Diagnosis Of Posterior Reversible Encephalopathy Syndrome Research Articles

Posterior reversible encephalopathy syndrome with functional and motor sequelae: A case report

Posterior Reversible Encephalopathy Syndrome (PRES) is a rare clinicoradiological neurological disorder, initially elucidated in 1996, Since this first description of PRES numerous case reports and case series, as well as retrospective observational studies describing the syndrome have been published. Importantly, no randomized controlled studies have been performed, We present the case of a young patient with no significant medical history who presented with neurological symptoms consistent with Posterior Reversible Encephalopathy Syndrome (PRES). Brain MRI confirmed the diagnosis of Posterior Reversible Encephalopathy Syndrome (PRES). Additionally, the etiological assessment yielded negative results. A notable aspect in our patient's case is the persistence of sequelae, characterized by dexterity and grip impairment on the right side, hindering various activities of daily living. The diagnosis of PRES is both radiological and clinical, with MRI playing a crucial role. Treatment is primarily symptomatic, sometimes requiring intensive care unit admission. Prognosis is favorable in the majority of cases, but severe complications and even death can occur. The reversibility of initial symptoms is well-documented in the literature. Long-term functional impairment outcomes following PRES have not been adequately characterized in the literature. Therefore, it is necessary to conduct follow-up screenings in more patients presenting both typical and atypical PRES syndromes. This will provide insight into whether the functional and motor sequelae of PRES are less reversible.

Posterior reversible encephalopathy syndrome: A rare complication of rituximab therapy in rheumatoid arthritis.

Rituximab, a murine-human chimeric monoclonal antibody targeting CD20-positive B lymphocytes, has established itself as an effective and relatively safe biologic therapy for patients with refractory rheumatoid arthritis. Most common side effects associated with its use include infusion related reactions and cytopenia. Rare adverse effects such as progressive multifocal leukoencephalopathy and posterior reversible encephalopathy syndrome (PRES) have also been reported. Diagnosis of PRES following rituximab treatment requires a high index of suspicion correlated with clinical and radiological features in individuals at risk. Early diagnosis and prompt treatment is associated with a favorable prognosis. We present a case of a young man who developed PRES following rituximab administration on account of active rheumatoid arthritis. Timely diagnosis and prompt treatment ensured his uneventful recovery without residual neurological deficit.

Posterior Reversible Leucoencephalopathy Syndrome: Case Series, Comments, and Diagnostic Dilemma.

To report a series of patients with clinical and radiological features suggestive of posterior reversible encephalopathy syndrome (PRES) related to diverse etiologies emphasizing its pathophysiological basis. Posterior reversible encephalopathy syndrome (PRES) may present with a broad range of clinical symptoms from headache and visual disturbances to seizure and altered mentation. Typical imaging findings include posterior-circulation predominant vasogenic edema. Although there are many well-documented diseases associated with PRES, the exact pathophysiologic mechanism has yet to be fully elucidated. Generally accepted theories revolve around disruption of the blood-brain barrier secondary to elevated intracranial pressures or endothelial injury induced by ischemia from a vasoconstrictive response to rising blood pressure or toxins/cytokines. While clinical and radiographic reversibility is common, long-standing morbidity and mortality can occur in severe forms. In patients with malignant forms of PRES, aggressive care has markedly reduced mortality and improved functional outcomes. Various factors that have been associated with poor outcome include altered sensorium, hypertensive etiology, hyperglycemia, longer time to control the causative factor, elevated C reactive protein, coagulopathy, extensive cerebral edema, and hemorrhage on imaging. Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS-spectrum disorders. Diagnosis of PRES in some circumstances can be challenging and structural imaging may not be sufficient to distinguish it from other differential diagnostic considerations like ADEM. Advanced imaging techniques, such as MR spectroscopy or positron emission tomography (PET) can provide additional information to determine the diagnosis. Such techniques are more useful to understand the underlying vasculopathic changes in PRES and may answer some of the unresolved controversies in pathophysiology of this complex disease. Eight patients with PRES resulting from different etiologies varying from pre-eclampsia/eclampsia, post-partum headache with seizures, neuropsychiatric systemic lupus erythematosus, snake bite, Dengue fever with encephalopathy, alcoholic liver cirrhosis with hepatic encephalopathy, and lastly reversible cerebral vasoconstriction syndrome (RCVS). Additionally, a diagnostic dilemma between PRES and acute disseminated encephalomyelitis (ADEM) was notable in one patient. Some of these patients did not have or only very transiently had arterial hypertension. PRES may underlie the clinical conundrum of headache, confusion, altered sensorium, seizures, and visual impairment. PRES need not necessarily be always associated with high blood pressure. Imaging findings may also be variable. Both clinicians and radiologists need to familiarize themselves with such variabilities.

An Unusual Case of Posterior Fossa Predominant Posterior Reversible Encephalopathy Syndrome (PRES) Leading to Acute Hydrocephalus (P10-7.002)

<h3>Objective:</h3> To discuss acute hydrocephalus as an unusual complication of Posterior Reversible Encephalopathy Syndrome. <h3>Background:</h3> Posterior Reversible Encephalopathy Syndrome (PRES) is a well-recognized clinical and radiologic syndrome typically characterized by parieto-occipital vasogenic edema along with neurological symptoms, that improves after management or withdrawal of offending factor, in most cases being acute uncontrolled hypertension. However, clinical presentation and radiological findings are variable and may encompass different cerebral structures. Although pathophysiology is not well established, failure of sympathetic autoregulation in the vertebrobasilar system is thought to be related. This failure leads to breakdown of the blood-brain barrier, hence the vasogenic-type edema appreciated on imaging. Acute obstructive hydrocephalus is a rare and potentially fatal complication of PRES. <h3>Design/Methods:</h3> A 56-year-old Caucasian female who presented due to encephalopathy, headache and falls in the setting of hypertensive emergency. CT (Computed Tomography) head showed hypodense pons, midbrain, cerebellum, and bilateral thalami with effacement of the fourth ventricle, ventriculomegaly in the lateral and 3rd ventricles with associated trans-ependymal edema. Due to initial concern of posterior fossa mass leading to acute hydrocephalus, neurosurgery was consulted, and extra ventricular drain (EVD) was placed for emergent CSF diversion. <h3>Results:</h3> MRI Brain with and without contrast showed no evidence of acute ischemia or enhancing lesions. Severe edema was redemonstrated within the pons, midbrain, bilateral thalami, and cerebellum. Susceptibility weighted imaging showed microhemorrhages confined to the brainstem. The patient’s clinical exam improved after blood pressure management and EVD placement. Repeat head CT showed decreased cerebral edema and improvement of fourth ventricle obstruction. Based on presentation of hypertensive encephalopathy with posterior circulation vasogenic edema that improved with blood pressure management, PRES diagnosis was made. <h3>Conclusions:</h3> Posterior fossa predominant PRES is an uncommon presentation that if overlooked may lead to life threatening complications. Early recognition of clinical manifestations and radiological findings may lead to favorable outcomes. <b>Disclosure:</b> Dr. Morales-Nunez has nothing to disclose. Dr. Baez-Lugo has nothing to disclose. Dr. Yalcin has nothing to disclose. Dr. Shah has nothing to disclose. Dr. Garcia has nothing to disclose. Dr. Nichols has nothing to disclose.

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.

P1490: CHILDREN WITH SICKLE CELL DISEASE AND MOYAMOYA SYNDROME: A 10-YEAR EXPERIENCE

Background: Sickle cell anemia (SCD) is a multiorgan disease. Involvement of the central nervous system (CNS) is associated with increased mortality. Cerebrovascular accidents, especially when associated with permanent neurological deficits, are one of the most devastating potential outcomes of SCD. SCD can be complicated by Moyamoya syndrome (MMS), which is characterized by progressive stenosis of the supraclinoid segment of carotid arteries and development of typical collaterals, resulting in increased risk for both ischemic and hemorrhagic strokes. Aims: We describe the clinical characteristics of children with SCD and MMS treated at our center over the last 10 years and define the prevalence of MMS in our setting. Methods: We conducted a retrospective, descriptive study of all patients with SCD who were diagnosed with cerebrovascular disease and with MMS in our institution from 2012 to 2021. Indications for neuroimaging [(magnetic resonance imaging (MRI) or magnetic resonance angiography (MRA)] in our center include recurrent episodes of syncope, epileptic seizures, neurocognitive changes, time-averaged maximum mean velocity (TAMMV) of the middle cerebral or intracranial internal carotid arteries (ICA) measured ≥200 cm/s or before starting regular transfusion regime. Relevant clinical data was collected and summarized using descriptive statistics. Results: The study included 109 patients with SCD and 19,3% of them (21 patients) required neuroimaging. Within these patients, 6 presented with acute focal neurological signs that resulted in a diagnosis of ischemic stroke in four patients, and posterior reversible encephalopathy syndrome diagnosis in the other two. Besides these, 2 patients (9,5%) were diagnosed by neuroimaging with MMS and 1 (4,8%) had a pattern suggestive of MMS on brain MRI not confirmed by angiography. Furthermore, whereas four (19%) patients had no imaging findings suggestive of cerebrovascular disease, they presented with TAMMV ≥200cm/s, so they were proposed to regular transfusion regimen for primary prevention of stroke. Regarding the patients diagnosed with SCD and MMS, MMS was diagnosed at 9 years old in one patient and at 10 years old in another. Both patients had a SS genotype and were diagnosed following an abnormal surveillance transcranial doppler study, since they were asymptomatic for the disease. One of the patients was under erythrocytapheresis protocol and achieved a HbS level <20%. Currently she is without critical stenosis or cerebrovascular events, maintaining only imaging follow-up. The other patient was under regular transfusion regimen with HbS levels between 40 and 50%, and has been proposed for sibling bone marrow transplantation and encephaloduroarteriosynangiosis. Summary/Conclusion: The prevalence of MMS in our center (1,8%) is lower than described in other studies. Recent guidelines recommend at least a one-time MRI screening in early-school-age children to detect cerebrovascular disease earlier and reduce the burden of CNS complications in children and adults with SCD. By following these new recommendations, we expect an earlier and more frequent diagnosis of MMS, thus reducing the morbidity of these patients.

Comparison of Clinical Manifestations, Laboratory, Neuroimaging Findings, and Outcomes in Children With Posterior Reversible Encephalopathy Syndrome (PRES) in Children With and Without Renal Disease

BackgroundTo demonstrate and compare the clinical manifestations, laboratory findings, and neuroimaging findings of posterior reversible encephalopathy syndrome (PRES) in children with and without underlying renal disease. MethodsThe study included 23 children with a diagnosis of PRES from January 2009 to March 2019. All data, including clinical manifestations, laboratory findings, underlying medical illness, and neuroimaging results, were obtained. ResultsSixteen had underlying renal disease. The median age of PRES onset was 10.3 years in children with renal disease and 9.8 years in children without renal disease. Higher blood pressure at the baseline, on admission, and at the onset of PRES was found in the renal disease group more than in the nonrenal disease group (P < 0.05). Seizures were likely seen in the renal disease group compared with the nonrenal disease group (P = 0.03). Generalized tonic-clonic seizures were the most common seizure type in both groups. An initial CT scan revealed vasogenic edema in 75% of the renal group and 85.7% of the nonrenal group. During a long-term follow-up, all children recovered without significant neurological deficits or subsequent epilepsy. ConclusionsHypertension and higher baseline blood pressure are more common in children with renal disease who develop PRES compared with nonrenal disease. Seizures are more common in the renal disease group. A computed tomographic (CT) scan can help with PRES diagnosis when magnetic resonance imaging is not available. All children with PRES recovered without significant neurological deficits or subsequent epilepsy.

Posterior Reversible Encephalopathy Syndrome: An Unusual Complication of Benzodiazepine Poisoning: A Case Report

Posterior Reversible Encephalopathy Syndrome (PRES), also known as Reversible Posterior Leukoencephalopathy Syndrome, presents with rapid onset symptoms, including headache, seizures, altered consciousness, and visual disturbance. It is seen most frequently in settings of acute hypertension and is usually related to eclampsia. Only a few cases in the literature described PRES syndrome following benzodiazepines. We present a young male with benzodiazepine poisoning brought to the hospital in a deep coma, hypoxia, acidosis, and shock. Diagnosis of PRES was made on history, clinical examination, and radiologic findings of symmetric bilateral hyperintensities on T2 weighted Magnetic Resonance Imaging (MRIs) representing vasogenic edema.

Posterior Reversible Encephalopathy Syndrome: An Unusual Complication of Benzodiazepine Poisoning: A Case Report

Posterior Reversible Encephalopathy Syndrome (PRES), also known as Reversible Posterior Leukoencephalopathy Syndrome, presents with rapid onset symptoms, including headache, seizures, altered consciousness, and visual disturbance. It is seen most frequently in settings of acute hypertension and is usually related to eclampsia. Only a few cases in the literature described PRES syndrome following benzodiazepines. We present a young male with benzodiazepine poisoning brought to the hospital in a deep coma, hypoxia, acidosis, and shock. Diagnosis of PRES was made on history, clinical examination, and radiologic findings of symmetric bilateral hyperintensities on T2 weighted Magnetic Resonance Imaging (MRIs) representing vasogenic edema.

Abstract TMP51: Predictors Of Severe Disease In Posterior Reversible Encephalopathy Syndrome

Background: Despite its name, posterior reversible encephalopathy syndrome (PRES) is not always fully reversible and there might be residual neurologic sequelae or even mortality in a significant minority of patients. Factors predisposing to severe disease course in PRES are largely unclear. The aim of this study was to evaluate the predictors of in-hospital mortality and adverse discharge disposition in patients hospitalized due to PRES. Methods: Hospitalizations due to the primary diagnosis of PRES with known discharge disposition were identified from the Nationwide Readmissions Database 2016-2018. Survey statistics were used to compute national estimates. Univariate and multivariable logistic regression analyses were used to identify factors associated with the study outcomes. P-value &lt;0.01 was considered significant. Results: There were 9,298 patients with PRES (mean±SD age: 56.2±17.6 years; female: 71.2%). Of these, 207 (2.2%) had in-hospital mortality and 4,002 (43.0%) had adverse discharge disposition, defined as discharge to a short-term hospital, a rehabilitation facility, home with in-home healthcare or hospice. Figure 1 shows the independent predictors of the study outcomes. Advanced age, history of cancer, concurrent sepsis, coma, ischemic stroke, and mechanical ventilation were associated with higher in-hospital mortality. Whereas advanced age, female sex, higher Charlson comorbidity index score, mechanical ventilation, and longer length of hospital stay were associated with a higher likelihood of adverse discharge disposition. Conclusion: Nearly half of the hospitalized patients with PRES have severe disease. This study identifies several clinical predictors of severe disease course in PRES.

Abstract WP82: Early Readmissions After Hospitalization For Posterior Reversible Encephalopathy Syndrome

Background: While most patients with posterior reversible encephalopathy syndrome (PRES) have clinical and radiological reversibility, a significant proportion can have residual neurologic sequelae and longstanding morbidity. The aim of the study was to evaluate the rate, etiologies, and risk factors for 90-day readmissions following hospitalization for PRES. Methods: Data were obtained from the Nationwide Readmissions Database 2016-2018. Hospitalizations with primary diagnosis of PRES, survival to discharge, and known discharge disposition were included in the study. Primary outcome was non-elective readmission within 90 days of discharge. Survival analysis was performed, and independent predictors of readmission were analyzed using multivariable Cox proportional hazards regression. P&lt;0.01 was considered significant. Results: There were 6,155 hospitalizations that met the study inclusion criteria (mean±SD age: 55.9±17.3 years, female: 71.0%). Non-elective readmission within 90 days of discharge occurred in 1,922 (31.2%) patients. The most common reasons for readmissions were infections (20.0%), hypertensive disorders (12.4%), fluid/electrolyte imbalance or renal problems (8.8%), gastrointestinal problems (7.2%), continued/recurrent symptoms related to PRES (6.3%), cardiac problems (5.5%), seizures (4.6%), and acute cerebrovascular events (4.4%). Age was inversely associated with risk of readmissions [hazards ratio (HR): 0.91 for every 10 years increase in age, 95% confidence interval (CI): 0.87-0.95, P &lt;0.001]. Patients with chronic kidney disease (HR: 1.24, 95% CI: 1.06-1.46, P 0.009) and higher Charlson comorbidity index score (HR: 1.10, 95% CI: 1.07-1.14, P &lt;0.001) were more likely to be readmitted. Further, patients with longer length of stay (HR: 1.01, 95% CI: 1.00-1.02, P 0.002) and those not discharged to home (HR: 1.35, 95% CI: 1.16-1.58, P &lt;0.001) during the index hospitalizations were also at a higher risk for readmission. Conclusions: Nearly one-third of patients with PRES get readmitted within 90 days of discharge. While most of the readmissions are due to non-neurologic etiologies, ongoing symptoms related to PRES, seizures, and acute cerebrovascular events contribute to about 15% of all readmissions.

Very Early Onset Severe Preeclampsia and Eclampsia Syndrome with Posterior Reversible Encephalopathy Syndrome- A Catastrophe

Early onset severe preeclampsia and eclampsia is a direct cause in the deadly triad of maternal mortality along with haemorrhage and sepsis with high perinatal mortality. Antiphospholipid Syndrome (APS) with Recurrent Pregnancy Loss (RPL) may also result in early onset severe preeclampsia, eclampsia and its catastrophic complications. Present report is a rarest case of a 33-year- old, unbooked G6A5 at 21+4 weeks gestation with eclampsia, HELLP (Haemolysis, Elevated liver Enzymes, Low Platelet count) syndrome, Posterior Reversible Encephalopathy Syndrome (PRES), Intrauterine foetal demise (IUFD) with RPL due to APS with Hepatitis B positive status. Patient was managed by a multidisciplinary team of Obstetricians, Intensivists, Cardiologists and Neurologists. Eclampsia was managed with magnesium sulphate (Pritchard’s regime), intravenous labetalol and delivery of stillborn male foetus weighing 645 gm achieved within 24 hours of eclamptic seizure. Lupus anticoagulant and beta-2 glycoprotein IgM (Immnoglobulin M) antibodies were positive in high titres confirming APS. Postdelivery patient had persistent headache with blurring of vision. A diagnosis of PRES was confirmed on Magnetic Resonance Imaging (MRI) brain. Patient was put on Levetiracetam and low molecular weight heparin and was discharged in satisfactory condition on seventh postpartum day with an advice to follow- up at two weeks, six weeks and 12 weeks. Early onset preeclampsia is encountered in Obstetrics but very early onset severe preeclampsia (&lt;24 weeks of gestation) and eclampsia with HELLP syndrome, PRES in a patient of RPL due to APS is rarest. Early diagnosis of APS and early onset preeclampsia with timely referral to tertiary care and multidisciplinary management can save women from severe dreaded complications with a good obstetric outcome in future.

Clinical, imaging spectrum and outcome of PRES

Background: Posterior reversible encephalopathy syndrome (PRES), also called the acute hypertensive encephalopathy and reversible leukoencephalopathy syndrome (RPLS), is a clinico-radiological syndrome presents with rapid onset of headache, seizures, loss of consciousness, visual loss and characterized by white matter vasogenic edema affecting parietal and occipital lobes of the brain predominantly. However, the imaging findings are variable and may occur in other locations such as the frontal lobes, thalami, basal ganglia and brainstem. Objective: To study the clinical, imaging spectrum and final disease outcome of PRES Materials And Methods: The study was conducted on 52 patients who presented with clinical features and radiological diagnosis of PRES The clinical, imaging features and outcome of each patient were analyzed Results: The study was conducted on 52 patients with age range from below 10yrs to 65 yrs. Patients presented with various symptoms of which seizures(84.6%) is most common followed by headache(59.6%), vomiting (36.5%), visual disturbances(32.7%), altered sensorium(15.3%), thalamic aphasia (5.7%), hemiparesis(3.8%), paresthesia(3.8%), ataxia(3.8%), Quadriparesis (3.8%) and facial numbness (1.9%) is the least. On MR imaging typical parieto-occipital lobe involvement(98%)is seen in most of the cases, however other atypical regions involved were frontal lobe(52%),temporal lobe (17.3%), cerebellum (27%),thalamus (15.3%), brainstem (13.4%), basal ganglia (9.6%) and corpus callosum (1.9%) Lesions in atypical locations also had lesions in typical locations in all cases except in one case of central PRES. Dominant parieto-occipital pattern was seen in 32%,superior frontal sulcus pattern in 27%,holohemispheric pattern in 34.6%,Partial or asymmetric expression of primary patterns in 5.2% cases Diffusion restriction and postcontrast enhancement was seen in less than 25% cases. Complete resolution of symptoms was seen in 90.5% cases,3.8% of them succumbed to death. On follow up 5.7% of cases showed persistence of symptoms. Conclusion: PRES is a clinico-radiological syndrome with varied clinical and imaging spectrum. Involvement of atypical regions is not uncommon. However lesions in atypical locations often have lesions in typical locations also.Atypical imaging features like restricted diffusion and contrast enhancement are also not uncommon. Knowledge of atypical lesion location, atypical imaging features is necessary for the clinicians and radiologists not to misdiagnose PRES in the appropriate clinical setting

Neurotoxic complications of chemotherapy in children: posterior reversible encephalopathy syndrome

Introduction. The syndrome of posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological condition caused by vasogenic brain edema due to elevated blood pressure. The development of this syndrome is associated with the toxic effect of drugs in the treatment of oncological diseases. In most cases, timely diagnosis and therapy of PRES determine the complete reversibility of symptoms. However, a poor prognosis is possible with the development of severe disabling consequences or death. In practice, there are difficulties in the diagnosis of PRES due to the lack of specificity of clinical manifestations and awareness of many doctors about the existence of this syndrome.The aim of the study was to present the clinical-radiological aspect of the syndrome of posterior reversible encephalopathy in children with oncological diseases.Materials and methods. The analysis of 8 medical histories of children with oncological pathology complicated by the syndrome of posterior reversible encephalopathy was carried out.Results. The study group was dominated by patients with hemato-oncological diseases. The development of PRES occurred during chemotherapy and in most cases was not associated with arterial hypertension. The clinical picture was dominated by such symptoms as impairment of consciousness, seizures and movement disorders in the form of paresis. When carrying out magnetic resonance imaging (MRI), the lesion of the white matter of the brain was determined, mainly in the projection of the parieto-occipital regions. During treatment, all patients showed reversibility of neurological symptoms with leveling of radiological signs.Discussion. When analyzing the clinical and radiological characteristics of PRES in children, a comparison of the data with clinical observations presented in the literature was noted. The role of chemotherapy as a triggering factor in the development of posterior reversible encephalopathy syndrome has been determined.Conclusion. The necessity of including the syndrome of posterior reversible encephalopathy in the list of differential diagnoses in patients receiving chemotherapy is shown.

Neuroimaging findings of posterior reversible encephalopathy syndrome (PRES) following haematopoietic stem cell transplantation in paediatric recipients

BackgroundHaematopoietic stem cell transplantation (HSCT) is used worldwide in various malignant and nonmalignant childhood diseases, including haematologic, genetic, autoimmune and metabolic disorders, and is the only curative treatment for many of these illnesses. The survival rates of many childhood diseases have been increased due to HSCT treatment. However, associated complications are still important for management. Central nervous system (CNS) complications in paediatric HSCT recipients can be associated with high morbidity and significantly contribute to mortality. Posterior reversible encephalopathy syndrome (PRES) is one of the most common CNS complications in patients with neurological symptoms following HSCT. Magnetic resonance imaging (MRI) is the modality of choice and shows typical bilateral vasogenic oedema at the posterior parts of the cerebral hemispheres; however, various atypical imaging manifestations can also occur. In this study, we retrospectively examined CNS complications in our paediatric HSCT recipients with a focus on the typical and atypical neuroimaging manifestations of PRES following HSCT.MethodsWe retrospectively reviewed the medical records of 300 consecutive paediatric HSCT recipients from January 2014 to November 2018. A total of 130 paediatric HSCT recipients who experienced neurological signs and symptoms and were evaluated with neuroimaging studies following HSCT were enrolled in the study. The timing of CNS complications was defined according to immune status, including the pre-engraftment period (< 30 days after HSCT), the early postengraftment period (30–100 days after HSCT), and the late postengraftment period (> 100 days after HSCT), which were defined as phases 1, 2 and 3, respectively.ResultsOverall, 130 paediatric HSCT recipients experienced neurological signs and symptoms and therefore underwent neuroimaging examinations. Among these 130 patients, CNS complications were present in 23 patients (17.6%, 23/130), including 13 (56.5%) females and 10 (43.5%) males with a median age of 8.0 years (range, 8 months to 18.0 years). Among these 23 patients, 14 cases of PRES (60.9%), 5 (21.7%) cases of leukoencephalopathy, 3 cases of acute subdural haemorrhage (ASDH) (13%) and 1 (4.3%) case of fungal CNS infection were identified by neuroimaging. On MRI, typical parietooccipital vasogenic oedema was present in 78.5% of the PRES cases (11/14). The following atypical neuroimaging manifestations were observed: isolated involvement of the bilateral frontal lobes in 1 case, isolated cerebellar vermis involvement in 1 case, and isolated basal ganglia involvement in 1 case. Restricted diffusion associated with cytotoxic damage was demonstrated in 2 of 14 cases, one of which also showed subacute cytotoxic injury with ADC pseudonormalization.ConclusionPaediatric HSCT recipients presenting with CNS signs and symptoms should be evaluated by neuroimaging studies for timely diagnosis and early management. PRES is the most common CNS complication and may present with atypical MRI manifestations, which should not dissuade a PRES diagnosis in appropriate clinical settings.

MRI characteristics of brain edema in preeclampsia/eclampsia patients with posterior reversible encephalopathy syndrome

BackgroundThe neuroimaging manifestations of eclampsia and preeclampsia often overlap, mainly presenting as posterior reversible encephalopathy syndrome (PRES). The purpose of this retrospective study was to compare the extent and nature of brain edema in eclampsia and preeclampsia patients with PRES based on MRI characteristics.MethodsOne hundred fifty women diagnosed with preeclampsia-eclampsia and undergoing cranial MRI were enrolled; 24 of these were diagnosed as having eclampsia. According to clinicoradiologic diagnosis of PRES, eligible patients were classified as having eclampsia with PRES (group E-PRES) and preeclampsia with PRES (group P-PRES). A scale on T2W FLAIR-SPIR images was established to evaluate the extent of brain edema, and the score of brain edema (SBE) of both groups was compared. In patients of the two groups who also underwent DWI sequence, the presence or absence of hyperintensity on DWI and hypointensity on ADC maps were determined to compare the nature of brain edema. Furthermore, clinical and biochemical data of the two groups were compared.ResultsThe incidence of PRES in eclampsia patients was significantly higher than that in preeclampsia patients (87.50% vs. 46.03%, P<0.001). The SBE of all regions and typical regions in group E-PRES patients were significantly higher than those in group P-PRES patients (15.88±8.72 vs. 10.90±10.21, P=0.021; 8.52±3.87 vs. 5.01±4.19, P=0.002; respectively). The presence of hyperintensity on DWI was determined more frequently in group E-PRES patients than group P-PRES patients (71.43% vs. 32.00%, P=0.024). Age, systolic blood pressure, white blood cell count, neutrophil count and percentage of neutrophils were significantly different between the two groups (P<0.05).ConclusionsCertain MRI characteristics that reflect the extent and nature of brain edema were different between eclampsia and preeclampsia patients with PRES. Additional prospective studies are still required to explore whether these MRI characteristics of brain edema may further become a potential predictor for eclamptic seizures in preeclampsia patients with PRES.

Posterieur reversibel encefalopathiesyndroom in het postpartum

A late postpartum complication The posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological syndrome characterized by acute neurological symptoms such as headaches, visual symptoms, altered consciousness and epileptic seizures in combination with subcortical vascular edema. One of the possible causes of PRES is pre-eclampsia. Observational studies show that some women with new-onset hypertension in the postpartum have an imbalance in pro- and anti-angiogenic factors, as in women with antepartum pre-eclampsia. Based on this case report, this potentially dangerous clinical entity is described. The 38-year-old primiparous patient presented 17 days after childbirth with a headache and severe hypertension. The diagnosis of PRES was made via an MRI. There was a good clinical response among chlorthalidone, bisoprolol and amlodipine. Sequential MRI images showed reversibility of the lesions. Regular blood pressure monitoring in women who recently gave birth, is important. New-onset hypertension is an alarm sign for pre-eclampsia in this patient group. Brain imaging is strongly recommended in case of the concomitant presence of risk factors of a headache. Early diagnosis of PRES and rapid treatment are necessary to prevent permanent neurological damage and mortality.

Mesial Temporal Sclerosis as Late Consequence of Posterior Reversible Encephalopathy Syndrome in Pediatric Hemato-oncologic Patients.

Drug resistant epilepsy has rarely been reported following posterior reversible encephalopathy syndrome (PRES), with few cases of mesial temporal sclerosis (MTS). The aim of this study was to report clinical and neuroimaging features of MTS subsequent to PRES in hemato-oncologic/stem cell transplanted children. Among 70 children treated in 2 pediatric hemato-oncologic Italian centers between 1994 and 2018 and presenting an episode of PRES, we retrospectively identified and analyzed a subgroup of patients who developed epilepsy and MTS. Nine of 70 patients (12.8%) developed post-PRES persistent seizures with magnetic resonance imaging evidence of MTS. One patient died few months after MTS diagnosis, because of hematologic complications; the remaining 8 patients showed unprovoked seizures over time leading to the diagnosis of epilepsy, focal in all and drug resistant in 4. At PRES diagnosis, all patients with further evidence of epilepsy and MTS suffered of convulsive seizures, evolving into status epilepticus in 3. In 3 patients a borderline cognitive level or intellectual disability were diagnosed after the onset of epilepsy, and 2 had behavioral problems impacting their quality of life. MTS and long-term focal epilepsy, along with potential cognitive and behavioral disorders, are not uncommon in older pediatric patients following PRES.

Clinical Presentation and Risk Factors for Poor Outcomes Among Adult Patients With Posterior Reversible Encephalopathy Syndrome: A Retrospective Cohort Study.

Posterior reversible encephalopathy syndrome (PRES) is an acute neurological condition with unknown global incidence, variable clinical presentation, and prognosis. To describe a cohort of patients with PRES with a focus on brain magnetic resonance imaging (MRI) patterns and their relationship with short-term clinical outcomes. Retrospective cohort study. The authors included patients if they were older than 15 years and had a PRES diagnosis on the basis of a positive brain MRI at any time during the in-hospital stay. Forty-four patients were included in the present analysis. The median age was 57 years (interquartile range, 32.0-68.5) and 70.5% were women. Hypertension (59.1%), history of transplantation (27.3%), previous chemotherapy (27.3%), chronic renal failure (38.6%), and autoimmune disease (15%) were the main comorbid conditions present. The classic triad of seizures, headache, and visual impairment was present in 18.0% of the cases. Eighty-six percent of patients were admitted to the intensive care unit, with 36.0% needing invasive life support. Brain MRI showed a dominant parieto-occipital pattern in 26 patients, whereas cytotoxic edema and bleeding were present in 27.3% and 29.6%, respectively. In-hospital mortality was 11.4%. The median modified Rankin Scale at hospital discharge was 1 (0-2.5). Risk factors associated with low modified Rankin Scale scores were: headache, visual impairment, and parieto-occipital pattern. Decreased level of consciousness and mechanical ventilation requirement were associated with greater discharge disability. Characteristic symptoms and signs of PRES and classic MRI patterns are associated with better clinical outcomes.

Abstract WP220: Can’t Breathe, Can’t See, Can’t Think: Chronic Obstructive Pulmonary Disease Increases the Risk of Posterior Reversible Encephalopathy Syndrome

Introduction: Several theories exist regarding the pathogenesis of posterior reversible encephalopathy syndrome (PRES). One theory suggests that PRES occurs when systemic blood pressure exceeds the upper limit of cerebral autoregulation. Endothelial dysfunction has been proposed as an alternative pathogenesis to account for PRES outside the setting of acute hypertension. This mechanism has been implicated in other conditions associated with PRES including autoimmune diseases, cytotoxic medications, sepsis, and eclampsia. The purpose of this study was to determine if COPD, a disease known to cause endothelial dysfunction, has a causative association with the development of PRES. Methods: A single center retrospective, age-matched, case-control study was performed from January 2013 to June 2019 comparing patients discharged with a primary diagnosis of PRES to a control group with acute ischemic stroke. Demographics, medical comorbidities, initial blood pressure, and clinical outcomes were compared between the two groups. For categorical variables, p-values were calculated using χ2 and Fisher’s exact tests. For continuous variables, p-values were calculated using two-sample t-tests. The effect of COPD and acute hypoxic respiratory failure on PRES status was investigated using multivariate logistic regression. Results: A total of 94 PRES subjects and 109 control subjects were included for analysis. Mean age did not differ between the two groups; however, the PRES group was more likely to be female (78.7% vs. 49.5%, p&lt;0.001). COPD was present in 26.6% (n=25) of cases and 11% (n= 12) of controls (odds ratio 4.12, p=0.003). Occurrence of hypertension did not differ significantly between the two groups (78.0% vs 86.2%). Among patients with PRES in the setting of COPD (n=25), 60% (n=16) did not meet criteria for hypertensive emergency. Controlling for hypertensive emergency status in a multivariate logistic regression analysis, patients with COPD were 3.21 times more likely to develop PRES (p= 0.004). Conclusions: To our knowledge, very few reports of PRES in the setting of COPD have been described in the literature and no association of PRES and COPD has been defined to date. Our data support the role of COPD as a risk factor in the development of PRES.