Posterior Reversible Encephalopathy Syndrome Associated with L-Asparaginase Treatment in Children: Literature Review and Six Case Reports.

L-asparaginase (L-ASNase) is an enzyme that shows targeted activity against Acute Lymphoblastic Leukemia (ALL) and similar lymphoid neoplasms by facilitating the breakdown of asparagine into L-aspartic acid, thereby reducing L-asparagine levels in leukemic cells. However, its therapeutic potential is hindered by its associated toxicity, leading to complications, such as thrombosis, hemorrhage, thrombocytopenia, fibrinolysis, hypersensitivity reactions, and the development of Posterior Reversible Encephalopathy Syndrome (PRES). This review compiles documented cases of PRES linked to treating B and T cell acute lymphoblastic leukemia in children using L-ASNase. Although this pathology is rare, understanding its management is crucial within ASNase-based chemotherapy protocols. As PRES lacks a specific treatment, focusing on symptomatic management becomes pivotal. Therefore, comprehending the underlying causes during L-ASNase treatment for acute lymphoblastic leukemia is essential. Understanding the etiology and clinical symptoms of this illness is critical for early diagnosis and treatment. The cases of PRES described in this review include instances in which this syndrome has appeared after the administration of L-ASNase in children. In some cases, PRES developed during induction therapy, while in others, it occurred during the reinduction phase. These cases resolved days after discontinuation of L-ASNase. The findings suggest a close relationship between drug administration and the appearance of brain lesions, as evidenced by the disappearance or decrease of these lesions when the drug was eliminated from the bloodstream.

Perfusion-Dependent Focal Neurologic Deficits in a Critically Ill Heart Transplant Recipient: A Case of Tacrolimus-Associated Reversible Cerebral Vasospasm Syndrome?

TACROLIMUS, a mainstay of immunosuppression after orthotopic heart transplantation (OHT), is associated with a broad range of side effects. Vasoconstriction caused by tacrolimus has been proposed as a mechanism underlying common side effects such as hypertension and renal injury. Neurologic side effects attributed to tacrolimus include headaches, posterior reversible encephalopathy syndrome (PRES), or reversible cerebral vasospasm syndrome (RCVS). Six case reports have been published describing RCVS in the setting of tacrolimus administration after OHT. The authors report a case of perfusion-dependent focal neurologic deficits attributed to tacrolimus-induced RCVS in an OHT recipient.

Visual Hallucinations as a Major Manifestation of Posterior Reversible Encephalopathy Syndrome: Case Report and Literature Review.

We evaluated a 48-year-old woman who had visual hallucinations (VHs) as a major presenting sign of posterior reversible encephalopathy syndrome (PRES). Despite her mild loss of vision, she described various hallucinations after awakening from a comatose state days after a motorcycle collision. VHs are usually accompanied by more severe loss of vision, yet our case and literature review indicate that sudden onset of formed VHs should suggest a possible diagnosis of PRES in patients who have large fluctuations in blood pressure, renal failure, or autoimmune dysfunction, as well as in patients taking cytotoxic agents.

Eclampsia and posterior reversible encephalopathy syndrome in a parturient complicated by SARS COVID-19 pneumonia.

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.

Posterior reversible encephalopathy syndrome: A neuropsychiatric manifestation of systemic lupus erythematosus.

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.

Emergencias hipertensivas neurológicas Aproximación diagnóstica y terapéutica con base en imágenes por resonancia magnética / Hypertensive neurological emergencies Diagnostic approach and treatment based on magnetic resonance imaging

RESUMEN El diagnóstico y el tratamiento de las complicaciones cerebrales de la emergencia hipertensiva constituyen un reto para los médicos de urgencias y terapia intensiva. Mediante una aproximación diagnóstica basada en las diferentes secuencias de las imágenes por resonancia magnética se llega al diagnóstico de encefalopatía hipertensiva, isquemia cerebral, hemorragia intracraneal, hemorragia subaracnoidea aneurismática, síndrome de encefalopatía posterior reversible y a su inmediato tratamiento ajustado a nuestro medio según las últimas guías de práctica clínica de la AHA/ASA (Asociación Americana del Corazón y Asociación Americana de Ataque Cerebrovascular).

SUMMARY The diagnosis and treatment of brain injury in patients with hypertensive emergencies is a challenge for the emergency department and intensive care doctors. The diagnosis of hypertensive encephalopathy, ischemic stroke, intracranial hemorrhage, aneurysmal subarachnoid hemorrhage and posterior reversible encephalopathy syndrome is achieved with different MRI sequences as well as their appropriate treatment according to the last AHA/ASA clinical practice guidelines.

Síndrome de encefalopatía posterior reversible. Presentación radiológica atípica / Posterior Reversible Encephalopathy Syndrome. Atypical radiological presentation / Síndrome de encefalopatia posterior reversível (PRES), apresentação radiológica atipica

Resumen: Introducción: El síndrome de encefalopatía posterior reversible (PRES por las siglas en inglés Posterior Reversible Encephalopathy Syndrome) es una entidad clínica caracterizada por cefalea, alteraciones visuales y crisis convulsivas. Ésta es una entidad subdiagnosticada, que tiene como fisiopatología la alteración del vaso de regulación cerebral y se puede presentar en féminas gestantes, lo que puede llegar a confundirse con eclampsia. Caso clínico: Paciente de 20 años sin antecedentes patológicos, con embarazo de término normoevolutivo. Presentó ruptura prematura de membranas e ingresó para conducción de trabajo; se preparó a la paciente para analgesia obstétrica; sin embargo, previamente al procedimiento presentó crisis convulsivas de características tonicoclónicas generalizadas. Se realizó un manejo avanzado de la vía aérea y se realizó una cesárea de urgencia; se obtuvo producto único vivo y fue ingresada a la Unidad de Cuidados Intensivos. El perfil toxémico no reportó alteraciones y la tomografía computarizada inicial no mostró alteraciones estructurales. Se le retiró a la paciente la ventilación mecánica 12 horas después. Se realizó una resonancia magnética que mostró en fase T2, hiperintensidad subcortical parieto-occipital derecho. Permaneció en vigilancia y fue egresada a piso sin complicaciones ni secuelas neurológicas. Conclusiones: El tratamiento del PRES va enfocado al control de la presión arterial, y el soporte orgánico y metabólico. No todos los enfermos con PRES se recuperan neurológicamente ad integrum; sin embargo, la proporción de pacientes que cursan sin secuelas es mayor en pacientes obstétricas. La resonancia magnética es el estudio de elección; el hallazgo característico es el edema simétrico de la materia blanca en la región occipital de los hemisferios cerebrales, en particular las regiones parieto-occipitales, no obstante, existen otro tipo de presentaciones radiológicas.

Abstract: Introduction: Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical entity characterized by headache, visual disturbances and convulsive seizures. It is a sub-diagnosed entity that, due to its trigger being cerebral vasoregulation disorders, may present in pregnant women, which may be confused with eclampsia. Clinical case: Patient of 20 years of age, without pathological antecedents, with term pregnancy, evolutionary norm. Who enters by exit of mucous plug, followed by premature rupture of membranes, so it is admitted for conduction of labor, however presents convulsive crisis, generalized clonic tonic. Advanced airway management is decided, and an emergency caesarean section is performed, obtaining a single live product, and the patient is admitted to the ICU. His toxemic profile was reported without alterations, his initial CT scan without structural alterations, and mechanical ventilation was removed twelve hours later. MRI is performed where subcortical right parieto-occipital hyperintensity is observed, being kept under surveillance and graduated to floor without complications. Conclusions: The treatment of PRES syndrome is focused on organic and metabolic supports and mainly on tension Figures. Not all are reversible, however, the pregnant patient is more frequent than present evolution without sequelae. Magnetic resonance is the study of choice for this clinical entity, and the typical findings are symmetrical edema of white matter in the posterior cerebral hemispheres, particularly the parieto-occipital regions, but there are variations.

Resumo: Introduçao: A síndrome da encefalopatia posterior reversível (PRES) é uma entidade clínica caracterizada por dor de cabeça, distúrbios visuais e convulsões. É uma entidade subdiagnosticada, cuja fisiopatologia é a alteração do vaso de regulação cerebral, pode ocorrer em mulheres grávidas, o que pode ser confundido com eclampsia. Caso clínico: Paciente de 20 anos, sem antecedentes patológicos, com gestação á término e evolução normal. Apresentou ruptura prematura de membranas e ingressou para indução do trabalho de parto, preparou-se para analgesia obstétrica, porém antes do procedimento apresentou convulsões com características tônicas clônicas generalizadas. Foi realizado manejo avançado das vias aéreas e cesariana de emergência, obtendo-se um único produto vivo, e foi admitida na Unidade de Terapia Intensiva. O perfil toxémico não relatou alterações, a tomografia computadorizada inicial não mostrou alterações estruturais e a ventilação mecânica foi removida doze horas depois. Foi realizada ressonância magnética que mostrou na fase T2, hiperintensidade subcortical parieto-occipital direita. A paciente permaneceu sob vigilância e recebeu alta sem complicações ou sequelas neurológicas. Conclusões: O tratamento do PRES, está focado no controle da pressão arterial, suporte orgânico e metabólico. Nem todos os pacientes com PRES se recuperam neurologicamente ad integrum, no entanto, a proporção de pacientes que não apresentam sequelas é maior em pacientes obstétricos. A ressonância magnética é o estudo de eleição; o achado característico é edema simétrico de substância branca na região occipital dos hemisférios cerebrais, particularmente regiões parietooccipitais, porém existem outros tipos de apresentações radiológicas.

Posterior reversible leukoencephalopathy syndrome: Case series and review of the literature. / Síndrome de leucoencefalopatía posterior reversible: serie de casos y revisión de la literatura.

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.

Síndrome De Encefalopatía Posterior Reversible De Etiología Atípica / Posterior Reversible Encephalopathy Síndrome Of Atypical Etiology

Resumen El síndrome de encefalopatía posterior reversible, es un síndrome clínico-radiológico, caracterizado por imágenes compatibles con edema vasogénico, cuya fisiopatología no está esclarecida por completo. Existen múltiples causas descritas de este síndrome, principalmente hipertensión arterial severa, insuficiencia renal, sepsis, preeclampsia o eclampsia, terapia inmunosupresora, entre otras. Presentamos a continuación el caso de un paciente de 38 años, postrasplante renal en tratamiento con micofenolato y prednisona, con falla renal del trasplante y anemia crónica, hospitalizado por sepsis de foco urinario más tuberculosis ganglionar, quien tras recibir un concentrado de glóbulos rojos presenta convulsiones tónico clónicas, elevación de Hb de 3.1 g/dl e imágenes compatibles con edema vasogénico y resolución casi completa de las mismas a los 16 días.

Abstract Posterior reversible encephalopathy syndrome is a clinical-radiological syndrome, characterized by images compatible with vasogenic edema, whose pathophysiology is not fully clarified. There are multiple described causes of this syndrome, mainly severe arterial hypertension, renal insufficiency, sepsis, preeclampsia or eclampsia, immunosuppressive therapy and others. We report a case of a 38-year-old man, renal post-transplant in treatment with mycophenolate and prednisone, with renal failure of the transplant and chronic anemia, hospitalized by urinary focus sepsis plus lymph node tuberculosis, who after receiving 1 concentrate of red blood cells presents seizures, Hb elevation of 3.1 g/dl and images compatible with vasogenic edema and almost complete resolution of them at 16 days.

Diagnosis and management of posterior reversible encephalopathy syndrome in systemic lupus erythematosus. Case report / Diagnóstico y manejo de síndrome de encefalopatía posterior reversible asociado a lupus eritematoso sistémico. Reporte de caso clínico

ABSTRACT Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological disorder that may include encephalopathy, seizures, headache, and visual disturbances. It is associated with conditions that induce endothelial damage, causing vasogenic cerebral edema that can be observed in magnetic resonance scans. It occurs in <1% of patients with systemic lupus erythematosus (SLE). It is usually resolved with timely treatment, but delays may lead to neurological sequelae or death. A case of PRES is presented in a patient with SLE with severe activity, a hypertensive emergency, and lupus glomerulonephritis debuting with epileptic status. The outcome was satisfactory with anticonvulsants, as well as treatment for her cerebral edema and hypertension, along with control of other causal factors.

RESUMEN El síndrome de encefalopatía posterior reversible (PRES) es un trastorno clínico-radiológico caracterizado por encefalopatía, convulsiones, cefalea y alteraciones visuales. Se asocia a entidades que ocasionan daño endotelial, causando edema cerebral vasogénico evidente en resonancia magnética. En pacientes con lupus eritematoso sistémico (LES) se presenta en < 1%. Con tratamiento oportuno usualmente resuelve; caso contrario puede producir secuelas neurológicas o muerte. Se reporta el caso de PRES en una paciente con LES con actividad severa, emergencia hipertensiva y glomerulonefritis lúpica que comienza con estatus epiléptico. Evolucionó satisfactoriamente con tratamiento anticonvulsivante, antiedema cerebral, antihipertensivo y control de los demás factores causales.

Posterior Reversible Encephalopathy Syndrome in a Normotensive Child after Allogeneic Hematopoietic Stem Cell Transplantation.

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.

Posterior reversible encephalopathy syndrome as a complication of Henoch-Schönlein purpura in a seven-year-old girl.

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.

Encefalopatía posterior reversible en preeclampsia / Reversible posterior encephalopathy in preeclampsia / Encefalopatia posterior reversível em pré-eclâmpsia

Resumen: El síndrome de encefalopatía posterior reversible (PRES por sus siglas en inglés Reversible Posterior Leukoencephalopathy Syndrome) es una entidad clínico-radiológica con datos claros de afección del lóbulo posterior por imagen por resonancia magnética. Fue descrito por primera vez en 1996 por Hinchey, se caracteriza clínicamente por cefalea, afección laberíntica, así como ceguera cortical, entre otros síntomas. Una de las poblaciones en riesgo de presentar esta complicación neurológica son las mujeres que padecen preeclampsia/eclampsia. El objetivo de este trabajo es exponer un caso de encefalopatía posterior reversible en una paciente con preeclampsia.

Abstract: Posterior reversible encephalopathy syndrome is a clinical-radiological entity, with clear data of vasogenic edema at the posterior lobe by magnetic resonance image. As First described in 1996 by Hinchey, clinical features are headache, labyrinthine affection, and cortical blindness among others symptoms preeclampsia/eclampsia among obstetric patients has been characterized for presenting as a risk factor for this neurologic complication. The aim of this paper is to present a clinical case of posterior reversible encephalopathy in a patient with pre-eclampsia.

Resumo: A síndrome da encefalopatia posterior reversível (PRES, sigla em Inglês) é uma entidade clínica radiológica, com clara evidência da afetação do lobo posterior por IRM.1 Descrita pela primeira vez em 1996 por Hinchey; clinicamente caracterizada por cefaleia, doença labiríntica e cegueira cortical entre outros sintomas. Uma das populações em risco de apresentar essa complicação neurológica são mulheres com pré-eclâmpsia/eclâmpsia. O objetivo deste trabalho é apresentar um caso de encefalopatia posterior reversível em uma paciente com pré-eclâmpsia.

Posterior reversible encephalopathy syndrome and association with systemic lupus erythematosus.

INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a neurological complex disorder with many clinical associations and causative factors. It is important to recognize this condition because early diagnosis and treatment usually result in its complete resolution, radiological imaging becoming the key for the correct diagnosis. METHODS: We retrospectively reviewed charts and magnetic resonance imaging findings in the University of Campinas from January 2005 to July 2015, selecting three cases of patients with systemic lupus erythematosus syndrome who developed PRES, for whom risk factors, characteristics, magnetic resonance imaging findings and neurological resolution were analyzed. We also conducted a review of the English-language literature. RESULTS: The three cases had neurological symptoms like acute onset of headache, altered mental status, cortical blindness and seizures. Brain magnetic resonance imaging demonstrated posterior cortical and white matter alterations involving posterior brain territories, which were more conspicuous on T2-weighted and fluid-attenuated inversion recovery. Spectroscopy, diffusion-weighted imaging and susceptibility-weighted imaging were also important for neuroradiological evaluation. Immunosuppressive drugs were taken in all cases. Partial clinical and radiological recovery was observed in two cases, and complete resolution was observed in the third patient. LITERATURE REVIEW: We found 52 cases of PRES in systemic lupus erythematosus patients. Almost all patients were women 94%, ranging from 8 to 62 years old. Posterior brain territory involvements were found in 98% of patients. Hemorrhagic complications involved 26% of patients, becoming a risk factor for clinical sequels. The total percentage of patients with no complete resolution of radiological findings on follow-up images was 27.5%. DISCUSSION: In patients with autoimmune disorders, endothelial dysfunction may occur secondary to autoimmunity and the use of cytotoxic drugs, supposedly facilitating the occurrence of more severe PRES. The hypothesis that patients with autoimmune diseases have a propensity to develop non-reversible lesions has been raised.

Porfiria aguda intermitente y su relación con el síndrome de encefalopatía posterior reversible: reporte de caso / Acute Intermittent Porphyria and it´s relationship with Posterior Reversible Encephalopathy Syndrome: Case Report

Las porfirias son un grupo heterogéneo de trastornos del metabolismo en el cual hay una deficiencia enzimática específica necesaria en la biosíntesis del grupo hemo. Dentro de estas se destaca la porfiria aguda intermitente como la forma más común, que se caracteriza por episodios de exacerbación o crisis neuroviscerales. Las manifestaciones clínicas son amplias, sin embargo, la presentación con síntomas del sistema nervioso central a diferencia de los síntomas disautonómicos o neuropáticos son poco frecuentes. Reportamos un caso que resalta la relación infrecuente entre la porfiria aguda intermitente y el síndrome de encefalopatía posterior reversible. Paciente femenina de 18 años con dolor abdominal persistente severo, hipertensión arterial, convulsiones, ceguera cortical y neuropatía axonal motora. Se le realizó una resonancia magnética nuclear (RMN) contrastada que evidenció lesiones hiperintensas corticales y cortico-subcorticales sugestivas de edema vasogénico, compatibles con síndrome de encefalopatía posterior reversible. Se hizo el diagnóstico de porfiria aguda intermitente por elevados niveles de resonancia magnética nuclear (PBG) y ácido 5-amino-levulínico (ALA) en orina y se inició tratamiento con hematina, terapia parenteral hiperglucida y el retiro de agentes porfirinogénicos. Los síntomas centrales así como los hallazgos imagenológicos se resolvieron de forma adecuada con el tratamiento. Este caso resalta la relación inusual entre la porfiria aguda intermitente y el síndrome de encefalopatía posterior reversible, en una paciente con crisis de dolor abdominal persistente, disautonomía, convulsiones, ceguera cortical y neuropatía axonal motora, síntomas que asociados nos deben sugerir estos diagnósticos.

The porphyrias are a heterogeneous group of metabolism disorders in which there is an enzymatic deficiency necessary for the pathway of heme biosynthesis. Within this group, Acute Intermittent Porphyria (AIP) is the most common disorder, characterized by episodes of neuro-visceral crisis. The clinical manifestation spectrum is wide, however symptoms originating from Central Nervous System (CNS) dysfunction are rare. We report a case that shows the infrequent relationship between AIP and Posterior Reversible Encephalopathy Syndrome (PRES) An 18-year-old female patient presented with severe persistent abdominal pain, hypertension, seizures, cortical blindness and motor axonal neuropathy. A brain contrasted MRI evidenced a cortical and cortico-subcortical high intensity lesion suggestive of vasogenic edema in frontal, parietal and occipital lobes, compatible with PRES. A diagnosis of AIP was also made due to high levels of PBG and ALA in the urine. Treatment consisted of hematin, intravenous sugar solution and the withdrawal of porphyrinogenic agents. The CNS-related symptoms and the brain lesions shown via imaging resolved appropriately with treatment. This case shows the unusual relationship between AIP and PRES, in a patient that presented with persistent abdominal pain, dysautonomia, seizures, cortical blindness and motor axonal neuropathy, symptoms that, as a whole, can suggest this diagnosis.

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.

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.(AU)

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.(AU)

Implicancias neurológicas de la preclampsia, más que solo eclampsia

La preeclampsia es una enfermedad multisistémica que afecta a la gestante; se constituye en la segunda causa de muerte materna en el Perú y produce una alta morbilidad. La disfunción neurológica producida por la preeclampsia abarca múltiples síntomas, siendo el más conocido la eclampsia. El entendimiento de la encefalopatía posterior reversible como fenómeno que explica todas las manifestaciones neurológicas de la preeclampsia abre un sinnúmero de posibilidades terapéuticas y plantea la interrogante sobre el pronóstico a largo plazo de las pacientes afectadas. El sulfato de magnesio se muestra como la mejor alternativa terapéutica para bloquear los fenómenos fisiopatológicos que podrían devenir en eclampsia, entendiéndola como una forma de encefalopatía posterior reversible.

Preeclampsia is a multisystemic disease that affects pregnant women; it is the second cause of maternal death in Peru and morbidity is high. The neurologic dysfunction in preeclampsia includes various symptoms, and eclampsia is the most known. Understanding reversible posterior encephalopathy as the explanation of all neurological manifestations in preeclampsia would evolve to endless therapeutic possibilities and interrogations on long term prognosis. Magnesium sulfate appears as the best therapeutic alternative in blocking pathophysiologic phenomena resulting in eclampsia, if it is considered as a reversible posterior encephalopathy.