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.

Cortical blindness secondary to posterior reversible encephalopathy syndrome, recovered by successful blood pressure management / Cegueira cortical secundária à síndrome de encefalopatia reversível posterior recuperada pelo controle bem sucedido da pressão arterial

ABSTRACT We report a case of cortical blindness secondary to posterior leukoencephalopathy syndrome, which was successfully treated by blood pressure management. A 66-year-old white man presented at the emergency room complaining of severe frontal headache, disorientation, and progressive blurred vision. The initial physical examination disclosed a blood pressure of 200/176 mmHg. One hour later the patient exhibited mental confusion and bilateral blindness. Computed tomography and magnetic resonance imaging were performed, and a systemic workup was conducted, resulting in a diagnosis of posterior leukoencephalopathy syndrome. This syndrome is a rare manifestation of systemic hypertension that requires proper diagnosis and management to avoid irreversible brain damage. Early recognition of this condition and prompt control of the patient's blood pressure are essential because they may bring about a reversal of the syndrome, which may otherwise result in permanent brain damage.

RESUMO Relatamos um caso de cegueira cortical secundário à síndrome de leucoencefalopatia posterior recuperado pelo controle bem sucedido da pressão arterial. Um homem branco de 66 anos de idade compareceu à emergência com queixa de dor de cabeça frontal severa, desorientação e embaçamento progressivo da visão. O exame físico inicial revelou uma pressão arterial de 200/176 mmHg. Uma hora depois, o paciente apresentou confusão mental e cegueira bilateral. Após a tomografia e a ressonância nuclear magnética, o tratamento sistêmico foram realizados e o diagnóstico de síndrome da leucoencefalopatia posterior foi realizado. A síndrome de leucoencefalopatia posterior é uma manifestação rara de hipertensão sistêmica que requer um diagnóstico e gerenciamento adequados para evitar danos cerebrais irreversíveis. O reconhecimento precoce desta condição e o controle imediato da pressão arterial são essenciais porque podem levar à reversão da síndrome, o que, de outra forma, pode resultar em dano cerebral permanente.

Síndrome de encefalopatía posterior reversible con afectación del tronco cerebral y médula espinal y retinopatía hipertensiva grado IV / Posterior reversible encephalopathy syndrome with brain stem and spinal cord involvement and grade iv hypertensive retinopathy

El síndrome de leucoencefalopatía posterior reversible se presenta con síntomas de edema cerebral e imágenes hiperintensas en las secuencias T2 y FLAIR en la resonancia magnética (RM) de cerebro. Las lesiones típicamente comprometen la región parietooccipital, aunque también pueden tener localizaciones atípicas. Presentamos el caso de una mujer de 33 años que consultó por trastornos visuales asociados con dolor de cabeza intenso e hipertensión grave (220/140 mmHg). El examen del fondo de ojo reveló retinopatía hipertensiva grado IV y la RM mostró lesiones hiperintensas en T2 y FLAIR a nivel centropontino, bulbo y médula espinal, las cuales regresaron tras el control sostenido de la presión arterial. (AU)

The posterior reversible leukoencephalopathy syndrome presents with symptoms of cerebral edema. On magnetic resonance imaging (MRI) of the brain, hyperintensities on T2 -weighted and FLAIR sequences typically affect the parietal-occipital region; however it may also have atypical locations. We report the case of a 33 year old patient with visual disturbances associated with intense headache and severe hypertension (220/140 mmHg). The Fundus examination revealed grade IV hypertensive retinopathy, and MRI showed hyperintense lesions in T2 and FLAIR affecting the pontine center, medulla and spinal cord, which regressed after sustain blood pressure control. (AU)

Posterior reversible encephalopathy syndrome presenting with seizures.

Hyperperfusion syndrome, previously known as posterior reversible encephalopathy syndrome (PRES), is a clinicoradiological entity with characteristic features on neuro-imaging. It is believed to be caused by vasogenic oedema, predominantly in the posterior cerebral hemispheres. We report the case of an elderly man who presented with convulsions and was diagnosed to have PRES due to hypertension.

Late postpartum preeclampsia with posterior reversible encephalopathy syndrome.

Posterior reversible encephalopathy syndrome is a reversible syndrome characterized by headache, seizures, altered mentation, and loss of vision associated with white matter changes on imaging. We report here a 27 year-old lady three weeks postpartum, presenting with posterior reversible encephalopathy syndrome. She was treated successfully with antihypertensives and showed dramatic improvement. This condition is important to recognize and needs to be treated promptly to prevent morbidity and mortality in pregnancy and postpartum.

Leucoencefalopatía reversible posterior: cuatro casos clínicos / Reversible posterior leukoencephalopathy syndrome in patients with immunosupressive treatment: Report of four cases

Reversible posterior leukoencephalopathy syndrome (PLS) is characterized by headache, clouding of sensorium, visual disturbances and seizures. It is associated to hypertension, renal disease or immunosuppressive therapy. We report three males, aged 9, 12 and 16 years and one female, aged 5 years wih PLS associated to immunosuppressive therapy. All had seizures and three had headache and clouding of sensorium. One case was associated to an hypertensive emergency, one to liver failure and one to high tacrolimus levels. Magnetic resonance imaging showed lesions in the white matter in two patients and in the gray matter in the other two. The lapse between the start of immunosuppressive treatment and neurological symptoms ranged from 4 days to 6 months. All received antiepileptic drugs and immunosuppresive therapy was changed or decreased, with complete clinical recovery.