Tacrolimus-Associated Posterior Reversible Encephalopathy Syndrome in Renal Transplant Recipient in Early Posttransplant Period: Case Report.

Posterior reversible encephalopathy syndrome is an emergency medical condition with varied causes presenting as reversible subcortical vasogenic brain edema caused by endothelial injury, resulting from changes in blood pressure or direct effects of cytokines on endothelium. Posterior reversible encephalopathy syndrome is manifested by neurologic symptoms. Common causes include hypertensive emergency, renal disease, preeclampsia, eclampsia, and immunosuppressive drugs. In this case report, a 17-year-old female patient on hemodialysis as a result of lupus nephritis who had previously undergone deceased donor organ transplant and was on triple immunosuppression presented with neurological symptoms of posterior reversible encephalopathy syndrome in the early posttransplant period. She was normotensive, and tacrolimus level was in desired level. She improved after cessation of tacrolimus from immunosuppression with complete resolution of radiological lesions. Posterior reversible encephalopathy syndrome can occur in solid-organ transplant recipients who are on tacrolimus as a part of immunosuppression.

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.

Reversible cerebral vasoconstriction syndrome in Guillain-Barre syndrome: a case report and literature review.

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by transient constriction of cerebral arteries, leading to severe headache and potential complications. The association between RCVS and Guillain-Barre syndrome (GBS) is rare and poorly understood and warrants further investigation. METHODS: A detailed case of RCVS in a patient with GBS was presented, followed by a comprehensive literature review. PubMed, Embase, and Google Scholar were searched for relevant cases and studies. RESULTS: The case involved a 62-year-old woman with GBS who developed RCVS. The literature review identified three additional reported cases. RCVS in GBS primarily affected middle-aged women and presented with a variety of neurological symptoms. Neuroimaging showed reversible vasoconstriction in the cerebral arteries, along with other complications such as posterior reversible encephalopathy syndrome, subarachnoid hemorrhage, and infarcts. While the treatment for GBS consisted mainly of intravenous immunoglobulin, specific treatments for RCVS remain unclear. CONCLUSIONS: The coexistence of RCVS and GBS is a rare occurrence. RCVS in GBS may result from the disruption of cerebral vascular tone regulation, possibly influenced by GBS-related dysautonomia and consequent high blood pressure. Recognizing RCVS in GBS patients is critical for appropriate management.

Posterior Reversible Encephalopathy Syndrome in Pediatric Hematopoietic Stem Cell Transplantation with Beta Major Thalassemia: The Association between the PRES Occurrence and Class of Beta Major Thalassemia.

INTRODUCTION: Allogeneic hematopoietic stem cell transplantation (HSCT) is the only definitive curative option for ß-major thalassemia patients (ß-MT). Posterior reversible encephalopathy syndrome (PRES) is a pervasive neurological complication which typically occurs following HSCT. ß-MT patients are prone to a higher PRES incidence due to long-term immunosuppression; thus, it is imperative that these patients are closely monitored for PRES after HSCT. PATIENTS AND METHODS: We included 148 pediatric patients with ß-MT who underwent HSCT between March 2015 and August 2022 in Children's Medical Center. Patients in this study were divided into two groups. The association between PRES and class of ß-MT and other risk factors were assessed and the overall survival rate was determined. RESULTS: Fourteen out of 112 patients (12%) with class I and II ß-MT developed PRES. However, PRES occurred in 11 out of 36 patients (30.5%) with ß-MT-III. Our results indicated that there was a significant association between class III ß-MT and the occurrence of (P = .004). Additionally, acute graft-versus-host disease (aGVHD) occurred in 80% and 44.7% of patients in the PRES and non-PRES groups, respectively (P = .001). The results of the Kaplan-Meier analysis revealed that the 5-year overall survival (OS) was 75.6% in the PRES group versus 95% in the non-PRES group, which was statistically significant (P = .001). CONCLUSION: Based on our results, pediatric ß-MT III patients are at a higher risk of developing PRES. Additionally, pediatric ß-MT patients with a history of aGVHD, regardless of disease class, are more likely to develop PRES. Considering these results, PRES has a higher chance of being the etiology of symptoms and should be considered more often in these patients.

The challenging clinical dilemma of posterior reversible encephalopathy syndrome in systemic lupus erythematosus.

BACKGROUND AND OBJECTIVE: Posterior reversible encephalopathy syndrome (PRES) in systemic lupus erythematosus (SLE) is a challenging clinical dilemma. A retrospective single-center study was performed to investigate the clinical features, risk factors, outcomes, and clinical determinants of the prognosis of PRES in SLE. METHODS: A retrospective study was performed from January 2015 to December 2020. 19 episodes of lupus PRES and 19 episodes of non-lupus PRES were identified. 38 cases of patients presenting with neuropsychiatric lupus (NPSLE) hospitalized during the same period were selected as controls. Survival status was acquired via outpatient and telephone follow-up in December 2022. RESULTS: The clinical neurological presentation of PRES in lupus patients was similar to that of the non-SLE-related PRES and NPSLE populations. Nephritis-induced hypertension is the predominant trigger of PRES in SLE. Disease flare and renal failure-triggered PRES were identified in half of the patients with SLE. The mortality rate of lupus-related PRES during the 2­year follow-up was 15.8%, the same as that of NPSLE. For patients with lupus-related PRES, multivariate analysis indicated that high diastolic blood pressure (OR =1.762, 95% CI: 1.031 ~ 3.012, p = 0.038), renal involvement (OR = 3.456, 95% CI: 0.894 ~ 14.012, p = 0.049), and positive proteinuria (OR = 1.231, 95% CI: 1.003 ~ 1.511, p = 0.047) were independent risk factors compared to NPSLE. A strong connection between the absolute counts of T and/or B cells and prognosis in lupus patients with neurological manifestations was found (p < 0.05). The lower the counts of T and/or B cells, the worse the prognosis. CONCLUSION: Lupus patients with renal involvement and disease activity are more likely to develop PRES. The mortality rate of lupus-related PRES is similar to that of NPSLE. Focusing on immune balance might reduce mortality.

Lithium-induced subacute diencephalic angio endotheliopathy.

A 65-years-old woman with bipolar affective disorder presented to our ED with a severe lithium intoxication and the recent onset of confusion, clumsiness, and tremors. Symptoms worsened to stupor and anarthria immediately after hospital admission. Gadolinium-enhanced brain Magnetic Resonance Imaging (MRI) showed signal hyperintensity involving both thalami in T2weighted (T2w)/Fluid Attenuated Inversion Recovery sequences (right > left), restricted areas of proton diffusivity at the level of both occipital lobes and a sharp contrast enhancement of thalami, rhombencephalon, and of leptomeninges from either the temporal, parietal, occipital lobes as well as from the cerebellar folia (right > left). These findings were consistent with a severe form of Posterior Encephalopathy known as Subacute Diencephalic Angio Endotheliopahty (SDAE). In addition, Magnetic Resonance Angiography revealed thrombosis of the right transverse and sigmoidal sinuses up to confluence with the jugular vein. The MRI picture resolved one month later after a course of high dosage dexamethasone. The patient deceased one month after discharge, mainly due to Diabetes Insipidusassociated hypernatremia. Dissecting the "Pandora's box" represented by complex MRI findings (SDAE and sinus thrombosis) in lithium-induced neurotoxicity is fundamental in timely recognizing this threating but potentially reversible clinical picture.

Postoperative Posterior Reversible Encephalopathy Syndrome after Coronary Artery Bypass Surgery.

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

BACKGROUND: Hereditary 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 PRESENTATION: A 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. CONCLUSIONS: This 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.

Posterior Reversible Encephalopathy Syndrome and Eclampsia in the Setting of Magnesium Toxicity: A Case Report.

Posterior reversible encephalopathy syndrome (PRES) is a rare neurologic condition and a feared complication of eclampsia. It is evidenced by acute neurologic dysfunction secondary to cerebral edema and is typically reversible in nature. Although it is a relatively new diagnosis, an increasing amount of literature has described its occurrence, including an association with hypomagnesemia. We present a case wherein a 24-year-old parturient developed PRES and eclampsia in the setting of symptomatic hypermagnesemia, requiring management with lorazepam after seizures developed. Here we detail her clinical course, including the unique challenges of treating eclampsia and PRES in the setting of magnesium toxicity.

Antiglomerular basement membrane antibody type rapidly progressive glomerulonephritis with seizures: Two cases and literature review.

BACKGROUND: Rapidly progressive glomerulonephritis (RPGN) is clinically manifestations as a rapidly progressive renal failure and pathologically as crescentic and necrotizing lesions with infiltration of inflammatory cells in the glomeruli. Uremic encephalopathy (UE) usually develops in patients who are suffering from acute or chronic renal failure. OBJECTIVE: The purpose of this article is to provide reference for clinical diagnosis and treatment of renal disease complicated with seizures. Patients Two cases of anti-glomerular basement membrane type rapidly progressive glomerulonephritis complicated with seizures were reported. MATERIALS & METHODS: In case 1, a 40-year-old woman was hospitalized for the treatment of nausea, anorexia, and fever. On admission, she presented with elevated serum inflammatory indicators, moderate anemia, and advanced acute kidney injury requiring hemodialysis. Her anti-glomerular basement membrane (GBM) antibody in serum and renal tissues was found to be extremely high. She was finally diagnosed with anti-GBM disease. She was treated with a combination of corticosteroid pulse therapy, oral cyclophosphamide and prednisolone, and plasma exchange, while continued to require maintenance hemodialysis for end-stage kidney disease. During treatment, she suddenly suffered blindness, seizure, and consciousness disturbance. She was diagnosed as posterior reversible leukoencephalopathy syndrome by magnetic resonance imaging (MRI). The posterior reversible leukoencephalopathy syndrome subsided quickly after control of her hypertension and reinforcement of immunosuppressive treatment. In case 2, the patient also developed epileptic symptoms on the basis of GBM disease, and was given treatment similar to that of Case 1, so that the epileptic symptoms were controlled. RESULT: Reversible posterior leukoencephalopathy syndrome, especially when accompanied by cerebral hemorrhage, may lead to irreversible and lethal neurological abnormalities, and nephrologists should, therefore, be aware of the potential risk of reversible posterior leukoencephalopathy syndrome in patients with anti-GBM disease. We can discuss the current two cases in the light of the previous literature.

Misdiagnosis of Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome in the Emergency Department.

Background Cerebrovascular dysregulation syndromes, posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS), are challenging to diagnose because they are rare and require advanced neuroimaging for confirmation. We sought to estimate PRES/RCVS misdiagnosis in the emergency department and its associated factors. Methods and Results We conducted a retrospective cohort study of PRES/RCVS patients using administrative claims data from 11 states (2016-2018). We defined patients with a probable PRES/RCVS misdiagnosis as those with an emergency department visit for a neurological symptom resulting in discharge to home that occurred ≤14 days before PRES/RCVS hospitalization. Proportions of patients with probable misdiagnosis were calculated, characteristics of patients with and without probable misdiagnosis were compared, and regression analyses adjusted for demographics and comorbidities were performed to identify factors affecting probable misdiagnosis. We identified 4633 patients with PRES/RCVS. A total of 210 patients (4.53% [95% CI, 3.97-5.17]) had a probable preceding emergency department misdiagnosis; these patients were younger (mean age, 48 versus 54 years; P<0.001) and more often female (80.4% versus 69.3%; P<0.001). Misdiagnosed patients had fewer vascular risk factors except prior stroke (36.3% versus 24.2%; P<0.001) and more often had comorbid headache (84% versus 21.4%; P<0.001) and substance use disorder (48.8% versus 37.9%; P<0.001). Facility-level factors associated with probable misdiagnosis included smaller facility, lacking a residency program (62.2% versus 73.7%; P<0.001), and not having on-site neurological services (75.7% versus 84.3%; P<0.001). Probable misdiagnosis was not associated with higher likelihood of stroke or subarachnoid hemorrhage during PRES/RCVS hospitalization. Conclusions Probable emergency department misdiagnosis occurred in ≈1 of every 20 patients with PRES/RCVS in a large, multistate cohort.

Rituximab-associated PRES in antibody-mediated kidney rejection: A case report.

Posterior Reversible Encephalopathy Syndrome (PRES) is a rare neurological disorder with a wide range of neurological symptoms. Different risk factors are known for PRES in patients with a history of kidney transplantation; these patients developing PRES were associated with immunosuppressants and cytotoxic drug therapies, including reports of rituximab therapy. Herein, we report a case of rituximab-associated PRES in the context of antibody-mediated kidney allograft rejection. A 29-year-old male patient with antibody-mediated kidney rejection was treated with rituximab, and then he developed PRES. The patient, who was transplanted with a kidney allograft five years earlier, was continuously treated with standard tacrolimus and mycophenolate mofetil therapy without any symptoms of PRES. Rituximab treatment was started to block an ongoing kidney rejection, and the patient received a second dose of rituximab four days prior to the hospital admission. At admission, the patient demonstrated symptoms of headache, nausea, and photophobia. The brain magnetic resonance imaging (MRI) showed changes consistent with PRES. After 12 days of hospitalization, he was discharged with a complete cessation of the initial symptoms. We postulate that possible endothelial dysfunction caused by rituximab may explain the condition leading to PRES. It is unclear whether rituximab, when used in kidney rejection patients who receive other immunosuppressants, may contribute to PRES.

SARS-CoV-2 associated posterior reversible encephalopathy syndrome (PRES) - a review of 82 cases.

OBJECTIVES: Severe, acute, respiratory syndromecoronavirus- 2 (SARS-CoV-2) infections can be complicated by central nervous system (CNS) disease. One of the CNS disorders associated with Coronavirus Disease-19 (COVID- 19) is posterior reversible encephalopathy syndrome (PRES). This narrative review summarises and discusses previous and recent findings on SARS-CoV-2 associated PRES. METHODS: A literature search was carried out in PubMed and Google Scholar using suitable search terms and reference lists of articles found were searched for further articles. RESULTS: By the end of February 2023, 82 patients with SARS-CoV-2 associated PRES were recorded. The latency between the onset of COVID-19 and the onset of PRES ranged from 1 day to 70 days. The most common presentations of PRES were mental deterioration (n=47), seizures (n=46) and visual disturbances (n=18). Elevated blood pressure was reported on admission or during hospitalisation in 48 patients. The most common comorbidities were arterial hypertension, diabetes, hyperlipidemia and atherosclerosis. PRES was best diagnosed by multimodal cerebral magnetic resonance imaging (MRI). Complete recovery was reported in 35 patients and partial recovery in 21 patients, while seven patients died. CONCLUSIONS: PRES can be a CNS complication associated with COVID-19. COVID-19 patients with mental dysfunction, seizures or visual disturbances should immediately undergo CNS imaging through multimodal MRI, electroencephalography (EEG) and cerebrospinal fluid (CSF) studies in order not to miss PRES.

Atypical and isolated posterior reversible encephalopathy syndrome in postpartum without preeclampsia: a case report.

Posterior reversible encephalopathy syndrome (PRES) is a neurological complication frequently found during brain exploration for severe preeclampsia when it is associated with neurological signs. Being a newly discovered entity, its mechanism of genesis is still based on a hypothesis not yet confirmed. The clinical case that we report highlights an atypical PRES syndrome occurring in postpartum without any signs of preeclampsia. The patient had suffered a state of convulsive dysfunction after delivery without hypertension and the diagnosis of PRES syndrome was confirmed based on the results of the brain computed tomography (CT) scan, she showed signs of clinical improvement on the fifth day of postpartum. Our case report calls into question the association between PRES syndrome and preeclampsia that we find in literature and puts a big question mark on the causal link between the two in pregnant women.

Headache and systemic lupus erythematosus: A narrative review.

OBJECTIVE: To review the epidemiology, the differential diagnosis, and the clinical and laboratory factors associated with the care management of headaches in patients with systemic lupus erythematosus (SLE). BACKGROUND: SLE is a chronic autoimmune disease and in 12%-95% of patients, the nervous system is involved. Headache is a frequently reported, although nonspecific, symptom that may potentially represent serious underlying diagnoses. Primary headaches may also occur in these patients, thereby causing a negative and significant impact on their quality of life. METHODS: This is a narrative review. A literature review was conducted on the PubMed platform using the following terms: (1) headache and (2) lupus. All articles considered relevant were included. No limitations were imposed for the publication date. RESULTS: Headache is a frequent symptom in patients with SLE. Although its prevalence is similar to the general population, headaches nonetheless tend to have a greater negative impact on these patients. Patients with SLE are more likely to experience headache due to vascular diseases such as cerebral venous sinus thrombosis, stroke, reversible cerebral vasoconstriction syndrome, posterior reversible encephalopathy syndrome, and vasculitis. Aseptic meningitis, neuroinfections, intracranial neoplasms, and intracranial hypertension or hypotension may also be a cause of headache in these patients. Although used in disease activity scores, the concept of lupus headache is controversial. CONCLUSIONS: Headache is a frequent symptom in patients with SLE. An appropriate approach enables the potentially serious conditions, which are the causes of secondary headaches, to be recognized and treated, together with an appropriate diagnosis and treatment of primary headaches.

Pathogenesis of acute encephalopathy in acute hepatic porphyria.

Acute encephalopathy (AE) can be a manifestation of an acute porphyric attack. Clinical data were studied in 32 patients during AE with or without polyneuropathy (PNP) together with 12 subjects with PNP but no AE, and 17 with dysautonomia solely. Brain neuroimaging was done in 20 attacks during AE, and PEPT2 polymorphisms were studied in 56 subjects, 24 with AE. AE manifested as a triad of seizures, confusion and/or blurred vision. Symptoms lasting 1-5 days manifested 3-19 days from the onset of an attack. 55% of these patients had acute PNP independent of AE. Posterior reversible encephalopathy syndrome (PRES) was detected in 42% of the attacks. These patients were severely affected and hyponatremic (88%). Reversible segmental vasoconstriction was rare. There was no statistical difference in hypertension or urinary excretion of porphyrin precursors among the patients with or without AE. In 94% of the attacks with AE, liver transaminases were elevated significantly (1.5 to fivefold, P = 0.034) compared to a normal level in 87% of the attacks with dysautonomia, or in 25% of patients with PNP solely. PEPT2*2/2 haplotype was less common among patients with AE than without (8.3% vs. 25.8%, P = 0.159) and in patients with PNP than without (9.5% vs. 22.9%, P = 0.207), suggesting a minor role, if any, in acute neurotoxicity. In contrast, PEPT2*2/2 haplotype was commoner among patients with chronic kidney disease (P = 0.192). Acute endothelial dysfunction in porphyric encephalopathy could be explained by a combination of abrupt hypertension, SIADH, and acute metabolic and inflammatory factors of hepatic origin.