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 and autoimmunity.

Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome characterized by acute or subacute onset of neurological symptoms (e.g., headache, seizure, confusion, vomiting, and diminished eyesight) and impaired endothelial barrier function of the cerebral circulation that leads to bilateral subcortical vasogenic edema, while exhibiting a "reversible" feature in most cases. Clinically, various predisposing or precipitating conditions have been identified, such as hypertension, autoimmune diseases, renal dysfunction/failure, preeclampsia/eclampsia, post-transplantation conditions, and certain therapeutic agents. Among several putative mechanisms, the immune activation hypothesis prevails, as up to 50% of patients with PRES harbor abnormalities related to autoimmunity, such as concurrent systemic lupus erythematosus. In this Review, we summarize the clinical and laboratory evidence that places PRES in the context of autoimmunity.

Clinical features and outcomes of children admitted to the pediatric intensive care unit due to posterior reversible encephalopathy syndrome.

OBJECTIVE: The aim of this study is to analyze the clinical features, neuroimaging findings and outcomes of the children admitted to our tertiary pediatric intensive care unit (PICU) due to posterior reversible encephalopathy syndrome (PRES). METHODS: This was a retrospective study where the hospital records of children admitted to PICU due to PRES between January 1, 2011 and January 1, 2021 were reviewed. RESULTS: We enrolled 14 patients with a median age of 8 years (IQR 2.2-14.2) to study. Eight (57 %) patients were male. All patients had comorbid illnesses such as hemophagocytic lymphohistiocytosis in 3, Β-cell acute lymphoblastic leukemia in 2, and different diagnosis in other patients as one one. Three patients had cardiac arrest, 9 patients had seizures, 5 patients had SE, 12 patients had altered mental status, 8 patients had hypertensive crisis, 1 patient had visual impairment. Thirteen patients had occipital involvement, 11 had parietal involvement, 4 had temporal involvement, 1 had thalamic involvement, 2 had cerebellar involvement, 1 had involvement of the corpus callosum, 1 had brainstem involvement, 1 had hippocampus involvement and 1 had involvement of the basal ganglia. Fourteen patients had supratentorial involvement while 3 had infratentorial involvement. Electroencephalogram was performed for 7 patients, out of which 6 revealed encephalopathy. Median PICU LOS was 19.5 days (IQR 13.2-49.2, minimum 2 - maximum 84 days). Five patients had neurologic sequelae. Four (28.5 %) patients died and ten patients survived. CONCLUSION: Co-occurence of hypertension and seizures should prompt consideration of PRES and urgent neuroimaging, particularly in patients on immunosuppressants or chemotherapeutics. Hypertension should be addressed aggressively in patients with PRES. Electroencephalographic monitoring should be performed if there is suspicion of SE or nonconvulsive SE. Despite its usually good prognosis, PRES can cause serious morbidity and mortality with delay in diagnosis or treatment.

Predictors of Hospital Outcomes Among Patients with Posterior Reversible Encephalopathy Syndrome.

OBJECTIVES: We aimed to identify independent predictors of the clinical outcomes of posterior reversible encephalopathy syndrome (PRES) in the Thai-Asian population. MATERIALS AND METHODS: This retrospective cohort study recruited PRES patients admitted in the hospital between 2006 and 2019. The baseline characteristics, clinical presentations, precipitating factors, neuroimaging characteristics, hospital complications, and hospital outcomes were compared between the groups with favourable (modified Rankin scale [mRS] score, 0-2) and unfavourable (mRS score, 3-6) clinical outcomes. Factors presenting with p-values < 0.5 in univariate analysis were analysed in the multiple logistic regression model to determine independent predictors of outcome. RESULTS: Among 136 PRES patients, 22.80% experienced unfavourable outcomes (mRS score, 3-6), and the mortality rate was 7.35%. The common presenting symptoms in the unfavourable clinical outcome group were impairment of consciousness (90.32%) and seizures (67.74%). Sepsis as a hospital complication (adjusted odds ratio [aOR], 32.95; 95% confidence interval [CI], 4.44-244.22, p = 0.001), acute kidney injury as a hospital complication (aOR, 9.94; 95% CI, 1.71-57.66; p = 0.010), and impairment of consciousness (aOR, 10.85; 95% CI, 1.72-68.53, p = 0.011) were independent predictors of unfavourable outcomes. On the other hand, headache was an independent protective factor (aOR, 0.164; 95% CI, 0.03-0.91; p = 0.039). CONCLUSIONS: Impairment of consciousness, sepsis as a hospital complication, and acute kidney injury were independent predictors of unfavourable clinical outcomes, whereas headache showed a preventive effect.

Posterior Reversible Encephalopathy Syndrome (PRES) in Palliative Medicine: Case Report and Discussion.

Posterior reversible encephalopathy syndrome (PRES) is associated with seizures, visual disturbances, headache, and altered mental status. Given its presentation, the diagnosis can be mistaken for other severe conditions. Palliative medicine consultants should be aware of PRES and be prepared to counsel families on the treatment and prognosis of this syndrome.

Co-Presentation of Posterior Reversible Encephalopathy Syndrome in a Patient with Post-Dural Puncture Headache.

INTRODUCTION: Post-dural puncture headache (PDPH) is a well-known complication of neuraxial anesthesia, but the occurrence of seizures and vision loss within a few days after dural puncture could be alarming. Posterior reversible encephalopathy syndrome (PRES) is associated with reversible edema and leukoencephalopathy in the posterior parieto-occipital cortex. We report the co-presentation of PRES and PDPH after labor epidural analgesia. CASE PRESENTATION: A 25-year-old multiparous African-American woman was admitted for evaluation of new-onset seizures and headache in the postpartum period. She had a recent history of multiple needle insertion attempts and inadvertent dural puncture during epidural analgesia for delivery. Soon after delivery, she was diagnosed with PDPH and was treated with an epidural blood patch, with no relief of symptoms. Six days later, she developed sudden-onset transient blindness, seizures, and altered sensorium, and magnetic resonance imaging of the brain revealed white matter changes suggestive of PRES. CONCLUSION: PRES is an uncommon complication of cerebrospinal fluid leak and intracranial hypotension. We report the occurrence of PRES in a patient with no known risk factors except a traumatic dural tap. Is it important to expand the differentials for headache after dural puncture to encompass PRES as a possibility, especially in patients with a delayed presentation of seizures and cortical blindness.

The pathophysiologic, diagnostic, and therapeutic aspects of posterior reversible encephalopathy syndrome during pregnancy.

Posterior reversible encephalopathy syndrome (PRES) is a neurological condition with a wide range of symptoms, including visual disturbances, headache, vomiting, seizures, and altered consciousness. This review describes the pathophysiology of PRES, as well as the clinical, diagnostic, and therapeutic intervention during pregnancy. The gold standard for diagnosis of PRES is Magnetic Resonance Imaging (MRI), helping to differentiate it from other similar conditions. The aim of this paper is to review the principal aspects of PRES, general care, blood pressure control, and seizures prevention while avoiding potential injuries to the mother and fetus in the event of pregnancy. We concluded that PRES can be effectively treated and reversed if prompt diagnostic action is made, and adequate care is initiated.

A case report of posterior reversible encephalopathy syndrome caused by ANCA-associated vasculitis case report and retrospective analysis.

INTRODUCTION: Posterior reversible encephalopathy syndrome is a reversible syndrome clinically manifested by significant variations in neuroimaging. Early treatment is indispensable to achieve the reversibility, which requires the punctual alleviation of its inducing factors. PATIENT CONCERNS: Most symptoms of posterior reversible encephalopathy syndrome (PRES) patients can be dispelled followed by a good prognosis after the inducement removal. The patient died due to the untimely diagnosis and treatment of the primary disease. Therefore, sufficient attention should be paid to the PRES induced by ANCA-associated vasculitis (AAV). DIAGNOSIS AND INTERVENTIONS: The patient is a middle-aged male, with acute onset, previous history of hypertension, cholecystectomy, intestinal obstruction, spontaneous renal rupture, etc. For this complaint, the manifestations are mainly blurred vision with convulsion and transient disturbance of consciousness. In 1 day of treatment, these symptoms were relieved, and he was diagnosed as PRES combined with the cranial imaging. The AAV of the patient was confirmed by spleen pathology. OUTCOMES: Despite the relief of most symptoms in 1 day of symptomatic treatment, it is highly likely that the patient eventually died of AAV, it is highly likely that the patient eventually died of AAV which will invade the vascular system due to the failure to treat the primary disease in time. LESSONS: For patients diagnosed as PRES, the punctual identification of cause should be performed, so as to diagnose and correct the cause and primary disease as soon as possible, accompanied with the dynamical observation of the relevant indicators for suspected patients to avoid systemic organ failure.

Posterior reversible encephalopathy syndrome (PRES) in a patient with moyamoya disease: A case report.

INTRODUCTION: Moyamoya disease (MMD) and posterior reversible encephalopathy syndrome (PRES) share similar pathophysiological characteristics of endothelial dysfunction and impaired cerebral autoregulation. However, there have never been any published studies to demonstrate the relationship between these 2 rare diseases. PATIENT CONCERNS: A 26-year-old Asian man presented with a throbbing headache, blurred vision, and extremely high blood pressure. We initially suspected acute cerebral infarction based on the cerebral computed tomography, underlying MMD, and prior ischemic stroke. However, the neurological symptoms deteriorated progressively. DIAGNOSIS: Cerebral magnetic resonance imaging indicated the presence of vasogenic edema rather than cerebral infarction. INTERVENTIONS AND OUTCOMES: An appropriate blood pressure management prevents the patient from disastrous outcomes successfully. Cerebral magnetic resonance imaging at 2 months post treatment disclosed the complete resolution of cerebral edema. The patient's recovery from clinical symptoms and the neuroimaging changes supported the PRES diagnosis. CONCLUSION: This report suggests that patients with MMD may be susceptible to PRES. It highlights the importance of considering PRES as a differential diagnosis while providing care to MMD patients with concurrent acute neurological symptoms and a prompt intervention contributes to a favorable clinical prognosis.

Eclampsia and posterior reversible encephalopathy syndrome in a parturient complicated by SARS COVID-19 pneumonia.

A primigravida at 32 weeks of gestation presented to us with eclampsia and Posterior Reversible Encephalopathy Syndrome (PRES) along with SARS COVID-19 pneumonia. Immediate termination of pregnancy was done under general anesthesia and patient was electively ventilated in view of increased oxygen requirements. Further therapy using magnesium sulphate, antihypertensives, steroids, and convalescent plasma was carried out. The condition of the patient steadily improved leading to her extubation on the 4th postoperative day and subsequent discharge on the 8th day of admission.

A rare case of posterior reversible encephalopathy syndrome in a patient with severe leptospirosis complicated with rhabdomyolysis and acute kidney injury; a case report.

BACKGROUND: Leptospirosis is a zoonotic spirochetal disease caused by Leptospira interrogans. The clinical presentation ranges from an asymptomatic state to a fatal multiorgan dysfunction. Neurological manifestations including aseptic meningitis, spinal cord and peripheral nerve involvement, cranial neuropathies and cerebellar syndrome are well recognized with varying frequencies among patients with this disease. Posterior reversible encephalopathy syndrome is a very rare occurrence in leptospirosis and only two cases are reported in the medical literature up to now. We report a case of posterior reversible encephalopathy syndrome in a patient with leptospirosis with rhabdomyolysis and acute kidney injury. CASE PRESENTATION: A 21 year-old male presented with fever and oliguric acute kidney injury with rhabdomyolysis. A diagnosis of leptospirosis was made and he was being managed according to the standard practice together with regular hemodialysis. The clinical condition was improving gradually. On day 8 of the illness, he developed headache and sudden painless complete bilateral vision loss followed by several brief generalized tonic clonic seizure attacks. Examination was significant for a Glasgow Coma Scale of 14/15, blood pressure of 150/90 mmHg and complete bilateral blindness. The findings of magnetic resonance imaging of the brain were compatible with posterior reversible encephalopathy syndrome. He was managed with blood pressure control and antiepileptics with supportive measures and standard treatment for leptospirosis and made a complete recovery. CONCLUSION: Posterior reversible encephalopathy syndrome, though very rare with leptospirosis, should be considered as a differential diagnosis in a patient with new onset visual symptoms and seizures, especially during the immune phase. Optimal supportive care together with careful blood pressure control and seizure management would yield a favourable outcome in this reversible entity.

A case report of reversible posterior encephalopathy syndrome with intracranial hemorrhage in a child.

INTRODUCTION: The objective is to analyze the clinical diagnosis and treatment of children with rescindable posterior encephalopathy syndrome (PRES) and intracranial hemorrhage (ICH) to improve the pediatrician's understanding of PRES combined with ICH in children. PATIENT CONCERNS AND DIAGNOSIS: After liver transplantation, the patient developed symptoms of epilepsy and coma. Meanwhile, massive necrosis of acute cerebral infarction and small hemorrhage was observed in the left cerebellar hemisphere and left occipital lobe, respectively. The above symptoms were initially diagnosed as PRES. INTERVENTIONS AND OUTCOMES: After adjusting the anti-rejection drug regimen, it was found that the child's neurological symptoms were relieved, and the limb motor function gradually recovered during follow-up. Imaging examination showed significant improvement on abnormal signals in brain. CONCLUSION: In general, children with PRES may further develop ICH and contribute to a poor prognosis. Early diagnosis, detection of risk factors and timely adjustment of medication regimen are the keys to prevent irreversible brain damage.

Posterior Reversible Encephalopathy Syndrome.

PURPOSE OF REVIEW: This review provides an updated discussion on the clinical presentation, diagnosis and radiographic features, mechanisms, associations and epidemiology, treatment, and prognosis of posterior reversible encephalopathy syndrome (PRES). Headache is common in PRES, though headache associated with PRES was not identified as a separate entity in the 2018 International Classification of Headache Disorders. Here, we review the relevant literature and suggest criteria for consideration of its inclusion. RECENT FINDINGS: COVID-19 has been identified as a potential risk factor for PRES, with a prevalence of 1-4% in patients with SARS-CoV-2 infection undergoing neuroimaging, thus making a discussion of its identification and treatment particularly timely given the ongoing global pandemic at the time of this writing. PRES is a neuro-clinical syndrome with specific imaging findings. The clinical manifestations of PRES include headache, seizures, encephalopathy, visual disturbances, and focal neurologic deficits. Associations with PRES include renal failure, preeclampsia and eclampsia, autoimmune conditions, and immunosuppression. PRES is theorized to be a syndrome of disordered autoregulation and endothelial dysfunction resulting in preferential hyperperfusion of the posterior circulation. Treatment typically focuses on treating the underlying cause and removal of the offending agents.

Optic pathways and brainstem involvement in posterior reversible encephalopathy syndrome.

Posterior reversible encephalopathy syndrome (PRES) is a neurological syndrome manifesting with acute focal signs, and concomitant neuroimaging findings of vasogenic oedema. It affects the parieto-occipital regions in a vast majority of cases, although atypical variants have been described comprising the brainstem, basal ganglia or spinal cord. We report the case of a 41-year-old woman, admitted for persistent headache and inferior altitudinal field defect in the right eye. She presented with severe, non-medicated, hypertension. Brain MRI showed findings compatible with atypical PRES, involving the brainstem and optic pathways. With antihypertensive therapy the headache remitted, although visual field remained and was interpreted in the context of a vascular aetiology-non-arteritic anterior ischaemic optic neuropathy. MRI was repeated 3 weeks later and showed almost complete reversal of the previous changes.

Reversible Cerebral Vasoconstriction Syndrome and Sickle Cell Disease: A Case Report.

Reversible cerebral vasoconstriction syndrome (RCVS), is rare in the pediatric population and is characterized by severe headaches and other neurologic symptoms. We present a case of RCVS occurring concomitantly with posterior reversible encephalopathy syndrome in an 8-year-old African American child with sickle cell disease (HbSS). Imaging studies including computed tomography, magnetic resonance imaging and cerebral angiography of the brain showed acute hemorrhagic stroke and a beaded appearance of peripheral cerebral vessels. In this report, we focus on the typical features of RCVS and discuss the underlying risk factors that may increase the risk in patients with HbSS disease.

Posterior Reversible Encephalopathy Syndrome in Childhood Hematological/Oncological Diseases: Multicenter Results.

The aim of the study was to analyze the characteristics of posterior reversible encephalopathy syndrome (PRES) cases treated at 10 different institutions in our country. Fifty-eight patients diagnosed with PRES were included in this study. The data of PRES cases from 10 departments of pediatric hematology/oncology were analyzed. The mean age of the patients at the time of diagnosis of PRES was 8.95±3.66 years. Most patients (80.4%) had a primary diagnosis of acute leukemia. Patients received chemotherapy (71.4%) and/or used steroids within 14 days before the diagnosis of PRES (85.7%). Hypertension was found in 83.9% of the patients. Twenty-six patients had infections and 22 of them had febrile neutropenia. The most common electrolyte disorders were hypocalcemia, hypomagnesemia, and hypopotassemia. Six patients had tumor lysis syndrome and 4 had inappropriate antidiuretic hormone syndrome. Magnetic resonance imaging was used for diagnosis in all patients. The most commonly involved regions by magnetic resonance imaging were occipital (58%), parietal (51%), and frontal lobes (45%), respectively. Twenty-five patients required intensive care and 7 patients were intubated. In conclusion, PRES may develop during the follow-up and treatment of hematological diseases. In addition to steroid and intense combined chemotherapies, immunosuppressive agents and hypertension are also factors that may be responsible for PRES.