Posterior reversible encephalopathy syndrome in early lyme neuroborreliosis.

We report a case of posterior reversible encephalopathy syndrome in an adult patient fulfilling criteria for proven early Lyme neuroborreliosis.

Risk factors of posterior reversible encephalopathy syndrome in patients with preeclampsia or eclampsia: A retrospective review.

Background and purpose:

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

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

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

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

Tacrolimus-induced posterior reversible encephalopathy syndrome following liver transplantation.

In this editorial, we talk about a compelling case focusing on posterior reversible encephalopathy syndrome (PRES) as a complication in patients undergoing liver transplantation and treated with Tacrolimus. Tacrolimus (FK 506), derived from Streptomyces tsukubaensis, is a potent immunosuppressive macrolide. It inhibits T-cell transcription by binding to FK-binding protein, and is able to amplify glucocorticoid and progesterone effects. Tacrolimus effectively prevents allograft rejection in transplant patients but has adverse effects such as Tacrolimus-related PRES. PRES presents with various neurological symptoms alongside elevated blood pressure, and is primarily characterized by vasogenic edema on neuroimaging. While computed tomography detects initial lesions, magnetic resonance imaging, especially the Fluid-Attenuated Inversion Recovery sequence, is superior for diagnosing cortical and subcortical edema. Our discussion centers on the incidence of PRES in solid organ transplant recipients, which ranges between 0.5 to 5 +ACU-, with varying presentations, from seizures to visual disturbances. The case of a 66-year-old male status post liver transplantation highlights the diagnostic and management challenges associated with Tacrolimus-related PRES. Radiographically evident in the parietal and occipital lobes, PRES underlines the need for heightened vigilance among healthcare providers. This editorial emphasizes the importance of early recognition, accurate diagnosis, and effective management of PRES to optimize outcomes in liver transplant patients. The case further explores the balance between the efficacy of immunosuppression with Tacrolimus and its potential neurological risks, underlining the necessity for careful monitoring and intervention strategies in this patient population.

Case report: Blood-transfusion induced posterior reversible encephalopathy syndrome.

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

Cerebral hypometabolism in a pediatric patient with clinically resolved posterior reversible encephalopathy syndrome.

A 4-year-old boy with Nuclear factor-kappa B Essential Modulator deficiency syndrome presented with encephalopathy post haematopoietic stem cell transplantation. MRI demonstrated T2/FLAIR-hyperintensities in the posterior cerebral cortex concerning for posterior reversible encephalopathy syndrome. Clinical improvement was appreciated following withdrawal of the suspected offending pharmacological agent (Cyclosporine). An 18F-FDG PET/CT performed 2 months later to screen for post-transplant lymphoproliferative disease demonstrated markedly reduced FDG uptake in the posterior cerebral cortex, involving the parietal and occipital lobes. We describe, to the best of our knowledge, the first case of profound cerebral hypometabolism in a child with clinically resolved posterior reversible encephalopathy syndrome.

Fatal posterior reversible encephalopathy syndrome after blood transfusion in a patient with myelodysplastic syndromes.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is known as a transfusion-related complication with typically favorable prognosis and no report fatalities. Pathological evaluation of PRES is also scarce. CASE REPORT: An 88-year-old female with myelodysplastic syndromes (MDS) attended our hospital because of a compression fracture and chronic heart failure with chronic anemia. While her hemoglobin levels improved from 4.6 to 8.0 g/dL and the pleural effusions substantially decreased following six units of red blood cell transfusion and diuretic therapy, a gradual decline in cognitive function and speech reduction was noted. PRES was diagnosed by magnetic resonance imaging of the head. Despite treatment of intensive supportive care, the patient fell into a coma by the 20th day and passed away on the 22nd day. Although the pathophysiological link between blood-transfusion-related PRES and its impact on survival is not fully understood, autopsy findings confirmed the diagnosis of PRES and revealed multiple cerebral hemorrhages that were not detected in earlier imaging studies. CONCLUSION: This case highlights the importance of vigilant monitoring and management of PRES, especially in high-risk populations such as elderly patients with multiple comorbidities or those with thrombocytopenia. Further studies are needed to elucidate the mechanisms of PRES in patients with hematologic diseases.

Posterior Reversible Encephalopathy Syndrome Associated with L-Asparaginase Treatment in Children: Literature Review and Six Case Reports.

L-asparaginase (L-ASNase) is an enzyme that shows targeted activity against Acute Lymphoblastic Leukemia (ALL) and similar lymphoid neoplasms by facilitating the breakdown of asparagine into L-aspartic acid, thereby reducing L-asparagine levels in leukemic cells. However, its therapeutic potential is hindered by its associated toxicity, leading to complications, such as thrombosis, hemorrhage, thrombocytopenia, fibrinolysis, hypersensitivity reactions, and the development of Posterior Reversible Encephalopathy Syndrome (PRES). This review compiles documented cases of PRES linked to treating B and T cell acute lymphoblastic leukemia in children using L-ASNase. Although this pathology is rare, understanding its management is crucial within ASNase-based chemotherapy protocols. As PRES lacks a specific treatment, focusing on symptomatic management becomes pivotal. Therefore, comprehending the underlying causes during L-ASNase treatment for acute lymphoblastic leukemia is essential. Understanding the etiology and clinical symptoms of this illness is critical for early diagnosis and treatment. The cases of PRES described in this review include instances in which this syndrome has appeared after the administration of L-ASNase in children. In some cases, PRES developed during induction therapy, while in others, it occurred during the reinduction phase. These cases resolved days after discontinuation of L-ASNase. The findings suggest a close relationship between drug administration and the appearance of brain lesions, as evidenced by the disappearance or decrease of these lesions when the drug was eliminated from the bloodstream.

Clinical features and outcomes of posterior reversible encephalopathy syndrome after heart transplantation: a case series.

Posterior reversible encephalopathy syndrome (PRES) is a rare neurological disease that may be associated with hypertension, autoregulatory failure, and the use of calcineurin inhibitors following heart transplantation (HT). In this article, we present a case series of PRES, discussing its potential causes and management strategies. Among the 126 HT recipients at our hospital, four were diagnosed with PRES. Three of these patients developed PRES within 7 days after HT. Prior to the onset of PRES, all patients experienced sustained hypertension, and strict blood pressure (BP) control was maintained. Three of the four patients recovered without PRES recurrence, while one patient died of sepsis after an episode of altered consciousness. Hypertension was observed in all patients prior to the onset of PRES, and the majority experienced symptom improvement with BP control. While most cases of PRES were reversible with conservative treatment, including the administration of antiepileptics, one irreversible case resulted in in-hospital mortality. Thus, PRES can have serious outcomes and is not invariably benign.

Posterior Reversible Encephalopathy Syndrome After Infliximab Infusion for Fistulizing Crohn's Disease.

Biologic therapy is the mainstay of treatment of complicated inflammatory bowel diseases, which has numerous potential side effects. Among these is a rare condition known as posterior reversible encephalopathy syndrome (PRES), which is a reversible neurological disorder that results in symptoms such as headache, nausea/vomiting, blurry vision, and seizure and is diagnosed based on specific clinical and radiological features. This report presents a case of a 19-year-old woman with fistulizing Crohn's disease who was treated with infliximab, but subsequently developed PRES, which was manifested as recurrent episodes of seizures and elevated blood pressure readings, was managed supportively with antiepileptic and antihypertensive medications and eventually made a full recovery, even after resuming infliximab. This case adds to the fewer than 10 previously reported cases of PRES associated with biological therapy for inflammatory bowel disease. It highlights the need to consider this complication when prescribing these drugs.

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.

Posterior reversible encephalopathy syndrome in immunocompromised children - A single-center study from South India.

This study describes the profile of children diagnosed with posterior reversible encephalopathy syndrome (PRES) in the pediatric hematology oncology unit and highlights the clinical features of PRES in immunosuppressed children. This retrospective study included 10 children diagnosed with PRES with a mean age of 6.8 years. Acute lymphoblastic leukemia was the most common primary diagnosis followed by post-hematopoietic stem cell transplant patients. Most cases of PRES occurred within one month of treatment initiation. Hypertension was noted in all at the time of diagnosis. Neuroimaging revealed bilateral lesions with parietal and occipital lobe involvement being the most common. All patients received corticosteroids as part of treatment for primary diagnosis. Controlling blood pressure was critical in managing PRES. Consideration of PRES as a clinical possibility in pediatric hematology oncology unit in children presenting with symptoms such as headache, seizures, and visual disturbances will aid in early diagnosis after ruling out other causes of these symptoms.

Successful Treatment with Mycophenolate Mofetil in a Patieint with Anti-MDA 5 Antibody-positive Interstitial Lung Disease and Posterior Reversible Encephalopathy syndrome: A Case Report.

A 59-year-old woman presented with a rash on the top part of her hands and pain in the wrist joint and was diagnosed with dermatomyositis complicated by interstitial pneumonia positive for anti-melanoma differentiation-associated gene 5 (MDA-5) antibody. However, the patient reported a severe headache following treatment with oral prednisolone and tacrolimus. Posterior reversible encephalopathy syndrome (PRES) was diagnosed based on the brain magnetic resonance imaging findings. Tacrolimus was discontinued, and mycophenolate mofetil was instead administered with a favorable outcome. Mycophenolate mofetil should therefore be considered as an alternative treatment for anti-MDA-5-positive interstitial lung disease in cases where calcineurin inhibitors cannot be used.

Electrifying Consequences: Posterior Reversible Encephalopathy Syndrome After an Electric Shock Injury.

Reversible posterior leukoencephalopathy syndrome, often referred to as posterior reversible encephalopathy syndrome (PRES), is a disorder characterized by acute cerebral dysfunction and is seen in conjunction with vasogenic edema on brain imaging. Headaches, visual issues, seizures, abnormal mentation, disturbances in awareness, and focal neurological symptoms are its defining features. In this case report, we present a 40-year-old male patient who developed PRES after experiencing a high-voltage electric shock.

Posterior reversible encephalopathy syndrome post stem cell transplantation in sickle cell disease: case series and literature review.

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a serious neurological syndrome that may develop following immunosuppressive therapy for stem cell transplantation (SCT). We report 8 patients with sickle cell disease (SCD) who developed PRES, which is likely to be related to immunosuppression. Methods: This is retrospective cohort analysis of the SCD registry at the King Faisal Specialist Hospital and Research Center (KFSHRC) in Riyadh, Saudi Arabia. Inclusion criteria included all adults SCD patients who underwent SCT from 2011 until 2022. We explored all cases of PRES in patients with SCT. PRES was diagnosed with MRI imaging showing reversible vasogenic cerebral edema associated with neurological symptoms including severe headache, seizures, encephalopathy, delirium, and visual disturbances. Results: During ten years follow-up (2011-2022) we found 8 patients with PRES (age range between 14 to 37 years at diagnosis) PRES occurred 8 to 124 days following SCT in 7 cases and one patient developed PRES 8 months prior to SCT. All patients were on immunosuppressive medications, including tacrolimus, cyclosporine, sirolimus and or mycophenolate mofetil. Headache, seizures, visual hallucinations, confusion, and drowsiness were the most common presenting symptoms. MRI showed abnormalities in the occipital, parietal and frontal lobes in most cases. Recovery was complete in all patients and no recurrences were noted. Two patients had graft versus host disease (GVHD). We compared risk factors for PRES among the 8 cases and 136 SCT in SCD patients who did not develop PRES. There was a significant association between PRES and imaging abnormalities, including previous bi-hemispheric infarctions (p = 0.001), and cerebral microbleeds (CBMs). PRES was strongly associated with presence (p = 0.006), size (p = 0.016) and number (p = 0.005) of CMBs. Conclusion: PRES can develop days to weeks following SCT in patients with SCD, and is associated with immunosuppressive therapy, previous bi-hemispheric infarctions and CMB. Prompt recognition and intervention leads to good recovery.

Posterior reversible encephalopathy syndrome: A case report.

Posterior Reversible Encephalopathy Syndrome (PRES) is a radio-clinical entity associating reversible damage of the central nervous system and typical brain imaging. The clinical context is often suggestive with, in half of cases, the use of vasoactive substances (cannabis, antidepressants, nasal decongestants) and/or postpartum. The etiologies are dominated by hypertensive encephalopathy, preeclampsia, eclampsia, immunosuppressive therapies, and systemic diseases. We report a case of posterior encephalopathy syndrome occurring in a young female without hypertension. It was about a 40-year-old female without hypertension underlying condition, received at the emergency department for headaches and generalized tonic-clonic seizures. The physical examination was unremarkable, and her blood pressure was 130/70 mm Hg. CT scan revealed bilateral white matter hypodensity in the posterior occipital regions and a right frontal subarachnoid hemorrhage. There was no aneurysmal malformation of the polygon of Willis and no cerebral thrombophlebitis. Brain MRI showed T2 and FLAIR hypersignal areas in the occipital and frontal cortico-subcortical regions, with no diffusion signal abnormalities or contrast enhancement, and a right frontal subarachnoid hemorrhagic lesion with no other impairment. The diagnosis of reversible posterior encephalopathy syndrome was made up, and the outcome was favorable under treatment. Posterior reversible encephalopathy syndrome is an uncommon but probably underdiagnosed condition. Hypertensive encephalopathy is the most common etiology. However, there would be cases of PRES without hypertension as shown in this observation.

Clinical and Radiological Features of Unilateral Posterior Reversible Encephalopathy Syndrome (PRES): A Case Report.

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological entity characterized by reversible vasogenic edema predominantly affecting the posterior regions of the cerebral hemispheres. However, unilateral presentation of PRES is an exceptionally rare manifestation. We describe the case of a 34-year-old woman who presented with left-sided hemiparesis, hemisensory loss, headache, and focal motor seizures. Brain CT revealed right anterior and posterior hypodensities in the right frontal and parietal subcortical locations, brain MRI showed vasogenic edema in the subcortical right cerebral hemisphere, and cerebral angiogram revealed diffuse narrowing of the left internal carotid artery just distal to the carotid bifurcation with no flow of contrast beyond the ophthalmic segment. The patient's symptoms resolved during her admission, MRI findings improved on repeated imaging, and she was ultimately diagnosed with unilateral PRES. Unilateral PRES is a complex and challenging diagnosis, and this case sheds light on the atypical radiological features of unilateral PRES possibly intricately linked with contralateral steno-occlusive disease of the carotid artery. It is essential to maintain the atypical variants of PRES as part of the differential diagnosis when encountering acute neurological symptoms and vasogenic edema on imaging in the context of contralateral steno-occlusive disease of the carotid artery.

Hemolysis, Elevated Liver Enzymes and Low Platelet Count Syndrome Complicated by Disseminated Intravascular Coagulation and Posterior Reversible Encephalopathy Syndrome: A Case Report.

A 22-year-old pregnant woman was transferred from an external medical facility after experiencing an eclamptic seizure linked to hemolysis, elevated liver enzymes and low platelet count syndrome (HELLP) syndrome, and posterior reversible encephalopathy syndrome (PRES). Her situation was further complicated by intrauterine fetal demise and disseminated intravascular coagulation, necessitating a comprehensive multidisciplinary approach. This report details the diagnostic process and challenges in managing this complex patient with diverse medical requirements. Emphasis is placed on the observed hemostatic abnormalities, and we delineate the nuances in our approach compared to managing a similar condition in a nonpregnant patient. Heightened awareness among healthcare professionals is imperative for prompt diagnosis and effective intervention in such uncommon neurological complications during pregnancy.

Posterior Reversible Encephalopathy Syndrome With Hemorrhagic Transformation in the Postoperative Period of a Kidney Transplant.

Patients with end-stage renal disease (ESRD) who undergo kidney transplantation are at an increased risk of developing surgical and/or medical complications. Posterior reversible encephalopathy syndrome (PRES) is a rare complication that occurs in 0.34% of kidney transplant patients. It is characterized by a combination of neurological manifestations, risk factors, and characteristic radiological findings in neuroimaging studies. The development of PRES has been associated with various medical conditions and factors, including hypertension, the use of cytotoxic and immunosuppressive drugs, acute or chronic kidney disease, pre-eclampsia/eclampsia, autoimmune diseases, and solid organ and bone marrow transplantation. This report presents the case of a 19-year-old woman diagnosed with ESRD on hemodialysis due to lupus nephritis who experienced an episode of PRES with intraparenchymal hemorrhage during the postoperative period of kidney transplantation. The case emphasizes the importance of closely monitoring these patients during this period to enable early diagnosis and timely treatment of complications, ensuring a favorable prognosis.

Posterior Reversible Encephalopathy Syndrome Presenting as Delirium With Psychosis and Agitation in the Postpartum Period.

Posterior reversible encephalopathy syndrome (PRES), which was first described in 1996, is a neurologic condition characterized by a combination of clinical and neuroimaging findings. PRES may arise in the context of preeclampsia, eclampsia, renal failure, and sepsis, among other conditions. Neuropsychiatric symptoms of PRES include altered mental status, agitation, and in some cases psychosis. PRES occurring in the postpartum period is understudied, especially with regard to its psychiatric manifestations. We aim to add to the literature a case of PRES associated with psychosis and agitation in a postpartum woman, highlighting clinical implications and offering suggestions for practice. A female in her late 20s, with no significant psychiatric or medical history, presented to the hospital at 29 weeks and one day of gestation following a witnessed seizure. She was found to be hypertensive and hyponatremic, was diagnosed with eclampsia, and underwent an emergent cesarean section due to fetal malpresentation. The next day, the patient developed paranoia with acute agitation, and the psychiatry team diagnosed her with delirium with psychosis/agitation secondary to her underlying medical condition. She required intramuscular medications for agitation, was placed in restraints, and was transferred to the ICU for sedation. Subsequently, CT and MRI scans of her head both indicated that she had developed PRES. The patient's delirium and psychotic behavior resolved after appropriate treatment of her eclampsia. To our knowledge, this case report is the second documented case in the literature, of a patient who presented with PRES characterized by agitation and psychotic features in the postpartum period. Due to the significant overlap in symptoms between delirium and postpartum psychosis, this case highlights the crucial importance of interdisciplinary collaboration for accurate diagnosis and prompt treatment of PRES in the postpartum period. The case also speaks to the importance of differentiating postpartum psychosis associated with a primary psychiatric disorder from delirium arising in postpartum patients with or without a previous psychiatric history.

Exploring the Impact of Neurophysiotherapy in Managing Leukoencephalopathy Challenges: A Case Report.

Leukoencephalopathy (LE), characterized by structural changes affecting cerebral white matter, presents a complex clinical picture with diverse etiologies. This case report details the presentation, clinical findings, and physiotherapy management of a 32-year-old female with colony-stimulating factor 1 receptor (CSF1R)-related leukoencephalopathy and a history of diabetes and hypertension. She suddenly stopped her medications, which led to the worsening of her condition. She presented with symptoms of headache, slurred speech, visual disturbances, cognitive impairment, and impaired balance and coordination, due to which her activities of daily living were affected. The symptoms highlighted the challenges and multidisciplinary approach required for its management. The patient exhibited neurological deficits, cognitive decline, and abnormal reflexes, with magnetic resonance imaging (MRI) revealing white matter abnormalities. Outcome measures demonstrated significant improvements in cognitive and functional abilities, emphasizing the effectiveness of tailored rehabilitation in managing the complexities of colony-stimulating factor 1 receptor-related leukoencephalopathy. A six-week physiotherapy rehabilitation program addressed various domains, including strength training, task-specific exercises, errorless learning, facial muscle retraining, balance exercises, visual restoration therapy, and mobility training. All these interventions effectively improved her functional capacity and made the patient independent in performing activities of daily living.