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.

Clinical characteristics analysis of children with reversible posterior leukoen-cephalopathy syndrome during the treatment of hematological tumor / 北京大学学报(医学版)

OBJECTIVE@#To analyze the clinical characteristics of patients with hematological tumor or disease before and after reversible posterior leukoen-cephalopathy syndrome (RPLS).@*METHODS@#Five patients were both from Peking University First Hospital Pediatric Hematology-oncology Department in the period from March 2012 to March 2017. The gender, age, BMI, underlying diseases, with or without renal damage, hypertension family history, clinical manifestations of convulsions, hemoglobin, and blood pressure, serum sodium levels before and after convulsion, and other data of the children with RPLS were retrospectively analyzed. In the meantime, we followed up the five patients for 6 months to 66 months, kept a watchful eye on their original condition and the recovery of symptoms and signs of the nervous system. The relevant literature was reviewed.@*RESULTS@#All of the subjects were females in school-age or pre-school age. The underlying diseases were malignant tumor associated with renal involvement or on one side of nephrectomy in 4 of these subjects, while the other one was refractory autoimmune hemolytic anemia. All of the subjects suffered from mild or moderate anemia. The day before RPLS occurred they received chemotherapy made up with cyclophosphamide, vincristine, and actinomycin-D, or the therapy with cyclosporin A and glucocorticoid. The clinical manifestations were afebrile convulsion after getting up in the mooring or in the afternoon. We observed elevation of blood pressure and cutting down of serum sodium compared with themselves. All of the cases recovered soon after management with diazepam, furosemide and amlodipine besylate. Four of them had a good outcomes and did not remain any sequela, while only one girl became childish in emotion and behavior, and then returned gradually to normal two years later. However, by long-term follow-up, the elevation of blood pressure was mainly reviewed in literature.@*CONCLUSION@#The patients attacked by RPLS, with hematology or oncology cases, could have the underlying disease of renal damage and anemia. Blood pressure elevation and serum sodium falling down at the same time may play an important role during the occurrence of RPLS. Remaining stable of blood pressure and electrolyte level together will possibly reduce or mitigate RPLS.

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.

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 leukoencephalopathy syndrome: postpartum focal neurologic deficits: a report of three cases and review of the literature

Posterior reversible encephalopathy syndrome presents with a variety of neurologic features, which, although devastating at some point, are potentially reversible on prompt recognition and institution of appropriated treatment. We report the management of three cases occurring in the last 4 years in our tertiary university hospital

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.