Symptoms Of Posterior Reversible Encephalopathy Syndrome Research Articles

Posterior reversible encephalopathy syndrome in children with acute lymphoblastic leukemia during remission induction chemotherapy: a single-center retrospective study.

This study investigated the clinical characteristics, cranial magnetic resonance imaging (MRI) features, MRI follow-up, and prognosis of children with acute lymphoblastic leukemia (ALL) who developed posterior reversible encephalopathy syndrome (PRES) during remission induction chemotherapy. We analyzed the age, gender, PRES symptoms and signs, cranial MRI findings, therapeutic effect, and prognosis of children with ALL who developed PRES during chemotherapy from January 2010 to December 2013 at the Hematology Oncology Center of Beijing Children's Hospital. Changes in cranial MRI findings were analyzed, and intelligence (IQ) and cognitive function were evaluated using the Wechsler Scale and the Wisconsin Card Score Test after the children completed chemotherapy. There were 850 children with newly diagnosed ALL in this period; 13 (1.5%), 6 boys and 7 girls, developed PRES. All were diagnosed as B-cell ALL. The median age at PRES onset was 7 years (2-11 years). The median day of PRES onset was day 28 (day 17-34) of remission induction chemotherapy. Of the 13 children with PRES onset and seizures, 4 had visual disturbances and 2 had consciousness disturbances. Cranial MRI showed hyperintensity in the subcortical white matter on T2-weighted axial and fluid-attenuated inversion recovery (FLAIR) images. The lesion locations were as follows: occipital lobe, 12 (92.3%) patients; frontal lobe, 7 (53.8%) patients; temporal lobe, 5 (38.4%) patients; parietal lobe, 3 (23.1%) patients; and cerebellum, 1 (7.7%) patient. There were 8 (61.5%) patients with vasogenic edema and 5 (38.5%) with cytotoxic edema. After treatment, all children recovered within one month, when their PRES symptoms were relieved, they continued to receive chemotherapy. However, 1 child (1.07%) died of severe central nervous system infection one year after PRES treatment, and 3 (25%) had recurrent seizures and were diagnosed with epilepsy after three months of PRES treatment. Their cranial MRIs showed cytotoxic edema, which was acute stage on day 15, with aggravated lesions on cranial MRI. The cranial MRI lesions returned to normal at one month in 3 (23.1%) patients, at three months in 6 (46.1%) patients, at one year in 8 (61.5%) patients, and at two years in 12 (92.3%) patients. The 12 surviving children all returned to school, and their full-scale, verbal, and performance IQs were normal, with no significant differences in intelligence or cognitive function compared with children with ALL without PRES during the same period. PRES can occur during remission induction chemotherapy treatment of children with ALL, but the incidence is low. Cranial MRI can be used for diagnosis and to characterize lesions. The children recover about a month after treatment, and cranial MRI lesions return to normal within two years. The time for complete resolution of MRI lesions differs, and children with cytotoxic edema have worse prognosis with sequelae, such as epilepsy, which requires close monitoring. PRES does not affect intelligence or cognitive development.

P15.01: Posterior Reversible Encephalopathy Syndrome in Transplant Patients: Diagnosis and Management

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a rare neurotoxic status composed of own brain imaging patterns. Depending on the location of the lesion, is characterized by a wide scale of neurologic symptoms. Although, the main cause of PRES is not fully enlightened, it is shown that, especially in solid organ transplant patients due to calcineurin inhibitors (CNI) toxicity, severe hypertension and any kind of infections are strongly related with PRES. The current therapy accepted is only supportive care. In this study we aim to evaluate diagnosis and management of PRES in our transplant patients. Method: From 3 November 1975 to 31 December 2021, we performed 3288 kidney transplant, 701 liver transplant and 142 heart transplants. Among these transplants 381 patient in kidney,334 patient in liver and 70 patients in heart were pediatric. 785 pediatric transplant patients were examined, and PRES was observed in 12 pediatric patients with median age 12 years (5 month - 18 year). 6 (50%) of these patients were male and median BMI is 18,5 (12,4 - 28,9). The radiology report database was analyzed for patients which PRES was cited in brain MR imaging text reports. Cases of PRES were included and searched for demographic values, laboratory values, clinical presentation, immunosuppressive they use, management we made and results. Results: Among PRES cases, 6 (50%) patients were liver transplant, 4 (33%) were kidney transplant and 2 (17%) were heart transplant patients. %66,5 (n:8) of the patients were using Tacrolimus as immunosuppressant. Causes of PRES were toxicity of Tacrolimus in 3 (25%) patients, hypertension in 4 (33%) patients, sepsis in 2 (16%) patients, hypomagnesemia in 1(8,5%) patient, hyponatremia in 1 (8,5%) patient and cyclosporine toxicity in 1(8,5%) patient. Symptoms of PRES were Seizure in 8 (66,5%) patients, vision change in 3 (25%) patients, status epilepticus in 1 (8,5%) patient and nausea/ vomiting in 1 (8,5%) patient. The average time between transplant and the diagnosis of PRES was 23 days (2 - 27 days). In brain MRI edema (n:5), hemorrhagic lesions (n:4), ischemia (n:1) and brain infarction (n:1) were observed. As medical treatment all patients consulted with pediatric neurology. Patients having hypertension (n:4) are treated with antihypertensives ACEİ combined with calcium canal blockers. Seizure is controlled with hydantoin at early period and levetiracetam (36.9 mg/kg/day) as maintenance treatment. Patients having hypertension are taken to hemodialysis. Also, patients related with tacrolimus toxicity (n:3) were switched to cyclosporine and the patient who had cyclosporine toxicity was switched to tacrolimus. End of the treatment period all patients were discharged successfully. Conclusion: PRES is a rare diagnosis which is having an unknown etiology and due to that doesn’t have a proven management strategy. When the patient is suspected of PRES, MRI should be performed immediately, in order to avoid complications, achieve a better recovery and treat PRES successfully.

Posterior Reversible Encephalopathy Syndrome: Incidence and Clinical Characteristics in Children With Cancer.

The etiology and outcomes of posterior reversible encephalopathy syndrome (PRES) in children with cancer are not well understood. We aim to determine the incidence of PRES, describe associated morbidity and mortality, and better understand risk factors in this patient population. A total of 473 children with a hematologic malignancy or postallogeneic hematopoietic cell transplantation between June 2015 and June 2020 were screened for PRES to determine incidence and whether age or underlying diagnosis are associated with development of PRES. We conducted a case-control study to evaluate whether comorbidities or chemotherapeutic agents are associated with PRES. Children with PRES were matched with 2 controls based on age and underlying diagnosis to identify additional risk factors. Fourteen patients developed PRES, with an incidence of 5.9/1000 people/year. Those diagnosed with PRES had commonly described PRES symptoms: hypertension, seizures, nausea/vomiting, altered mental status, and headaches. All patients received an magnetic resonance imaging, and most had findings consistent with PRES. Hematopoietic cell transplantation was associated with the development of PRES. The use of Etoposide was associated with PRES but comorbidities, steroids and calcineurin inhibitors were not. While PRES was infrequent in this population, it is associated with high morbidity and mortality, with ICU admissions and an overall hospital mortality, because of secondary causes, of 29%.

Posterior Reversible Encephalopathy Syndrome: Occurrence and Clinical Characteristics in Children with Cancer

This project was funded, in part, with support from the Indiana Clinical and Translational Sciences Institute funded, in part by UL1TR002529 from the National Institutes of Health. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. 
 
 Background: The diagnosis and outcomes of posterior reversible encephalopathy syndrome (PRES) in children with cancer are not well understood. We aim to determine the incidence of PRES in pediatric oncology patients, describe the associated morbidity and mortality, and to better understand risk factors in this patient population. 
 
 Project Methods: We screened 473 children with a hematologic malignancy or post-allogeneic hematopoietic cell transplantation (HCT) between June 2015 and June 2020 for PRES to determine incidence and if age or diagnosis is associated with PRES. To evaluate if comorbidities or chemotherapeutic agents are associated with PRES, we conducted a case-control study. Children with PRES were matched with two controls based on age and diagnosis to identify additional risk factors for PRES development. Incidence was calculated over the 5-year period. Mann Whitney U and Chi-Squared or Fisher Exact Tests were performed using SPSS v26 to compare continuous and categorical variables respectively. 
 
 Results: Fourteen (3.0%) patients developed PRES, resulting in an incidence of 5.9/1000 people/year. Median age was not different between those that developed PRES [14 years (IQR: 11, 17.25)] and those that did not [9.6 years (IQR: 3.8, 15.4)], (p=0.421). Allogenic HCT was associated with the development of PRES (p=0.019). PRES symptoms were common: hypertension (100%), seizures (79%), nausea/vomiting (50%), altered mental status (50%), and headaches (43%). All received an MRI, and 79% had findings consistent with PRES. The majority (79%) of patients with PRES were admitted to the PICU and 4 (29%) later died. After 2:1 matching, we found that the use of Etoposide (p=0.008) was associated with PRES but comorbidities were not. 
 
 Conclusion and Implications: While PRES was infrequent in this population, it carries a high morbidity with most requiring PICU admission and a high associated hospital mortality of 29%. The use of etoposide and HCT were associated with PRES.

MARIJUANA-INDUCED HYPERTENSION: A 'PRES'-ING MATTER

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiographic condition of varied etiologies which typically involves the white matter of the posterior cerebral hemispheres. Posterior circulation vasogenic edema is seen on MRI. Although the full extent of PRES is not well understood, patients of all age groups have been affected. Features include hypertension, seizures, and altered consciousness. It is prudent to consider PRES in stroke patients who present with vision abnormalities and altered mentation. We present a case with symptoms of PRES in the setting of marijuana induced hypertensive encephalopathy. CASE PRESENTATION: A 41-year-old male with history of previous CVA, seizures, hypertension, and polysubstance abuse presented after being found with altered mentation. The patient remained agitated despite receiving sedatives, required mechanical ventilation for airway protection. His BP was 180/104. He is a chronic cannabis user. The patient’s previous CVA, 5 years ago, resulted after CO poisoning and hypertensive emergency with subsequent encephalopathy. MRI at that time showed acute infarcts involving b/l occipital, temporal, parietal lobes, & thalami resulting in posterior CVA/cortical blindness, with development of seizures. MRA demonstrated vertebral artery dissection. MRI on admission revealed development of subcortical white matter signal changes in the parieto-occipital and frontal white matter. These findings are suggestive of PRES in this hypertensive patient, suggesting that this is an unusual presentation of the consequences of his chronic THC use causing hypertensive encephalopathy with recurrent PRES. DISCUSSION: There are various pathogeneses for development of PRES. Our patient’s presentation 5 years ago suggests that there was a component of both hypertension and hypoperfusion from ischemic stroke. Normal autoregulation preserves cerebral blood flow through arteriolar constriction/dilatation. As BP increases, cerebral arterioles dilate causing increased cerebral blood flow and brain hyperperfusion causing breakdown of the blood-brain-barrier permitting extravasation of blood products into brain parenchyma. This vasogenic edema contributes to symptoms of PRES. His current presentation is more due to hypertension, believed to be marijuana induced. CVS effects of marijuana are tachycardia, hypertension, increased levels of carboxyhemoglobin. Cannabinoids bind to cannabinoid receptors, CB1-CNS and CB2-immune system. There are few cases of cannabis induced ischemic stroke & hypertensive encephalopathy associated PRES. It is important to highlight that marijuana can result in systemic complications. CONCLUSIONS: Although called posterior reversible encephalopathy syndrome, it is not always reversible. PRES should be considered in patients presenting similarly to evaluate with appropriate imaging and begin treatment based on the comorbidities of the patient. Reference #1: Qiang Zhang, George Richerson, Nandakumar Narayanan Neurology Apr 2017, 88 (16 Supplement) P4.066 Reference #2: Uhegwu N, Bashir A, Hussain M, Dababneh H, Misthal S, Cohen-Gadol A. Marijuana induced Reversible Cerebral Vasoconstriction Syndrome. J Vasc Interv Neurol. 2015;8(1):36-38. DISCLOSURES: No relevant relationships by Laith Al-janabi, source=Web Response No relevant relationships by Saad Chaudhry, source=Web Response No relevant relationships by Yashar Eshman, source=Web Response No relevant relationships by Padmini Giri, source=Web Response No relevant relationships by Victoria Gonzalez, source=Web Response

Posterior reversible encephalopathy syndrome in children with acute lymphoblastic leukemia during remission induction chemotherapy: a single-center retrospective study.

This study investigated the clinical characteristics, cranial magnetic resonance imaging (MRI) features, MRI follow-up, and prognosis of children with acute lymphoblastic leukemia (ALL) who developed posterior reversible encephalopathy syndrome (PRES) during remission induction chemotherapy. We analyzed the age, gender, PRES symptoms and signs, cranial MRI findings, therapeutic effect, and prognosis of children with ALL who developed PRES during chemotherapy from January 2010 to December 2013 at the Hematology Oncology Center of Beijing Children's Hospital. Changes in cranial MRI findings were analyzed, and intelligence (IQ) and cognitive function were evaluated using the Wechsler Scale and the Wisconsin Card Score Test after the children completed chemotherapy. There were 850 children with newly diagnosed ALL in this period; 13 (1.5%), 6 boys and 7 girls, developed PRES. All were diagnosed as B-cell ALL. The median age at PRES onset was 7 years (2-11 years). The median day of PRES onset was day 28 (day 17-34) of remission induction chemotherapy. Of the 13 children with PRES onset and seizures, 4 had visual disturbances and 2 had consciousness disturbances. Cranial MRI showed hyperintensity in the subcortical white matter on T2-weighted axial and fluid-attenuated inversion recovery (FLAIR) images. The lesion locations were as follows: occipital lobe, 12 (92.3%) patients; frontal lobe, 7 (53.8%) patients; temporal lobe, 5 (38.4%) patients; parietal lobe, 3 (23.1%) patients; and cerebellum, 1 (7.7%) patient. There were 8 (61.5%) patients with vasogenic edema and 5 (38.5%) with cytotoxic edema. After treatment, all children recovered within one month,when their PRES symtoms were relieved, they continued to receive chemotherapy. However, 1 child (1.07%) died of severecentral nervous system infection one year after PRES treatment, and 3 (25%) had recurrent seizures and were diagnosed with epilepsy after three months of PRES treatment. Their cranial MRIs showed cytotoxic edema, which was acute stage on day 15, with aggravated lesions on cranial MRI. The cranial MRI lesions returned to normal at one month in 3 (23.1%) patients, at three months in 6 (46.1%) patients, at one year in 8 (61.5%) patients, and at two years in 12 (92.3%) patients. The 12 surviving children all returned to school, and their full-scale, verbal, and performance IQs were normal, with no significant differences in intelligence or cognitive function compared with children with ALL without PRES during the same period. PRES can occur during remission induction chemotherapy treatment of children with ALL, but the incidence is low. Cranial MRI can be used for diagnosis and to characterize lesions. The children recover about a month after treatment, and cranial MRI lesions return to normal within two years. The time for complete resolution of MRI lesions differs, and children with cytotoxic edema have worse prognosis with sequelae, such as epilepsy, which requires close monitoring. PRES does not affect intelligence or cognitive development.

Posterior Reversible Encephalopathy Syndrome Secondary to Cyclophosphamide in the Treatment of Pulmonary Renal Syndrome

SESSION TITLE: Nervous System Disorders in the ICU 2 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a clinical radiological syndrome, carrying a 15% mortality risk. It can lead to permanent neurological deficits and death if not identified and treated early. CASE PRESENTATION: A 22-year-old female with a history of Sjogren’s syndrome was admitted to the intensive care unit for acute respiratory failure. She was diagnosed with diffuse alveolar hemorrhage after bronchoalveolar lavage, along with concurrent acute glomerulonephritis. Labs were significant for creatinine of 1.47 mg/dL, as well as protein >500, large blood, and 54 RBCs/hpf on urinalysis. Urine protein to creatinine ratio was 8.3. Glomerulonephritis workup was significant for ANA 1:5120, positive anti-MPO IgG, but negative anti-dsDNA, negative anti-GBM, negative HIV and hepatitis serologies, as well as a negative syphilis screen. The patient was initially diagnosed with pulmonary renal syndrome due to MPO-associated vasculitis. She was treated with high dose pulse steroids, cyclophosphamide induction on day 2 of ICU, and 5 days of plasmapheresis with improvements in respiratory status, but not in renal function. On day 5 the patient developed acute nausea, vomiting, and tonic-clonic seizures. Her vital signs were significant for a blood pressure of 170/110 mmHg. Neurological exam was evident for lethargic appearance, and a right pronator drift. Based on documentation, a progressive increase in her blood pressure was observed since the start of cyclophosphamide. Non-contrast MRI of the brain confirmed posterior reversible encephalopathy syndrome (PRES), with evidence of vasogenic edema in the bilateral parietal, occipital lobes, and cerebellum. DISCUSSION: The most common signs and symptoms of PRES are acute hypertension, seizure, nausea and vomiting, and altered consciousness. The incidence of PRES is unknown but is reported in ages 2-90, and occurs more frequently in women. Reported risk factors associated with PRES are hypertension, renal disease, immunosuppressive therapy, eclampsia/preeclampsia, and autoimmune diseases. The pathogenesis is not fully understood, however current hypotheses suggest disruption of vascular autoregulation in the brain, leading to vascular leakage, vasogenic edema, and hemorrhage. Diagnosis involves a high degree of clinical suspicion, as there are no current guidelines available. Aggressive blood pressure control with rapid lowering of the diastolic pressure below 100 mmHg within 2-6 hours is the mainstay of therapy, along with removal of the inciting agent. CONCLUSIONS: It is important to recognize PRES early and initiate the appropriate management. Unlike its name, PRES is not always posterior, and may not always be reversible. If not treated, it can lead to permanant neurological disability and death. Reference #1: Pedraza, R. Posterior Reversible Encephalopathy Syndrome: A Review, Crit Care & Shock (2009) 12:135-143 DISCLOSURE: The following authors have nothing to disclose: Di Pan, Basera Sabharwal, Franco Vallejo No Product/Research Disclosure Information

Posterior reversible encephalopathy syndrome in a renal transplanted patient

Patient: Male, 28Final Diagnosis: Posterior reversible encephalopathy syndromeSymptoms: Headache • pain around umblical region • seizures • visual disturbancesMedication: Mycophenolate mofetilClinical Procedure: Treatment of parasitosis • antiepileptic treatment • control of hypertension • changing mycophenolate mofetil to everolimusSpecialty: TransplantologyObjective:Unusual or unexpected effect of treatmentBackground:Posterior reversible encephalopathy syndrome (PRES) is characterized by reversible neurological findings with clinical hallmarks such as headache, confusion, seizures, cortical visual disturbances, and other focal neurological signs.Case Report:A 28-year-old male patient was hospitalized secondary to diarrhea and abdominal pain. He had renal transplantation due to renal amyloidosis secondary to familial Mediterranean fever (FMF). In his clinical follow-up, he had seizures, hemiparesis, blurred vision, and vomited an Ascaris lumbricoides. MRI results led to diagnosis of PRES. Mycophenolate mofetil was changed to everolimus, his systolic blood was pressure kept below 140 mm hg, and his intestinal parasitosis was treated. During follow-up, he had no pain and no diarrhea. His neurological symptoms turned to normal within 48 hours and neuroradiological findings returned to normal within 2 weeks.Conclusions:PRES is a rare disorder of unknown incidence in renal transplantation patients. Early diagnosis is very important to prevent irreversible neurological sequelae. PRES is totally reversible with cessation of the offending agent, rapid control of hypertension, and treatment of the underlying disease. For early diagnosis and to reduce morbidity and mortality, stool sample examination should be made in patients taking immunosuppressive drugs.

Posterior Reversible Encephalopathy Syndrome

Posterior reversible encephalopathy syndrome (PRES) is a rare neurologic disorder characterized by an acute increase in blood pressure, and by headaches, altered mental status, seizures, and visual loss. It is usually seen on computed tomographic scans as white-matter vasogenic edema predominantly affecting the posterior occipital and parietal lobes of the brain. Risk factors include malignant hypertension, eclampsia, medications such as immunosuppressants (including tacrolimus and cyclosporine), chemotherapy, biotherapy, and renal failure. Early recognition of the signs and symptoms of PRES, particularly identifying and treating high blood pressure, can prevent permanent neurologic disability.

Blood Pressure Fluctuations in Posterior Reversible Encephalopathy Syndrome

Posterior reversible encephalopathy syndrome (PRES) can be a consequence of hypertensive crisis and is often associated with rapid fluctuations in blood pressure (BP). However, the role of these BP changes in the pathogenesis of PRES has not been formally studied. Our objective was to analyze the relationship between BP fluctuations and the occurrence of PRES. We identified consecutive patients who developed PRES in the hospital and compared them with randomly selected controls matched for age, gender, and history of hypertension (HTN). Systolic BP (SBP) and diastolic BP (DBP) were collected at 2-hour intervals over a 48-hour window before the onset of PRES symptoms. A profile of changes in the values of SBP, DBP, mean arterial pressure (MAP), and pulse pressure (PP) over the 48-hour window was summarized for each individual by calculating a single number (M value) using the approach by Service et al. Comparisons of these summary numbers between the 2 groups (cases and controls) were made with the Wilcoxon signed rank test because of the smaller sample size and paired nature of the data. All tests were 2-sided, and P < .05 was considered statistically significant. We analyzed the BP profiles in 25 cases of PRES and 25 controls. The median age of PRES patients was 54 years (range 31-72). Fourteen of them (56%) had a history of HTN. Hypertensive encephalopathy was considered the underlying cause of PRES in 13 patients (52%). At the time of the first symptoms of PRES, the mean SBP was 182 ± 20 mm Hg (range 218-145), DBP 95 ± 16 mm Hg (range 134-62), MAP 124 ± 15 (range 152-93), and PP 87 ± 18 (range 123-46). While BP was higher in PRES cases, the severity of HTN was variable and BP fluctuations were not significantly more common than in controls (P = .38 for SBP, .79 for DBP, .25 for MAP, and .73 for PP, respectively). Although acute HTN is frequent in patients with PRES, BP fluctuations do not appear to be more common in hospitalized patients who develop PRES compared with controls matched for age and history of HTN. Other predisposing factors must therefore contribute to the development of PRES.