scholarly journals Acute Intermittent Porphyria, Posterior Reversible Encephalopathy Syndrome and Hypertensive Emergency, A Review of The Literature

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Mohamed A. Ahmed ◽  
Mahmoud Abdelnabi ◽  
Abdallah Almaghraby
Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Molly Bates ◽  
Kyle Darpel ◽  
Nneka Amadife ◽  
Adam Dugan ◽  
Jessica D Lee

Introduction: Several theories exist regarding the pathogenesis of posterior reversible encephalopathy syndrome (PRES). One theory suggests that PRES occurs when systemic blood pressure exceeds the upper limit of cerebral autoregulation. Endothelial dysfunction has been proposed as an alternative pathogenesis to account for PRES outside the setting of acute hypertension. This mechanism has been implicated in other conditions associated with PRES including autoimmune diseases, cytotoxic medications, sepsis, and eclampsia. The purpose of this study was to determine if COPD, a disease known to cause endothelial dysfunction, has a causative association with the development of PRES. Methods: A single center retrospective, age-matched, case-control study was performed from January 2013 to June 2019 comparing patients discharged with a primary diagnosis of PRES to a control group with acute ischemic stroke. Demographics, medical comorbidities, initial blood pressure, and clinical outcomes were compared between the two groups. For categorical variables, p-values were calculated using χ2 and Fisher’s exact tests. For continuous variables, p-values were calculated using two-sample t-tests. The effect of COPD and acute hypoxic respiratory failure on PRES status was investigated using multivariate logistic regression. Results: A total of 94 PRES subjects and 109 control subjects were included for analysis. Mean age did not differ between the two groups; however, the PRES group was more likely to be female (78.7% vs. 49.5%, p<0.001). COPD was present in 26.6% (n=25) of cases and 11% (n= 12) of controls (odds ratio 4.12, p=0.003). Occurrence of hypertension did not differ significantly between the two groups (78.0% vs 86.2%). Among patients with PRES in the setting of COPD (n=25), 60% (n=16) did not meet criteria for hypertensive emergency. Controlling for hypertensive emergency status in a multivariate logistic regression analysis, patients with COPD were 3.21 times more likely to develop PRES (p= 0.004). Conclusions: To our knowledge, very few reports of PRES in the setting of COPD have been described in the literature and no association of PRES and COPD has been defined to date. Our data support the role of COPD as a risk factor in the development of PRES.


2020 ◽  
Vol 20 (6) ◽  
pp. 486-488
Author(s):  
Grace Swart ◽  
Su San Lim ◽  
Martin Jude

Acute intermittent porphyria is a rare genetic condition in which disrupted haem synthesis causes overproduction of porphyrin precursors. Occasionally, it is associated with posterior reversible encephalopathy syndrome (PRES), presenting with headache, confusion, seizures and visual disturbance. We describe a patient with acute intermittent porphyria who presented with seizures and PRES, and who had previous unexplained severe abdominal pain. Acute intermittent porphyria should be considered as a possible cause of PRES, especially in those with unexplained abdominal pain, since delays in its diagnosis can result in permanent complications.


2014 ◽  
Vol 14 (4) ◽  
pp. 344-347 ◽  
Author(s):  
Nicholas Ettinger ◽  
Matthew Pearson ◽  
Fred S. Lamb ◽  
John C. Wellons

In this report, the authors describe the case of a teenage boy who presented with hypertensive emergency, posterior reversible encephalopathy syndrome, and hydrocephalus due to fourth ventricle outlet obstruction. Posterior reversible encephalopathy syndrome is a well-characterized but uncommon syndrome in children that is generally triggered by severe hypertension. The unusual clinical picture of this patient, who had isolated cerebellar edema leading to obstructive hydrocephalus, has been rarely described in children.


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