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Abstract(s)
Introdução: PANDAS é a abreviatura inglesa de “Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections” (Distúrbios Neuropsiquiátricos Autoimunes Pediátricos Associados a Infeções Estreptocócicas). O termo é utilizado para descrever um subgrupo de crianças e adolescentes que apresentam um início súbito de Transtorno Obsessivo-Compulsivo (TOC) e/ou tiques, após uma infeção estreptocócica de grupo A.
Objetivos: Clarificar a epidemiologia, a fisiopatologia, as bases para o diagnóstico, e a orientação terapêutica bem como o prognóstico desta síndrome.
Métodos: Pesquisa bibliográfica abrangente, com base em artigos de diversos especialistas na área da Pediatria, Psiquiatria, Neurologia e Infeciologia, efetuada nas bases de dados: PubMed, UpToDate, ScienceDirect, Medscape e Scielo, entre setembro de 2018 e março de 2019, utilizando os termos: PANDAS; PANS; Obsessive-compulsive disorder; Tics; Streptococcus infection. Foram incluídos artigos nos idiomas Espanhol, Italiano, Inglês e Português, sem limitação temporal.
Resultados: O mecanismo proposto para a patogénese de PANDAS, sugere que exista inicialmente uma infeção por estreptococo beta-hemolítico grupo A, num hospedeiro com suscetibilidade genética, causando uma resposta imunitária anormal que se traduz na produção de anticorpos antineuronais, com atingimento de estruturas cerebrais, nomeadamente os gânglios da base, com repercussões no normal funcionamento neurobiológico, levando assim à apresentação dos sintomas característicos desta patologia. É um diagnóstico sobretudo clínico e complexo, obedecendo atualmente a cinco critérios: presença de TOC e/ou tiques; exigência etária (entre os 3 anos e a puberdade); início agudo e percurso episódico; associação com infeção estreptocócica do grupo A; e associação com anomalias neurológicas. O tratamento com antibioterapia, imunomoduladores, terapêutica farmacológica neuropsiquiátrica dirigida, bem como a associação de terapia cognitivo-comportamental, é recomendado por se revelar eficaz no controlo e remissão dos sintomas.
Conclusão: Apesar da controvérsia do tema em relação aos mecanismos propostos para explicar a etiologia e respetiva correlação com a fisiopatologia, e não obstante ser necessário realizar investigação adicional para explicar a heterogeneidade dos casos de PANDAS, o não reconhecimento desta síndrome, e a instituição de terapêutica dirigida de forma não atempada, piora inequivocamente o outcome clínico, diminuindo a qualidade de vida das crianças a curto, médio e longo prazo.
Introduction: PANDAS is the English abbreviation for "Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections". The term is used to describe a subgroup of children and adolescents who have a sudden onset of Obsessive-Compulsive Disorder (OCD) and/or tics after a group A streptococcal infection. Objectives: Clarify the epidemiology, physiopathology, the basis for diagnosis and the therapeutic orientation, as well as the prognosis of this syndrome. Methods: Comprehensive bibliographic research was carried out, based on articles of several specialists in Pediatrics, Psychiatry, Neurology and Infeciology, carried out in the PubMed, UpToDate, ScienceDirect, Medscape and Scielo databases, between September 2018 and March 2019, using the terms: PANDAS; PANS; Obsessive-compulsive disorder; Tics; Streptococcus infection. Articles in Spanish, Italian, English and Portuguese were included, without any temporal limitation. Results: The proposed mechanism for the pathogenesis of PANDAS suggests that there is, initially, a group A beta-hemolytic streptococcal infection, in a host with genetic susceptibility, causing an abnormal immune response that results in the production of antineuronal antibodies, reaching brain structures, as the basal ganglia, with repercussions on the normal neurobiological functioning, leading to the presentation of the characteristic symptoms of this pathology. Mainly, the diagnose is clinical and complex, currently obeying five criteria: presence of OCD and/or tics; age requirement (between 3 years and puberty); acute onset and episodic course; association with group A streptococcal infection; and association with neurological abnormalities. Treatment with antibiotic therapy, immunomodulators, targeted neuropsychiatric pharmacology, as well as the association of cognitive-behavioral therapy, is recommended because it is effective in controlling and remission of symptoms. Conclusion: Despite the controversy over the mechanisms proposed to explain the etiology and its correlation with pathophysiology, and although the further research needed to explain the heterogeneity of PANDAS cases, the non-recognition of this syndrome, and the institution of targeted therapy in a non-timely manner, unequivocally worsens the clinical outcome, reducing children's quality of life in the short, medium and long term.
Introduction: PANDAS is the English abbreviation for "Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections". The term is used to describe a subgroup of children and adolescents who have a sudden onset of Obsessive-Compulsive Disorder (OCD) and/or tics after a group A streptococcal infection. Objectives: Clarify the epidemiology, physiopathology, the basis for diagnosis and the therapeutic orientation, as well as the prognosis of this syndrome. Methods: Comprehensive bibliographic research was carried out, based on articles of several specialists in Pediatrics, Psychiatry, Neurology and Infeciology, carried out in the PubMed, UpToDate, ScienceDirect, Medscape and Scielo databases, between September 2018 and March 2019, using the terms: PANDAS; PANS; Obsessive-compulsive disorder; Tics; Streptococcus infection. Articles in Spanish, Italian, English and Portuguese were included, without any temporal limitation. Results: The proposed mechanism for the pathogenesis of PANDAS suggests that there is, initially, a group A beta-hemolytic streptococcal infection, in a host with genetic susceptibility, causing an abnormal immune response that results in the production of antineuronal antibodies, reaching brain structures, as the basal ganglia, with repercussions on the normal neurobiological functioning, leading to the presentation of the characteristic symptoms of this pathology. Mainly, the diagnose is clinical and complex, currently obeying five criteria: presence of OCD and/or tics; age requirement (between 3 years and puberty); acute onset and episodic course; association with group A streptococcal infection; and association with neurological abnormalities. Treatment with antibiotic therapy, immunomodulators, targeted neuropsychiatric pharmacology, as well as the association of cognitive-behavioral therapy, is recommended because it is effective in controlling and remission of symptoms. Conclusion: Despite the controversy over the mechanisms proposed to explain the etiology and its correlation with pathophysiology, and although the further research needed to explain the heterogeneity of PANDAS cases, the non-recognition of this syndrome, and the institution of targeted therapy in a non-timely manner, unequivocally worsens the clinical outcome, reducing children's quality of life in the short, medium and long term.
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Keywords
Obsessive-Compulsive Disorder Pandas Pans Streptococcus Infection Tics