scholarly journals Posterior Reversible Encephalopathy Syndrome as Presenting Form of Very Early Systemic Sclerosis

2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
María Isabel Pedraza ◽  
Julia Barbado ◽  
Marina Ruiz ◽  
Ángel Luis Guerrero
Keyword(s):  
Systemic Sclerosis ◽  
Arterial Hypertension ◽  
Blood Brain Barrier ◽  
Posterior Reversible Encephalopathy Syndrome ◽  
Vascular Endothelium ◽  
Brain Barrier ◽  
Posterior Reversible Encephalopathy ◽  
Blood Brain ◽  
Reversible Encephalopathy ◽  
Early Systemic Sclerosis

Introduction. Posterior Reversible Encephalopathy Syndrome (PRES) is an increasingly recognized clinical and radiological entity with a wide spectrum of symptoms. Its mechanism depends on failure of the blood-brain barrier due to high systemic blood pressure (BP) and loss of integrity of vascular endothelium related with different triggers.Methods. We aim to report a case of PRES induced by arterial hypertension and very early systemic sclerosis (SSc) not previously known.Results. A 64-year-old female was admitted due to 1-week pulsating headache more prominent on frontal scalp, accompanied by phonophobia, photophobia, and facial flushing. Neurological exam revealed brisk deep tendon reflex. Brain magnetic resonance imaging (MRI) showed subcortical lesions mainly located in posterior regions. BP was monitored and episodic arterial hypertension was detected. In laboratory tests positive anti-topoisomerase I antibodies were detected. BP was controlled with angiotensin-converting-enzyme inhibitors and headache improved. In a new MRI a month later improvement of white matter lesions was observed. Capillaroscopy showed “active pattern,” considered typical of SSc.Conclusion. In SSc anti-endothelial cell antibodies impair vascular endothelium and liberation of vasoconstrictors leads to BP increasing and disruption of blood-brain barrier autoregulation mechanisms. PRES can be the first manifestation of very early SSc and this entity should be considered even in absence of skin lesions or Raynaud phenomenon.

scholarly journals Central-Variant Posterior Reversible Encephalopathy Syndrome with Albuminocytologic Dissociation

2018 ◽  
Vol 10 (1) ◽  
pp. 29-33 ◽  
Author(s):  
Sang-Woo Lee ◽  
Seung-Jae Lee
Keyword(s):  
Blood Brain Barrier ◽  
Posterior Reversible Encephalopathy Syndrome ◽  
Vasogenic Edema ◽  
Brain Magnetic Resonance Imaging ◽  
Cerebral Hemispheres ◽  
Brain Barrier ◽  
Posterior Reversible Encephalopathy ◽  
Blood Brain ◽  
Normal White Blood Cell ◽  
Reversible Encephalopathy

Posterior reversible encephalopathy syndrome (PRES) is a disorder of reversible vasogenic brain edema which mainly involves the parieto-occipital lobes in various clinical settings. The main mechanism is known to be cerebral autoregulation failure and endothelial dysfunction leading to the disruption of the blood-brain barrier. We report the case of a 47-year-old woman with PRES which involved the brain stem and thalami, sparing the cerebral hemispheres. She was admitted to the emergency room because of acute-onset confusion. Her initial blood pressure was 270/220 mm Hg. Routine blood lab tests showed pleocytosis, hyperglycemia, and azotemia. Brain magnetic resonance imaging (MRI) showed a lesion of vasogenic edema involving nearly the whole area of pons, the left side of the midbrain, and the bilateral medial thalami. Cerebrospinal fluid (CSF) examination revealed an increased level of protein with normal white blood cell count. With conservative care, the patient markedly recovered 3 days after symptom onset, and a follow-up MRI confirmed complete resolution of the vasogenic edema. This case suggests that PRES can rarely involve the “central zone” only, sparing the cerebral hemispheres, which may be confused with other neurological diseases. Besides, the CSF albuminocytologic dissociation may suggest the disruption of the blood-brain barrier in patients with PRES.

scholarly journals Recurrent Posterior Reversible Encephalopathy Syndrome Potentially Related to AIDS and End-Stage Renal Disease: A Case Report and Review of the Literature

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Olivia Hui-Chiun Chang ◽  
Alexandra Stanculescu ◽  
Chi Dola ◽  
William Benjamin Rothwell
Keyword(s):  
Renal Disease ◽  
Blood Brain Barrier ◽  
Posterior Reversible Encephalopathy Syndrome ◽  
End Stage Renal Disease ◽  
Brain Barrier ◽  
Posterior Reversible Encephalopathy ◽  
Blood Brain ◽  
Stage Renal Disease ◽  
End Stage ◽  
Reversible Encephalopathy

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological syndrome that is characterized by clinical features including headache, altered mental status, cortical blindness, seizures, and other focal neurological signs as well as subcortical edema without infarction on neuroimaging. Under the umbrella of hypertensive encephalopathy, PRES is defined by reversible cerebral edema due to dysfunction of the cerebrovascular blood-brain barrier unit. The pathophysiology of PRES is thought to result from abnormalities in the transmembrane flow of intravascular fluid and proteins caused by two phenomena: one, cerebral autoregulatory failure and two, loss of integrity of the blood-brain barrier. PRES is not a common disease in patients with human immunodeficiency virus (HIV) and AIDS with only three previously reported cases. Both the HIV and end-stage renal disease appear to further compromise the blood brain barrier. Although uncommon, PRES recurrence has been described. To the best of our knowledge, this is the first report demonstrating recurrent PRES in a HIV patient on hemodialysis for end-stage renal disease.

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