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Posterior Reversible Encephalopathy Syndrome: Clinical Features and Outcome.

ABSTRACT: Background: Posterior reversible encephalopathy syndrome (PRES) is an acute neurotoxic syndrome that is characterized by a spectrum neurological and radiological feature from various risk factors. Common neurological symptoms includes headache, impairment in level of consciousness, seizures, visual disturbances, and focal neurological deficits. Common triggering factors include blood pressure fluctuations, renal failure, eclampsia, exposure to immunosuppressive or cytotoxic agents and autoimmune disorders. The classic radiographic findings include bilateral subcortical vasogenic edema predominantly affecting the parieto-occipital regions but atypical features include involvement of other regions, cortical involvement, restricted diffusion, hemorrhage, contrast enhancement. This review is aimed to summarize the updated knowledge on the typical and atypical clinical and imaging features, prognostic markers and identify gaps in literature for future research. Methods: Systematic literature review using PUBMED search from 1990 to 2019 was performed using terms PRES was performed. Results: While clinical and radiographic reversibility is common, long-standing morbidity and mortality can occur in severe forms. In patients with malignant forms of PRES, aggressive care has markedly reduced mortality and improved functional outcomes. Although seizures were common, epilepsy is rare. Various factors that have been associated with poor outcome include altered sensorium, hypertensive etiology, hyperglycemia, longer time to control the causative factor, elevated C reactive protein, coagulopathy, extensive cerebral edema, and hemorrhage on imaging. Conclusion: Large prospective studies that accurately predict factors that are associated with poor outcomes, determine the pathophysiology, and targeted therapy are required.

SUBMITTER: Hinduja A

PROVIDER: S-EPMC7034490 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Posterior Reversible Encephalopathy Syndrome: Clinical Features and Outcome.

Hinduja Archana A

Frontiers in neurology 20200214

<b>Background:</b> Posterior reversible encephalopathy syndrome (PRES) is an acute neurotoxic syndrome that is characterized by a spectrum neurological and radiological feature from various risk factors. Common neurological symptoms includes headache, impairment in level of consciousness, seizures, visual disturbances, and focal neurological deficits. Common triggering factors include blood pressure fluctuations, renal failure, eclampsia, exposure to immunosuppressive or cytotoxic agents and aut ...[more]

PMID: 32117030

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Project description:ObjectiveTo identify and define clinical associations and radiologic findings of posterior reversible encephalopathy syndrome (PRES).Patients and methodsPatients prospectively diagnosed as having PRES from October 1, 2005, through April 30, 2009, were pooled with retrospectively identified patients admitted from August 1, 1999, through September 30, 2005. We performed a detailed review of clinical information, including demographics, presenting symptoms, medical history, and risk factors. All patients underwent computed tomography of the brain or magnetic resonance imaging. Findings on magnetic resonance imaging were analyzed independently by 2 neuroradiologists.ResultsWe identified 120 cases of PRES in 113 patients (mean age, 48 years). Mean peak systolic blood pressure was 199 mm Hg (minimum-maximum, 160-268 mm Hg), and mean peak diastolic blood pressure was 109 mm Hg (minimum-maximum, 60-144 mm Hg). Etiologies of PRES included hypertension (n=69 [61%]), cytotoxic medications (n=21 [19%]), sepsis (n=8 [7%]), preeclampsia or eclampsia (n=7 [6%]), and multiple organ dysfunction (n=1 [1%]). Autoimmune disease was present in 51 patients (45%). Clinical presentations included seizures (n=84 [74%]), encephalopathy (n=32 [28%]), headache (n=29 [26%]), and visual disturbances (n=23 [20%]). In the 115 cases (109 patients) for which magnetic resonance imaging findings were available, the parieto-occipital regions were the most commonly involved (n=108 [94%]), followed by the frontal lobe (n=88 [77%]), temporal lobe (n=74 [64%]), and cerebellum (n=61 [53%]). Cerebellar involvement was significantly more frequent in patients with a history of autoimmunity (P=.008), and patients with sepsis were more likely to have cortical involvement (P<.001).ConclusionA substantial proportion of patients with PRES have underlying autoimmune conditions that may support endothelial dysfunction as a pathophysiologic mechanism. On brain imaging, the location and severity of vasogenic edema were mostly similar for the different clinical subgroups.

Project description:Background and purposePosterior reversible encephalopathy syndrome (PRES) is known to occur after solid organ transplantation (SOT), potentially associated with cyclosporine and tacrolimus. In this study, we assess the frequency and clinical and imaging characteristics of PRES after SOT.Materials and methodsWe identified 27 patients (13 men and 14 women; age range, 22-72 years) who developed PRES after SOT. Features noted included SOT subtype, incidence and timing of PRES, infection and rejection, mean arterial pressure (MAP), and toxicity brain edema.ResultsPRES developed in 21 (0.49%) of 4222 patients who underwent transplantation within the study period (no significant difference among SOT subtypes). Transplantation was performed in 5 patients before the study period, and 1 patient underwent transplantation elsewhere. In consideration of all 27 patients, PRES typically developed in the first 2 months in patients who had SOT of the liver (9 of 10 patients) and was associated with cytomegalovirus (CMV), mild rejection, or systemic bacterial infection. PRES also typically developed after 1 year in patients who had SOT of the kidney (8 of 9 patients) and was associated with moderate rejection or bacterial infection. Toxicity MAP was significantly lower (P < .001) in liver transplants (average MAP, 104.8 +/- 16 mm Hg) compared with that in kidney transplants (average MAP, 143 +/- 20 mm Hg). Toxicity brain edema was significantly greater (P < .001) in patients who had liver transplants and developed PRES compared with patients who had undergone kidney transplants despite severe hypertension in those who had the kidney transplants.ConclusionPatients who had undergone SOTs have a similar low incidence of developing PRES. Differences between those who have had liver and kidney transplants included time after transplant, toxicity MAP, and PRES vasogenic edema noted at presentation. In patients who have undergone kidney transplants, severely elevated MAP was associated with reduced, not greater, brain edema.

Project description:Background: Accurate diagnosis and timely treatment for posterior reversible encephalopathy syndrome (PRES) with atypical regions are very important in clinical practice. However, until now, little has been known about the clinical and MRI manifestations of this disease. Therefore, the aim of this study is to investigate the clinical and MRI features of PRES to promote clinical management and deepen our understanding of this disease. Materials and Methods: Data from six PRES patients with atypical regions were collected from our hospital. Data from another 550 cases were obtained by searching the PubMed, EMBASE and Web of Science databases with the keywords "posterior reversible encephalopathy syndrome" "PRES" "reversible posterior leukoencephalopathy" "RPLS" "hypertensive encephalopathy" "hyperperfusion encephalopathy" or "reversible posterior cerebral edema encephalopathy." The clinical and MRI features of these 556 cases were analyzed together. Results: A total of 305 patients were female, and 248 were male, with a median age of 34 years. The information on sex and age of three patients was not available. The most common symptom was headache (282/556, 50.7%), followed by altered mental status (243/556, 43.7%), seizures (233/556, 41.9%), visual disturbances (194/556, 34.9%), nausea/vomiting (130/556, 23.4%), and focal neurological deficits (101/556, 18.2%). Hypertension (425/556, 76.4%), renal diseases (152/556, 27.3%), immunosuppressant drugs (79/556, 14.2%), and chemotherapy/chemoradiotherapy (59/556, 10.6%) were the major predisposing factors. The atypical regions of the lesions were the cerebellum (331/556, 59.5%), basal ganglia (135/556, 24.3%), periventricular/deep white matter (125/556, 22.5%), pons (124/556, 22.3%), brainstem (115/556, 20.7%), thalamus (114/556, 20.5%), midbrain (48/556, 8.6%), spinal cord (33/556, 5.9%), and medulla (29/556, 5.2%). Additionally, the following typical regions were observed: occipital (278/556, 50.0%), parietal (234/556, 42.1%), frontal (150/556, 27.0%), and temporal (124/556, 22.3%) lobes. The major treatments were antihypertensives (358/515, 69.5%), antiepileptics/sedation (126/515, 24.5%), discontinuation/switching agents (67/515, 13.0%), and steroids (54/515, 10.5%). The median time of the clinical state improved and abnormal neuroimaging resolved is 2-3 weeks after appropriate treatment. Conclusion: The common symptoms of PRES with atypical regions include headaches, altered mental status, seizures, visual disturbances, nausea or vomiting, and focal neurological deficits. The frequent predisposing factors include hypertension, renal diseases, immunosuppressant drugs and chemotherapy/chemoradiotherapy. MRI features are mainly characterized by vasogenic edema in central zones always accompanied by typical regions. Most cases can be reversed in 2-3 weeks when promptly recognized and properly treated.

Dataset Information

Posterior Reversible Encephalopathy Syndrome: Clinical Features and Outcome.

Publications

Posterior Reversible Encephalopathy Syndrome: Clinical Features and Outcome.

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