Posterior Reversible Encephalopathy Syndrome Research Articles

Posterior Reversible Encephalopathy Syndrome in Children and Adolescents.

Posterior reversible encephalopathy syndrome, or PRES, is a constellation of severe, acute hypertension and specific brain imaging findings. This may be caused by failure of the cerebral autoregulatory system to manage acute or severe changes in blood pressure. The incidence in children is unknown but estimated to be more common in children with predisposing factors including renal disease, autoimmune disease, malignancy, solid organ transplantation, stem cell transplantation, hypertension, sepsis, and exposure to certain medications. Management of PRES includes addressing hypertension, removing offending agents when possible, and anti-epileptic medications. Most children with PRES recover completely, but recurrence is possible. Lack of resolution of imaging findings likely portends a worse prognosis.

Intracerebral hemorrhagic developing in a patient with posterior reversible encephalopathy syndrome; a case report

We describe a case of hemorrhagic posterior reversible encephalopathy syndrome (PRES) in a 19-year-old woman, who had undergone a cesarean section for epilepsy and lupus-related chronic renal failure 2 months ago, and presented to the emergency department with complaints of blurred vision. The neurological and ophthalmological examinations were normal. Despite the initial imaging being normal, subsequent magnetic resonance images showed hyperintensity in bilateral occipital lobes and cerebellum (indicative of PRES) and watershed ischemia in addition to intracerebral hemorrhage. Brain surgery was performed, external ventricular drainage was placed and she was monitored postoperatively with routine care in the intensive care unit where she remained intubated for a month, after which she died of the consequences of her condition.

Alcohol-related posterior reversible encephalopathy syndrome: a case report of a patient managed with a benzodiazepine-sparing regimen for alcohol withdrawal

ABSTRACT We report a case of posterior reversible encephalopathy syndrome (PRES) during treatment for alcohol withdrawal syndrome with gabapentin and clonidine. The patient developed severe hypertension, confusion and tremor, culminating in bilateral vision loss and a seizure. Imaging revealed posterior cerebral edema. Treatment with benzodiazepines, antihypertensives, and anti-seizure medications led to resolution. One year later, imaging showed resolution of the findings. We review the associated literature and propose the recognition of a PRES sub-entity, Alcohol-Related PRES (ARPRES), which can appear in the setting of alcohol withdrawal syndrome, chronic alcohol use, and acute alcohol intoxication, with or without hypertension.

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

IntroductionPosterior 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.MethodsThis 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.ResultsDuring 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.ConclusionPRES 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 associated with antibiotic therapy: a case report and systematic review.

Posterior reversible encephalopathy syndrome (PRES) is an acute neurological condition associated with different etiologies, including antibiotic therapy. To date, most data regarding antibiotic-related PRES are limited to case reports and small case series. Here, we report a novel case description and provide a systematic review of the clinico-radiological characteristics and prognosis of available cases of PRES associated with antibiotic therapy. We performed a systematic literature search in PubMed and Scopus from inception to 10 January 2024, following PRISMA guidelines and a predefined protocol. The database search yielded 12 subjects (including our case). We described the case of a 55-year-old female patient with PRES occurring one day after administration of metronidazole and showing elevated serum neurofilament light chain protein levels and favorable outcome. In our systematic review, antibiotic-associated PRES was more frequent in female patients (83.3%). Metronidazole and fluoroquinolones were the most reported antibiotics (33.3% each). Clinical and radiological features were comparable to those of PRES due to other causes. Regarding the prognosis, about one third of the cases were admitted to the intensive care unit, but almost all subjects (90.0%) had a complete or almost complete clinical and radiological recovery after prompt cessation of the causative drug. Antibiotic-associated PRES appears to share most of the characteristics of classic PRES. Given the overall good prognosis of the disease, it is important to promptly diagnose antibiotic-associated PRES and discontinue the causative drug.

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

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

P026 Neurofascin antibodies complicating a case of severe systemic lupus erythematosus

Abstract Background/Aims Juvenile-onset systemic lupus erythematosus (jSLE) is an autoimmune disease that results in increased disease activity and damage compared to adult-onset SLE. There is increased medication burden, severe organ manifestations and higher incidence of renal, cardiovascular and neuropsychiatric (NP) involvement. Anti-nervous system (NS) antibodies are seen across immune mediated diseases including SLE. Myelin oligodendrocyte glycoprotein (MOG) and neurofascin (NF) 186 antibodies are particularly associated with higher frequencies of NP-SLE. These patients can present with the distinct clinical phenotype associated with that antibody (i.e., demyelination with MOG, neuropathy with NF186). Methods We present an SLE patient with progressive neurological features and anti-NF antibodies who responds well to ocrelizumab after modest response to initial immunotherapy. Results An 18 year old female has active jSLE including malar rash, alopecia, arthralgia, aphthous ulcers, headaches. She presents with lower limb and ascending abdominal paraesthesia and left 6th cranial nerve palsy alongside a recent respiratory tract infection. ANA 1:5120 homogenous pattern, high dsDNA, low C3 and C4, thrombocytopenia and antibodies to Sm, Ro, RNP, anti-Rib-P and strongly positive lupus anticoagulant antibodies and low titre anti-cardiolipin (aCL) IgG and IgM. Lumbar puncture is unremarkable. Nerve conduction studies (NCS) demonstrate progressive predominant motor inflammatory neuropathy without demyelination. CT, MRI brain and MRI spine are normal. She is treated as atypical Guillain-Barre Syndrome (GBS)/ Miller-Fischer with 5 days IV immunoglobulin 2g/kg and also for active SLE with 3 days 500mg IV methylprednisolone. She is intubated following worsening dysphagia, dysarthria, muscle weakness and reduced consciousness. Plasma exchange and pulsed cyclophosphamide are subsequently given. Eye and facial movements worsen with NCS showing worsening patchy axonal sensorimotor polyneuropathy. Serial MRI brain is suggestive of posterior reversible encephalopathy syndrome (PRES). Autonomic instability is seen with labile blood pressure and tachycardia. Given the unusual presentation, anti-NF antibodies are tested and identified at 1:3200 positive, NF155 and 186, both IgG1 and IgG2. Given previous adverse reaction to rituximab, she received ocrelizumab which lead to a marked improvement in her sensorimotor symptoms and functionality. C3 and C4 levels show considerable improvement which is sustained and dsDNA levels normalise. B cell depletion therapy was chosen following a small case series of patients with neurofascin antibodies who developed severe autoimmune neuropathies. Half of these patients responded well to rituximab. One proposed mechanism being the targeting of immature CD20+ B cells inhibits plasmablast generation, which may be responsible for neurofascin antibody production early on in the disease. Conclusion Anti-NS antibodies are seen in SLE patients and their presence can suggest increased frequency of NP-SLE suggesting a potential future role as a biomarker. One should consider anti-NF antibody testing in patients with a GBS-like illness with progressive neuropathy, respiratory involvement and autonomic dysfunction despite standard immunotherapy. Disclosure P. Yee: None. M. Mouyis: None.

E029 Intracranial haemorrhage in scleroderma renal crisis; an unusual presentation

Abstract Background/Aims Scleroderma renal crisis is a life-threatening complication of systemic sclerosis (SSc), which typically occurs in the first 5 years after disease onset. Several risk factors for development of renal crisis are well recognised, including diffuse skin involvement, presence of RNA polymerase 3 antibodies and preceding use of corticosteroids. Methods A 66 year old female 6 years post diagnosis of stable diffuse systemic sclerosis/ polymyositis overlap (PM Scl 100 positive) presented to her GP with a 1 week history of fatigue. She had been seen 2 months earlier by her rheumatologist with stable, mild skin involvement. She had no preceding history of hypertension or other significant co-morbidity. A month earlier she received antibiotics for a chest infection from which she made a slow but complete recovery. She was found to have shingles and started a course of oral acyclovir. Blood tests revealed acute kidney injury stage 3 (eGFR 10 from baseline 70). However, she deteriorated overnight, waking with headache and disturbed colour vision. This quickly deteriorated to bilateral blindness precipitating admission to the emergency department (ED). On arrival she was conscious but severely hypertensive with a BP of 240/105. She received ramipril, amlodipine and a GTN infusion with limited efficacy. CT head (CTH) showed a left occipitoparietal bleed. She then experienced a seizure in ED, which was terminated with lorazepam. She was intubated in the post ictal phase. Repeat CTH following the seizure showed a right frontal bleed extending into the subarachnoid space. Repeat CTH at 48 hours showed progression of the right frontal and left parieto-occipital intracerebral haemorrhages, with evidence of posterior reversible encephalopathy syndrome. Lumbar puncture was normal (VZV negative). Treatment was commenced with intravenous hydralazine. On day 2 of admission she required haemofiltration and subsequently regular renal dialysis. Results SSc was diagnosed in 2016, since when her main issue had been severe Raynaud’s phenomenon for which she took sildenafil. Her muscle disease had only ever manifested as a modestly raised CPK, which settled with a brief course of low dose prednisolone at diagnosis. She has never required DMARDs. She had mild reflux symptoms. Skin tightening was noted distally over her fingers, forearms, lower legs and feet. Annual echocardiography showed nothing to suggest developing pulmonary arterial hypertension. With supportive management she has made a remarkable neurological recovery and has returned to her pre-morbid level of function. She remains dialysis dependent at 4 months. Conclusion This lady lacked a single recognised risk factor for developing a renal crisis, and this occurred quite unexpectedly following a reassuring clinic visit just 2 months prior to presentation. As rheumatologists we must remain vigilant for features of a renal crisis and avoid being falsely reassured by a low risk profile. Disclosure L. Parker: None. N. Buchanan: None. E.L. Williams: None.

The Treatment of Steroid-Refractory Severe Gastrointestinal Acute Graft-Versus-Host Disease in Children after Allogeneic Hematopoietic Stem Cell Transplantation: A Single-Centre Experience

Acute graft-versus-host disease is a common complication of allogeneic hematopoietic stem cell transplantation and is a major cause of morbidity and mortality. Systemic steroid therapy is the first-line treatment for acute graft-versus-host disease, although about half of patients will become refractory to treatment. We aimed to evaluate treatment options by reviewing available alternatives for patients with steroid-refractory acute graft-versus-host disease by comparing data from recently published studies. We retrospectively studied the safety and efficacy of treatment in 22 children with steroid‐dependent/refractory acute GVHD between the years 2010 and 2023. Seven (31.8%) out of 22 patients with acute graft-versus-host disease were still alive. The seven surviving patients have been followed for an average of 1141 (±403) days. 15 non-responders with grade III/IV acute graft-versus-host disease died from causes associated with acute graft-versus-host disease and/or other conditions. Among 15 patients who died, the number of patients who received all three treatments, MSC, ECP and infliximab, was 5 (33%). The most common cause of death was infection (8 cases in 15 patients, 53.3%). Other causes of death were gastrointestinal hemorrhages (n=5, 33.3%), and intracranial hemorrhages (n=2, 13.3%). When factors causing morbidity were evaluated, it was observed that three patients developed posterior reversible encephalopathy syndrome, two patients developed hepatic veno-occlusive disease, and one patient developed hypertension. The estimated probability of survival after 1 year was 31.8%, and the median survival was 655 days. We believe that it would be crucial to show the safety and efficacy of novel treatments in comprehensive, randomized clinical trials.

PRESenting a Challenge: Posterior Reversible Encephalopathy Syndrome (PRES) in pediatric patients of Guillain-Barré Syndrome (GBS) a case series and review of literature

BackgroundGuillain-Barre 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. MethodsMedical records of seventy-five 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. ResultsAll 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. ConclusionGBS-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.

Neuropsychiatric manifestation in patients with systemic lupus erythematosus: A unique case series

Neuropsychiatric systemic lupus erythematosus (NPSLE) affects the brain, spinal cord, or other nerves and leads to neurological and psychiatric symptoms directly related to SLE. We report a series of four female patients with NPSLE as the first presentation for SLE. None of them were previously diagnosed with SLE. The first case involved a 50-year-old patient who presented with back pain and lower limb weakness for three weeks associated with numbness. A lumbar puncture was done, and CSF analysis showed high WBC and normal glucose levels with negative CSF cultures for bacteria. The patient was diagnosed with aseptic meningitis and treated with antibiotics and antiviral medication without improvement. So she was treated as a case of SLE aseptic meningitis with steroids. In the second case, a 20-year-old patient had a generalized tonic-clonic seizure with the acute manifestation of hypertensive urgency. Magnetic resonance imaging (MRI) was done and suggested posterior reversible encephalopathy syndrome (PRES). The third case was for a 38-year-old patient who complained of dizziness, general weakness, and convulsion. On review of the systems, the patients had positive symptoms of SLE. Anti-nuclear antibody (ANA) and double-stranded ds-DNA were positive. Computed tomography (CT) scans were done and showed a thymoma. The patient was diagnosed with SLE in association with focal seizures and thymoma. In the last case, a 44-year-old patient presented with tongue heaviness and right-side weakness. MRI revealed small acute lacunar infarction scattered at both cerebral hemispheres and the right side of the cerebellum, and chest CT showed pulmonary embolism (PE). The patient was diagnosed with an acute ischemic stroke and PE related to SLE. In conclusion, the neurological manifestations of SLE are a significant issue for both patients and clinicians. Here we report a unique case series and describe the prognosis and outcome of the treatment in these cases.

Perioperative Cerebrovascular Accidents in Spine Surgery: A Retrospective Descriptive Study and A Systematic Review with Meta-Analysis.

Perioperative cerebrovascular accidents (CVAs) related to spine surgery, although rare, can lead to significant disabilities. More studies on spine surgeries are required to identify those at risk of perioperative CVAs. The characteristics and outcomes of patients that experienced CVAs during spine surgery were assessed through a retrospective descriptive study and meta-analysis. Patients aged ≥18 years who underwent spine surgery under general anesthesia at a hospital between April 2011 and March 2023 were examined. Of the 2,391 initially identified patients, 2,346 were included after excluding 45 who underwent debridement for surgical site infections. Subsequently, a meta-analysis including the present retrospective descriptive study was conducted. Databases such as PubMed and Google Scholar were searched for original peer-reviewed articles written in English. Of the 2,346 patients, 4 (0.17%) (three men, one woman) exhibited perioperative CVAs associated with spine surgery. The CVAs were diverse in nature: one case of cerebral hemorrhage resulting from dural injury during posterior occipitocervical fusion, two cases of cerebral infarctions after lumbar laminectomy and anterior thoracic fusion due to anticoagulant discontinuation, and one case of posterior reversible encephalopathy syndrome following microscopic lumbar discectomy due to gestational hypertension. The subsequent meta-analysis included three studies (n=186,860). It showed several risk factors for perioperative CVAs, including cervical level (pooled odds ratio [OR]=1.33), hypertension (pooled OR=2.27), atrial fibrillation (pooled OR=8.78), history of heart disease (pooled OR=2.47), and diabetes (pooled OR=2.13). It was speculated that the potential risk factors for the four perioperative CVA cases of spine surgery in this retrospective descriptive study were intraoperative dural injury, preoperative anticoagulant discontinuation, and gestational hypertension history. The meta-analysis revealed that cervical spine surgery, hypertension, atrial fibrillation, heart disease, and diabetes increased the CVA risk. This highlights the need for risk assessment, preoperative optimization, and postoperative care to reduce spine surgery-associated perioperative CVAs.

POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME IN ECLAMPTIC PATIENTS: THE RELATIONSHIP BETWEEN BLOOD PRESSURE, CRANIAL INVOLVEMENT, AND SEIZURE RECURRENCE.

Posterior reversible encephalopathy syndrome(PRES) is a clinic radiological disorder characterized by headache, epileptic seizure, encephalopathy, visual impairment, and focal neurological deficits. Gestational hypertension, which is a significant risk factor for PRES, may cause significant morbidity and mortality among pregnant women. Twenty-four patients with PRES caused by eclampsia who were admitted to our hospital in the last 5 years were included in this study. Blood pressure at admission, number of regions with vasogenic edema in the brain, and recurrent seizures were noted. Patients were divided into three groups: mild, moderate, and severe. Using Kruskal-Wallis and Pearson Chi-square tests, there was no statistical significance between the groups in terms of cranial involvement(P: 0.471). However, binary logistic regression analysis showed that seizure recurrence increased in correlation with blood pressure (P: 0.04) Limitations: PRES is a rare syndrome associated with several etiologies. In our study, only patients with PRES due to eclampsia were included. Therefore, the number of included patients was limited (24 participants). PRES may occur in eclamptic patients with mild, moderate or severe blood pressure values. Evaluation by MRI is needed to confirm the diagnosis. Early and rapid treatment is essential for reducing the morbidity and mortality among pregnant women.

Analysis of drug-induced posterior reversible encephalopathy syndrome using the food and drug administration adverse drug events reporting system database

ABSTRACT Objective In this retrospective pharmacovigilance study, we gathered data on drug-induced posterior reversible encephalopathy syndrome (PRES). Our goal was to identify the primary suspect drugs in PRES by analyzing the Food and Drug Administration Adverse Events Reporting System (FAERS) database. Methods We identified and analyzed reports of PRES listed in the FAERS database between 2004 and 2021. Using the reporting odds ratio and 95% confidence interval, we evaluated the safety signals for each of the drugs associated with PRES. Results We reviewed 11,077 reports of adverse events corresponding to PRES. The primary suspect drug categories were antineoplastics, immunosuppressants, and glucocorticoids. PRES was 24.77% more likely to occur in females than in males. Drug-induced PRES usually occurs in individuals with cancer, those who have undergone an organ/stem cell transplant, and those with autoimmune conditions. Conclusion Our results show that the drugs most commonly suspected to cause PRES were antineoplastics, immunosuppressants, and glucocorticoids. Future studies are needed to illuminate the pathophysiological alterations that underlie PRES. In the meantime, prescribers and patients should be made aware of the potential risks of PRES associated with pharmaceutical therapy, and the summaries of product characteristics for individual drugs should be updated to include this information.

Association between renal insufficiency and lesion characteristics of posterior reversible encephalopathy syndrome.

Posterior reversible encephalopathy syndrome (PRES) is characterized by cerebral blood flow dysregulation and the blood-brain barrier (BBB) disruption. While renal insufficiency has been considered a factor in BBB fragility, the relationship between renal insufficiency and the PRES lesions volume remains unclear. This observational study was performed retrospectively. PRES patients were categorized into two groups with renal insufficiency, defined as an estimated glomerular filtration rate (eGFR) of less than 60mL/min/1.73m2 on the day of symptom occurrence. Lesion volume was measured using fluid-attenuated inversion recovery (FLAIR) imaging, and the brain was divided into nine regions. The volume of the parietal-occipital-temporal lobe was considered typical, while the other six regions were labeled as atypical. The study included 200 patients, of whom 94 (47%) had renal insufficiency. Patients with renal insufficiency had a larger lesion volume (144.7 ± 125.2cc) compared to those without renal insufficiency (110.5 ± 93.2cc; p = 0.032); particularly in the atypical lesions volume (49.2 ± 65.0 vs. 29.2 ± 44.3cc; p = 0.013). However, there was no difference in the reversibility of the lesions (35.2 ± 67.5 vs. 18.8 ± 33.4cc; p = 0.129). Multiple regression analysis revealed that decreases in eGFR (β = -0.34, 95% CI -0.62-0.05, p = 0.020) were positively associated with total lesion volume. Our findings suggest that PRES patients with renal insufficiency experience more severe lesion volumes, likely due to the atypical brain regions involvement. The lesions involving atypical regions may have a similar pathophysiology to typical lesions, as the PRES lesions reversibility was found to be similar between individuals with and without renal insufficiency.

Frequency of ischaemic stroke and intracranial haemorrhage in patients with reversible cerebral vasoconstriction syndrome (RCVS) and posterior reversible encephalopathy syndrome (PRES) - A systematic review.

Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) may cause ischaemic stroke and intracranial haemorrhage. The aim of our study was to assess the frequency of the afore-mentioned outcomes. We performed a PROSPERO-registered (CRD42022355704) systematic review and meta-analysis accessing PubMed until 7 November 2022. The inclusion criteria were: (1) original publication, (2) adult patients (≥18 years), (3) enrolling patients with PRES and/or RCVS, (4) English language and (5) outcome information. Outcomes were frequency of (1) ischaemic stroke and (2) intracranial haemorrhage, divided into subarachnoid haemorrhage (SAH) and intraparenchymal haemorrhage (IPH). The Cochrane Risk of Bias tool was used. We identified 848 studies and included 48 relevant studies after reviewing titles, abstracts and full text. We found 11 studies on RCVS (unselected patients), reporting on 2746 patients. Among the patients analysed, 15.9% (95% CI 9.6%-23.4%) had ischaemic stroke and 22.1% (95% CI 10%-39.6%) had intracranial haemorrhage. A further 20.3% (95% CI 11.2%-31.2%) had SAH and 6.7% (95% CI 3.6%-10.7%) had IPH. Furthermore, we found 28 studies on PRES (unselected patients), reporting on 1385 patients. Among the patients analysed, 11.2% (95% CI 7.9%-15%) had ischaemic stroke and 16.1% (95% CI 12.3%-20.3%) had intracranial haemorrhage. Further, 7% (95% CI 4.7%-9.9%) had SAH and 9.7% (95% CI 5.4%-15%) had IPH. Intracranial haemorrhage and ischaemic stroke are common outcomes in PRES and RCVS. The frequency reported in the individual studies varied considerably.

Tacrolimus-associated neurotoxicity isolated to the brainstem: two illustrative cases and a systematic review of the literature.

Tacrolimus-associated neurotoxicity (TAN) manifests with wide clinical spectrum, ranging from mild tremors to severe encephalopathy. The isolated involvement of the brainstem is a rarely documented presentation of TAN, and its clinical and diagnostic characteristics are unclear. We report two cases of brainstem-isolated TAN (bi-TAN). Moreover, we performed a systematic review of the literature on bi-TAN and extracted data concerning demographics, clinical characteristics, radiological features, and management. The systematic literature search followed PRISMA guidelines and a pre-defined protocol. Eleven patients, including our two, were identified (mean age: 41.3years, ± 18.8; five males, 45%). Speech disturbance was the most common clinical presentation (45%). The mean latency from Tacrolimus initiation to bi-TAN onset was 26days (± 30.8). Tacrolimus serum level tested above the reference range in three patients (mean: 26.83 ± 5.48). Brain MRI showed T2-FLAIR hyperintensities; three showed restricted diffusion on ADC maps. Neurological symptoms resolved completely in seven patients (63%) after Tacrolimus withdrawal or dose reduction. Our findings suggest that bi-TAN could represent a brainstem variant of posterior reversible encephalopathy syndrome. Recognition of bi-TAN as a potential cause of isolated brainstem lesions is crucial to disentangle the diagnostic work-up and ensure prompt withdrawal or reduction of the offending agent.

A study on correlation between neuroimaging and maternal outcome in eclampsia

Background: Hypertensive disorders remain among the most significant complications of pregnancy. Neurological complications of eclampsia are a major contributor to morbidity and mortality that is associated with eclampsia. Neuroimaging studies have revolutionized visualization of hypertensive encephalopathy aids in timely intervention and a favorable maternal and perinatal outcome. Aims and Objectives: (1) To study the spectrum of neuroimaging findings in patients with eclampsia. (2) To correlate these findings with the maternal outcome. Materials and Methods: A prospective observational study was undertaken in the Department of Obstetrics and Gynecology, Mysore Medical College and research institute Mysore, for 18 months. All the eclamptic mothers during the study were included in the study. They were studied in terms of neuroimaging and its correlation with maternal outcomes. Results: The incidence of eclampsia was 7.14% which is relatively high, attributed to the fact that being a tertiary care center many cases are referred. Higher incidence was seen in primigravida (72.4%) and lower maternal age of 18–20 years (48%). Cesarean section was indicated in 50% of deliveries indicating immediate action was necessary for better fetomaternal outcome. They presented with varied imminent symptoms such as headache in 20.4%, vomiting in 22.4%, and visual blurring in 22.4%. About 50% presented with altered sensorium. On non-contrast computed tomography brain, 64.3% (63) had normal findings, 17.3% (17) had posterior reversible encephalopathy syndrome (PRES) with a sensitivity of 68.42% and specificity of 85%, whereas on magnetic resonance imaging (MRI) brain, 36.26% had no abnormalities, 40.81% had PRES with a sensitivity of 86.84%, and specificity of 85%. About 58.6% had an uneventful maternal outcome. The others were hemolysis, elevated liver enzymes, and low platelet syndrome seen in 9.18% of patients, acute kidney injury in 6.12%, postpartum hemorrhage seen in 5.1%. The maternal mortality rate was 7.66%, the most common cause of death was intracranial hemorrhage. Conclusion: The common neuroimaging findings in eclampsia are cerebral edema, PRES, cerebral venous thrombosis, infarcts, hemorrhage, and hypertensive leukoencephalopathy. Although some abnormalities seen in neuroimaging studies are incidental and transient without chronic neurologic sequelae, both CT and MRI findings correlate with the clinical presentation and maternal outcome but MRI correlates better compared to CT and can be a better imaging modality in eclampsia patients and is indicated in all patients of eclampsia.

Reversible cerebral vasoconstriction syndrome and posterior reversible encephalopathy syndrome following vaccination: analysis of the VAERS database and systematic review.

This study aimed to analyze the Vaccine Adverse Event Reporting System (VAERS) database and systematically review the literature to provide a comprehensive analysis of reversible cerebral vasoconstriction syndrome (RCVS) and posterior reversible encephalopathy syndrome (PRES) secondary to vaccination. The authors analyzed the VAERS database and conducted a systematic review following PRISMA guidelines. The inclusion criteria for VAERS data were a score of ≥3 on the RCVS2 score and/or radiographic findings consistent with the diagnosis of RCVS or PRES. The systematic review was registered with PROSPERO. Our combined data set included 29 cases (9 RCVS and 20 PRES). Most cases were women (72.4%) with a mean age of 50.7 years (SD 19.4 years). Most cases were associated with COVID-19 mRNA vaccines (58.6% Moderna, 20.7% Pfizer). Hypertension (37.9%), hyperlipidemia (13.7%), chronic kidney disease (CKD) (10.3%), and end-stage renal disease (6.8%) were common comorbidities. Furthermore, 20.6% (6/29) of cases were on immunosuppression therapy for various reasons. The mean time to symptom onset was 10.49 days after vaccination (SD 18.60), and the mean duration of hospitalization was 7.42 days (SD 5.94). The symptoms reported the most frequently were headache (41.3%), elevated blood pressure (31.0%), and emesis (17.2%). Typical radiographic findings included T2/FLAIR hyperintensities affecting the parieto-occipital lobes, indicative of vasogenic and/or cytotoxic edema. This study provides a comprehensive analysis of postvaccine RCVS and PRES. Both disease states were seen most often in those with pre-existing risk factors such as female sex, age over 50, hypertension, renal disease, and immunosuppression. Vaccines and their associated immune response may cause endothelial dysfunction leading to cerebral vasospasm and loss of cerebral autoregulation. However, further research is required to understand the underlying pathophysiological mechanisms. Despite the associations found, the absolute risk of these syndromes remains extremely low compared to the immense benefits of vaccination.

OPHTHALMOLOGIC MANIFESTATIONS REVEALING POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME: A CASE REPORT

Posterior reversible encephalopathy syndrome (PRES) is a rare clinico-radiological syndrome, secondary to many diseases especially high blood pressure. First described by Hinchey in 1996, pathophysiology is not clear. Clinical manifestations are headache, seizures, altered mental state such as confusion or coma, and also visual disturbances, such as hemianopia, blurred vision, diplopia, or cortical blindness. We report the case of a Moroccan 47-years old patient who arrived at the ophthalmology emergency presenting a sudden vision loss and horizontal diplopia, associated with headache. General examination found a high blood pressure at 240/110mmhg. The funduscopy of both eyes found hypertensive retinopathy. A brain MRI was realized showing bilateral occipital cortex T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities. The diagnosis of Posterior reversible encephalopathy syndrome was retained.