Posteriores reversibles Enzephalopathiesyndrom (PRES)

Background

Posterior reversible encephalopathy syndrome (PRES) is an acute neurological disease. Although there are some case reports of patients with a poor clinical outcome, studies about PRES and intensive care medicine are rare.

Patients and methods

The medical records of our department were screened for the diagnosis of PRES. Data of 26 patients were analyzed retrospectively.

Results

Of the 26 PRES patients, 65.4% were treated in the intensive care unit (n=13) or stroke unit (n=4) for a mean duration of 3.9 days (range: 1–9 days). During admission, the correct diagnose was suspected for only 1 patient. Generalized seizures occurred in 88.5%. Only 1 patient needed artificial ventilation (>12 h).

Conclusion

Patients with PRES are frequently treated in the intensive care unit/stroke unit because of the clinical occurrence of the syndrome as a severe neurological disease. On the other hand, there are many life-threatening differential diagnoses. Epileptic seizures are the most frequent symptoms. Especially the combination of disturbed vision and epileptic seizures make the diagnosis of PRES more likely.     

Das Posteriore Reversible Enzephalopathie-Syndrom (PRES)

During 2004 to 2006 we observed six patients developing posterior reversible encephalopathy syndrome (PRES) during intensive medicine treatment at our hospital. Underlying diagnoses were intracranial hemorrhage (n=3), surgery of a neoplasm (n=2), and hypertensive crisis (n=1). Symptoms had been seizures, visual disturbances, and alteration of vigilance in two patients, respectively. The presumed factors for development of a PRES were hypertension (n=2), renal insufficiency with hypertension (n=1) and hypopotassemia, respectively (n=1), administration of serotonine reuptake inhibitors combined with hypertension (n=1), and unknown (n=1). Acute treatment consisted in lowering of blood pressure, anticonvulsive therapy, balanced fluid therapy, correction of electrolyte imbalance, withdrawal of presumed noxious agents, and expectant regimen, respectively. Symptoms were reversible in all patients and long-term treatment was necessary in two patients with convulsions and two patients with hypertension.     

Recurrent posterior reversible encephalopathy syndrome (PRES)

Posterior reversible encephalopathy syndrome is a proposed cliniconeuroradiological entity characterized by headache, altered mental status, cortical blindness, seizures, and other focal neurological signs, and a diagnostic magnetic resonance imaging picture. A variety of different etiologies have been reported like hypertension, pre-eclampsia/eclampsia, cyclosporin A or tacrolimus neurotoxicity, uraemia and porphyria. With early diagnosis and prompt treatment, the syndrome is usually fully reversible. We report a case of recurrent PRES of unknown aetiology following intensive care unit treatment and only moderately elevated blood pressure. Clinicians as well as radiologists must be familiar with this clinically frightening, underdiagnosed condition to assure timely diagnosis and treatment to prevent persistent deficits.     

Posterior reversible encephalopathy syndrome (PRES) and chronic kidney disease

Posterior reversible encephalopathy (PRES) is a recently described syndrome, defined by clinical and neuroimaging features. Chronic kidney disease patients may be especially vulnerable to this syndrome because they are frequently exposed to several of its possible causes, including uremia and hypertension. In its most severe form, PRES can manifest clinically as seizures, coma or death. However, if properly diagnosed and treated, this syndrome can be completely reversible. Therefore, neuroimaging methods, especially brain magnetic resonance is fundamental for its diagnosis because it shows brain edema in characteristic pattern, and excludes causes of seizures or coma. An important example is the case of a young hypertensive chronic kidney disease patient on peritoneal dialysis, brought to the emergency room comatous with generalized tonic-clonic seizures; the cerebral magnetic resonance imaging features were impressive. Anti-hypertensive therapy and hemodialysis allowed complete recovery. The reversibility of this syndrome depends on timely diagnosis and therapy and therefore it should be kept in mind in the differential diagnosis of seizures. or coma in chronic kidney disease patients.     

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.     

Unusual neurologic manifestations (II): posterior reversible encephalopathy syndrome (PRES) in the context of juvenile systemic lupus erythematosus

Posterior reversible encepalopathy syndrome (PRES), or reversible posterior leukoencephalopathy, is a neurologic condition characterized by recognizable pattern of altered mental status, headache, visual changes and seizures in association with findings indicating a predominantly posterior leucoencephalopathy on imaging studies. It has rarely been described in children. We report two cases of pediatric systemic lupus erythematosus (SLE) complicated by PRES and review the literature.     

Aspirin-Intolerant Asthma (AIA) Assessment Using the Urinary Biomarkers, Leukotriene E(4) (LTE(4)) and Prostaglandin D(2) (PGD(2)) Metabolites

The clinical syndrome of aspirin-intolerant asthma (AIA) is characterized by aspirin/nonsteroidal anti-inflammatory drug intolerance, bronchial asthma, and chronic rhinosinusitis with nasal polyposis. AIA reactions are evidently triggered by pharmacological effect of cyclooxygenase-1 inhibitors. Urine sampling is a non-invasive research tool for time-course measurements in clinical investigations. The urinary stable metabolite concentration of arachidonic acid products provides a time-integrated estimate of the production of the parent compounds in vivo. AIA patients exhibits significantly higher urinary concentrations of leukotriene E(4) (LTE(4)) and 1,15-dioxo-9α-hydroxy-2,3,4,5-tetranorprostan-1,20-dioic acid (tetranor-PGDM), a newly identified metabolite of PGD(2), at baseline. This finding suggests the possibility that increased mast cell activation is involved in the pathophysiology of AIA even in a clinically stable condition. In addition, lower urinary concentrations of primary prostaglandin E(2) and 15-epimer of lipoxin A(4) at baseline in the AIA patients suggest that the impaired anti-inflammatory elements may also contribute to the severe clinical outcome of AIA. During the AIA reaction, the urinary concentrations of LTE(4) and PGD(2) metabolites, including tetranor-PGDM significantly and correlatively increase. It is considered that mast cell activation probably is a pathophysiologic hallmark of AIA. However, despite the fact that cyclooxygenease-1 is the dominant in vivo PGD(2) biosynthetic pathway, the precise mechanism underlying the PGD(2) overproduction resulting from the pharmacological effect of cyclooxygenease-1 inhibitors in AIA remains unknown. A comprehensive analysis of the urinary concentration of inflammatory mediators may afford a new research target in elucidating the pathophysiology of AIA.     

Expression, purification, and characterization of human hemoglobins Gower-1 (zeta(2)epsilon(2)), Gower-2 (alpha(2)epsilon(2)), and Portland-2 (zeta(2)beta(2)) assembled in complex transgenic-knockout mice

Embryonic zeta- and epsilon-globin subunits assemble with each other and with adult alpha- and beta-globin subunits into hemoglobin heterotetramers in both primitive and definitive erythrocytes. The properties of these hemoglobins-Hbs Gower-1 (zeta(2)epsilon(2)), Gower-2 (alpha(2)epsilon(2)), and Portland-2 (zeta(2)beta(2))-have been incompletely described as they are difficult to obtain in quantity from either primary human tissue or conventional expression systems. The generation of complex transgenic-knockout mice that express these hemoglobins at levels between 24% and 70% is described, as are efficient methods for their purification from mouse hemolysates. Key physiological characteristics-including P(50), Hill coefficient, Bohr effect, and affinity for 2,3-BPG-were established for each of the 3 human hemoglobins. The stability of each hemoglobin in the face of mechanical, thermal, and chemical stresses was also determined. Analyses indicate that the zeta-for-alpha exchange distinguishing Hb Portland-2 and Hb A alters hemoglobin O(2)-transport capacity by increasing its P(50) and decreasing its Bohr effect. By comparison, the epsilon-for-beta exchange distinguishing Hb Gower-2 and Hb A has little impact on these same functional parameters. Hb Gower-1, assembled entirely from embryonic subunits, displays an elevated P(50) level, a reduced Bohr effect, and increased 2,3-BPG binding compared to Hb A. The data support the hypothesis that Hb Gower-2, assembled from reactivated epsilon globin in individuals with defined hemoglobinopathies and thalassemias, would serve as a physiologically acceptable substitute for deficient or dysfunctional Hb A. In addition, the unexpected properties of Hb Gower-1 call into question a common hypothesis for its primary role in embryonic development.     

Comparison of FEV(3), FEV(6), FEV(1)/FEV(3) and FEV(1)/FEV(6) with usual spirometric indices

Background and objective: Pulmonary function tests play an important role in the management of pulmonary diseases. One of the tests that are widely used is spirometry. Performing an acceptable spirometry manoeuvre according to the standards set by the American Thoracic Society/European Respiratory Society is difficult. The aim of this study was to compare forced expiratory volume in 3 s (FEV₃) and forced expiratory volume in 6 s (FEV₆) with forced vital capacity (FVC), and forced expiratory volume in 1 s FEV₁/FEV₃ and FEV₁/FEV₆ with FEV₁/FVC, in order to substitute the usual spirometric manoeuvres with manoeuvres that are easier to perform. Methods: In a cross‐sectional study, spirometry was performed for 588 subjects who were referred for occupational health evaluations. The accuracy of FEV₃, FEV₆, FEV₁/FEV₃ and FEV₁/FEV₆ was compared with that of FVC and FEV₁/FVC. Chi‐square tests and kappa tests were used to analyse the data. Results: Individuals with normal (n = 297) and abnormal spirometry (n = 291) were evaluated. The sensitivity, specificity, positive predictive value and negative predictive value of FEV₁/FEV₆, as compared with that of FEV₁/FVC for detecting obstruction, were 93.56, 99.32, 98.95 and 96.09, respectively. The sensitivity, specificity, positive predictive value and negative predictive value of FEV₆, as compared with that of FVC for detecting restriction, were 96.68, 98.65, 96.68 and 98.65, respectively. Conclusions: FEV₆ and FEV₁/FEV₆ can be used as surrogates for FVC and FEV₁/FVC, respectively, and these parameters showed acceptable sensitivity, specificity, positive predictive value and negative predictive value for occupational health evaluations.