia na comunidade científica, com a sua validade como entidade diagnóstica questionada pela incongruência dos resultados nos vários estudos desenvolvidos sobre a sua etiologia, fisiopatologia e tratamento.
Com este trabalho de revisão bibliográfica pretende-se apresentar os progressos realizados e o actual conhecimento existente acerca da sua prevalência, etiologia, fisiopatologia, apresentação clínica, diagnóstico, terapêutica e prognóstico.
PANDAS (Pediatric Autoimmune Neuropsychiatric Disorder associated with Streptococcal Infections) is a controversial syndrome that aims to characterize the clinical presentation of a subgroup of children who develop, in an abrupt and disruptive way, obsessive-compulsive disorder and/or tic disorder accompanied by other neurological manifestations such as choreiform movements after an infection by group A B-Hemolytic Streptococcus, presenting an episodic course with relapses and remissions. The relevance of this diagnosis relates not only to the impact of the symptoms on the quality of life of these children, but mainly to the underlying etiopathophysiology hypothesis. Several authors argue that it is a post-infectious autoimmune process resulting from molecular mimicry between streptococcal antigens and neuronal antigens, a perspective that advocates a therapeutic approach focused on etiology. PANDAS has raised controversy in the scientific community, with its validity as a diagnostic entity questioned by the incongruity of results in the various studies developed on its etiology, pathophysiology and treatment. This bibliographic review aims to present the progress made and the current existing knowledge about its prevalence, etiology, pathophysiology, clinical presentation, diagnosis, therapy and prognosis.
PANDAS (Pediatric Autoimmune Neuropsychiatric Disorder associated with Streptococcal Infections) is a controversial syndrome that aims to characterize the clinical presentation of a subgroup of children who develop, in an abrupt and disruptive way, obsessive-compulsive disorder and/or tic disorder accompanied by other neurological manifestations such as choreiform movements after an infection by group A B-Hemolytic Streptococcus, presenting an episodic course with relapses and remissions. The relevance of this diagnosis relates not only to the impact of the symptoms on the quality of life of these children, but mainly to the underlying etiopathophysiology hypothesis. Several authors argue that it is a post-infectious autoimmune process resulting from molecular mimicry between streptococcal antigens and neuronal antigens, a perspective that advocates a therapeutic approach focused on etiology. PANDAS has raised controversy in the scientific community, with its validity as a diagnostic entity questioned by the incongruity of results in the various studies developed on its etiology, pathophysiology and treatment. This bibliographic review aims to present the progress made and the current existing knowledge about its prevalence, etiology, pathophysiology, clinical presentation, diagnosis, therapy and prognosis.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2020
Palavras-chave
PANDAS POC pediátrica Tiques Auto-imunidade Streptococcus Pediatria