Isolated pons involvement in Posterior Reversible Encephalopathy Syndrome: Case report and review of the literature

Abstract

BackgroundPosterior Reversible Encephalopathy Syndrome (PRES) is a clinical-radiological syndrome, usually reversible and with a favorable prognosis, which recognizes a variety of etiologies and clinical patterns and is likely due to an impairment in cerebral blood flow autoregulation. It is typically characterized by subcortical, predominantly parieto-occipital, vasogenic brain oedema in patients with acute-subacute neurological symptoms. Infratentorial oedema on neuroimaging has been mostly described in association with the typical supratentorial pattern and seldom as isolated.Case reportWe report a case of PRES with isolated pons involvement on MRI. A woman affected by Turner syndrome, epilepsy, slight mental deficiency, obesity and hypothyroidism, experienced a progressive gait and standing impairment, worsening in the last 2 weeks. At admission blood pressure was 220/110 mmHg. Brain MRI showed a wide FLAIR signal hyperintensity on T2-weighted sequences affecting the entire pons, without contrast enhancement. Clonidine, doxazosine, furosemide and telmisartan were effective in restoring normal blood pressure. Pons hyperintensity completely resolved on MRI 3 weeks later, together with return to normal neurological examination.ConclusionsThough isolated infratentorial involvement in PRES recognizes several causes, hypertension, which is a common feature in Turner syndrome, would have played a key role in our case with solely pons MRI T2-hyperintensity.

Clinical and MRI Features of Posterior Reversible Encephalopathy Syndrome With Atypical Regions: A Descriptive Study With a Large Sample Size

“…Therefore, it is very important to study the clinical and MRI features of PRES with atypical regions to improve clinical management. However, to our knowledge, most of the previous studies of PRES with atypical regions have been case reports or small case series lacking a comprehensive summary with a large sample (4,17). Therefore, in this study, we investigate the clinical and MRI features of PRES with atypical regions in a large sample by retrospectively collecting data from patients in our hospital and from the patients reported in the literature by searching the PubMed, EMBASE, and Web of Science databases.…”

Isolated pontine involvement in posterior reversible encephalopathy syndrome with coincidental acute ischaemic stroke

“…The most commonly affected region is the parieto-occipital lobe followed by the frontal lobes, basal ganglia, cerebellum, brainstem and thalamus 7. Even though the central variant of PRES is widely described, infratentorial involvement and particularly diffuse involvement of the pons is rare 8. There are anecdotal case reports in the medical literature with isolated brainstem involvement in PRES and concomitant acute ischaemic cerebral infarctions 9…”

A Case Report: Isolated Pontine Lesion in Hypertensive of a Pregnant Patient with Idiopathic Thrombocytopenia

“…Posterior reversible encephalopathy syndrome (PRES) is a clinical condition characterised by headache, visual disturbances, seizures, and confusion. 1,2 The main problem lies in the impairment of cerebral blood flow autoregulation which, in turn, leads to endothelial dysfunction and vasogenic brain oedema. 1 PRES is often related to an acute increase in arterial blood pressure, is clinically indistinguishable from hypertensive encephalopathy, and has been associated with pre-eclampsia, renal failure, infections, immunosuppressive agent requirement, and hypercalcaemia.…”

“…1,2 The main problem lies in the impairment of cerebral blood flow autoregulation which, in turn, leads to endothelial dysfunction and vasogenic brain oedema. 1 PRES is often related to an acute increase in arterial blood pressure, is clinically indistinguishable from hypertensive encephalopathy, and has been associated with pre-eclampsia, renal failure, infections, immunosuppressive agent requirement, and hypercalcaemia. It presents with abnormal findings on CT and MRI indicating cerebral oedema in the occipital, temporal, and parietal lobes.…”