Síndrome da encefalopatia posterior reversível em paciente com COVID-19 submetida à oxigenação por membrana extracorpórea / Posterior reversible encephalopathy syndrome in a patient submitted to extracorporeal membrane oxygenation for COVID-19

RESUMO Uma mulher com 63 anos de idade compareceu ao pronto-socorro com história aguda de febre, prostração e dispneia. Recebeu diagnóstico de quadro grave da COVID-19 e síndrome do desconforto respiratório agudo. Apesar de suporte clínico intensivo, cumpriu os critérios para ser submetida à oxigenação venovenosa por membrana extracorpórea. No dia 34, após 7 dias de desmame da sedação com evolução positiva de seu quadro neurológico, apresentou uma crise tônico-clônica generalizada limitada, não relacionada com desequilíbrio hidroeletrolítico ou metabólico, que levou à necessidade de investigação diagnóstica. Seus exames de imagem cerebral revelaram síndrome da encefalopatia posterior reversível. Este caso enfatiza a questão das complicações neurológicas em pacientes com COVID-19 grave e a importância do diagnóstico e suporte precoces.

ABSTRACT A 63-year-old woman presented to the emergency department with an acute history of fever, prostration and dyspnea. She was diagnosed with severe COVID-19 acute respiratory distress syndrome and, despite optimized critical care support, met the indications for veno-venous extracorporeal membrane oxygenation. On day 34, after 7 days of wean sedation with a positive evolution of neurologic status, she presented a limited generalized tonic-clonic seizure not related to hydroelectrolytic or metabolic imbalance, which led to a diagnostic investigation; her brain imaging tests showed a posterior reversible encephalopathy syndrome. This case emphasizes the issue of neurological complications in patients with severe COVID-19 infection and the importance of early diagnosis and support.

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.

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.

Serie de casos de encefalopatía posterior reversible y eclampsia / Case series of reversible posterior encephalopathy and eclampsia

El síndrome de encefalopatía Posterior Reversible es una condición clínica y radiológica, con diversas manifestaciones neurológicas, también llamada la encefalopatía hipertensiva aguda y síndrome de leuco encefalopatía posterior reversible (SLPR), es un síndrome neurotóxico de vasoregulación cerebral clásicamente caracterizada por edema parieto-occipital bilateralmente simétrica. Sin embargo, los hallazgos de imagen son variables y pueden ocurrir en otras ubicaciones tales como los lóbulos frontales, tálamo, los ganglios basales y el tronco cerebral. TC y RM imágenes suelen mostrar áreas de edema vasogénico simétricamente distribuidos, principalmente en el territorio de la circulación posterior. Típicamente, las anormalidades afectan a la materia blanca, pero la corteza cerebral también ha sido afectada en algunos casos. El mecanismo del síndrome no se entiende por completo. Es un trastorno de la autorregulación cerebrovascular. Un estado hiperperfusión con una ruptura de la barrera sangre-cerebro conduce a la extravasación de fluido que contiene sangre o macromoléculas, que resulta en edema cortical o subcortical...(AU)

Reversible Posterior encephalopathy syndrome is a clinical and radiological condition, with various neurological manifestations, also called acute hypertensive encephalopathy and reversible posterior leukoencephalopathy syndrome (RPLS), is a neurotoxic syndrome of cerebral vasoregulation classically characterized by parieto-occipital edema bilaterally symmetric However, the imaging findings are variable and may occur in other locations such as the frontal lobes, thalamus, basal ganglia and brain stem. CT and MRI images usually show areas of symmetrically distributed vasogenic edema, mainly in the territory of the posterior circulation. Typically, abnormalities affect the white matter, but the cerebral cortex has also been affected in some cases. The mechanism of the syndrome is not completely understood. It is a disorder of cerebrovascular self-regulation. A hyperperfusion state with a rupture of the blood-brain barrier leads to the extravasation of fluid containing blood or macromolecules, resulting in cortical or subcortical edema ... (AU)

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.

Encefalopatía reversible posterior en una niña con lupus eritematoso sistémico: Presentación de un caso / Posterior reversible encephalopathy in a girl with systemic lupus erythematosus: Report of a case

La encefalopatía posterior reversible es una patología de baja frecuencia en pediatría. Las manifestaciones clínicas características son cefaleas, convulsiones, trastornos visuales y de conciencia asociadas a imágenes típicas en la resonancia magnética del sistema nervioso. Por lo general, se manifiesta en pacientes con eclampsia, trasplante de órganos sólidos, enfermedades hematológicas, renales y autoinmunes, entre otras causas menos frecuentes, y suele desencadenarse luego de un cuadro de hipertensión arterial o el uso de drogas inmunosupresoras. Factores patogénicos menos habituales, como transfusión sanguínea, uso de inmunoglobulinas o una infección subyacente, pueden estar asociados. Se describe una paciente con lupus eritematoso sistémico, que desarrolló la encefalopatía al estar expuesta a múltiples factores etiopatogénicos.

Posterior reversible encephalopathy is a rare disease in children. Clinical manifestations include headache, seizures, visual disturbances and altered consciousness associated with typical magnetic resonance images of the nervous system. The syndrome usually manifests in patients with eclampsia, solid organ transplantation, haematologic, renal and autoimmune diseases among other less common causes and it is often triggered after a hypertensive crisis or use of immunosuppressive drugs. Less common pathogenic factors as blood transfusion, use of immunoglobulins or an underlying infection can be associated. In this case a girl with systemic lupus erythematosus and exposed to multiple etiopathogenic factors developed posterior reversible encephalopathy.

Clinical, imagiological and etiological spectrum of posterior reversible encephalopathy syndrome / Espectro clínico, imagiológico e etiológico da síndrome de encefalopatia posterior reversível

Objective Analyze the cases of posterior reversible encephalopathy syndrome (PRES) admitted in a Neurology Department during an 8-year period. Method Retrospective observational study in a central hospital in the north of Portugal. Results 14 patients were identified, mean age 52.3 years. Precipitating factors included: eclampsia, isolated arterial hypertension, spinal trauma and autonomic dysreflexia, Guillain-Barré syndrome, sepsis, sarcoidosis and pulmonary cryptococcosis and drugs. Most patients presented posterior-predominant vasogenic edema lesions, however 64.2% presented frontal lesions and in 42.8% cerebellum was involved. Four patients also had acute ischemic lesions and 1 had hemorrhagic lesions. During follow-up 10 patients recovered fully, 2 recovered partially, 1 suffered a recurrence and 2 died in hospital. Conclusion PRES has many etiological factors. The terms posterior and reversible should be revised because PRES frequently involves other brain regions and it is not always reversible. PRES patients may develop life-threatening complications and mortality is not negligible. .

Objetivo Análise dos casos de síndrome de encefalopatia posterior reversível (PRES) internados em um Serviço de Neurologia durante oito anos. Método Estudo restrospectivo observacional num hospital central do norte de Portugal. Resultados Identificaram-se 14 casos, idade média de 52,3 anos. Os factores precipitantes foram: eclâmpsia, hipertensão arterial isolada, traumatismos vertebro-medulares com disfunção autonómica, síndrome de Guillain-Barré, sépsis, sarcoidose e criptococose pulmonar e fármacos. A maioria dos doentes apresentou lesões edematosas de predomínio posterior, contudo 64,2% apresentaram lesões frontais e 42,8% apresentaram também lesões cerebelosas. Quatro doentes tinham lesões isquémicas agudas e um apresentou lesões hemorrágicas. Durante o seguimento, 10 doentes recuperaram totalmente, 2 recuperaram com sequelas, 1 teve recidiva e 2 faleceram durante o internamento. Conclusão A PRES apresenta muitos factores precipitantes. As designações posterior e reversível deverão ser reequacionadas dado que a PRES afecta outras zonas do cérebro e nem sempre é reversível, apresentado complicações e mortalidade não ...

Síndrome de encefalopatía posterior reversible: presentación de un caso clínico pediátrico / Posterior reversible encephalopathy syndrome: presentation of a pediatric case report

The Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical and neuro-radiological syndrome described several decades ago. This syndrome was characterized by headache, frequent seizures, nausea, vomiting, decreased vision and variable confusion. The clinical manifestations may be of varying magnitude and should be recognized as an early management is associated with increased likelihood of full recovery of neurological disturbances. Magnetic resonance imaging (MRI) reveals the typical pattern of bilateral hyper intensities on fluid attenuated inversion recovery imaging predominantly in the parietal-occipital regions. The possible triggers may be abrupt arterial hypertension, impaired renal function, immunosuppressive therapies, eclampsia / pre-eclampsia, chemotherapy, autoimmune diseases and other inflammatory conditions. PRES is involved in the differential diagnosis of acute encephalopathy, leukoencephalopathies, CNS tumor and other refractory seizures. The prognosis is generally good and the recurrence rare. The clinical resolves within days and the earlier management in inpatient pediatric or medicine services withdrawal of medication related in the acute phase. RNM lesions are resolved more slowly and the injury may be remaining any month in hemorrhagic vascular compromise.

El Síndrome de Encefalopatía Posterior Reversible, de la denominación inglesa “Posterior reversible encephalopathy syndrome” (PRES), es un síndrome clínico neuro-radiológico descrito hace algunas décadas, caracterizado por cefalea, convulsiones frecuentes, náuseas, vómitos, disminución de la visión, con compromiso variable de la conciencia. Las manifestaciones clínicas pueden ser de diversa magnitud y deben ser reconocidas, ya que un manejo precoz se relaciona con mayor probabilidad de recuperación completa del compromiso neurológico. El estudio con resonancia nuclear magnética (RNM) cerebral precoz permite demostrar imágenes hiperdensas en diferentes localizaciones, principalmente en las regiones parietooccipitales. Este síndrome se ha relacionado con varios posibles factores desencadenantes, como la hipertensión arterial aguda, compromiso de la función renal, uso de inmunosupresores, eclampsia/pre eclampsia, quimioterapia, enfermedades autoinmunes, entre otras condiciones. Constituye parte del estudio de diagnóstico diferencial de encefalopatías agudas, leucoencefalopatías, procesos tumorales de SNC, crisis convulsivas refractarias, entre otros. El pronóstico en general es favorable, con recuperación clínica en días y con adecuado manejo en unidad de hospitalizados, con control de las condiciones desencadenantes y/o del fármaco relacionado. Las alteraciones en RNM se resuelven mucho más lentamente que la clínica, pudiendo persistir lesiones por meses, principalmente en caso de compromiso hemorrágico-vascular.

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.

Encefalopatía posterior reversible como primera manifestación del Síndrome de Guillain-Barré / Posterior reversible encephalopathy as the first manifestation of Guillain-Barré syndrome. Report of one case

Background: We report a 56year old male hypertensive, who presented with a posterior reversible encephalopathy syndrome (PRES) as an initial manifestation of Guillain-Barré syndrome (GBS). His first symptoms were right hemiparesis and hemihypoesthesia, followed by headache, dizziness, dysarthria and a general feeling of discomfort. On the third day, flaccid tetraparesis, impairment of consciousness, epileptic seizures and respiratory failure appeared, along with severe hypertension. Cerebral Magnetic Resonance Imaging showed the characteristic PRES lesions. Cerebrospinal fluid analyses revealed albumin-cytological dissociation and nerve conduction studies showed an axonal demyelinating polyradiculoneuropathy, which confirmed the diagnosis of GBS. Treatment with intravenous immunoglobulin was given together with antihypertensive therapy and mechanical ventilation, achieving an important clinical and imaging remission of PRES, but maintaining tetraparesis during the hospitalization. Twelve months after discharge and regular motor rehabilitation, the patient achieved complete autonomy on the activities of daily living. It has been postulated that the autonomic failure and the elevation of circulating pro-inflammatory cytokines in GBS may be the cause of a breach in the blood-brain barrier, thus causing PRES, that can completely remit with an adequate management.

Síndrome de encefalopatía posterior reversible (PRES) en dos casos clínicos de eclampsia / Posterior reversible encephalopathy syndrome (pres) in two clinical cases of eclampsia

The Posterior Reversible Encephalopathy Syndrome is a neurological condition described on 1996, developed in patients with complex systemic conditions, especially pregnant women with preeclampsia that at the same time presented neurological signs as seizures, headache, visual loss, and vomiting in addition to posterior brain edema, visible on neuroimaging and located in parietal and occipital lobes, that usually reverses completely. We present two clinical cases with PRES and eclampsia, antenatal and post-partum, both with seizure and confirmed brain edema with Nuclear Magnetic Resonance. Both had a favorable evolution after the anticonvulsivant and anti-hypertensive therapy were done. We discuss the controversy over pathophysiolgical mechanisms, the new methods for diagnosis and management and the importance of multidisciplinary approach.

El Síndrome de Encefalopatía Posterior Reversible (PRES) es una entidad descrita en 1996 que ocurre en pacientes con condiciones sistémicas complejas, especialmente embarazadas con pre-eclampsia, en que se agregan signos y síntomas neurológicos destacando convulsiones tónico-clónicas, cefalea, trastornos visuales y vómitos acompañados de edema cerebral posterior transitorio visible con técnicas de neuro-imágenes y localizado más frecuentemente en lobulos parietales y occipital. Presentamos dos casos clínicos con PRES y Eclampsia, antenatal y postparto, en las que después del episodio convulsivo con Resonancia Nuclear Magnética se confirmó imagen de edema cerebral propia de PRES y que en relación con tratamiento anti-convulsivante e hipotensor, tuvieron una evolución favorable. Se discuten la controversia de mecanismos fisiopatológicos, las posibilidades para diagnóstico y manejo oportunos y la importancia del manejo multidisciplinario.

Posterior Reversible Encephalopathy Syndrome.

PRES stands for Posterior Reversible Encephalopathy Syndrome. It is a proposed clinoneuroradiological entity that is characterized by headache, altered mental status, cortical blindness, seizure and other focal neurological signs and diagnostic magnetic resonance imaging picture. A variety of different etiologies have been reported like hypertension, preeclampsia, eclampsia, cyclosporine (and tacrolimus) neurotoxicity, uremia, sepsis, porphyria, SLE and interferon therapy. With early diagnosis and treatment the syndrome is usually fully reversible. A case of reversible encephalopathy is reported here in a 7-day postpartum female patient with marked elevation of blood pressure. Clinician and radiologist must be familiar with this clinically frightening, under diagnosed condition to assure timely diagnosis and treatment so as to prevent persistent defect.

Reversible posterior leukoencephalopathy syndrome: a retrospective study in King Chulalongkorn Memorial Hospital.

BACKGROUND: Reversible posterior leukoencephalopathy (RPLE) or reversible posterior cerebral edema syndrome is a syndrome characterized by transient focal or diffused neurological deficits and reversible neuroimaging changes. RPLE is often associated with hypertension and systemic illness. The classical Magnetic Resonance Imaging (MRl) feature of RPLE is predominately involvement of bilateral posterior cerebral white matter. Due to availability of MRI technology, this entity has been reported more frequently MATERIAL AND METHOD: The clinical recordings were searched from data bases at King Chulalongkorn Memorial Hospital from 2003 to 2005. Keywords were "leukoencephalopathy" and "hypertensive encephalopathy". Neuroimaging criteria for the diagnosis of RPLE were bilateral symmetrical cortical-subcortical white matter lesions predominantly affecting the occipital lobe. The data were reviewed. Cases with RPLE were recruited and analyzed. RESULTS: Nine patients (female:male, 8:1), ranged in age from 17 to 39 years (average, 26 years) were analyzed. Five patients had acute hypertension associated with renal failure, 1 had acute hypertension without renal impairment, 2 had eclampsia and 1 was associated with cyclosporine therapy. Most common clinical symptoms were seizure and cortical blindness. MRI revealed bilateral occipital white matter edema in 7/8 patients while computerized tomography demonstrated this feature in 3/9 patients. Other MRI abnormalities were detected in frontal lobes, parietal lobes, deep grey nuclei, brainstem and cerebellum. The patients were treated with antihypertensive drugs, antiepileptics and withdrawal from immunosuppressive therapy. In 8 patients, the neurological deficits recovered within 2 weeks. The case with cyclosporine therapy had residuals in the form of limb weakness and spasticity. CONCLUSION: RPLE is associated with hypertension, systemic autoimmune diseases, renal impairment, immunosuppressive therapy or eclampsia. The neuroimaging findings reveal characteristic white matter vasogenic edema in occipital lobes as well as other cortical areas and deep grey matter. Good clinical outcomes occur after prompt symptomatic treatment with antihypertensive drugs, antiepileptics or withdrawal from immunosuppressive therapy and repeated neuroimaging may not be necessary.