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OBJECTIVE: To review the epidemiology, the differential diagnosis, and the clinical and laboratory factors associated with the care management of headaches in patients with systemic lupus erythematosus (SLE). BACKGROUND: SLE is a chronic autoimmune disease and in 12%-95% of patients, the nervous system is involved. Headache is a frequently reported, although nonspecific, symptom that may potentially represent serious underlying diagnoses. Primary headaches may also occur in these patients, thereby causing a negative and significant impact on their quality of life. METHODS: This is a narrative review. A literature review was conducted on the PubMed platform using the following terms: (1) headache and (2) lupus. All articles considered relevant were included. No limitations were imposed for the publication date. RESULTS: Headache is a frequent symptom in patients with SLE. Although its prevalence is similar to the general population, headaches nonetheless tend to have a greater negative impact on these patients. Patients with SLE are more likely to experience headache due to vascular diseases such as cerebral venous sinus thrombosis, stroke, reversible cerebral vasoconstriction syndrome, posterior reversible encephalopathy syndrome, and vasculitis. Aseptic meningitis, neuroinfections, intracranial neoplasms, and intracranial hypertension or hypotension may also be a cause of headache in these patients. Although used in disease activity scores, the concept of lupus headache is controversial. CONCLUSIONS: Headache is a frequent symptom in patients with SLE. An appropriate approach enables the potentially serious conditions, which are the causes of secondary headaches, to be recognized and treated, together with an appropriate diagnosis and treatment of primary headaches.

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Posterior reversible encephalopathy syndrome is a rare clinical and radiological syndrome characterized by vasogenic edema of the white matter of the occipital and parietal lobes, which are usually symmetrical, resulting from a secondary manifestation of acute dysfunction of the posterior cerebrovascular system. We describe a case of posterior reversible encephalopathy syndrome secondary to SARS-CoV-2 infection in a 9-year-old boy who developed acute hypoxemic respiratory failure and required assisted mechanical ventilation. The child developed multisystem inflammatory syndrome, and he was monitored in the pediatric intensive care unit and was provided mechanical ventilation and vasoactive agents for hemodynamic support. Additionally, he developed pulmonary and extrapulmonary clinical manifestations along with neuropsychiatric manifestations that required close follow-up and were verified using brain magnetic resonance imaging for timely intervention. Currently, there are few reports of children with posterior reversible encephalopathy syndrome associated with multisystem inflammatory syndrome.

A síndrome da encefalopatia posterior reversível é uma rara síndrome clínica e radiológica caracterizada por edema vasogênico da matéria branca dos lobos occipital e parietal, que geralmente são simétricos, resultante de uma manifestação secundária de disfunção aguda do sistema cerebrovascular posterior. Descrevemos um caso de síndrome de encefalopatia posterior reversível secundária à infecção por SARS-CoV-2 em um menino de 9 anos de idade que desenvolveu insuficiência respiratória hipoxêmica aguda e necessitou de ventilação mecânica assistida. A criança desenvolveu síndrome inflamatória multissistêmica e foi monitorada na unidade de terapia intensiva pediátrica, tendo-lhe sido fornecidos ventilação mecânica e agentes vasoativos para suporte hemodinâmico. Além disso, desenvolveu manifestações clínicas pulmonares e extrapulmonares juntamente de manifestações neuropsiquiátricas que necessitavam de seguimento cuidadoso, tendo sido verificadas por ressonância magnética cerebral para intervenção oportuna. Atualmente, há poucos relatos de crianças com síndrome da encefalopatia posterior reversível associada à síndrome inflamatória multissistêmica.

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BACKGROUND: Guillain-Barré syndrome is the most common cause of flaccid paralysis, with multiple known clinical variants. Autonomic dysfunction, although frequently reported in the clinical course, is often overlooked in the pediatric population and is usually not the initial presenting symptom in this age group CASE PRESENTATION: We present the case of a previously healthy 17-year-old who arrived at the Emergency Department complaining of gastrointestinal symptoms associated with lipothymia. An initial electrocardiogram (ECG) showed sustained sinus bradycardia subsequently associated with arterial hypertension. Structural and inflammatory cardiac pathology were ruled out, as well as auriculoventricular conduction block and posterior reversible encephalopathy syndrome. On the ninth day after initial symptoms, the patient presented sensory and motor nerve disturbances with the cerebrospinal fluid analysis showing a clear albumin-cytologic dissociation, consistent with an atypical presentation of GBS with autonomic dysfunction. Immunoglobulin therapy was administered, developing subsequent aseptic meningitis, that required discontinuation of previous therapy and treatment with plasmapheresis. Clinical improvement was achieved with full motor function recovery. CONCLUSION: This case illustrates a Guillain-Barré syndrome variant in which autonomic dysfunction preceded neurologic deficit, a finding uncommon in children, emphasizing this as an important differential diagnosis for severe bradycardia in pediatric patients.

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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.

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A primigravida at 32 weeks of gestation presented to us with eclampsia and Posterior Reversible Encephalopathy Syndrome (PRES) along with SARS COVID-19 pneumonia. Immediate termination of pregnancy was done under general anesthesia and patient was electively ventilated in view of increased oxygen requirements. Further therapy using magnesium sulphate, antihypertensives, steroids, and convalescent plasma was carried out. The condition of the patient steadily improved leading to her extubation on the 4th postoperative day and subsequent discharge on the 8th day of admission.

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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.

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.

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Posterior Reversible Encephalopathy Syndrome (PRES) is an acute neurological syndrome clinically characterized by seizures, altered mental status, headache, and visual disturbances. It is caused by a variety of abnormalities in the endothelial function that ultimately result in vasogenic edema in the circulation of the central nervous system. This is reflected by the neuroimaging findings, that most often show reversible parieto-occipital edema. An important proportion of patients with PRES present with Systemic Lupus Erythematosus (SLE), and its complications, as their sole risk factors. This review describes the relationship between these two clinical entities and explains the pathophysiological models that have been proposed to describe the development of PRES. We explain how SLE can cause alterations in every pathway implicated in the development of PRES. Given the relatively high frequency and the distinct clinical course, PRES in the setting of SLE might be best described as a distinct neuropsychiatric syndrome associated with SLE.

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OBJECTIVE: To describe clinical manifestations, antecedents, comorbidities and associated treatments, imaging findings, and follow-up in patients with posterior reversible encephalopathy syndrome. METHODS: A retrospective, descriptive analysis of admitted patients was performed between June 2009 and May 2014 in a third-level care hospital. We evaluated age, sex, comorbidities, symptoms, values of blood pressure at admission, renal function, medication and time elapsed until the disappearance of symptoms. RESULTS: Thirteen patients were included. In all, 77% of them had a history of hypertension at baseline and 85% had impaired renal function. The most prevalent comorbidity was renal transplantation, and 85% had deterioration of renal function. Five of the patients had undergone renal transplantation. The most common clinical manifestation was seizures. All had subcortical lesions and bilateral parietooccipital involvement was the finding most frequently observed. CONCLUSION: This syndrome should be taken into account in the differential diagnoses of patients presenting with acute neurological syndromes and the abovementioned risk factors.

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Posterior reversible encephalopathy syndrome (PRES) is an uncommon clinicoradiological syndrome that is characterized by acute neurological symptoms such as headache, convulsion, visual disturbance, and altered consciousness. The characteristic magnetic resonance (MR) finding is vasogenic edema, predominantly in the subcortical areas of the posterior parietal and occipital lobes on T2-weighted and fluid-attenuated inversion recovery (FLAIR) sequences. Herein, we described a rare case of PRES induced by cyclosporine (CsA) after an allogeneic hematopoietic stem cell transplantation (HSCT) from a sibling donor.

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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.

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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.

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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.

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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)

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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.

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Introduction Henoch-Schönlein purpura is a multisystem small vessel vasculitis. Neurologic manifestations are uncommon. Posterior reversible encephalopathy syndrome is a rare complication of Henoch-Schönlein purpura with typical clinical and neuroimaging findings that occurs most commonly in the setting of severe hypertension and renal injury. Case presentation A seven-year-old girl was admitted to our institution presenting with clinical and laboratory findings suggestive of Henoch-Schönlein purpura. Glucocorticoid therapy was initiated, but five days following her admission, she developed altered consciousness, seizures, arterial hypertension, and cortical blindness. Brain MRI scan revealed areas of vasogenic oedema in parieto-occipital lobes, consistent with posterior reversible encephalopathy syndrome. She was immediately initiated on antihypertensives and antiepileptics, which successfully improved her neurologic symptoms. Further laboratory work-up disclosed a rapidly progressive glomerulonephritis secondary to Henoch-Schönlein purpura that was the likely cause of her sudden blood pressure elevation. Immunosuppressive therapy was undertaken, and at one-year follow-up, the patient exhibited complete renal and neurologic recovery. Conclusion Posterior reversible encephalopathy syndrome is a severe complication of Henoch-Schönlein purpura. If promptly diagnosed and treated, children with Henoch-Schönlein purpura presenting with posterior reversible encephalopathy syndrome usually have a good prognosis. Clinicians should be familiar with the characteristic presentation of posterior reversible encephalopathy syndrome and be aware that hypertension and renal injury may predispose Henoch-Schönlein purpura patients to developing this complication.

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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.

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Henoch-Schönlein purpura is a common small-vessel vasculitis that presents in children as nonthrombocytopenic, nontender, and palpable purpura, arthritis/arthralgia, and abdominal pain. Central nervous system involvement is a reported, but likely, under-recognized complication. We present an 8-year-old boy with Henoch-Schönlein purpura and posterior reversible encephalopathy syndrome, after proposed mechanism and treatment considerations.

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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.

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