ABSTRACT
Background and purpose:
Posterior reversible encephalopathy syndrome (PRES) is characterized by vasogenic edema, usually reversible, with the prominent involvement of the parietal and occipital lobes. The exact etiopathogenesis leading to PRES is unknown. Because signs of eclampsia and preeclampsia in neuroimaging often overlap and manifest as PRES, we aimed to evaluate whether demographic, clinical, and laboratory parameters predict PRES in patients with preeclampsia or eclampsia.
213 pre-eclampsia or eclampsia patients with cranial imaging were retrospectively examined. We recorded the patients’ demographic information, systolic blood pressure (SBP), diastolic blood pressure (DBP), mean arterial pressure (MAP), hemogram, biochemical indicators, clinical symptoms, and imaging features.
. Results:Of all patients, 69% (n = 147) had preeclampsia while 31% (n = 66) had eclampsia, and 24.4% (n = 53) were diagnosed with PRES. The mean age of patients who developed PRES was 25.81 ± 6.07 years and thus significantly less than that of patients who did not develop PRES (p = .000). Patients with PRES had significantly higher mean SBP (p = .015), DBP (p = .009), and MAP (p = .003) than patients without PRES, along with significantly higher aspartate aminotransferase (ASAT; p = .001), alanine aminotransferase (ALAT; p = .001) blood urea nitrogen (BUN; p = .001), white blood cell (WBC; p = .003), neutrophil (p = .001), and hemoglobin (Hb; p = .027) levels, but significantly lower albumin (p = .000) levels.
. Conclusion:Age, high blood pressure, and BUN, neutrophil, and WBC levels were predictors of the development of PRES in patients with preeclampsia and eclampsia. Early neuroimaging considering those predictors should be performed to diagnose PRES in patients with preeclampsia and eclampsia.
.Subject(s)
Eclampsia , Posterior Leukoencephalopathy Syndrome , Pre-Eclampsia , Humans , Female , Pregnancy , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Posterior Leukoencephalopathy Syndrome/etiology , Posterior Leukoencephalopathy Syndrome/physiopathology , Pre-Eclampsia/blood , Pre-Eclampsia/physiopathology , Eclampsia/diagnostic imaging , Retrospective Studies , Adult , Risk Factors , Blood PressureABSTRACT
PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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.
Subject(s)
Posterior Leukoencephalopathy Syndrome , Humans , Posterior Leukoencephalopathy Syndrome/physiopathology , Child , Adolescent , Hypertension/physiopathology , Prognosis , Magnetic Resonance ImagingABSTRACT
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.
Subject(s)
Guillain-Barre Syndrome , Posterior Leukoencephalopathy Syndrome , Humans , Guillain-Barre Syndrome/therapy , Guillain-Barre Syndrome/complications , Guillain-Barre Syndrome/physiopathology , Male , Posterior Leukoencephalopathy Syndrome/etiology , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Posterior Leukoencephalopathy Syndrome/therapy , Posterior Leukoencephalopathy Syndrome/physiopathology , Child , Adolescent , Child, PreschoolABSTRACT
OBJECTIVES: 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. DESIGN: Twenty-four patients with PRES caused by eclampsia who were admitted to our hospital in the last 5 years were included in this study. PARTICIPANTS/MATERIALS, SETTING, METHODS: Blood pressure at admission, the number of regions with vasogenic edema in the brain, and recurrent seizures were noted. Patients were divided into three groups: mild, moderate, and severe. RESULTS: Using Kruskal-Wallis and Pearson χ2 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). CONCLUSION: PRES may occur in eclamptic patients with mild, moderate, or severe blood pressure values. Evaluation by magnetic resonance imaging is needed to confirm the diagnosis. Early and rapid treatment is essential for reducing morbidity and mortality among pregnant women.
Subject(s)
Eclampsia , Magnetic Resonance Imaging , Posterior Leukoencephalopathy Syndrome , Recurrence , Seizures , Humans , Female , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Posterior Leukoencephalopathy Syndrome/physiopathology , Posterior Leukoencephalopathy Syndrome/etiology , Posterior Leukoencephalopathy Syndrome/complications , Pregnancy , Eclampsia/physiopathology , Adult , Seizures/etiology , Seizures/physiopathology , Blood Pressure/physiology , Young Adult , Risk FactorsABSTRACT
BACKGROUND: 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. METHODS: 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 60 mL/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. RESULTS: The study included 200 patients, of whom 94 (47%) had renal insufficiency. Patients with renal insufficiency had a larger lesion volume (144.7 ± 125.2 cc) compared to those without renal insufficiency (110.5 ± 93.2 cc; p = 0.032); particularly in the atypical lesions volume (49.2 ± 65.0 vs. 29.2 ± 44.3 cc; p = 0.013). However, there was no difference in the reversibility of the lesions (35.2 ± 67.5 vs. 18.8 ± 33.4 cc; p = 0.129). Multiple regression analysis revealed that decreases in eGFR (ß = -0.34, 95% CI -0.62-0.05, p = 0.020) were positively associated with total lesion volume. CONCLUSION: 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.
Subject(s)
Magnetic Resonance Imaging , Posterior Leukoencephalopathy Syndrome , Renal Insufficiency , Humans , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Posterior Leukoencephalopathy Syndrome/pathology , Posterior Leukoencephalopathy Syndrome/physiopathology , Posterior Leukoencephalopathy Syndrome/complications , Female , Male , Renal Insufficiency/pathology , Renal Insufficiency/physiopathology , Retrospective Studies , Middle Aged , Adult , Brain/pathology , Brain/diagnostic imaging , Aged , Glomerular Filtration Rate/physiologyABSTRACT
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.
Subject(s)
Electroencephalography , Epilepsy , Hematologic Neoplasms , Magnetic Resonance Imaging , Posterior Leukoencephalopathy Syndrome , Seizures , Adolescent , Child , Child, Preschool , Epilepsy/diagnostic imaging , Epilepsy/epidemiology , Epilepsy/physiopathology , Female , Hematologic Neoplasms/diagnostic imaging , Hematologic Neoplasms/epidemiology , Hematologic Neoplasms/therapy , Humans , Male , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Posterior Leukoencephalopathy Syndrome/epidemiology , Posterior Leukoencephalopathy Syndrome/physiopathology , Retrospective Studies , Sclerosis , Seizures/diagnostic imaging , Seizures/epidemiology , Seizures/physiopathologyABSTRACT
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.
Subject(s)
Brain Edema , Hypertension , Moyamoya Disease , Nicardipine/administration & dosage , Posterior Leukoencephalopathy Syndrome , Adult , Antihypertensive Agents/administration & dosage , Brain/diagnostic imaging , Brain Edema/diagnostic imaging , Brain Edema/etiology , Brain Edema/therapy , Diagnosis, Differential , Humans , Hypertension/diagnosis , Hypertension/etiology , Hypertension/therapy , Magnetic Resonance Imaging/methods , Male , Moyamoya Disease/complications , Moyamoya Disease/diagnosis , Moyamoya Disease/physiopathology , Moyamoya Disease/therapy , Neurologic Examination/methods , Posterior Leukoencephalopathy Syndrome/complications , Posterior Leukoencephalopathy Syndrome/diagnosis , Posterior Leukoencephalopathy Syndrome/physiopathology , Posterior Leukoencephalopathy Syndrome/therapy , Prognosis , Tomography, X-Ray Computed/methods , Treatment OutcomeABSTRACT
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.
Subject(s)
Acute Kidney Injury/complications , Leptospirosis/complications , Posterior Leukoencephalopathy Syndrome/etiology , Rhabdomyolysis/complications , Acute Kidney Injury/diagnosis , Acute Kidney Injury/physiopathology , Acute Kidney Injury/therapy , Brain/diagnostic imaging , Diagnosis, Differential , Humans , Leptospirosis/diagnosis , Leptospirosis/physiopathology , Leptospirosis/therapy , Magnetic Resonance Imaging , Male , Posterior Leukoencephalopathy Syndrome/diagnosis , Posterior Leukoencephalopathy Syndrome/physiopathology , Posterior Leukoencephalopathy Syndrome/therapy , Rhabdomyolysis/diagnosis , Rhabdomyolysis/physiopathology , Rhabdomyolysis/therapy , Treatment Outcome , Young AdultABSTRACT
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.
Subject(s)
Comorbidity , Hyperglycemic Hyperosmolar Nonketotic Coma/complications , Posterior Leukoencephalopathy Syndrome/complications , Adult , Humans , Hyperglycemic Hyperosmolar Nonketotic Coma/physiopathology , Male , Posterior Leukoencephalopathy Syndrome/physiopathologySubject(s)
Acute Kidney Injury/physiopathology , COVID-19/physiopathology , Hypertension/physiopathology , Posterior Leukoencephalopathy Syndrome/physiopathology , Acute Kidney Injury/complications , Acute Kidney Injury/therapy , COVID-19/complications , COVID-19/therapy , Humans , Hypertension/complications , Hypertension/drug therapy , Male , Middle Aged , Posterior Leukoencephalopathy Syndrome/complications , Posterior Leukoencephalopathy Syndrome/diagnosis , Posterior Leukoencephalopathy Syndrome/therapy , SARS-CoV-2 , Tomography, X-Ray ComputedABSTRACT
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.
Subject(s)
Bile Ducts/abnormalities , Biliary Atresia/surgery , Cerebral Infarction , Immunosuppression Therapy , Intracranial Hemorrhages , Posterior Leukoencephalopathy Syndrome , Risk Adjustment/methods , Biliary Atresia/diagnosis , Biliary Atresia/etiology , Brain/diagnostic imaging , Brain Damage, Chronic/etiology , Brain Damage, Chronic/prevention & control , Cerebral Infarction/diagnosis , Cerebral Infarction/etiology , Child, Preschool , Early Diagnosis , Humans , Immunosuppression Therapy/adverse effects , Immunosuppression Therapy/methods , Intracranial Hemorrhages/diagnosis , Intracranial Hemorrhages/etiology , Liver Transplantation/methods , Magnetic Resonance Imaging/methods , Male , Posterior Leukoencephalopathy Syndrome/diagnosis , Posterior Leukoencephalopathy Syndrome/etiology , Posterior Leukoencephalopathy Syndrome/physiopathology , Posterior Leukoencephalopathy Syndrome/therapy , Tomography, X-Ray Computed/methodsABSTRACT
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.
Subject(s)
Endothelium/physiopathology , Headache/physiopathology , Posterior Leukoencephalopathy Syndrome/physiopathology , Seizures/physiopathology , Vision Disorders/physiopathology , Acute Chest Syndrome/epidemiology , Aminolevulinic Acid/analogs & derivatives , Anemia, Sickle Cell/epidemiology , Autoimmune Diseases/epidemiology , Blood-Brain Barrier/metabolism , Brain Edema/diagnostic imaging , Brain Edema/physiopathology , COVID-19/epidemiology , Cerebrovascular Circulation/physiology , Cytokines/metabolism , Eclampsia/epidemiology , Female , Homeostasis/physiology , Humans , Hypertension/physiopathology , Magnetic Resonance Imaging , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Posterior Leukoencephalopathy Syndrome/epidemiology , Posterior Leukoencephalopathy Syndrome/therapy , Pre-Eclampsia/epidemiology , Pregnancy , Prognosis , Renal Insufficiency/epidemiology , SARS-CoV-2 , Vasospasm, Intracranial/physiopathologyABSTRACT
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.
Subject(s)
Hypertension/diagnostic imaging , Hypertension/physiopathology , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Posterior Leukoencephalopathy Syndrome/physiopathology , Acute Disease , Adolescent , Child , Child, Preschool , Electroencephalography/methods , Electroencephalography/trends , Female , Follow-Up Studies , Humans , Magnetic Resonance Imaging/methods , Magnetic Resonance Imaging/trends , MaleSubject(s)
Immunosuppressive Agents/adverse effects , Posterior Leukoencephalopathy Syndrome/chemically induced , Posterior Leukoencephalopathy Syndrome/diagnosis , Tacrolimus/adverse effects , Adolescent , Delayed Diagnosis , Female , Humans , Lung Transplantation , Magnetic Resonance Imaging , Posterior Leukoencephalopathy Syndrome/physiopathology , Vision Disorders/chemically induced , Vision Disorders/diagnosis , Vision Disorders/physiopathology , Visual Acuity/physiologyABSTRACT
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.
Subject(s)
Outcome Assessment, Health Care , Posterior Leukoencephalopathy Syndrome/pathology , Posterior Leukoencephalopathy Syndrome/physiopathology , Adult , Aged , Comorbidity , Female , Headache/etiology , Hospital Mortality , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Posterior Leukoencephalopathy Syndrome/complications , Posterior Leukoencephalopathy Syndrome/mortality , Retrospective Studies , Risk Factors , Seizures/etiology , Vision Disorders/etiologyABSTRACT
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.
Subject(s)
Cerebrovascular Circulation/physiology , Hypertensive Encephalopathy , Posterior Leukoencephalopathy Syndrome , Vasospasm, Intracranial , Female , Humans , Male , Posterior Leukoencephalopathy Syndrome/physiopathologyABSTRACT
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.
Subject(s)
Betacoronavirus/pathogenicity , Coronavirus Infections/virology , Endothelium, Vascular/virology , Pneumonia, Viral/virology , Posterior Leukoencephalopathy Syndrome/virology , Blood Pressure , COVID-19 , Coinfection , Coronavirus Infections/diagnosis , Coronavirus Infections/immunology , Coronavirus Infections/physiopathology , Endothelium, Vascular/physiopathology , Female , HIV Infections/immunology , HIV Infections/physiopathology , HIV Infections/virology , Host-Pathogen Interactions , Humans , Immunosuppressive Agents/adverse effects , Middle Aged , Pandemics , Pneumonia, Viral/diagnosis , Pneumonia, Viral/immunology , Pneumonia, Viral/physiopathology , Posterior Leukoencephalopathy Syndrome/diagnosis , Posterior Leukoencephalopathy Syndrome/immunology , Posterior Leukoencephalopathy Syndrome/physiopathology , Prognosis , Risk Factors , SARS-CoV-2ABSTRACT
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.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/adverse effects , Cognition/drug effects , Leukoencephalopathies/chemically induced , Leukoencephalopathies/physiopathology , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Breast Neoplasms/drug therapy , Case-Control Studies , Dose-Response Relationship, Drug , Humans , Methotrexate , Posterior Leukoencephalopathy Syndrome/chemically induced , Posterior Leukoencephalopathy Syndrome/physiopathology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Prevalence , Prospective Studies , Severity of Illness IndexSubject(s)
Anticonvulsants/therapeutic use , Calcium Channel Blockers/therapeutic use , Coronavirus Infections/physiopathology , Pneumonia, Viral/physiopathology , Posterior Leukoencephalopathy Syndrome/physiopathology , Status Epilepticus/drug therapy , Verapamil/therapeutic use , Aged , Anemia/etiology , Anticoagulants/therapeutic use , Antiviral Agents/therapeutic use , Betacoronavirus , COVID-19 , Coronavirus Infections/complications , Coronavirus Infections/drug therapy , Drug Combinations , Enzyme Inhibitors/therapeutic use , Humans , Hydroxychloroquine/therapeutic use , Lopinavir/therapeutic use , Lymphopenia/etiology , Male , Pandemics , Pneumonia, Viral/complications , Pneumonia, Viral/drug therapy , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Posterior Leukoencephalopathy Syndrome/etiology , Ritonavir/therapeutic use , SARS-CoV-2 , Status Epilepticus/etiology , COVID-19 Drug TreatmentABSTRACT
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.