Posterior reversible encephalopathy syndrome in a known case of sickle-beta-thalassemia: a case presentation

Abstract

Abstract Background Posterior reversible encephalopathy syndrome is a reversible condition, which occurs in response to acute changes in blood pressure due to failure of posterior circulatory autoregulation. Posterior reversible encephalopathy syndrome in sickle cell patients has been rarely reported previously in the setting of severe crisis or uncontrolled hypertension. Case presentation We report a very rare case of posterior reversible encephalopathy syndrome that occurred in a 10-year-old male child with sickle-beta-thalassemia which was detected at 4 years of age and required 2 units of packed red blood cells transfusion in last 6 years. At the time of the presentation, he was hypertensive with blood pressure 140/100 mm of Hg (> 99th percentile) and had focal seizures with magnetic resonance imaging findings suggestive of posterior reversible encephalopathy syndrome. Patient was treated with anticonvulsant and antihypertensives and had complete recovery of symptoms. Conclusions Posterior reversible encephalopathy syndrome is a rare neurological presentation in a sickle-beta thalassemia; this case guides in differentiating it from other common neurological manifestations in sickel-beta thalassemia and emphasis on early management as it is completely reversible.