POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME (PRES) SECONDARY TO VASO-OCCLUSIVE CRISIS IN A SICKLE CELL ANEMIA PATIENT: A CASE REPORT

Abstract

Background: Posterior Reversible Encephalopathy Syndrome (PRES) is a clinico-radiological diagnosis, characterized by distinctive neuroimaging features and non-specific neurological symptoms including: visual disturbances, altered consciousness, headache and seizures. The neuroimaging alterations are reversible and predominantly posterior in the parieto-occipital region. PRES is classically suspected in patients with severe hypertension, renal failure, autoimmune disorders, eclampsia, or immunosuppressant medications. Also patients with sickle cell disease are exposed to different forms of brain insults as part of their disease process. So far, PRES has been reported in a few patients with sickle cell disease with some of these patients having recurrent episodes.Case: This case report presents a 35-years-old Sudanese male known case of Sickle Cell Disease presenting with a full presenting picture of PRES included sudden onset of headache, seizures, visual disturbances, and altered consciousness. While the patient had exhibited near-all clinical traits of SCD, it was the vaso-occlusive crisis -in form of priapism- that the most evident of symptoms and signs which had evolved to PRES.Discussion: Few hypotheses have been established regarding the pathophysiology of PRES. One believes that the sudden onset of hypertension causes breakdown in brain autoregulation especially in the occipital area. This leads to hyperperfusion and subsequent extravasation of proteins and fluids, forming a local vasogenic oedema. Another suggests that the endothelial dysfunction due to sepsis and eclampsia is the culprit mechanism of injury despite its association with ischemia and vasospasm. Patients with sickle cell disease are exposed to different forms of brain insults as part of their disease process, PRES has been reported in a few patients with sickle cell disease with some of these patients having recurrent episodes. We are reporting this unique case sickle cell disease and PRES to further highlight the coexistence of the two conditions.Conclusion: This study highlights the potential association between sickle cell disease and the development of PRES. As it is well explained as a result from the vaso-occlusive crisis that takes place in brain blood vessels which leads to hypo-perfusion of the brain and thus brain ischemia, also explained by the endothelial injury in PRES-associated conditions which may lead to vascular instability and vasoconstriction.