Eclampsia, HELLP and PRES in a 16-week partial molar pregnancy.

Eclampsia spectrum disorders are a set of serious complications of pregnancy that commonly present after 20 weeks of gestation. There is an association between molar pregnancy, a gestational trophoblastic disease resulting from abnormal fertilisation and gametogenesis, and eclampsia spectrum disorders which can result in manifestation of pre-eclamptic symptomatology earlier than 20 weeks of gestation. We report a case of a gravida 1 para 0 in her mid 20s at 16-weeks gestation presenting with partial hydatidiform mole who developed eclampsia, haemolysis, elevated liver enzymes and low platelets syndrome and posterior reversible encephalopathy syndrome. Ultrasound findings were consistent with molar pregnancy and pathology confirmed partial molar pregnancy with triploid 69, XYY karyotype. This case highlights the early onset potential of eclampsia spectrum disorders in molar pregnancies while suggesting screening such patients for hypertensive disorders.

PRESenting a Challenge: Posterior Reversible Encephalopathy Syndrome in Pediatric Patients With Guillain-Barré Syndrome: A Case Series and Review of Literature.

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

Chemotherapy-associated hemorrhagic posterior reversible encephalopathy syndrome (PRES) with considerations for circle of Willis variants on cerebral blood flow and autoregulation: A case report.

RATIONALE: Hodgkin lymphoma, a lymphatic system cancer, is treated by chemotherapy, radiation therapy, and hematopoietic stem cell transplantation. Posterior reversible encephalopathy syndrome (PRES) is a rare neurotoxic effect associated with several drugs and systemic conditions. This case study emphasizes the potential risks of intensive chemotherapy regimens and postulates the impact of the circle of Willis variants on the heterogeneity of hemispheric lesions in PRES. PATIENT CONCERNS: A 42-year-old woman diagnosed with stage IIA nodular sclerosing Hodgkin lymphoma and chronic thrombocytopenia presented after 6 years of initial diagnosis and 4 years post-haploidentical transplant. She underwent planned chemotherapy with ifosfamide, carboplatin, and etoposide. DIAGNOSES: She developed an alteration in her mental status. A computerized tomography scan and angiogram of the head and neck revealed findings consistent with PRES and a left fetal-type posterior cerebral artery with an aplastic A1 segment of the left anterior cerebral artery. One hour later she was found comatose with clinical sequelae of an uncal herniation. INTERVENTIONS: Subsequent events led to emergent intubation, and administration of 23.4% hypertonic saline. A repeat computerized tomography scan showed a right intraparenchymal hemorrhage with fluid-fluid levels measuring up to 4.7 cm, bilateral subarachnoid hemorrhage, right uncal herniation, and 15 mm of leftward midline shift. She emergently underwent a right decompressive hemi-craniectomy. OUTCOMES: An magnetic resonance imaging of the brain demonstrated bilateral cytotoxic edema involving the parieto-occipital lobes. Despite interventions, the patient's neurological condition deteriorated, leading to a declaration of brain death on the 8th day. LESSONS: This case underscores the importance of recognizing the severe neurological complications, including PRES, associated with chemotherapeutic treatments in Hodgkin lymphoma. PRES may also be exacerbated by coagulopathies such as thrombocytopenia in this case. The circle of Willis variants may influence cerebral blood flow, autoregulation, and other factors of hemodynamics, leading to increased susceptibility to both radiographic lesion burden and the worst clinical outcomes.

Clinical relevance of reversible cerebral vasoconstriction syndrome in pregnant women with posterior reversible encephalopathy syndrome: review of case reports in Japan.

We systematically reviewed case reports of posterior reversible encephalopathy syndrome (PRES), and investigated the characteristics of PRES in pregnant Japanese women and the clinical relevance of reversible cerebral vasoconstriction syndrome (RCVS) in pregnant women with PRES. Articles were collected using the PubMed/Medline and Ichushi-Web databases. This review was ultimately conducted on 121 articles (162 patients). The clinical characteristics of PRES, individual sites of PRES lesions, edema types, and clinical characteristics of RCVS in PRES cases were examined. The most common individual site of PRES lesion was the occipital lobe (83.3%), followed by the basal ganglia, parietal lobe, frontal lobe, brain stem, cerebellum, temporal lobe, thalamus, and splenium corpus callosum (47.5, 42.6, 24.7, 16.1, 9.3, 5.6, 4.3, and 0.0%, respectively). Edema types in 79 cases with PRES were mainly the vasogenic edema type (91.1%), with very few cases of the cytotoxic edema type (3.8%) and mixed type (5.1%). Among 25 PRES cases with RCVS, RCVS was not strongly suspected in 17 (68.0%) before magnetic resonance angiography. RCVS was observed at the same time as PRES in 13 cases (approximately 50%), and between days 1 and 14 after the onset of PRES in the other 12. These results suggest that the basal ganglia is a frequent site of PRES lesions in pregnant women. RCVS may occur at or after the onset of PRES, even if there are no symptoms to suggest RCVS.

Should Calcineurin Inhibitors/Sirolimus Be Ceased Completely In Posterior Reversible Encephalopathy Syndrome?

BACKGROUND: To investigate the relationship between immunosuppressive treatments and posterior reversible encephalopathy syndrome (PRES) in transplant patients. METHODS: We presented a retrospective study of 4 cases of PRES in transplant patients. Patient records were reviewed to identify potential risk factors, clinical presentations, radiological findings, and immunosuppressive treatments used. RESULTS: Our analysis revealed a potential association between immunosuppressive treatments and the development of PRES in transplant patients. Specifically, we found that adjusting or switching immunosuppressive treatments can improve outcomes and prevent the recurrence of PRES. CONCLUSION: Our findings highlight the importance of recognizing PRES as a potential complication of immunosuppressive treatments in transplant patients. Early detection and management, including a review of immunosuppressive treatments, may improve patient outcomes and prevent further complications.

Association of Posterior Reversible Encephalopathy Syndrome (PRES) with Preeclampsia with Severe Symptoms and Eclampsia in South East Part of Bangladesh.

Posterior reversible encephalopathy syndrome (PRES) is a pathology seen not only in precelampsia with severe symptoms and eclampsia but in a varicty of diseases/ conditions. With the availability of neuroimaging, it is possible to know the exact underlying Central nervous system (CNS) pathology in preeclampsia with severe symptoms and eclampsia and thus therapy can be targeted. Preeclampsia with severe symptoms and eclampsia remains to be an important cause of maternal morbidity and mortality in both the developing and developed world. The objective of this study was to evaluate the association of Posterior reversible encephalopathy syndrome (PRES) by MRI (Magnetic resonance imaging) with preeclampsia with severe symptoms and eclampsia in south east part of Bangladesh. This cross-sectional observational study was performed among women suffering from preeclampsia with severe symptoms and eclampsia who attended at Obstetrics & Gynaecology department of Chittagong Medical College Hospital (CMCH), Bangladesh from January 2021 to June 2021. According to inclusion/exclusion criteria 50 samples were taken by convenient sampling for this study. A detail history was taken and complete general physical and gynecological examination was performed. Required data was collected through preset questionnaire. Neuroimaging reports were reviewed by both neurologist and radiologist. Data was analyzed by using windows based computer software device, SPSS 25.0. Results obtained from this study will be used to make a statement regarding aggressive management for cerebral vasospasm in severe preeclampsia and eclamptia related PRES. PRES has been reported to be reversible but late recognition or incorrect treatment can cause irreversible brain damage. Institution of early treatment leads to resolution of symptoms without any neurologic deficit and thus reduces maternal morbidity and mortality. PRES is a cliniconeuroradiologic entity. This study can aware doctors regarding prompt diagnosis of PRES in peripartum period among patient suffering from preeclampsia with severe symptoms and eclampsia by imaging aside clinical findings. A conclusive decision can be made to improve the outcome in this potentially life threatening but reversible condition.

Sporadic Creutzfeldt-Jakob Disease Initially Presenting With Posterior Reversible Encephalopathy Syndrome: A Case Report.

INTRODUCTION: Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal neurodegenerative condition caused by prion proteins. Cortical and subcortical diffusion-weighted imaging restriction on magnetic resonance imaging (MRI) is associated with sCJD. Posterior reversible encephalopathy syndrome (PRES) results from impaired vessel autoregulation due to an identifiable trigger, which is associated with subcortical fluid-attenuated inversion recovery changes on MRI. We report a case of sCJD initially presenting with PRES. CASE REPORT: A 70-year-old woman presented to an outside hospital with progressive confusion and difficulty in managing activities of daily living. Initial examination revealed stuporous mental state and stimulus-induced myoclonus. MRI revealed bilateral subcortical occipital lobe T2-fluid-attenuated inversion recovery hyperintensities without contrast enhancement suggestive of PRES. Electroencephalogram (EEG) revealed frequent generalized periodic discharges meeting criteria for nonconvulsive status epilepticus. Clinical examination and EEG did not improve despite escalating antiseizure medications. Initial lumbar puncture was unremarkable. She was transferred to our hospital with a presumptive diagnosis of PRES, although there was no clear trigger. Continuous EEG revealed ongoing generalized periodic discharges with myoclonic activity meeting criteria for myoclonic seizures that were refractory to multiple antiseizure medications. Repeat MRI showed resolution of PRES but revealed subtle diffuse cortical diffusion-weighted imaging restriction. Repeat lumbar puncture was performed and 14-3-3 and real-time quaking-induced conversion returned positive, confirming sCJD. CONCLUSIONS: This case reports highlights that sCJD can present with neuroimaging consistent with PRES. The diagnosis of sCJD should be considered in patients with PRES who continue to show neurological decline despite optimal management and radiographic improvement of PRES on MRI. Further research is needed to identify a pathophysiological relationship between these clinical phenotypes.

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

PURPOSE: Identify MR features predictive of poor outcomes in non-oncologic pediatric PRES. METHOD: A 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. RESULTS: One 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. CONCLUSION: Diffusion restriction and hemorrhage on initial MR scans were the two independent predictors of poor outcomes in non-oncologic pediatric patients.

Posterior reversible encephalopathy syndrome and acute ischemic stroke: an underreported association.

INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a rare and complex disorder with variable clinical presentation and a typical magnetic resonance imaging (MRI) pattern of vasogenic edema with typical and atypical locations. It is often triggered by other diseases and drugs and the most prototypical association is with persistently elevated arterial pressure values. Among the potential cerebrovascular complications, intracranial bleeding has been described, but ischemic stroke is uncommonly reported. METHODS: We are presenting a case of a male patient with prolonged and sustained arterial hypertension acutely presenting with lacunar ischemic stroke involving the right corona radiata and composite MRI findings with the association of chronic small vessel disease (SVD) markers, acute symptomatic lacunar stroke, and atypical, central variant, posterior fossa dominant PRES. In the MRI follow-up, the white matter hyperintensities in T2-fluid attenuated inversion recovery (FLAIR sequences) due to PRES. DISCUSSION: The pathophysiology of PRES is not yet fully known, but the association with markedly increased values of arterial pressure is typical. In this context, ischemic stroke has not been considered in the clinical and neuroradiological manifestations of PRES and it has been only occasionally reported in the literature. In this case, the main hypothesis is that sustained hypertension may have triggered both manifestations, PRES, and ischemic stroke and the last one allowed to diagnose the first one. CONCLUSIONS: Atypical variants of PRES are not so rare and it may also occur in typical triggering situations. The association with ischemic stroke is even rarer and it may add some clues to the pathomechanisms of PRES.

Experimental Models of Posterior Reversible Encephalopathy Syndrome: A Review From Pathophysiology to Therapeutic Targets.

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

[Posterior reversible encephalopathy syndrome in children with hematological diseases]. / Sindrom zadnei obratimoi entsefalopatii u detei s zabolevaniyami sistemy krovi.

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

Case report: A case of posterior reversible encephalopathy in postpartum preeclampsia.

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