Posterior Reversible Encephalopathy Syndrome Research Articles

Recurrent exercise-induced acute kidney injury associated with hypouricemia: a case report and literature review

BackgroundHereditary renal hypouricemia (RHUC) is a heterogenous disorder characterized by defective uric acid (UA) reabsorption resulting in hypouricemia and increased fractional excretion of UA. RHUC is an important cause of exercise-induced acute kidney injury (EIAKI), nephrolithiasis and posterior reversible encephalopathy syndrome (PRES). We present here an unusual case of a patient with RHUC who presented with recurrent EIAKI and had two heterozygous mutations in the SLC2A9 gene.Case presentationA 43-year old man was admitted to our clinic because of bilateral loin pain, nausea and sleeplessness for 3 days after strenuous exercise. The laboratory results revealed increased levels of blood urea nitrogen (BUN) (15 mmol/l) and serum creatinine (Scr) (450 μmol/l), while the UA level was extremely low at 0.54 mg/dl, and his fractional excretion of urate (FE-UA) was 108%. The patient had an episode of acute kidney injury after playing soccer approximately 20 years ago, and on routine physical examination, his UA was less than 0.50 mg/dl. In view of the marked hypouricemia and high FE-UA, a diagnosis of RHUC was suspected, which led us to perform mutational screening of the SLC22A12 and SLC2A9 genes. DNA sequencing revealed no mutation in SLC22A12 gene, but two heterozygous mutations in the SLC2A9 gene.ConclusionsThis is a rare report of a patient with RHUC2 due to the mutation of SLC2A9. And this unique symptom of EIAKI and decreased or normal serum concentrations of UA warrant more attention as an early cue of RHUC.

The Association of Hypertension With Posterior Reversible Encephalopathy Syndrome in Systemic Lupus Erythematosus Patients: A Systematic Review.

Posterior reversible encephalopathy syndrome (PRES), also known as reversible posterior leukoencephalopathy syndrome (RPLS), is a rare disorder that most commonly affects the posterior part of the brain. Two common causes of PRES are hypertension and autoimmune diseases such as systemic lupus erythematosus (SLE). This systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 recommendations and aimed at finding the association between hypertension and PRES in SLE patients. We searched medical databases such as PubMed, PubMed Central(PMC), Cochrane Library, and Multidisciplinary Digital Publishing Institute (MDPI) for relevant medical literature. The identified papers were screened, subjected to inclusion and exclusion criteria, and ran through quality appraisal tools, after which 16 papers were finalized. The finalized papers explored the roles of hypertension in SLE patients diagnosed with PRES. In this review, we identified a link between hypertension and PRES-SLE patients. We aimed to explain the role of hypertension in the development of PRES in SLE patients. This study also explains the different treatment modalities to be used for treating the patients presenting with PRES and differentiates other neuropsychiatric illnesses commonly present in SLE patients from PRES. It's important to make an accurate clinical diagnosis by understanding the clinical features and neuroimaging results of PRES for future care since it may even be incurable in some circumstances.

Posterior reversible encephalopathy syndrome and stem cell transplantation in sickle cell disease: Case series and literature review

Posterior reversible encephalopathy syndrome and stem cell transplantation in sickle cell disease: Case series and literature review

COVID-19 and Brain complications in adult and pediatric patients: A review on neuroimaging findings

In this review, we will discuss the neuroimaging findings of patients with COVID-19 from the outbreak (late December 2019) to the end of October 2021. PubMed, Scopus, Google Scholar, Science Direct, ProQuest, Web of Science and the World Health Organization database (January 01, 2020, to October 30, 2021) were searched for related published articles. In each of the databases, the appendix search strategies were performed and the below keywords were used: COVID-19”OR“coronavirus disease 2019” AND “brain MRI” OR “brain magnetic resonance imaging” OR “brain CT” OR “neuroimaging”. In total, neuroimaging findings of 1550 patients, with ages from 1-96 years, were analyzed. Most brain neuroimaging findings include hyperintensity, Cerebral venous thrombosis, intraventricular and subarachnoid hemorrhage, infarction, leukoencephalopathy, acute ischemic strokes and posterior reversible encephalopathy syndrome (PRES) in adult patients and severe encephalopathy, stroke, infarction, CNS infection/demyelination, neuritis or polyradiculitis, venous thrombosis, Guillain-Barré syndrome, and longitudinally extensive myelitis, and myositis in pediatric patients. Our findings showed that the most important complication of the coronavirus is not just respiratory complications, because although transiently, COVID-19-related brain complications are seen in pediatrics as well as adults, and families should pay more attention to health care.

Posterior Reversible Encephalopathy Syndrome in an Older Patient in the COVID-19 and Paxlovid Era

Posterior Reversible Encephalopathy Syndrome in an Older Patient in the COVID-19 and Paxlovid Era

Evaluation of inpatients neurology consultations in the department of gynecology, obstetrics, and reproductive medicine of a tertiary hospital in Turkey

Background & Objective: It is important to be aware of hormonal changes in neurological disease in neurology training. We aimed to investigate the most common disorders which resulted in the clinicians from the Department of Gynecology, Obstetrics, and Reproductive Medicine (GORM) to ask for neurology consultation from the Department of Neurology. Methods: All the neurology consultations requested from the Department of GORM between 2015 and 2023 at Harran University Hospital, Sanliurfa, Turkey, were retrospectively evaluated with regard to demographic characteristics, the reason for referral, neurologic diagnoses, and treatment, imaging features. SPSS statistical software version 22 (IBM Inc. NC, USA) was used to perform all statistical analyses. Results: There were 333 patients with a mean age of 32.5 years (range 17-68) seeking consultations; 287 had obstetric problems, and 46 had gynecological issues. In the obstetric group, pre-eclampsia and Posterior Reversible Encephalopathy Syndrome (PRES) were the most frequent final neurological diagnoses, while headache and acute cerebrovascular disease were the most common frequent problems in the gynecological group. The number of consultations rose from 23 in 2015 to 66 in 2022. The predicted rate of final diagnosis of GORM residents was 58%. The neurologist’s contribution has substantial influence on the management of 208 (62%) patients. Conclusion: Neurologists have a significant contribution to the management of GORM inpatients. In neurological training, importance should be given to the effect of hormonal changes on neurological diseases in neurology training.

Prediction of posterior reversible encephalopathy syndrome (PRES) due to obstetric causes

Prediction of posterior reversible encephalopathy syndrome (PRES) due to obstetric causes

Neuromyelitis Optica Spectrum Disorders Associated to Posterior Reversible Encephalopathy Syndrome: Pathogenesis, Diagnosis, Treatment and Evolution: A Case Report and Literature Review

Neuromyelitis Optica Spectrum Disorders Associated to Posterior Reversible Encephalopathy Syndrome: Pathogenesis, Diagnosis, Treatment and Evolution: A Case Report and Literature Review

Post-operative posterior reversible encephalopathy syndrome in an adult who recently underwent coronary artery bypass surgery

Post-operative posterior reversible encephalopathy syndrome in an adult who recently underwent coronary artery bypass surgery

Posterior Reversible Leukoencephalopathy With Hemorrhagic Features: A Case Series.

Introduction Posterior reversible leukoencephalopathy syndrome (PRES) is a clinical-radiological condition characterized by reversible subcortical vasogenic cerebral edema of acute or subacute onset in circumstances that disrupt capillary permeability, unfrequently accompanied by cytotoxic and/or hemorrhagic lesions. We describe a case series of PRES with hemorrhagic features. Subjects and methods Electronic medical records of hospitalized patients diagnosed with PRES from January 2009 to December 2021 were collected. Demographic data, medical history, clinical presentation, and outcome were recorded. Variables were compared between patients with and without hemorrhagic features using the Wilcoxon-Mann-Whitney test with a statistical significance level of p<0.05. Results Over a 12-year period, 33 patients were diagnosed with PRES, of whom 10 had hemorrhagic features: seven cortical microbleeds, two intraparenchymal hematomas, and one subarachnoid hemorrhage. Half of the patients were women, with a median age of 45.8 years (interquartile range (IQR) 21.8), and were admitted for non-neurological reasons. The sample included nine transplant recipients (six solid organa, three bone marrowa), with four patients in the immediate post-transplant period. PRES occurred in the context of infections and blood pressure fluctuations under cytotoxic drugs, such as immunosuppressants. Seventy percent showed improvement/resolution on neuroimaging at a median of 70 days (IQR 62.9). The three major hemorrhages occurred in the context of thrombocytopenia. The recorded in-hospital mortality was 10%. When compared to PRES without hemorrhagic features, patients with hemorrhagic features had a lower use of corticosteroids (50% vs. 78.8%; p=0.02) and a higher presence of restrictive lesions on neuroimaging (60% vs. 17%; p=0.04), with no differences in the other analyzed variables. Conclusion Patients with PRES and hemorrhagic features had a lower use of corticosteroids and a higher presence of restrictive lesions on neuroimaging. Further studies are needed to better understand the clinical implications and management of PRES with hemorrhagic manifestations.

Dengue-Associated Posterior Reversible Encephalopathy Syndrome Occurring in a Woman with 32 Weeks Pregnancy: A Case Report from Bangladesh

Posterior reversible encephalopathy syndrome (PRES) is an increasingly well recognised reversible clinicoradiological condition. Dengue can cause various neurologic complications like encephalitis, myelitis, Guillain-Barré syndrome (GBS), and myositis, which are increasingly being recognized in addition to the more common manifestations of plasma leakage and coagulopathy. Posterior reversible encephalopathy syndrome (PRES) is rare in dengue, although the pathophysiology of endothelial dysfunction likely underlies both. Here we report a case of dengue-associated posterior reversible encephalopathy syndrome in a pregnant woman J Bangladesh Coll Phys Surg 2023; 41: 99-100

Oligoclonal Bands in CSF May be Associated with Chronic Graft Versus Host Disease after Haploidentical Hematopoietic Stem Cell Transplantation

Introduction Haploidentical hematopoietic stem cell transplantation (haplo-HSCT) is a straightforward and effective therapy for hematology patients, and chronic graft-versus-host disease (cGVHD) is recognized as a major and important complication of haplo-HSCT. cGVHD can affect a number of organs, such as the skin, eyes, oral mucosa, liver, and lung.In addition to classical manifestations, cGVHD can imitate almost any autoimmune disease, and its central nervous system (CNS) involvement is exceedingly rare. In our studies, IgG synthesis and anti-myelin oligodendrocyte glycoprotein antibody were risk factors for CNS demyelination ( Annals of Hematology, 2018), aGVHD and hypertension were associated with posterior reversible encephalopathy syndrome ( Bone Marrow Transplantation, 2020), and aGVHD and cGVHD were identified as factors for idiopathic inflammatory demyelinating disease ( Am J Hematol, 2021). However, there are limited data available on cGVHD in the CNS. Therefore, we performed a thorough assessment of cGVHD, including examining the CSF of patients with cGVHD. Methods We performed a retrospective nested case‒control study in patients receiving haplo-HSCT between January 2013 and June 2022 in our center. In total, 86 patients suffering from cGVHD who also received CSF examinations were analyzed, of whom 42 patients showed evidence of CNS-cGVHD. Written informed consent was, and ethical approval was obtained before sample collection by the Ethics Committee of Peking University People's Hospital. The CSF samples were collected by lumbar puncture (LP). At least 1 ml of CSF was obtained from all patients by nontraumatic LP. All CSF samples were routinely screened for infection with bacterial, fungal, and virological pathogens, examined by histopathology and analyzed by fluorescence-activated cell sorting to identify tumor cells. Immune biomarkers were also evaluated. Results The median time from transplantation to cGVHD was 236 days. In the 86 cGVHD patients, aGVHD (HR 0.431, 95% CI 0.229-0.812, P=0.009), CSF protein (HR 0.634, 95% CI 0.484-0.784, P=0.)and oligoclonal bands (OCB) (HR 0.527, 95% CI 0.312-0.890, P=0.017) were identified as risk factors for the onset of cGVHD after haplo-HSCT. Moreover, we combined the variables to establish a nomogram for the cumulative incidence of cGVHD. The test showed that the fitness of the multivariate logistic regression model was suitable (P = 0.9516). The area under the ROC curve was 0.741. There were no differences in overall survival (OS), disease-free survival (DFS), or the cumulative incidence of relapse (CIR) between the cGVHD group and the control group without cGVHD. Nonetheless, there was a significant difference in nonrelapse mortality (NRM) (P=0.009). The 2-year cumulative NRM was 48.2% (95% CI 45.8%-50.6%) versus 12.6% (95% CI 12.2%-13.0%) between the cGVHD and without cGVHD groups, respectively. Then, we performed multivariate analysis for data from 42 patients who were diagnosed with CNS-cGVHD. The underlying diseases (HR 1.334, 95% CI 1.027-1.733, P=0.030) and CSF proteins (HR 0.313, 95% CI 0.126-0.776, P=0.012) were independently associated with the occurrence of CNS-cGVHD. Conclusions In conclusion, CSF immunity may be associated with the onset of cGVHD after haplo-HSCT, especially OCB, and the identification of cGVHD increases NRM after haplo-HSCT. Further multicenter prospective studies are needed to confirm the results and establish the standard diagnostic criteria and therapy for CNS-cGVHD.

A Novel Adult Case of Recurrent Acute Pancreatitis Caused by Hypercalcemia With Concurrent Manifestation of Posterior Reversible Encephalopathy Syndrome Revealing Undiagnosed Primary Hyperparathyroidism due to an Underlying Parathyroid Adenoma.

Hypercalcemia-induced posterior reversible encephalopathy syndrome (PRES) is a rare entity primarily associated with iatrogenic vitamin D/calcium overdose, malignancy, or, infrequently, primary hyperparathyroidism. We present a novel case of an adult male from rural India who experienced recurrent acute pancreatitis caused by hypercalcemia with concurrent manifestation of PRES. Diagnostic evaluation revealed markedly elevated serum calcium levels and parathyroid hormone concentrations, consistent with primary hyperparathyroidism. Imaging studies identified a parathyroid adenoma near the right thyroid lobe, subsequently surgically excised. This case underscores the importance of considering primary hyperparathyroidism as an underlying cause of PRES, especially in the absence of acute arterial hypertension or autonomic dysfunction. Early recognition and intervention are essential in mitigating the morbidity and mortality of PRES.

Pediatric posterior reversible encephalopathy syndrome: Can MR imaging features predict outcomes in non-oncologic patients?

PurposeIdentify MR features predictive of poor outcomes in non-oncologic pediatric PRES. MethodA six-year search of all non-oncologic pediatric patients with clinical and MR features of PRES was performed. Modified Rankin scores were used to classify clinical outcomes into good versus poor, then clinical and MR features were compared among groups. Univariate and multivariate analysis was performed to identify MR predictors of poor outcomes for various imaging features, and p-values < 0.05 were considered statistically significant. ResultsOne hundred and forty-one patients (mean age 10.1 ± 3.0 years, male to female ratio 1:1.1) were included. Clinically, nephrotic syndrome (p = 0.03), focal deficits (p = 0.04), longer hospitalization (p < 0.001), and mechanical ventilation (p < 0.001) were significantly associated with poor outcomes. Univariate analysis revealed that deep grey matter nuclei (OR = 5.29, 95 % CI: 1.6–18.0) and cerebellar edema patterns (OR = 3.49, 95 % CI: 1.3–9.5), cytotoxic edema (OR = 63.6, 95 % CI:16.5–244.2), hemorrhage (OR = 16.58, 95 % CI: 4.3–64.2), and severe PRES patterns (OR = 11.0, 95 % CI: 3.5–34.7) on MR were all significantly associated with poor outcomes (p-values = 0.008 and 0.014, <0.001, <0.001, and < 0.001, respectively). This remained true for cytotoxic edema (OR = 84.26, 95 % CI: 17.3–410.9, p-value < 0.001) and hemorrhage (OR = 44.56, 95 % CI: 6.9–289.7, p-value < 0.001) on multivariate analysis. ConclusionDiffusion restriction and hemorrhage on initial MR scans were the two independent predictors of poor outcomes in non-oncologic pediatric patients.

Posterior Reversible Encephalopathy Syndrome

(N Engl J Med. 2023;388:2171–2178) Posterior reversible encephalopathy syndrome (PRES) most often affects individuals with acute hypertension or elevated blood pressure or those who have been exposed to certain drugs or toxic agents. PRES often presents with acute neurological deficits, but these are often resolved relatively quickly if promptly diagnosed and treated. This review article describes the pathology, features, epidemiology, management, and implications of PRES.

Newly identified adverse events for gemcitabine using the Food and Drug Administration Adverse Event Reporting System

ABSTRACT Background Our research aimed to identify previously undocumented adverse events (AEs) in the gemcitabine drug insert with the goal of informing clinical practice. Methods We extracted adverse events associated with gemcitabine use through 2023 using the Food and Drug Administration Adverse Event Reporting System (FAERS) database. Four algorithms (Reporting Odds Ratio, Proportional Reporting Ratio, Bayesian Confidence Propagation Neural Network, and Empirical Bayesian Geometric Mean) were employed to detect new AE signals. AEs were considered positive signals only if they were detected by all four algorithms. Results From 2014 to 2023, a total of 42,360 AEs were reported in 14,905 individuals following gemcitabine use. These AEs totaled 437 preferred terms (PTs) across 20 system organ classes (SOCs). We identified unexpected AEs related to the ocular disorders, the nervous system, and the ear and the labyrinth. The ocular organ system will present with retinopathy, purtscher retinopathy, choroidal effusion, amaurosis, necrotizing scleritis, etc. The nervous system may experience reversible posterior encephalopathy syndrome, cerebellar syndrome, cauda equina syndrome, athetosis, transverse myelitis, etc. The ears and labyrinth may exhibit ototoxicity. Conclusion Our study identified previously undetected signals following gemcitabine treatment, thereby providing new insights for future medication guidance.

Posterior reversible encephalopathy syndrome occurring in heart transplant recipients under a hypertensive state

Posterior reversible encephalopathy syndrome occurring in heart transplant recipients under a hypertensive state

Posterior reversible encephalopathy syndrome in a patient with late postpartum eclampsia.

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. We report here a woman who suffered from headache, generalized tonic-clonic seizures, and cortical blindness in the late postpartum period. Posterior reversible encephalopathy syndrome. The patient was treated with amlodipine besylate tablets for hypertension, dehydration with mannitol and glycerin fructose, and antispasmodic treatment with sodium valproate and oxcarbazepine. 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. 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.

NCMP-26. PANCEREBELLITIS ASSOCIATED WITH CISPLATIN THERAPY IN A PATIENT WITH OROPHARYNGEAL/ NASOPHARYNGEAL SQUAMOUS CELL CARCINOMA

Abstract Cisplatin, a platinum-based drug, is one of the oldest and most widely used chemotherapeutic agents. It exerts its anti-tumor effect by forming DNA inter-strand and intra-strand cross-links, resulting in lesions that eventually activate apoptotic pathways. Cisplatin remains widely used for the treatment of several solid tumors, though this is often limited by its toxicity which can affect many organ systems, including nephrotoxicity, hepatotoxicity, ototoxicity, and neurotoxicity. A dose-dependent peripheral sensory neuropathy is the most reported cisplatin-induced neurotoxicity. Central nervous system (CNS) toxicity is less common, likely due to the large molecular size of cisplatin preventing its passage through the blood-brain barrier (BBB). There have been reports of cognitive impairment, posterior reversible encephalopathy syndrome, and optic neuritis associated with cisplatin. We present a case of a 38-year-old patient who was treated with concurrent cisplatin and radiation therapy for oropharyngeal/nasopharyngeal squamous cell carcinoma, and around a month after the end of therapy, was found to have imaging findings concerning for pancerebellitis. Extensive workup into infectious causes of cerebellitis was negative. Although there are no reports in the literature of cerebellitis due to cisplatin, it could be the culprit in this case. Animal studies have shown cerebellar dysfunction occurring in the setting of cisplatin exposure. Moreover, it is thought that the drug can reach the CNS if there is a disruption of the BBB, this is supported by the fact that CNS toxicity is more seen in patients who have primary CNS neoplasia, CNS metastatic disease, radiation exposure to the head, among other mechanisms that could potentially disrupt the BBB. Thus, it is important to consider CNS adverse effects, including cerebellitis, in patients receiving cisplatin, especially among those with a potentially compromised BBB.

Abstract 18707: Concomitant Occurrence of Takotsubo Cardiomyopathy and Posterior Reversible Encephalopathy Syndrome: An Uncommon Encounter With Shared Pathophysiology

CASE PRESENTATION: A 61-year-old African American woman presented in an unconscious state after a seizure. She was febrile, tachycardic, tachypneic alongside elevated lactate, leukocyte counts, and procalcitonin levels. Initial high-sensitivity troponin I was notably raised at 4,217 ng/l, with ekg revealing a new onset left bundle branch block (LBBB). Patient was intubated and needed pressor support. Echocardiogram revealed hypokinesis in the basal-mid left ventricle, with a significantly decreased ejection fraction (EF) of 40%. Chest imaging showed a consolidation in the left lower lobe, and Hemophilus influenzae was identified in the sputum culture. A subsequent lumbar puncture yielded elevated leukocytes and proteins, suggesting a secondary meningitic process. DISCUSSION: She was started on antibiotic therapy for pneumonia and meningitis. LV hypokinesis was thought to be due to Takotsubo cardiomyopathy (TTC) or myocardial infarction. Although uncommon LBBB can be seen in TTC. Her clinical presentation, echocardiogram findings further points her LBBB and troponinemia towards TTC than Myocardial infarction. Patient was managed conservatively. Neuroimaging later on revealed restricted diffusion in bilateral occipital and parietal lobes, consistent with Posterior Reversible Encephalopathy Syndrome (PRES). A delayed coronary angiogram after clinical stabilization ruled out obstructive atherosclerotic disease. Weakness and ataxia improved over the course. EF at discharge was 80%.This case illustrates the possibility of concomitant TTS and PRES, conditions hypothesized to arise following intense physical or emotional stress, in this case, likely triggered by pneumonia and meningitis. This clinical scenario underscores the importance of awareness and understanding of these two entities and their potential for simultaneous presentation, providing valuable insights for clinical decision-making in such complex cases.