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Seizure following cerebrospinal fluid (CSF) rhinorrhoea surgery or surgery of the skull base almost always implies postoperative meningoencephalitis, unless proven otherwise. Here, we present the case of a middle-aged female in her 40's who underwent surgical CSF fistula closure and developed seizure on the eighth postoperative day. She was diagnosed to have posterior reversible encephalopathy syndrome (PRES). Early diagnosis and prompt initiation of treatment ensured that she had a complete recovery. Although not reported in the literature, PRES should always be a differential diagnosis in such situations, as delay in diagnosis may result in significant morbidity and rarely mortality.

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Posterior reversible encephalopathy syndrome (PRES) is characterized by nonspecific symptoms, including not only pronounced non-focal and various focal neurological signs but also specific neuroimaging features, including vasogenic edema affecting predominantly the posterior area. PRES usually develops in the setting of acute arterial hypertension. However, it is not uncommon for PRES to develop in non-hypertensive patients, including people with autoimmune disorders (multiple sclerosis, neuromyelitis optica spectrum disorder, etc). PRES could also be due to the toxic effects of drugs or other substances. The pathophysiological mechanisms of PRES include impaired autoregulation of cerebral blood flow due to acute arterial hypertension and toxic endotheliotropic effects of endogenous and exogenous factors.

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Background and purpose:

Posterior reversible encephalopathy syndrome (PRES) is characterized by vasogenic edema, usually reversible, with the prominent involvement of the parietal and occipital lobes. The exact etiopathogenesis leading to PRES is unknown. Because signs of eclampsia and preeclampsia in neuroimaging often overlap and manifest as PRES, we aimed to evaluate whether demographic, clinical, and laboratory parameters predict PRES in patients with preeclampsia or eclampsia.

213 pre-eclampsia or eclampsia patients with cranial imaging were retrospectively examined. We recorded the patients’ demographic information, systolic blood pressure (SBP), diastolic blood pressure (DBP), mean arterial pressure (MAP), hemogram, biochemical indicators, clinical symptoms, and imaging features.

Of all patients, 69% (n = 147) had preeclampsia while 31% (n = 66) had eclampsia, and 24.4% (n = 53) were diagnosed with PRES. The mean age of patients who developed PRES was 25.81 ± 6.07 years and thus significantly less than that of patients who did not develop PRES (p = .000). Patients with PRES had significantly higher mean SBP (p = .015), DBP (p = .009), and MAP (p = .003) than patients without PRES, along with significantly higher aspartate aminotransferase (ASAT; p = .001), alanine aminotransferase (ALAT; p = .001) blood urea nitrogen (BUN; p = .001), white blood cell (WBC; p = .003), neutrophil (p = .001), and hemoglobin (Hb; p = .027) levels, but significantly lower albumin (p = .000) levels.

Age, high blood pressure, and BUN, neutrophil, and WBC levels were predictors of the development of PRES in patients with preeclampsia and eclampsia. Early neuroimaging considering those predictors should be performed to diagnose PRES in patients with preeclampsia and eclampsia.

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Posterior reversible encephalopathy syndrome (PRES) is an increasingly recognized clinical entity associated with a variety of medical conditions. It is commonly considered in the presentation of uncontrolled, severe hypertension. However, more recently, it has been described in the setting of blood transfusion, particularly in those with chronic anemia, even in the absence of severe hypertension. We describe a patient who presented to the emergency department 12 days after large blood transfusion for severe, chronic anemia with headache, vision loss, expressive aphasia and a change in mental status, with only mild blood pressure elevation, who was ultimately diagnosed with PRES and refractory non-convulsive status epilepticus. Emergency physicians are often the first to initiate blood transfusion for those with a low hemoglobin. Therefore, it is prudent to proceed with caution in transfusing those with chronic anemia. It is also important for the emergency physician to keep PRES on the differential for those presenting with a neurologic complaint after correction of their chronic anemia, even in the absence of severe hypertension.

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Transplantation-associated thrombotic microangiopathy (TA-TMA) is a well-recognized serious complication of hematopoietic stem cell transplantation (HSCT). The understanding of TA-TMA pathophysiology has expanded in recent years. Dysregulation of the complement system is thought to cause endothelial injury and, consequently, microvascular thrombosis and tissue damage. TA-TMA can affect multiple organs, and each organ exhibits specific features of injury. Central nervous system (CNS) manifestations of TA-TMA include posterior reversible encephalopathy syndrome, seizures, and encephalopathy. The development of neurological dysfunction is associated with a significantly lower overall survival in patients with TA-TMA. However, there are currently no established histopathological or radiological criteria for the diagnosis of CNS TMA. Patients who receive total body irradiation (TBI), calcineurin inhibitors (CNI), and severe acute and chronic graft-versus-host disease (GVHD) are at a high risk of experiencing neurological complications related to TA-TMA and should be considered for directed TA-TMA therapy. However, the incidence and clinical manifestations of TA-TMA neurotoxicity remain unclear. Studies specifically examining the involvement of CNS in TMA syndromes are limited. In this review, we discuss clinical manifestations and imaging abnormalities in patients with nervous system involvement in TA-TMA. We summarize the mechanisms underlying TA-TMA and its neurological complications, including endothelial injury, evidence of complement activation, and treatment options for TA-TMA.

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BACKGROUND: Guillain-Barré syndrome (GBS) is an autoimmune disorder characterized by demyelination of peripheral nerves. GBS-associated posterior reversible encephalopathy syndrome (PRES) is a rare and potentially life-threatening complication in the pediatric population. We aimed to report and analyze the clinical features, management, and outcomes of three cases of GBS-associated PRES in our setting in the light of the existing literature. METHODS: Medical records of 75 pediatric patients with GBS were reviewed for autonomic changes and GBS-associated PRES. Thirty-one developed dysautonomia while three were identified to have PRES. Clinical, radiological, laboratory, and treatment data were collected and analyzed. RESULTS: All three patients were male and presented with symptoms of acute flaccid paralysis and respiratory distress requiring mechanical ventilation. All three patients experienced various complications, including hypertension, seizures, and hyponatremia, and were subsequently diagnosed with PRES. Multimodal intensive care resulted in patient improvement and discharge in an ambulatory state after an average of 104 days of care. CONCLUSIONS: GBS-associated PRES is a rare and potentially life-threatening complication that can occur in pediatric patients with GBS. Our findings suggest that early recognition, prompt intervention, and multimodal intensive care can improve patient outcomes. Further studies are needed to determine optimal treatment strategies for GBS-associated PRES.

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OBJECTIVES: Posterior reversible encephalopathy syndrome (PRES) is a clinic radiological disorder characterized by headache, epileptic seizure, encephalopathy, visual impairment, and focal neurological deficits. Gestational hypertension, which is a significant risk factor for PRES, may cause significant morbidity and mortality among pregnant women. DESIGN: Twenty-four patients with PRES caused by eclampsia who were admitted to our hospital in the last 5 years were included in this study. PARTICIPANTS/MATERIALS, SETTING, METHODS: Blood pressure at admission, the number of regions with vasogenic edema in the brain, and recurrent seizures were noted. Patients were divided into three groups: mild, moderate, and severe. RESULTS: Using Kruskal-Wallis and Pearson χ2 tests, there was no statistical significance between the groups in terms of cranial involvement (p = 0.471). However, binary logistic regression analysis showed that seizure recurrence increased in correlation with blood pressure (p = 0.04). LIMITATIONS: PRES is a rare syndrome associated with several etiologies. In our study, only patients with PRES due to eclampsia were included. Therefore, the number of included patients was limited (24 participants). CONCLUSION: PRES may occur in eclamptic patients with mild, moderate, or severe blood pressure values. Evaluation by magnetic resonance imaging is needed to confirm the diagnosis. Early and rapid treatment is essential for reducing morbidity and mortality among pregnant women.

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IgA vasculitis (IgAV), formerly known as Henoch-Scholein purpura, is a small vessel vasculitis, most commonly seen in pediatric patients, that can affect numerous internal organs including the kidneys, lungs, gastrointestinal tract, and the central nervous system (CNS). CNS manifestations of this condition include hypertensive encephalopathy, thrombosis, optic neuropathy, seizures, CNS vasculitis, and a more recently described phenomenon known as posterior reversible encephalopathy syndrome (PRES). Symptoms of PRES include hypertension, altered mental status, and seizures caused by vasogenic disruption of the blood-brain barrier, and the condition is diagnosed by characteristic edema-related gray-white matter changes in the parieto-occipital lobes on magnetic resonance imaging. Herein, we present a rare case of PRES as a presenting sign of IgAV to increase awareness about this unusual association.

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INTRODUCTION: Allogeneic hematopoietic stem cell transplantation (HSCT) is the only definitive curative option for ß-major thalassemia patients (ß-MT). Posterior reversible encephalopathy syndrome (PRES) is a pervasive neurological complication which typically occurs following HSCT. ß-MT patients are prone to a higher PRES incidence due to long-term immunosuppression; thus, it is imperative that these patients are closely monitored for PRES after HSCT. PATIENTS AND METHODS: We included 148 pediatric patients with ß-MT who underwent HSCT between March 2015 and August 2022 in Children's Medical Center. Patients in this study were divided into two groups. The association between PRES and class of ß-MT and other risk factors were assessed and the overall survival rate was determined. RESULTS: Fourteen out of 112 patients (12%) with class I and II ß-MT developed PRES. However, PRES occurred in 11 out of 36 patients (30.5%) with ß-MT-III. Our results indicated that there was a significant association between class III ß-MT and the occurrence of (P = .004). Additionally, acute graft-versus-host disease (aGVHD) occurred in 80% and 44.7% of patients in the PRES and non-PRES groups, respectively (P = .001). The results of the Kaplan-Meier analysis revealed that the 5-year overall survival (OS) was 75.6% in the PRES group versus 95% in the non-PRES group, which was statistically significant (P = .001). CONCLUSION: Based on our results, pediatric ß-MT III patients are at a higher risk of developing PRES. Additionally, pediatric ß-MT patients with a history of aGVHD, regardless of disease class, are more likely to develop PRES. Considering these results, PRES has a higher chance of being the etiology of symptoms and should be considered more often in these patients.

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ABSTRACT: Posterior reversible encephalopathy syndrome, or PRES, is often an unrecognized sequela of uncontrolled hypertension that is associated with a significant risk of mortality. It is poorly understood and can lead to endothelial dysfunction with disturbance of the blood-brain barrier and subsequent brain edema. Headache, seizures, encephalopathy, and visual disturbances are among the most common symptoms associated with PRES. Testing and evaluation should be quickly initiated not only to solidify the diagnosis but also to rule out others such as infection or stroke. Prompt identification of the symptoms of PRES can potentially reverse long-term side effects such as life-long seizures or visual deficits.

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Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological entity characterized by nonspecific symptomatology (eg, headache, visual disturbances, encephalopathy, and seizures) and classically cortical and subcortical vasogenic edema predominantly affecting the parietooccipital region. PRES etiologies are usually dichotomized into toxic PRES (eg, antineoplastic drugs, illicit drugs) and clinical condition-associated PRES (eg, acute hypertension, dysimmune disorders). Although the pathophysiology of PRES remains elusive, 2 main pathogenic hypotheses have been suggested: cerebral hyperperfusion due to acute hypertension and cerebral hypoperfusion related to endothelial dysfunction. Research into the pathogenesis of PRES has emerged through the development of animal models in the last decade. The motivation for developing a suitable PRES model is 2-fold: to fill in knowledge gaps of the pathophysiological mechanisms involved, and to open new perspectives for clinical assessment of pharmacological targets to improve therapeutic management of PRES. All current models of PRES have a hypertensive background, on which other triggers (acute hypertension, inflammatory, drug toxicity) have been added to address specific facets of PRES (eg, seizures). The initial model consisted in inducing a reduced uterine perfusion pressure that mimics preeclampsia, a leading cause of PRES. More recently, a model of stroke-prone spontaneously hypertensive rats on high-salt diet, originally developed for hypertensive small vessel disease and vascular cognitive impairment, has been studied in PRES. This review aims to discuss, depending on the research objective, the benefits and limitations of current experimental approaches and thus to define the desirable characteristics for studying the pathophysiology of PRES and developing new therapies.

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RATIONALE: Posterior reversible encephalopathy syndrome (PRES) is a rare complication commonly associated with headache and acute changes in blood pressure that results from a variety of causes, culminating in vasogenic cerebral edema in the occipital and parietal lobes of the brain. PATIENT CONCERNS: We report here a woman who suffered from headache, generalized tonic-clonic seizures, and cortical blindness in the late postpartum period. DIAGNOSES: Posterior reversible encephalopathy syndrome. INTERVENTIONS: The patient was treated with amlodipine besylate tablets for hypertension, dehydration with mannitol and glycerin fructose, and antispasmodic treatment with sodium valproate and oxcarbazepine. OUTCOMES: On day 2, the patient became conscious, headache and vision improved. One week later, symptoms and signs disappeared, blood pressure returned to normal, and brain MRI lesions disappeared in re-examination. LESSONS: Eclampsia associated with PRES is reversible in most cases, but it is a serious and potentially life-threatening obstetric emergency. If adequate treatment is provided in a timely manner, most women will make a full recovery. Attention needs to be paid to timely and adequate treatment, as well as appropriate follow-up and support for patients with PRES.

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RATIONALE: Posterior reversible encephalopathy syndrome is a neurological condition characterized by headache, convulsions, altered consciousness, and visual disturbance with specific radiological features, which is characterized by contrast enhancement in the occipital lobe on T2-weighted image. We report a case of sudden visual impairment of both eyes 6 days after childbirth diagnosed as postpartum preeclampsia and posterior reversible encephalopathy syndrome (PRES) through radiological examination. PATIENT CONCERNS: A 31-year-old female patient with headache and visual disturbance visited the clinic. DIAGNOSIS: Visual acuity was light perception in the right eye and hand motion in the left eye; pupillary light reflections of both eyes were normal. In the field of view test, the waveform was not observed in the defect pattern visual field power test, and the amplitude was greatly reduced in the visual field test. 1+ proteinuria was observed on urine test and magnetic resonance imaging showed contrast enhancement under both parietal and occipital cortex. INTERVENTIONS: Hospitalization was done for blood pressure control and examination of related disease under suspicion of PRES caused by postpartum preeclampsia. OUTCOMES: Four weeks after diagnosis, vision and visual field defects recovered to normal, and the previously observed lesion on magnetic resonance imaging completely improved 3 months after the initial visit, and it was diagnosed as PRES. LESSONS: PRES in postpartum preeclampsia can cause rapid vision and symptoms, visual field loss, and accurate follow-up diagnosis with relevant imaging and clinical patterns can improve vision.

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OBJECTIVE: To assess risk factors (RF) and severity grade of Posterior reversible encephalopathy syndrome (PRES) in children with hematological diseases. MATERIAL AND METHODS: We analyzed cases of PRES in children during chemotherapy (CT) and after allogeneic hematopoietic stem cell transplantation (allo-HSCT). We estimated the following RF: arterial hypertension, steroid therapy, CT, immunosuppressive therapy (IST), infection and renal injury. RESULTS: Thirty-five cases of PRES occurred in 32 patients (8 after allo-HSCT and 27 during CT) were included in this study. In the most of cases (94.3%), there were 2 and more RF. An increase in blood pressure level (88.6%), CT and IST (82.8%) administration, steroid therapy (71.4%) were the most significant for PRES development. Infectious process and the decline in renal function played a lesser role in this syndrome (31.4% and 14%). At the initial presentation of PRES, there were seizures (94.3%), a decrease of consciousness (28.6%), headache, vision disturbances and stomachache (20%). In the most of cases (91.4%), the 2nd and 3d grade according to the Common Terminology Criteria for Adverse Events (CTCAE 5.0) were observed. Brain magnetic resonance imaging (MRI) revealed the vasogenic edema of temporal (88.6%), occipital (74.3%), frontal (40%) lobes and the cerebellum (22.9%) more often than the cytotoxic edema (p=0.03). The cytotoxic edema was observed in the thalamus and the basal ganglia (2.9%) more often than in other parts of the brain (p<0.01). CONCLUSION: The majority of PRES cases are caused by more than two RF. Arterial hypertension does not have a leading role among its causes. There is a significant correlation between the grade of PRES according to CTCAE 5.0 score and RF number (p<0.05).

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