Reversible posterior encephalopathy syndrome in a 10-year-old child / Síndrome da encefalopatia reversível posterior em uma criança de 10 anos

Abstract Introduction: The posterior reversible encephalopathy (PRES) syndrome encompasses a set of clinical-radiological findings associated with severe systemic arterial hypertension. This case report proposes to discuss the identification, diagnosis, and management of PRES in the pediatric population. Case presentation: Female patient, 10 years old, admitted to the emergency room with complaint of oliguria and generalized edema. At the initial physical exam, the only alteration present was anasarca. The diagnostic investigation revealed nephrotic syndrome, and clinical treatment was started. She evolved on the 8th day of hospitalization with peak hypertension, sudden visual loss, reduced level of consciousness, nystagmus, and focal seizures requiring intubation. She was transferred to the Intensive Care Unit, with neurological improvement, after the established therapy. CT scan revealed a discrete hypodense area in the white matter of the occipital lobe and anteroposterior groove asymmetry, compatible with PRES. Discussion: PRES is due to vasogenic cerebral edema of acute or subacute installation. Symptoms include headache and altered consciousness, stupor, coma, neurological deficits, seizures and cortical blindness. Nephropathies are the main cause of PRES in pediatrics. Magnetic resonance imaging with diffusion of molecules is the gold standard for diagnosis. The initial treatment objectives are the reduction of blood pressure, antiepileptic therapy, correction of hydroelectrolytic and acid-base disorders and management of intracranial hypertension. Conclusion: PRES is associated with acute hypertension. Early diagnosis and proper management may determine a better prognosis and minimize the severity of the clinical course.

Resumo Introdução: A Síndrome de Encefalopatia Posterior Reversível (SEPR) engloba um conjunto de achados clínico-radiológicos, associados a hipertensão arterial sistêmica grave. Este relato de caso propõe discutir a identificação, o diagnóstico e o manejo de SEPR na população pediátrica. Apresentação do caso: Paciente do sexo feminino, 10 anos, admitida em pronto-atendimento com queixa de oligúria e edema generalizado. Ao exame físico inicial, a única alteração presente era anasarca. A investigação diagnóstica revelou síndrome nefrótica, iniciando-se tratamento clínico. Evoluiu no 8º dia de internação com pico hipertensivo, perda visual súbita, redução do nível de consciência, nistagmo e crises convulsivas focais, demandando intubação. Foi transferida para Unidade de Terapia Intensiva, com melhora neurológica, após a terapêutica instituída. Tomografia de crânio evidenciou área de hipodensidade discreta em substância branca do lobo occipital e assimetria anteroposterior de sulcos, compatível com SEPR. Discussão: A SEPR decorre de edema cerebral vasogênico de instalação aguda ou subaguda. Sintomas descritos incluem cefaleia e alteração de consciência, estupor, coma, déficits neurológicos, convulsões e cegueira cortical. As nefropatias constituem as principais causas de SEPR em pediatria. A ressonância magnética com difusão de moléculas é o padrão-ouro para o diagnóstico. Os objetivos iniciais são a redução dos níveis pressóricos, terapia antiepiléptica, correção de distúrbios hidroeletrolíticos e do equilíbrio ácido-básico, e manejo da hipertensão intracraniana. Conclusão: A SEPR ocorre associada à hipertensão aguda. O diagnóstico precoce e manejo adequado podem determinar melhor prognóstico e minimizar a gravidade do curso clínico.

Reversible posterior encephalopathy syndrome in a 10-year-old child.

INTRODUCTION: The posterior reversible encephalopathy (PRES) syndrome encompasses a set of clinical-radiological findings associated with severe systemic arterial hypertension. This case report proposes to discuss the identification, diagnosis, and management of PRES in the pediatric population. CASE PRESENTATION: Female patient, 10 years old, admitted to the emergency room with complaint of oliguria and generalized edema. At the initial physical exam, the only alteration present was anasarca. The diagnostic investigation revealed nephrotic syndrome, and clinical treatment was started. She evolved on the 8th day of hospitalization with peak hypertension, sudden visual loss, reduced level of consciousness, nystagmus, and focal seizures requiring intubation. She was transferred to the Intensive Care Unit, with neurological improvement, after the established therapy. CT scan revealed a discrete hypodense area in the white matter of the occipital lobe and anteroposterior groove asymmetry, compatible with PRES. DISCUSSION: PRES is due to vasogenic cerebral edema of acute or subacute installation. Symptoms include headache and altered consciousness, stupor, coma, neurological deficits, seizures and cortical blindness. Nephropathies are the main cause of PRES in pediatrics. Magnetic resonance imaging with diffusion of molecules is the gold standard for diagnosis. The initial treatment objectives are the reduction of blood pressure, antiepileptic therapy, correction of hydroelectrolytic and acid-base disorders and management of intracranial hypertension. CONCLUSION: PRES is associated with acute hypertension. Early diagnosis and proper management may determine a better prognosis and minimize the severity of the clinical course.