Clinical characteristics and prognosis of posterior reversible encephalopathy syndrome with spinal cord involvement / 中华神经科杂志

Objective:To investigate the clinical manifestations, imaging features and prognosis of posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI).Methods:The clinical data of 1 patient with PRES-SCI admitted to the Department of Neurology of the First Affiliated Hospital of Zhengzhou University in November 2021 were analyzed, and the data of 38 patients with PRES-SCI reported in domestic and foreign databases and this patient were collected for pooled analysis.Results:The main clinical manifestations of 39 PRES-SCI patients (including this patient) included headache (79.5%, 31/39), visual disturbance (79.5%, 31/39), vomiting (46.2%, 18/39), disturbance of consciousness (38.5%, 15/39), limb weakness (28.2%, 11/39) and seizure (23.1%, 9/39). There were up to 97.4% (38/39) of patients who had significantly elevated blood pressure. The imaging feature was long-segment spinal cord lesion involving central gray matter. Approximately 89.7% (35/39) of the spinal cord lesions originated from the junction of the medulla oblongata and the cervical spinal cord. The median number of abnormal spinal cord segments corresponding to the vertebral body was 9 (the shortest was 4 and the longest was the entire spinal cord). Thirty-eight patients had brain lesions, and the most frequently involved sites were medulla oblongata (82.1%, 32/39), occipital lobe (46.2%, 18/39), pons (43.6%, 17/39), parietal lobe (41.0%, 16/39), and cerebellum (38.5%, 15/39). Among 38 patients completing the follow-up, 31 patients (81.6%) were clinically recovered.Conclusions:Posterior reversible encephalopathy syndrome could involve the spinal cord. For patients with long-segment spinal cord lesions and significantly increased blood pressure or other risk factors, PRES-SCI should be considered. Timely identification and treatment could make most patients recovery.

Posterior reversible encephalopathy syndrome in the pediatric population: a pictorial essay / Síndrome de encefalopatia posterior reversível na população pediátrica: uma revisão de imagens

Abstract Posterior reversible encephalopathy syndrome (PRES) is a rare disease attributed to an increase in blood pressure that exceeds the autoregulatory capabilities of the cerebral vasculature, resulting in brain edema. Although PRES primarily affects adults, the pediatric population is also at risk. Radiologists must be aware of that risk because the imaging features on brain MRI are often atypical, especially in pediatric patients. Over a 6-year period, nine pediatric patients were diagnosed with PRES at our institution. Here, those patients are evaluated retrospectively regarding demographic characteristics, clinical profiles, imaging aspects, and outcomes. In this pictorial essay, we review the typical and atypical imaging findings of PRES in pediatric patients, demonstrating that it should be considered in patients with a clinical profile suggestive of the diagnosis, given that prompt, effective treatment is important for full recovery, thus avoiding major morbidity and mortality in such patients.

Resumo A síndrome de encefalopatia posterior reversível (PRES) é uma doença rara relacionada com uma falha no mecanismo autorregulatório vascular, resultando em edema cerebral. Apesar de primariamente descrita em adultos, a população pediátrica encontra-se em igual risco de desenvolver esta condição e o radiologista deve estar atento porque frequentemente a ressonância magnética apresenta características de imagem atípicas. Em um período de seis anos, nove pacientes pediátricos com o diagnóstico de PRES foram avaliados, retrospectivamente, no que diz respeito a sua demografia, antecedentes médicos, características de imagem e prognóstico. Neste ensaio analisamos características de imagem típicas e atípicas de pacientes pediátricos com o diagnóstico de PRES, mostrando que este diagnóstico deve ser considerado quando está presente um quadro clínico adequado, uma vez que o tratamento rápido e eficaz é importante para a recuperação completa, evitando morbimortalidade maior neste grupo etário.

Magnetic resonance imaging changes and clinical features of reversible posterior leukoencephalopathy syndrome / 中南大学学报(医学版)

OBJECTIVES@#Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare clinical imaging syndrome. The causes of RPLS are complex and diverse, the pathogenesis is not yet clear. The onset is urgent and the onset age span is large, ranging from children to the elderly. The clinical symptoms of RPLS have no significant specificity, which can be manifested as headache, blurred vision, disturbance of consciousness or seizures. Clinicians have little knowledge on the disease, which may lead to misdiagnosis or missed diagnosis. This study aims to analyze and summarize the MRI changes and clinical characteristics regarding RPLS patients, so as to provide basis for rapid diagnosis and timely intervention for this disease.@*METHODS@#The clinical data and complete imaging data of 77 patients with RPLS diagnosed in Xiangya Hospital of Central South University from January 2012 to March 2021 were retrospectively collected. The main image data include T1 weighted imaging (T1WI), T2 weighted imaging (T2WI), T2 liquid attenuation inversion recovery (T2-FLAIR), diffusion weighted imaging (DWI) (b value=1 000×10-6 mm2/s), and apparent diffusion coefficient (ADC). The case group included 63 patients who underwent DWI examination, and 71 normal controls matched in age and sex. The characteristics of patients' magnetic resonance signals and the ADC value of 19 regions of interest (ROI) were analyzed. The differences in bilateral ADC value in the case group, the difference of ADC value between the case group and the normal control group, and the difference of ADC value in the case group before and after treatment were compared.@*RESULTS@#Compared with the normal control group, the ADC value of the right frontal lobe, bilateral parietal lobe, bilateral thalamus, bilateral head of caudate nucleus, left lenticular nucleus, right internal capsule, bilateral temporal lobe and pons in the case group were significantly higher (all P<0.01). There was no significant difference in ADC value of bilateral sides of the case group and before and after treatment in the case group (all P>0.01). The lesions of RPLS were widely distributed and multiple, usually high signal in the posterior parieto temporo occipital lobe or pons of the brain, and involved the cortex and subcortical white matter. Most of them were bilateral, but not completely symmetrical.@*CONCLUSIONS@#The imaging manifestations of RPLS and the occurrence and development of clinical symptoms are basically synchronous. The imaging manifestations are specific. Magnetic resonance imaging can show the range of involvement of RPLS. ADC value can provide information on the severity of the disease and predict the prognosis. There are few reversible diseases. It is very important to fully understand and timely diagnose the disease.

Leucoencefalopatía posterior reversible en pacientes trasplantados. Presentación clínica, radiológica y evolución / Posterior reversible encephalopathy syndrome in transplanted patients. Clinical features and imaging follow up

Resumen El síndrome de leucoencefalopatía posterior reversible (PRES) es un desorden neurológico agudo caracterizado por cefalea, alteración de la conciencia, convulsiones y alteraciones visuales, con imágenes de edema vasogénico reversible en regiones cerebrales posteriores. Nos propusimos describir una serie de casos de pacientes trasplantados que desarrollaron PRES, caracterizando su presentación, evolución clínica, imágenes y terapéutica. Se analizaron historias clínicas informatizadas desde enero 2009 hasta enero 2019. Se recabaron datos demográficos, antecedentes clínicos, motivos y días de internación, tiempos desde el trasplante a la presentación clínica y diagnóstico. Se evaluó la mejoría/resolución en estudios por imágenes y la supervivencia anual. Se identificaron 27 pacientes con PRES; 22 trasplantados de órgano sólido de 1647 totales (1.3%) y 5 de médula ósea de 617 totales (0.8%). La media de edad fue de 38.2 años (DS 19.5), 62.9% de sexo femenino, 59.2% (16) antes del año del trasplante. Las comorbilidades más frecuentes enfermedad renal (14; 51%) e hipertensión arterial (11; 40%). Se realizó tomografía axial computarizada (TAC) a 23 pacientes (85.1%), siendo patológica en 11 (47.8%), y resonancia magnética nuclear (RMN) a 25 (92.6%), mostrando patrón característico en 17 (62.9%), con mejoría/resolución antes del año en 20 (74%). El tratamiento fue sintomático, modificando la inmunosupresión. Se registraron 5 óbitos durante la internación y otros 3 antes del año, con una supervivencia anual del 70.3% (19). La población de trasplantados, en crecimiento en nuestro medio, es particularmente susceptible al PRES. Tanto su presentación en estudios por imágenes, como su comorbilidad, difieren de otras poblaciones.

Abstract Posterior reversible encephalopathy syndrome (PRES) is an acute neurological disorder characterized by headache, encephalopathy, seizures and visual disturbances, with reversible vasogenic edema in posterior brain areas. The aim of this research was to describe a case series of transplanted patients who developed PRES, characterize their presentation, treatment, clinical and imaging evolution. Electronic medi cal records were analyzed from January 2009 to January 2019. Demographic data, clinical backgrounds, causes of admission, hospital length of stay and time from transplantation to PRES were collected. Image improvement/ resolution and annual survival were assessed. We identified 27 patients with PRES; 22 of 1647 total solid-organ transplant (1.3%) and 5 of 617 total bone marrow transplant (0.8%). The mean age at presentation was 38.2 years (SD 19.5), 62.9% female, 59.2% (16) before the year of transplantation. The most common comorbidities were kidney disease (14; 51%) and high blood pressure (11; 40%). Computed axial tomography (CT) was per formed in 23 patients (85.1%), with pathological findings in 11 (47.8%). Magnetic resonance imaging (MRI) of 25 (92.6%), showed a characteristic pattern in 17 (62.9%) with improvement/resolution before the year in 20 (74%). Treatment was symptomatic, modifying immunosuppression. Five deaths were recorded during hospital stay and another 3 before the year of admission, with an annual survival of 70.3% (19 patients). Organ transplant trend is growing in our region. These patients are particularly susceptible to PRES, with a different imaging presentation and comorbidities from other populations.

Síndrome de encefalopatía posterior reversible en paciente con diálisis peritoneal. Reporte de caso / Reversible posterior encephalopathy syndrome in a patient with peritoneal dialysis. Case report

Resumen Se presenta el caso de una paciente de 66 años con antecedente de enfermedad renal crónica desde hace 8 años y en terapia con diálisis peritoneal, quien acudió al servicio de urgencias por presentar deterioro del estado de conciencia asociado a convulsiones y emergencia hipertensiva. La paciente fue diagnosticada con síndrome de encefalopatía posterior reversible y luego de instaurado el tratamiento indicado la sintomatología cedió; después de un mes de evolución se observó resolución de las lesiones halladas en la resonancia magnética. Esta patología se asocia principalmente a hipertensión arterial severa de rápida instauración en correlación con diversas entidades clínicas como lo fue la hipertensión arterial en el presente caso.

Abstract We present the case of a 66-year-old patient with a history of chronic kidney disease for 8 years, on peritoneal dialysis therapy, who went to the emergency service for presenting deterioration in the state of consciousness associated with seizures, and hypertensive emergency, after treatment the symptoms subside and resolution of the lesions found on the MRI after one month of evolution, classified as posterior reversible encephalopathy syndrome. Considering this diagnosis in patients with chronic kidney injury associated with arterial hypertension.

Encefalopatía posterior reversible en niño trasplantado de corazón / Posterior reversible encephalopathy in a child transplanted of heart

Resumen La encefalopatía posterior reversible es un síndrome clínico-radiológico caracterizado por hipertensión arterial, cefalea, alteraciones visuales, convulsiones y delirium. Radiológicamente, se observa edema vasogénico en regiones subcorticales con predilección por los lóbulos posteriores del cerebro. Ha sido asociada con trasplante de órgano sólido, hipertensión arterial y uso de medicamentos inmunosupresores, como los anticalcineurínicos. Se reporta el caso de un niño de seis años quien inició con cefalea, cifras tensionales elevadas, irritabilidad, agresividad y por momentos agitado cinco días después de recibir un trasplante ortotópico de corazón y un día después de inicio de tacrolimus. El tratamiento incluyó manejo sintomático con antihipertensivos y anticonvulsivantes, además del aumento progresivo del inmunosupresor hasta niveles terapéuticos en sangre para disminuir el riesgo de rechazo del injerto. El síndrome de encefalopatía posterior reversible debe ser considerado en pacientes trasplantados de corazón que reciben inhibidores de la calcineurina y presentan síntomas neurológicos.

Abstract Posterior reversible encephalopathy is a clinic-radiological syndrome characterized by arterial hypertension, headache, visual disturbances, seizures and delirium. Radiologically, vasogenic edema is observed in subcortical regions with predilection for the posterior lobes of the brain. It has been associated with solid organ transplantation, arterial hypertension and the use of immunosuppressive drugs such as anticalcineurin. We report the case of a six-year-old boy who started with headache, elevated blood pressure, irritability, aggressiveness and at times agitated five days after receiving an orthotopic heart transplant and one day after starting tacrolimus. His treatment included symptomatic management with antihypertensives and anticonvulsants, in addition to a progressive increase in immunosuppressant to therapeutic blood levels to reduce the risk of graft rejection. Reversible posterior encephalopathy syndrome should be considered in heart transplant recipients receiving calcineurin inhibitors who present neurological symptoms.

Anti-contactin associated protein-like 2 antibody encephalitis complicated with reversible posterior encephalopathy syndrome: a case report / 中华神经科杂志

Anti-contactin associated protein-like 2 (CASPR2) antibody encephalitis is a rare autoimmune encephalitis with variable clinical symptoms and atypical imaging manifestations. The prognosis of the patients with severe disease is poor. Reversible posterior leukoencephalopathy syndrome is rarely reported in autoimmune encephalitis. The clinical data, diagnosis and treatment of a patient with anti-CASPR2 antibody encephalitis complicated with reversible posterior encephalopathy syndrome were reported, in order to improve the understanding of clinicians on the rare disease complicated with atypical imaging manifestations.

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.

Síndrome da encefalopatia posterior reversível: uma patologia pouco descrita no idoso / Posterior reversible encephalopathy syndrome: a disease rarely described in older adults

A síndrome da encefalopatia posterior reversível, conhecida como PRES, é rara e pouco descrita em idosos e é caracterizada pelo início subagudo de um conjunto de sinais clínicos e radiológicos e uma variedade de sintomas neurológicos, como cefaleia, crises convulsivas e transtornos da cognição. Na grande maioria dos pacientes, a apresentação clínica inclui pressão arterial elevada e emergência hipertensiva. A ressonância magnética (RM) é o exame padrão-ouro para o diagnóstico imaginológico dessa entidade. O quadro clínico e as alterações de imagens podem se tornar reversíveis caso seja detectada precocemente e tratada a causa base da síndrome. Os autores apresentam o caso clínico de uma idosa de 87 anos, internada para tratamento de pneumonia comunitária retornando ao setor de emergência 24 horas após a alta hospitalar apresentando sintomas neurológicos visuais complexos. Ao exame de RM, observaram-se lesões de hipodensidades occipitais bilaterais, sugestivas de edema vasogênico, compatível com PRES. Após o rigoroso controle da pressão arterial, verificou-se a reversibilidade total dessas lesões cerebrais.

Posterior reversible encephalopathy syndrome (PRES) is a disease rarely described in older adults. It is characterized by subacute onset of a set of clinical and radiological signs and a variety of neurological symptoms, such as headaches, seizures, and cognitive disorders. In the vast majority of patients, clinical presentation includes high blood pressure and hypertensive emergency. Magnetic resonance imaging (MRI) is the gold standard for diagnosing this condition using imaging findings. When the underlying cause is promptly recognized and treated, symptoms and imaging abnormalities may be completely reversible. The authors report the clinical case of an 87-year-old woman first admitted for treatment of community-acquired pneumonia. She returned to the emergency department 24 hours after discharge presenting with complex visual and neurological symptoms. An MRI scan showed lesions of bilateral occipital hypodensities, suggestive of vasogenic edema and compatible with PRES. Complete regression of brain lesions was observed after tight control of hypertension.

Encefalopatía posterior reversible en el curso de una emergencia hipertensiva, serie de cuatro niños hospitalizados en el Centro Hospitalario Pereira Rossell / Posterior reversible encephalopathy during a hypertensive emergency in 4 children hospitalized at the Pereira Rossell Pediatric Hospital / Encefalopatia reversível durante uma emergência hipertensiva em 4 crianças hospitalizadas no Centro Hospitalar Pereira Rossell

Introducción: la encefalopatía posterior reversible (PRES) es un complejo sindromático caracterizado por cefaleas, crisis epilépticas, trastornos visuales y alteración del sensorio asociado a cambios en la sustancia blanca de lóbulos occipitales y parietales en la resonancia nuclear magnética. Se relaciona con diversas causas que provocan edema cerebral, entre las que se destaca la hipertensión arterial (HTA) y los tratamientos inmunosupresores. Descripción de casos: se incluyeron cuatro niños hospitalizados entre el 1 de enero de 2005 y el 31 de diciembre de 2015 en el Centro Hospitalario Pereira Rossell. La mediana de edad fue 11,5 años. La manifestación clínica inicial en tres de los cuatro pacientes fue disnea y edemas generalizados. Todos presentaron hipertensión endocraneana y convulsiones generalizadas. La tomografía computada fue la primera imagen solicitada en todos los pacientes, evidenciado lesiones hipodensas córtico-subcorticales occipitales, parietales y frontales. Se realizó resonancia magnética en tres casos con hallazgos característicos. Todos presentaron síndrome nefrítico con cifras de tensión arterial mayores al P99, insuficiencia renal reversible, disminución de C3 y aumento del antiestreptolisina O (AELO). El control de HTA requirió politerapia en unidad de cuidado crítico. Ninguno presentó secuelas neurológicas. Conclusiones: la PRES es una asociación o manifestación poco habitual de la emergencia hipertensiva. Es necesaria la sospecha e identificación por parte del pediatra con el fin de realizar un abordaje terapéutico adecuado y precoz.

Introduction: posterior reversible encephalopathy (PRES) is a complex syndrome characterized by headaches, epileptic seizures, visual disturbances and sensorial alterations associated to changes of white matter in parieto-occipital regions shown in magnetic nuclear resonance. It is linked to various causes that provoke cerebral edema including arterial hypertension (AH) and immunosuppressive treatments. Clinical cases: 4 children admitted between January 1st, 2005 and December 31st, 2015 at the Pereira Rossell Pediatric Hospital. Median age: 11.5 years. The initial clinical manifestation in 3 of 4 patients was dyspnea and generalized edema. All of them presented endocranial hypertension and generalized convulsions. CT was the first requested image to all patients, and it was clear that all of them had experienced occipital cortical-subcortical, parietal and frontal hypodense lesions. In three of the cases we performed a Magnetic Resonance that showed typical findings. All of them presented nephritic syndrome with arterial hypertension over P99, reversible renal failure, a decrease of C3 and an increase of Antistreptolysin O (AELO). HTA control required polytherapy at the critical care unit. None of them presented neurological sequelae. Conclusions: PRES is an association or unusual manifestation of hypertensive emergency. Early detection by pediatricians seems essential to provide an appropriate therapeutic approach.

Introdução: a encefalopatia posterior reversível (PRES) é uma síndrome complexa caracterizada por dores de cabeça, convulsões epilépticas, distúrbios visuais e alterações sensoriais associadas à mudança da substância branca nas regiões parieto-occipitais observadas na ressonância magnética nuclear. Está ligada a várias causas que provocam o edema cerebral, incluindo hipertensão arterial (HA) e tratamentos imunossupressores. Casos clínicos: 4 crianças internadas entre 1º de janeiro de 2005 e 31 de dezembro de 2015 no Hospital Pediátrico Pereira Rossell. Idade mediana: 11,5 anos. A manifestação clínica inicial em 3 dos 4 pacientes foi dispneia e edema generalizado. Todos apresentaram hipertensão endocraniana e convulsões generalizadas. A TC foi a primeira imagem solicitada e todos os pacientes mostraram lesões occipitais corticais-subcorticais, parietais e hipodensas frontais evidentes. Em três dos casos, a Ressonância Magnética mostrou achados típicos. Todos apresentaram síndrome nefrítica com hipertensão arterial acima de P99, insuficiência renal reversível, diminuição de C3 e aumento de antiestreptolisina O (AELO). O controle de HTA requereu politerapia na unidade de terapia intensiva. Nenhum dos pacientes apresentou sequelas neurológicas. Conclusões: A PRES é uma associação ou manifestação de emergência hipertensiva pouco comum. A detecção precoce do pediatra é essencial para fornecer uma abordagem terapêutica adequada.

Using the Reduced Uterine Perfusion Pressure model of preeclampsia to study the blood brain barrier permeability / Uso do modelo de Redução da Pressão de Perfusão Uterina de pré-eclâmpsia para estudar a permeabilidade da barreira hematoencefálica

AIMS: To use the Reduced Uterine Perfusion Pressure (RUPP) model for preeclampsia to describe and evaluate the blood brain barrier permeability in pregnant rats. METHODS: Forty-one pregnant Wistar rats were divided into different intervention groups between 13 to 15 days of gestation: PregnantControl (PC; n=12), Reduced Uterine Perfusion Pressure (RUPP; n=15), Invasive Blood Pressure-Control (IBP; n=7) and Reduced Uterine Perfusion Pressure and Invasive Blood Pressure (RUPP-IBP; n=7). The 14 rats of groups IBP and RUPP-IBP had their mean arterial pressure measured at day 21. All animals were then sacrificed, administered Evans Blue dye through the tail vein and perfused with paraformaldehyde 4%. Brains were removed and evaluated by a blinded pathologist. Results are presented as means and standard errors. Comparisons between the groups were performed using Student's t-test for continuous variables and Fisher's exact test for categorical variables. Statistical significance was set as a p value less than 0.05. RESULTS: Mean arterial pressure averaged 85.4±2.2 mmHg in the IPB group and 102.5±8.3 mmHg in the RUPP-IPB group (p=0.002). Among all the RUPP rats (RUPP and RUPP-IBP groups), 82% had a positive staining with Evans Blue dye for at least one of the brain hemispheres, while none of the pregnant control rats (PC and IBP groups) had brain staining (p<0.001). CONCLUSIONS: In this study, altered permeability of the blood brain barrier was successfully reproduced in pregnant rats exposed to the RUPP protocol. Therefore, we concluded that the RUPP model is a valid surrogate to study blood brain barrier abnormalities.

OBJETIVOS: Usar o modelo de Redução da Pressão de Perfusão Uterina / Reduced Uterine Perfusion Pressure (RUPP) de pré-eclâmpsia para descrever e avaliar a permeabilidade da barreira hematoencefálica. MÉTODOS: Quarenta e uma ratas Wistar prenhes foram estratificadas em diferentes grupos de intervenção entre 13 a 15 dias de gestação: grupo controle (PC; n=12), grupo modelo de redução da pressão de perfusão uterina (RUPP; n=15), grupo monitorização invasiva da pressão arterial (IBP; n=7) e grupo redução da pressão de perfusão uterina e monitorização invasiva da pressão arterial (RUPP-IBP; n=7). As 14 ratas dos grupos IBP e RUPP-IBP tiveram sua pressão arterial média aferida no dia 21. Logo após todos os animais foram sacrificados e foi administrado o corante Azul de Evans pela veia da cauda, seguido de formaldeído 4%. Os cérebros foram removidos e avaliados por um patologista cegado para os grupos. Os resultados são apresentados em médias e erros padrão. As comparações entre os grupos foram realizadas utilizando o teste t de Student para variáveis contínuas e o teste exato de Fisher para variáveis categóricas. A significância estatística foi definida como um valor de p inferior a 0,05. RESULTADOS: As médias e desvios padrões da pressão arterial média foram 85,4±2,2 mmHg no grupo IPB e 102,5±8,3 mmHg no grupo RUPP-IPB (p=0,002). Entre todas as ratas RUPP (grupos RUPP e RUPP-IBP), 82% tiveram marcação positiva pelo corante em pelo menos um dos hemisférios cerebrais, enquanto nenhuma das ratas controle (grupos PC e IBP) teve marcação cerebral positiva (p<0,001). CONCLUSÕES: Neste estudo, a permeabilidade alterada da barreira hematoencefálica foi reproduzida com sucesso em ratas prenhes expostas ao protocolo RUPP. Portanto, concluímos que o modelo RUPP é um substituto válido para estudar anormalidades da barreira hematoencefálica.

Posterior reversible leukoencephalopathy syndrome (PRES) after kidney transplantation: a case report / Síndrome da leucoencefalopatia posterior reversível (PRES) após transplante renal: um relato de caso

ABSTRACT Introduction: Posterior reversible leukoencephalopathy syndrome (PRES) was first described by Hinchey in 1996. The syndrome is characterized by altered level of consciousness, headache, visual changes, and seizures associated with a vasogenic edema of the white matter that occurs predominantly in the occipital and parietal lobes. Imaging tests such as computed tomography (CT) and especially magnetic resonance imaging (MRI) support the diagnosis. Case Report: We report a case of a 48-year-old female patient who underwent a deceased donor kidney transplant and received tacrolimus as a part of the immunosuppressive regimen. Five weeks after transplantation she was admitted to the emergency due to sudden onset of confusion, disorientation, visual disturbances, and major headache. PRES was suspected and the diagnosis confirmed by brain MRI. Tacrolimus was withdrawn and rapid improvement of the neurological signs occurred leading to the conclusion that this drug triggered the syndrome. Conclusion: PRES is an unusual complication after organ transplantation and should be considered in the appropriate clinical setting. Physicians must be aware of this condition in order to provide early detection and appropriate treatment since delay in removing the cause may lead to permanent sequelae.

RESUMO Introdução: A síndrome da leucoencefalopatia posterior reversível (PRES) foi descrita pela primeira vez por Hinchey, em 1996. A síndrome é caracterizada por nível alterado de consciência, cefaleia, alterações visuais e convulsões associadas a edema vasogênico da substância branca, oriundo predominantemente nos lobos occipital e parietal. Exames de imagem como tomografia computadorizada (TC) e em particular a ressonância magnética (MRI) corroboram o diagnóstico. Relato de caso: relatamos o caso de uma paciente do sexo feminino de 48 anos submetida a transplante renal com doador falecido em regime imunossupressor com tacrolimus. Cinco semanas após o transplante, a paciente deu entrada no pronto-socorro por conta de aparecimento súbito de confusão, desorientação, distúrbios visuais e cefaleia intensa. As suspeitas diagnósticas recaíram sobre PRES, e a confirmação foi feita por imagens de ressonância magnética do cérebro. Tacrolimus foi suspenso e os sinais neurológicos da paciente melhoraram rapidamente, indicando que o medicamento desencadeou a síndrome. Conclusão: PRES é uma complicação incomum após o transplante de órgãos e deve ser considerada em ambiente clínico adequado. O corpo clínico deve estar ciente dessa patologia, de modo a detectá- -la precocemente e dar início ao tratamento, uma vez que atrasos na eliminação da causa podem levar a sequelas permanentes.

Unilateral Posterior Reversible Encephalopathy Syndrome Secondary to Renal Artery Obstruction: A Case Report / 대한소아신경학회지

Posterior reversible encephalopathy syndrome (PRES, or posterior leukoencephalopahty syndrome) is a neurological condition caused by reversible cortical/subcortical vasogenic brain edema secondary to hypertension, cytotoxic drugs, immunosuppressants, autoimmune diseases, renal disease, eclampsia or pre-eclampsia. It is characterized by acute neurological symptoms such as headache, seizures, visual disturbances, and impaired levels of consciousness. Brain imaging usually reveals bilateral, cortical/subcortical vasogenic edema. Completely unilateral PRES constituted only 2.6% of the cases in a previous study. Here we report the case of a pediatric patient with completely unilateral PRES. A 13-year-old boy was admitted with acute gastroenteritis. On the fourth day of hospitalization, he started to complain of headache and vomiting. He then developed generalized tonic-clonic seizure 3 times. His blood pressure was 180/121 mmHg during the first seizure, 188/112 mmHg during the second seizure and 152/92 mmHg during the third seizure. T2-weighted imaging with fluid attenuation by inversion recovery (T2 FLAIR) demonstrated high-signal intensity in the cortical gyri of the left frontal, parietal, and occipital lobes. Follow-up magnetic resonance imaging (MRI) was performed 2 weeks after the seizure onset, which indicated a significant improvement in the patient's condition. Abdominal pelvic computed tomography (CT) and renal CT angiography showed abnormal narrowing of the left renal artery. In summary, we present a case report of unilateral PRES secondary to renovascular hypertension due to left renal arterial obstruction.

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, eclampsia y síndrome de hellp / Reversible posterior encephalopathy syndrome, eclampsia and hellp syndrome

Resumen El síndrome de encefalopatía posterior reversible (PRES) es una entidad caracterizada por alteraciones clínicas y radiológicas debidas a un desbalance en la regulación de los vasos sanguíneos cerebrales. En la paciente obstétrica, el síndrome es más frecuente en la eclampsia y el síndrome de hemólisis, enzimas hepáticas elevadas y trombocitopenia (HELLP). Objetivo Caracterizar el PRES a nivel fisiopatológico, clínico, diagnóstico y terapéutico en la eclampsia y el síndrome de HELLP. Metodología Búsqueda bibliográfica con los términos MESH "Posterior Leukoencephalopathy Syndrome", "Pregnancy", "HELLP syndrome", "Eclampsia". Resultados La eclampsia es una de las condiciones más asociadas al PRES, las pacientes son primigestas, el curso clínico y los hallazgos imagenológicos son menos graves, el tratamiento debe enfocarse en la causa desencadenante, en este contexto debe optarse por desembarazar, administrar fármacos para manejar la hipertensión y las convulsiones. La literatura de PRES y HELLP se limita a series y reportes de caso. Las características propias de la fisiopatogenia del síndrome de HELLP pueden exagerar la respuesta vascular e incrementar el riesgo de sangrado lo que podría predisponer a un curso clínico más agresivo. El pronóstico del PRES en la paciente obstétrica es bueno, no se ha reportado riesgo de recurrencias en esta población. Conclusión Se necesitan estudios con un mayor seguimiento y número de pacientes que permitan aclarar las discordancias existentes. El conocimiento y manejo precoz del PRES contribuiría a la reducción de la morbimortalidad materna y las secuelas neurológicas a largo plazo.

ABSTRACT Posterior reversible encephalopathy syndrome (PRES) is an entity characterized by clinical and radiological manifestations due to an imbalance in cerebral blood vessels. In the obstetric patient, this syndrome is more frequent in eclampsia and hemolysis, elevated liver enzymes and thrombocytopenia (HELLP) syndrome. Aim To characterize PRES at physiopathological, clinical, diagnostic and therapeutic context in eclampsia and HELLP syndrome. Methodology Bibliographic search with the MESH terms "Posterior Leukoencephalopathy Syndrome", "Pregnancy", "HELLP Syndrome", "Eclampsia". Results Eclampsia is one of the most associated conditions with PRES, patients are primigravida, clinical course and imaging findings are less severe. The treatment should focus on the triggering cause, so emergent delivery and drugs to manage hypertension and seizures must be chosen. The literature on PRES and HELLP is limited to series and case reports. The characteristics of the pathophysiology of HELLP syndrome may exaggerate vascular response and increase the risk of bleeding, which may predispose to a more aggressive clinical course. Prognosis of PRES in the obstetric patient is good, there is not risk of recurrences reported in this population. Conclusion Studies with a greater follow-up and number of patients would clarify the existing discordances. Knowledge and early management of PRES would contribute to the reduction of maternal morbidity and mortality and long-term neurological sequelae.

Síndrome de encefalopatía posterior reversible: reporte de un caso fatal y análisis de factores predictores de mal pronóstico / Posterior reversible encephalopathy syndrome: Report of a fatal case and analysis of predictive factors of a poor prognosis

El síndrome de encefalopatía posterior reversible es una condición que responde a múltiples causas y presenta características clínicas o radiológicas distintivas; los intensivistas y los médicos de urgencias deben conocerlo con el fin de hacer el diagnóstico y ordenar el tratamiento oportuno. Se presenta un caso fatal de síndrome de encefalopatía posterior reversible, en el cual se determinaron los factores de riesgo relacionados con el resultado final. Un hombre de 60 años sin antecedentes médicos ingresó por urgencias con depresión de la conciencia, convulsiones y tensión arterial elevada. Las imágenes de la tomografía revelaron un hematoma cerebeloso posterior, y las de resonancia magnética mostraron zonas isquémicas, edema vasogénico que se extendía desde los tálamos hacia el tallo cerebral, los pedúnculos cerebelosos medios y la sustancia blanca profunda de los hemisferios cerebelosos, así como zonas de transformación hemorrágica. A pesar del tratamiento médico y quirúrgico recibido, el paciente falleció. Se determinaron los factores de riesgo que se han descrito como causa de muerte en este síndrome. Este caso demuestra que dicho síndrome puede ocurrir sin que se hayan detectado factores de riesgo desencadenantes y pone en evidencia la necesidad de su reconocimiento temprano para establecer una intervención adecuada y evitar daños o un desenlace fatal. Además, abre el camino a nuevos estudios sobre la propensión a desarrollarlo y las medidas preventivas que pueden adoptarse.

Posterior reversible encephalopathy syndrome is an illness with multiple causes and distinctive clinicalradiological characteristics that should be known by intensivists and emergency room physicians for a timely diagnosis and treatment. A fatal case of posterior reversible encephalopathy syndrome is presented, and the risk factors related to the outcome are identified. A 60-year-old man without a relevant medical history arrived at the emergency room presenting with depressed consciousness, seizures, and high blood pressure. Tomographic images revealed a posterior cerebellar hematoma. Resonance images showed ischemic zones, vasogenic edema from the thalamus to the brain stem, middle cerebellar peduncles, deep white matter of the cerebral hemispheres, and zones of hemorrhagic transformation. Despite medical-surgical management, the patient died. The risk factors described as the cause of the fatal outcome were identified. This case demonstrates that posterior reversible encephalopathy syndrome can occur without triggering risk factors and highlights the need for early recognition to establish an appropriate intervention to avoid injury or a fatal outcome. Cases of posterior reversible encephalopathy syndrome provide opportunities to investigate the susceptibility for the development of this condition and to establish appropriate preventive measures.

Transient visual loss after lung surgery in a healthy patient: posterior reversible encephalopathy syndrome: A case report

Postoperative visual loss is a rare complication of general anesthesia in patients undergoing lung surgery. If the visual complication is permanent, it can greatly affect the patient's quality of life. Posterior reversible encephalopathy syndrome (PRES) leads to visual disturbances and may be associated with hypertension, renal disease, eclampsia, and chemotherapy. Although PRES is usually reversible, delayed diagnosis and treatment can result in permanent damage. We herein report a case of PRES in a patient with no medical history. The patient's symptoms included somnolence, visual loss, and headache. He was treated with conservative therapy, and his vision abruptly recovered three days after surgery. He was discharged from the hospital without neurologic complications 13 days after surgery.

Risk factors analysis of reversible posterior leukoencephalopathy syndrome in pre-eclampsia or eclampsia gravida / 中华妇产科杂志

Objective To investigate the risk factors of reversible posterior leukoencephalopathy syndrome (RPLS) in pre-eclampsia or eclampsia gravida. Methods This study was conducted in the Third Affiliated Hospital of Guangzhou Medical University between January 2013 and March 2016. A total of 100 patients who had no severe neurological diseases and were diagnosed pre-eclampsia or eclampsia, and underwent brain MRI were collected retrospectively. They were divided into 2 groups according to the MRI results, the RPLS group (n=49) and the non-RPLS group (n=51). The medical history, clinical symptoms and the results of laboratory examination were analyzed by the logistic regression, in order to explore the risk factors.Results In single factor analysis, HELLP syndrome, pregnancy associated with other diseases, poor prenatal care, grade 3 hypertension, elevated systolic blood pressure or diastolic blood pressure, elevated WBC, aspartate transaminase (AST), alanine aminotransferase (ALT), uric acid (UA) and lactate dehydrogenase (LDH), decreased platelet (PLT), headache, visual changes, seizures and conscious disturbance were more frequent in the RPLS group than those in the non-RPLS group (all P<0.05). According to the multivariate logistic regression analysis, the elevated WBC (OR=1.291, 95%CI:1.058-1.575, P=0.012), UA (OR=1.008,95%CI:1.001-1.016,P=0.032) and headache (OR=18.260, 95%CI:3.562- 93.607, P=0.000) were the independent risk factors.Conclusions Maternal history, clinical symptoms and some laboratory examinations might help in the early diagnosis of RPLS in pre-eclampsia or eclampsia gravida. Headache, the elevation of WBC and UA were the most significant factors.

Clinical analysis of children having primary nephrotic syndrome complicated with posterior reversible encephalopathy syndrome on treatment of immunosuppressants / 中华肾脏病杂志

Objective To explore the relationship between posterior reversible encephalopathy syndrome (PRES) and the treatment of immunosuppressants such as cyclosporine A (CsA) and tacrolimus (FK506) in children with nephrotic syndrome.Methods The clinical data of nephrotic syndrome children with PRES caused by immunosuppressants who were hospitalized in Guangzhou First People's Hospital from June 2014 to May 2017 were collected.Their clinical characteristics,imaging features,treatments and prognosis were analyzed.Results A total of 23 children were enrolled,including 13 children with CsA and 10 children with FK506.In the concurrent of PRES 20 cases were in the activity stage of nephrotic syndrome,with large amounts of urinary protein,obvious edema,hypoalbuminemia and hyperlipidemia;while 3 cases were in the remission of nephrotic syndrome.The main clinical symptoms of PRES were hypertension,headache,epileptic attack,consciousness disorder,visual disorder and so on.Sixty-nine point six percent of children were using high dose immunosuppressive agents,and 78.3％ had high drug concentration.The cranial magnetic resonance imaging (MRI) results of 17 patients showed that they had T1 weighted (T1WI) hypointense,T2 weighted (T2WI) and fluid-attenuated inversion recovery (FLAIR) images hyperintense,as well as iso-and slight hypointense of diffusion-weighted image (DWI) in parietal-occipital regions or complicated with frontal lobes or basal nuclei region.Computer tomography (CT) examinations of 6 cases showed low-density focus of the occipital lobes.Children were relieved muscular spasm,debased intracranial hypertension,improved circulation,discontinued or reduced immunosuppressants at the onset of PRES.After these treatments,21 patients' symptoms and signs disappeared within one week;two patients suffered convulsions 2 times in one week,but recovered after one month.After three months 5 children had MRI and CT re-examination and it showed that their brain lesions disappeared.Conclusions PRES may be related to the dose and blood concentration of immunosuppressive agents.The immunosuppressants for nephrotic syndrome children should be increased gradually with low initiating doses.Physicians need to be precautious to prevent the occurrence of PRES once neurological symptoms occur.

Clinical significance of combined detection of peripheral miR-182 and IL-17 in the early diagnosis of patients with eclampsia complicated with cerebral infarction in ICU / 中华危重病急救医学

Objective To observe the effect of peripheral blood microRNA-182 (miR-182) combined with interleukin-17 (IL-17) in the early diagnosis of cerebral infarction (CI) in patients with eclampsia. Methods A prospective non-randomized controlled study was conducted. The patients with eclampsia admitted to intensive care unit (ICU) of Liaocheng People's Hospital from January 1st, 2013 to September 30th 2016 were enrolled. Cerebral imaging was conducted in 7 days after admission to make a definite diagnosis of the occurrence of CI, excluding patients with cerebral hemorrhage. Patients were divided into CI group and non-CI group. Twenty healthy women of childbearing age were selected as control group. Peripheral venous blood of all patients with eclampsia at 1 day after admission, the expression of miR-182 was detected by real-time fluorescence quantitative polymerase chain reaction (PCR), regulatory T cells (Treg) and T helper 17 cells (Th17) ratio was detected by flow cytometry, and the level of plasma IL-17 was detected by enzyme linked immunosorbent assay (ELISA). Pearson method was used to analyze the correlation between the indexes. The receiver operating characteristic curve (ROC) was used to analyze the diagnostic value of each index for CI in patients with eclampsia. Results In the 30 patients with eclampsia, there were 13 cases of CI, including 10 case of cerebral venous thrombosis (CVT) and 3 cases of arterial thrombus; 17 cases of non-CI, including 15 cases of reversible posterior leukoencephalopathy syndrome (RPLS) and 2 cases without obvious abnormalities. Compared with control group, the levels of miR-182, Th17% and IL-17 in non-CI group and CI group were significantly higher, and the Treg% was significantly lower. The levels of parameters mentioned above were further increased in CI group than those in non-CI group [miR-182 (2-ΔΔCt): 2.35±0.79 vs. 1.75±0.56, Th17%: (5.16±1.89)% vs. (3.93±1.92)%, IL-17 (ng/L):37.45±6.20 vs. 26.65±5.13, all P < 0.05]. Pearson correlation analysis showed that miR-182 was positively correlated with Th17% and IL-17 (r1 = 0.761, r2 = 0.842, both P < 0.01). ROC curves showed that when the cut-off value of miR-182 was 2.88, the diagnosis sensitivity of preeclampsia CI was 84.6%, the specificity was 82.4%, and area under the ROC curve (AUC) was 0.816 [95%CI confidence interval (95%CI) = 0.641-0.992]; when cut-off value of IL-17 was 34.44 ng/L, diagnosis of preeclampsia CI the sensitivity was 71.5%, the specificity was 85.3%, and AUC was 0.773 (95%CI = 0.602-0.945); when miR-182 was combined with IL-17, the diagnosis sensitivity was 92.3%, specificity was 83.6%, and AUC was 0.896 (95%CI = 0.759-1.032). Conclusions To some extent the expression of miR-182 and IL-17 in peripheral blood can predict the occurrence of CI in early stage. When the two are used together, the predictive value is better.