Posterior Reversible Encephalopathy Syndrome in a Patient With Multiple System Atrophy.
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ABSTRACT:
SUBMITTER: Debray S
PROVIDER: S-EPMC6174484 | biostudies-literature | 2017 Sep-Oct
REPOSITORIES: biostudies-literature
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Posterior Reversible Encephalopathy Syndrome in a Patient With Multiple System Atrophy.
Debray Sarah S Van de Vondel Saartje S Tousseyn Thomas T Thal Dietmar Rudolf DR Dewil Maarten M
Movement disorders clinical practice 20170525 5
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Project description:The posterior reversible encephalopathy syndrome (PRES) is a neurological disorder of (sub)acute onset characterized by varied neurological symptoms, which may include headache, impaired visual acuity or visual field deficits, disorders of consciousness, confusion, seizures, and focal neurological deficits. In a majority of patients the clinical presentation includes elevated arterial blood pressure up to hypertensive emergencies. Neuroimaging, in particular magnetic resonance imaging, frequently shows a distinctive parieto-occipital pattern with a symmetric distribution of changes reflecting vasogenic edema. PRES frequently develops in the context of cytotoxic medication, (pre)eclampsia, sepsis, renal disease or autoimmune disorders. The treatment is symptomatic and is determined by the underlying condition. The overall prognosis is favorable, since clinical symptoms as well as imaging lesions are reversible in most patients. However, neurological sequelae including long-term epilepsy may persist in individual cases.
| S-EPMC5533845 | biostudies-other
Project description:AudienceEmergency medicine residents of all levels.IntroductionPosterior reversible encephalopathy syndrome (PRES) is a clinically significant cause of seizures, headache, neurologic deficit, and hypertensive emergency that is not uncommon in the emergency department. Posterior reversible encephalopathy syndrome was initially described as a clinical syndrome in 1996.1 It is an important cause of hypertensive emergency that is not often covered in depth in the emergency medicine curriculum since the true incidence and disease process continues to be researched.Populations who are at most risk for PRES include those with chronic hypertension, chronic renal disease, autoimmune disease, and immune suppression.2 Patients with PRES will often present with varied forms of encephalopathy and sometimes even focal neurologic symptoms that would suggest a cerebral vascular accident. These neurologic symptoms can include visual complaints and headache. Seizures are also frequently reported in association with PRES.3Early identification and appropriate management of PRES decreases morbidity and mortality without chronic neurologic sequelae. The pillars of diagnosis and management can be initiated in the emergency department. This includes a diagnosis made by a thorough history and physical exam and cerebral imaging.4 The mainstay of management is parenteral anti-hypertensives with proper blood pressure monitoring.5.Educational objectivesBy the end of the simulation, the learner will be able to: 1) manage an acute seizure 2) discuss imaging modalities to diagnose PRES 3) discuss medical management of PRES.Educational methodsThis simulation exercise is meant to be presented as a traditional medium-to-high-fidelity medical simulation case. With minor adjustments, it could be utilized as a low-fidelity case or an oral exam case.Research methodsThe educational content and general usefulness of this simulation was evaluated by open verbal (qualitative) feedback from a convenience sample of random participants following a completion of the case and debriefing by a participant group (n=30) of emergency medicine residents at a large 3-year residency training program.ResultsThe overall feedback was positive. Participants felt that it was a good opportunity to practice identifying PRES and managing it in a safe learning environment. They especially appreciated learning more about the pathophysiology of PRES, the high-risk factors for PRES, and management of the condition.DiscussionPosterior reversible encephalopathy syndrome, an uncommon condition, presents similar to many other benign and common complaints. It is crucial to be able to differentiate PRES from other causes of headache, visual disturbance, and seizures. It is important to keep PRES in mind when considering hypertensive emergencies. Many PGY-1 residents struggled to diagnose and treat PRES because it was often not on their differential, and this case helped broaden their differential. PGY-2 and PGY-3 were more frequently able to appropriately diagnose and treat PRES in this patient but found the case to be helpful in their decision-making and learning more about PRES pathophysiology. This case and associated high-yield debriefing session were effective for learners of all levels.TopicsPosterior reversible encephalopathy syndrome (PRES), altered mental status, seizure, headache, hypertensive emergency.
| S-EPMC10332763 | biostudies-literature
Project description:Graft-versus-host disease (GVHD) is an immune triggered process leading to severe immune dysregulation and organ dysfunction until death and it is one of the worst medical complications after a transplant. Patients with GVHD may have several neurological alterations: during this acute severe phase there is coexistence of various and nonspecific neurological symptoms. We are reporting a case of a 53 year old woman with severe GVHD after bone marrow transplant with acute neurological signs and symptoms. MRI study showed findings consistent with Posterior reversible encephalopathy syndrome and Wernicke encephalopathy.
| S-EPMC6545363 | biostudies-literature
Project description: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.
| S-EPMC7034490 | biostudies-literature
Project description:BackgroundPosterior reversible encephalopathy syndrome (PRES) can cause short-term cerebrovascular complications, such as brain infarction and hemorrhage. We hypothesized that PRES is also associated with an increased long-term risk of stroke.MethodsWe performed a retrospective cohort study in the United States using statewide all-payer claims data from 2016 to 2018 on all admissions to nonfederal hospitals in 11 states. Adults with PRES were compared with adults with renal colic (negative control) and transient ischemic attack (TIA; positive control). Any stroke and the secondary outcomes of ischemic and hemorrhagic stroke were ascertained using International Classification of Diseases, Tenth Revision, Clinical Modification codes. We excluded prevalent stroke. We used time-to-event statistics to calculate incidence rates and Cox proportional hazards analyses to evaluate the association between PRES and stroke, adjusting for demographics and stroke risk factors. In a sensitivity analysis, outcomes within 2 weeks of index admission were excluded.ResultsWe identified 1606 patients with PRES, 1192 with renal colic, and 38 216 with TIA. Patients with PRES had a mean age of 56±17 years; 72% were women. Over a median follow-up of 0.9 years, the stroke incidence per 100 person-years was 6.1 (95% CI, 5.0-7.4) after PRES, 1.0 (95% CI, 0.62-1.8) after renal colic, and 9.7 (95% CI, 9.4-10.0) after TIA. After statistical adjustment for patient characteristics and risk factors, patients with PRES had an elevated risk of stroke compared with renal colic (hazard ratio [HR], 2.3 [95% CI, 1.7-3.0]), but lower risk than patients with TIA (HR, 0.67 [95% CI, 0.54-0.82]). In secondary analyses, compared with TIA, PRES was associated with hemorrhagic stroke (HR, 2.0 [95% CI, 1.4-2.9]). PRES was associated with ischemic stroke when compared with renal colic (HR, 1.9 [95% CI, 1.4-2.7]) but not when compared with TIA (HR, 0.49 [95% CI, 0.38-0.63]). Results were similar with 2-week washout.ConclusionsPatients with PRES had an elevated risk of incident stroke.
| S-EPMC9613524 | biostudies-literature
Project description:Posterior reversible encephalopathy syndrome (PRES) is a reversible neuroradiological syndrome characterized by reversible vasogenic edema. The pathophysiological mechanism is still unclear, but PRES may be triggered by various etiologies. To date, only a few PRES cases linked to cerebrospinal fluid (CSF) hypovolemia were reported. The association between PRES and CSF hypovolemia needs to be explored. We presented a case of PRES with CSF hypovolemia as a result of an inadvertent dural puncture and reviewed the literature to identify the clinical characterization and pathophysiological mechanism of PRES following CSF hypovolemia. A total of 31 cases of PRES-CSF hypovolemia was included for analysis. The median age was 33 years, with a notable female predominance (87.1%). Fifteen patients (48.4%) didn't have either a history of hypertension nor an episode of hypertension. The most common cause of CSF hypovolemia was epidural or lumbar puncture (n = 21), followed by CSF shunt (n = 6). The median interval between the procedure leading to CSF hypovolemia and PRES was 4 days. Seizure, altered mental state, and headache were the most frequent presenting symptom. The parietooccipital pattern was most frequent (71.0%). Conservative management remains the mainstay of treatment with excellent outcomes. Three patients had a second episode of PRES. CSF hypovolemia is a plausible cause of PRES via a unique pathophysiologic mechanism including arterial hyperperfusion and venous dysfunction. Patients with CSF hypovolemia is more susceptible to PRES, which is potentially life-threatening. Given that CSF hypovolemia is a common complication of anesthetic, neurological, and neurosurgical procedures, PRES should be early considered for prompt diagnosis and appropriate management.
| S-EPMC7324723 | biostudies-literature
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.
| S-EPMC8128592 | biostudies-literature
Project description:Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) are relatively uncommon neurological disorders, but their detection has been increasing mainly due to clinical awareness and spreading of magnetic resonance imaging (MRI). Because these syndromes share some common clinical and radiologic features and occasionally occur in the same patient, misdiagnosis may occur. PRES is characterized by varied neurological symptoms including headache, impaired visual acuity or visual field deficit, confusion, disorders of consciousness, seizures, and motor deficits often associated to peculiar neuroradiological pattern even if uncommon localization and ischemic or hemorrhagic lesions were described. RCVS is a group of diseases typically associated with severe headaches and reversible segmental vasoconstriction of cerebral arteries, often complicated by ischemic or hemorrhagic stroke. Pathophysiological basis of PRES and RCVS are still debated but, because they share some risk factors and clinical features, a possible common origin has been supposed. Clinical course is usually self-limiting, but prognosis may fluctuate from complete recovery to death due to complications of ischemic stroke or intracranial hemorrhage. Neuroradiological techniques such as digital angiography and MRI are helpful in the diagnostic pathway and a possible prognostic role of MRI has been suggested. This review will serve to summarize clinical, neuroradiological features and controversies underlying both syndromes that may mislead the diagnostic pathway and their possible relationship with pathophysiology, clinical course, and prognosis.
| S-EPMC7033494 | biostudies-literature
Project description:While posterior reversible encephalopathy syndrome (PRES) is often characterized by an inflammatory cerebrospinal-fluid (CSF) profile, knowledge of immune cell patterns in PRES is lacking. Thus, we retrospectively characterized CSF and peripheral blood (PB) from 15 PRES patients, which we analyzed by multidimensional flow cytometry (FC). Results were compared to 72 controls, as well as to 9 patients with progressive multifocal leukoencephalopathy (PML, as a relevant differential diagnosis) and 15 multiple sclerosis patients (MS, as a classical neuroinflammatory disorder), respectively. Total protein level in CSF from PRES patients was elevated compared to that in controls, but not to MS and PML. In-depth FC analysis revealed no differences for adaptive immune cells (B cells, plasma cells, CD4+, and CD8+ T cells) in PB or CSF of PRES compared to controls. In contrast, we observed alterations of the adaptive immune response in CSF of PML and MS compared to PRES, indicating that the adaptive immune response is not a driver of disease in PRES. Indeed, PRES was characterized by an innate immune response with CD14++/CD16+ (intermediate) monocytes elevated in PB and CSF, while CD14++/CD16- (classical) monocytes were decreased in PB from PRES patients as compared to controls. Levels of CD14++/CD16+ monocytes correlated with the duration of hospital stay as a surrogate marker for disease severity in PRES patients. Our findings argue for a role of innate rather than adaptive immunity in the pathophysiology of PRES. The observed shift in monocyte subsets might provide valuable diagnostic clues for the clinical management of these patients.
| S-EPMC8310851 | biostudies-literature
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.
| S-EPMC2861971 | biostudies-literature