Mesial Temporal Sclerosis as Late Consequence of Posterior Reversible Encephalopathy Syndrome in Pediatric Hemato-oncologic Patients.

OBJECTIVES: Drug resistant epilepsy has rarely been reported following posterior reversible encephalopathy syndrome (PRES), with few cases of mesial temporal sclerosis (MTS). The aim of this study was to report clinical and neuroimaging features of MTS subsequent to PRES in hemato-oncologic/stem cell transplanted children. MATERIALS AND METHODS: Among 70 children treated in 2 pediatric hemato-oncologic Italian centers between 1994 and 2018 and presenting an episode of PRES, we retrospectively identified and analyzed a subgroup of patients who developed epilepsy and MTS. RESULTS: Nine of 70 patients (12.8%) developed post-PRES persistent seizures with magnetic resonance imaging evidence of MTS. One patient died few months after MTS diagnosis, because of hematologic complications; the remaining 8 patients showed unprovoked seizures over time leading to the diagnosis of epilepsy, focal in all and drug resistant in 4. At PRES diagnosis, all patients with further evidence of epilepsy and MTS suffered of convulsive seizures, evolving into status epilepticus in 3. In 3 patients a borderline cognitive level or intellectual disability were diagnosed after the onset of epilepsy, and 2 had behavioral problems impacting their quality of life. CONCLUSIONS: MTS and long-term focal epilepsy, along with potential cognitive and behavioral disorders, are not uncommon in older pediatric patients following PRES.

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.

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.

Posterior reversible encephalopathy syndrome complicating hyperosmolar hyperglycemic syndrome.

Hyperosmolar hyperglycemic syndrome is a life-threatening diabetic emergency that manifests as altered mental status. An otherwise healthy 40-year-old man presented to our emergency department with headache and severe hypertension. Brain magnetic resonance imaging showed evidence of posterior reversible encephalopathy syndrome, a usually reversible neurological syndrome characterized by subcortical vasogenic oedema. This first reported case details posterior reversible encephalopathy syndrome concurrent with hyperosmolar hyperglycemic syndrome. Prompt diagnosis and management were essential to avoid non-reversible consequences.

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.

Posterior reversible encephalopathy syndrome (PRES) due to acute hypertension in children: 12 years single-center experience.

The objective of the study is to evaluate the clinical and neuroradiological findings, the risk factors for recurrence and the prognosis in patients with posterior reversible encephalopathy syndrome developed secondary to acute hypertension in children. The study was conducted between 2008 and 2019 at Mersin University Faculty of Medicine. A total of 49 episodes were evaluated retrospectively in 38 patients with PRES secondary to acute hypertension. The demographic data, etiology, and clinical and neuroradiological findings were recorded. Twenty-one (55.3%) patients were female; the mean age was 11.8 years. The etiology of acute hypertension in 29 (76.3%) patients was end-stage renal disease (ESRD). The most common clinical findings were seizure (81.6%) and altered consciousness (79.6%). Status epilepticus developed in eight (16.3%) episodes. MRI lesions were atypical in 33 episodes (67.3%). In eight (21%) patients, PRES recurred. Irreversible brain damage was detected after PRES in three (7.8%) patients. C-reactive protein and erythrocyte sedimentation rate were elevated in 82.2% and 71.4% of the episodes, respectively. A statistically significant relationship was found between the recurrence, the duration of hospitalization at the PICU, SE and the occurrence of irreversible lesion (p = 0.013, p = 0.015, p = 0.001 respectively). Also, there were statistically significant relationships between recurrence and ESRD; epilepsy and recurrences; SE and irreversible brain damage (p = 0.02, p = 0.012, p = 0.025 respectively). Although PRES is usually known to have a good prognosis, the mortality and morbidity rates may increase in the long-term follow-up as in our study. In this study, the etiology, the presence of status epilepticus, PICU history, atypical MRI lesions and increased inflammatory markers were found to be important for the prognosis in PRES.

Clinical Presentation and Risk Factors for Poor Outcomes Among Adult Patients With Posterior Reversible Encephalopathy Syndrome: A Retrospective Cohort Study.

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is an acute neurological condition with unknown global incidence, variable clinical presentation, and prognosis. OBJECTIVES: To describe a cohort of patients with PRES with a focus on brain magnetic resonance imaging (MRI) patterns and their relationship with short-term clinical outcomes. METHODS: Retrospective cohort study. The authors included patients if they were older than 15 years and had a PRES diagnosis on the basis of a positive brain MRI at any time during the in-hospital stay. RESULTS: Forty-four patients were included in the present analysis. The median age was 57 years (interquartile range, 32.0-68.5) and 70.5% were women. Hypertension (59.1%), history of transplantation (27.3%), previous chemotherapy (27.3%), chronic renal failure (38.6%), and autoimmune disease (15%) were the main comorbid conditions present. The classic triad of seizures, headache, and visual impairment was present in 18.0% of the cases. Eighty-six percent of patients were admitted to the intensive care unit, with 36.0% needing invasive life support. Brain MRI showed a dominant parieto-occipital pattern in 26 patients, whereas cytotoxic edema and bleeding were present in 27.3% and 29.6%, respectively. In-hospital mortality was 11.4%. The median modified Rankin Scale at hospital discharge was 1 (0-2.5). Risk factors associated with low modified Rankin Scale scores were: headache, visual impairment, and parieto-occipital pattern. Decreased level of consciousness and mechanical ventilation requirement were associated with greater discharge disability. CONCLUSIONS: Characteristic symptoms and signs of PRES and classic MRI patterns are associated with better clinical outcomes.

Cerebral Hyperperfusion Syndrome and Related Conditions.

Cerebral vasoconstriction is a normal physiological response under determined conditions to preserve a normal cerebral blood flow. However, there are several syndromes, with impaired cerebral autoregulation and cerebral vasoconstriction, not related with infection or inflammation, which share the same radiological and clinical presentation. We review here the cerebral hyperperfusion syndrome and related conditions such as hypertensive encephalopathy, posterior reversible encephalopathy syndrome, and reversible cerebral vasoconstriction syndrome. These syndromes might share the same pathophysiological mechanism with endothelial damage, cerebral vasoconstriction, blood-brain barrier disturbance, cerebral edema, and, occasionally, intracerebral hemorrhage, with fatal cases described in all. Despite knowledge of these syndromes, they still remain unknown to us. Why these entities present in some patients and not in others goes further than the actual understanding of these diseases. We have to consider that a genetic susceptibility and molecular disturbances may be involved. Thus, more studies are needed in order to better characterize such syndromes.

Posterior Reversible Encephalopathy Syndrome in Patients with Coronavirus Disease 2019: Two Cases and A Review of The Literature.

INTRODUCTION: Encephalopathy is a common complication of coronavirus disease 2019. Although the encephalopathy is idiopathic in many cases, there are several published reports of patients with posterior reversible encephalopathy syndrome in the setting of coronavirus disease 2019. OBJECTIVE: To describe the diverse presentations, risk factors, and outcomes of posterior reversible encephalopathy syndrome in patients with coronavirus disease 2019. METHODS: We assessed patients with coronavirus disease 2019 and a diagnosis of posterior reversible encephalopathy syndrome at our institution from April 1 to June 24, 2020. We performed a literature search to capture all known published cases of posterior reversible encephalopathy syndrome in patients with coronavirus disease 2019. RESULTS: There were 2 cases of posterior reversible encephalopathy syndrome in the setting of coronavirus 2019 at our institution during a 3-month period. One patient was treated with anakinra, an interleukin-1 inhibitor that may disrupt endothelial function. The second patient had an underlying human immunodeficiency virus infection. We found 13 total cases in our literature search, which reported modest blood pressure fluctuations and a range of risk factors for posterior reversible encephalopathy syndrome. One patient was treated with tocilizumab, an interleukin-6 inhibitor that may have effects on endothelial function. All patients had an improvement in their neurological symptoms. Interval imaging, when available, showed radiographic improvement of brain lesions. CONCLUSIONS: Risk factors for posterior reversible encephalopathy syndrome in patients with coronavirus disease 2019 may include underlying infection or immunomodulatory agents with endothelial effects in conjunction with modest blood pressure fluctuations. We found that the neurological prognosis for posterior reversible encephalopathy syndrome in the setting of coronavirus disease 2019 infection is favorable. Recognition of posterior reversible encephalopathy syndrome in this patient population is critical for prognostication and initiation of treatment, which may include cessation of potential offending agents and tight blood pressure control.

Prevalence of leukoencephalopathy and its potential cognitive sequelae in cancer patients.

Due to the rising use of chemotherapy treatment in cancer patients and growing survival rates, therapy-induced neurotoxic side effects are increasingly reported. Given the ambiguity about the prevalence and severity of leukoencephalopathy, one of such toxic side effects, in non-central nervous system (CNS) cancer patients, we performed a systematic literature search using the PubMed/Medline database to summarize existing literature regarding leukoencephalopathy epidemiology in non-CNS cancer patients and its potential cognitive sequelae. The search was based on the following terms: ('MRI' OR 'T2-weighted MRI' OR 'FLAIR') AND ('cancer' OR 'tumour' OR 'leukaemia' OR 'neoplasms') AND ('chemotherapy' OR 'radiotherapy') AND ('posterior reversible encephalopathy' OR 'leukoencephalopathy' OR 'cerebral ischaemia' OR 'stroke'). Thirty-two studies discussing the occurrence of leukoencephalopathy in cancer patients were included, of which the majority investigated Acute Lymphoblastic Leukaemia (ALL) patients (n = 22).Regularly scanned ALL patients showed a prevalence of leukoencephalopathy between 17 - 87%, and 15 - 83% of patients presented with leukoencephalopathy when only scanned after a CNS event. When diagnosed with posterior reversible encephalopathy syndrome, 100% of patients showed leukoencephalopathy because its diagnosis is based in part on observable lesions. An increased prevalence was observed in ALL patients treated with higher doses of methotrexate (5 g/m2 MTX, 42 - 87%) when compared to lower doses (< 5 g/m2, 32 - 67%). By contrast, in breast cancer patients, white matter lesions were mainly detected in case of neurological symptoms, but not (yet) clearly associated with chemotherapy administration. However, chemotherapy treatment was associated with more infratentorial microbleeds in breast cancer patients . Up to 50% of other (neurologically asymptomatic) solid tumour patients presented white matter lesions, even years after treatment. When cognitive data were investigated, lesioned patients showed lower scores on neurocognitive tests in 50% of studies, years after ending therapy.In conclusion, leukoencephalopathy is well-documented for ALL patients (with a focus on methotrexate), but there is a lack of knowledge for other intravenous chemotherapeutics, other oncological populations, wider age ranges and possible risk factors (e.g. history of CNS event). Furthermore, the long-term neuropsychological impact and potential risk for neurodegenerative processes due to leukoencephalopathy remains inconclusive. Hence, large international databanks, epidemiological and prospective case-control studies are necessary to stratify risk groups for CNS-related side effects.

Case 279: Central-Variant Posterior Reversible Encephalopathy Syndrome.

HistoryA 25-year-old woman with recently diagnosed systemic lupus erythematosus and class IV lupus nephritis confirmed with biopsy and treated with mycophenolate mofetil presented with a 2-day history of progressively worsening edema of her face and lower extremities. She had no antecedent infection or vaccination. She was admitted to the hospital and treated with methylprednisolone, furosemide, and C1 esterase inhibitor. On hospital day 2, she experienced a witnessed generalized tonic-clonic seizure. At that time, she became hypoxic and was intubated for airway protection. Her laboratory study results preceding the seizure were remarkable for hyponatremia, with a blood sodium level of 122 mEq/L (122 mmol/L) (normal range, 135-145 mEq/L [134-145 mmol/L]), which was corrected to 137 mEq/L (137 mmol/L) over 48 hours. Same-day cerebrospinal fluid analysis was unremarkable, and unenhanced head CT findings (not shown) were normal, with no evidence of intracranial hemorrhage or edema.Her subsequent hospital course was complicated by renal failure requiring continuous renal replacement therapy, hypertension (systolic blood pressure ranging from 140 to 190 mm Hg), anemia requiring blood transfusions, thrombocytopenia, and pneumonia. She remained intubated with a limited neurologic examination due to sedative medications until hospital day 10. After extubation, she was noted to have a right gaze preference. She was able to speak in short phrases and follow simple commands. Neurologic examination was notable for drowsiness, right gaze deviation, direction-changing torsional nystagmus, horizontal ophthalmoplegia, and generalized symmetric weakness without upper motor neuron signs. The following day (hospital day 11), unenhanced MRI of the brain was performed along with MR angiography of the brain. Biopsy of the temporal artery was normal, without evidence of inflammation.

Posterior reversible encephalopathy syndrome (PRES): presentation, diagnosis and treatment.

Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder which is characterised by variable symptoms, which include visual disturbances, headache, vomiting, seizures and altered consciousness. The exact pathophysiology of PRES has not been completely explained, but hypertension and endothelial injury seem to be almost always present. Vasoconstriction resulting in vasogenic and cytotoxic edema is suspected to be responsible for the clinical symptoms as well as the neuro-radiological presentation. On imaging studies, Symmetrical white matter abnormalities suggestive of edema are seen in the computer tomography (CT) and magnetic resonance imaging (MRI) scans, commonly but not exclusively in the posterior parieto-occipital regions of the cerebral hemispheres. The management is chiefly concerned with stabilization of the patient, adequate and prompt control of blood pressure, prevention of seizures and timely caesarean section in obstetric cases with pre-eclampsia/eclampsia. In conclusion, persistently elevated blood pressures remain the chief culprit for the clinical symptoms as well as the neurological deficits. Early diagnosis by diffusion weighted MRI scans, and differentiation from other causes of altered sensorium i.e. seizures, meningitis and psychosis, is extremely important to initiate treatment and prevent further complications. Although most cases resolve successfully and carry a favorable prognosis, patients with inadequate therapeutic support or delay in treatment may not project a positive outcome.

Neuroradiological perspectives of severe preeclampsia and eclampsia spectrum - Correlation from posterior reversible encephalopathy syndrome.

OBJECTIVES: Posterior reversible encephalopathy syndrome (PRES) is the neuroradiological appearance of eclampsia. This study was conducted to understand the neuroradiological aspects of severe preeclampsia by recognition of PRES. We hypothesized that posterior reversible encephalopathy syndrome is the sole background manifestation of neurological involvement in preeclampsia patients. Detecting and demonstrating the extent of PRES and hence, cerebral involvement of preeclampsia was the main objective. STUDY DESIGN: It was a prospective and observational study conducted in a tertiary referral center of northern India. A total of 75 women were recruited on admission to the emergency room, 30 of them with cerebral symptoms i.e., headache, visual disturbances and depressed consciousness, 30 patients of severe preeclampsia who were neurologically asymptomatic and 15 of eclampsia patients. Magnetic resonance imaging of the brain was performed in postpartum period within 48 h of delivery. OUTCOME MEASURES: Correlation of the MRI brain picture with the neurological disease status of preeclampsia and the symptoms of cerebral involvement and identification of predictors in development of PRES. RESULTS: PRES was identified in 86.7% of patients in eclampsia group (P value - 0.001), also in 20% and 26.6% of symptomatic and asymptomatic severe preeclampsia patients too (P value - 0.06 and 0.195). There was a shorter mean duration of high blood pressure records in those who developed PRES. CONCLUSION: True cerebral involvement of preeclampsia believed to be translated as PRES was witnessed even in asymptomatic severe preeclampsia patients and hence, extending the indications of neuroimaging in such patients. Treatment focused on PRES would induce early reversibility and avoid long term neurological sequel.

Psychiatric Morbidity and Its Prognosis in Posterior Reversible Encephalopathy Syndrome.

Posterior reversible encephalopathy syndrome (PRES) is a clinically and radiologically diagnosed disorder distinguished by subcortical vasogenic cerebral edema. To date, its presentation has been described through summarized neurological categories, such as seizures, headaches, "confusion," and "altered mental function." This retrospective case series identified all cases of clinically confirmed, radiologically diagnosed PRES resulting in treatment in a large teaching hospital from 2010 to 2019. The authors conducted a search for the term "reversible encephalopathy" in the hospital clinical radiology information system, followed by an audit of scan reports and clinical records. The most common reasons for psychiatric referral were addictions, acute psychosis, depression, suicidality, and treatment refusal. Multidisciplinary staff should consider PRES as a rare, organic differential diagnosis for acute mental state changes. Physicians should be aware of elevated rates of post-PRES psychiatric symptoms and consider whether psychiatric consultation may enhance recovery.

Posterior Reversible Encephalopathy Syndrome in Children:: A Prospective Follow-up Study.

AIM: To evaluate clinical and radiologic presentation, and neurologic outcome of pediatric posterior reversible encephalopathy syndrome (PRES). PATIENTS AND METHODS: The study included 24 children (14 males and 10 females) diagnosed with PRES who were prospectively followed for 2 years. They were evaluated using Wechsler Intelligence Scale, electroencephalograph (EEG), and brain magnetic resonance imaging (MRI). RESULTS: The mean age of the studied patients at the time of diagnosis of PRES was 6 years (±2.2). Chemotherapy for cancer represented 66.7% of the causes of PRES in the studied children, followed by renal disorders and immunosuppressive agents for hematopoietic stem cell transplantation. Twenty-seven attacks of PRES were reported as 3 children developed a second attack of PRES. Normal intelligence quotient was found in 95.8% of studied children after PRES. Residual abnormalities in follow-up MRI were demonstrated in 3 children. Epilepsy and residual MRI lesions were reported in 2 of the 3 children with recurrent PRES. Residual lesions in follow-up MRI and epilepsy were more significantly reported after recurrent PRES (P < .05). CONCLUSIONS: Neoplastic, renal disorders and hematopoietic stem cell transplantation represent the main disorders associated with PRES in children. Chemotherapeutic drugs, immunosuppressants, and hypertension are the main risk factors for pediatric PRES. The outcome of pediatric PRES is good, but long-term neurologic sequelae can occur, mainly epilepsy and residual MRI abnormalities. Recurrence of PRES is infrequently reported in children receiving chemotherapeutic or immunosuppressive drugs. Recurrent PRES is a risk factor for long-term neurologic sequelae.