Posterior reversible encephalopathy syndrome secondary to asymptomatic poststreptococcal glomerulonephritis in a child with sickle cell anemia: a case report

Abstract

BackgroundPosterior reversible encephalopathy syndrome is a neurotoxic condition that occurs as a result of the failure of posterior circulatory autoregulation in response to acute changes in blood pressure. Overperfusion with resultant disruption of the blood-brain barrier results in vasogenic edema, but not infarction. Posterior reversible encephalopathy syndrome can be the presenting feature of postinfectious glomerulonephritis, which has been reported in approximately 5% of hospitalized children, and it has been reported in very few cases of adult patients with sickle cell anemia. We report a very rare case of posterior reversible encephalopathy syndrome that occurred in a child with sickle cell anemia. This presentation should be differentiated from other neurologic manifestations that occur in patients with sickle cell anemia, because management is totally different.Case presentationWe report what is to our knowledge the first reported case of a 9-year-old Saudi girl with sickle cell anemia who developed posterior reversible encephalopathy syndrome secondary to asymptomatic poststreptococcal glomerulonephritis. This occurred after full recovery from acute chest syndrome and severe vaso-occlusive crisis.ConclusionsThe purpose of this report is to emphasize that all efforts should be made to explore the causes of different neurologic manifestations that occur in patients with sickle cell anemia, because this will require different pathways of management.

Challenges of Diagnosing Pediatric Posterior Reversible Encephalopathy Syndrome in Resource Poor Settings: A Narrative Review

“…Recurrent episodes of PRES in same individuals have been reported 30 , 43 especially in patients undergoing dialysis. 44 , 45 It is therefore imperative to closely monitor blood pressure in such patients and avoid further precipitants.…”

“…In the pediatric patient, PRES evolves over a few hours but can persist for several days and can be mistaken for a psychosis, drug intoxication, or psychogenic states. 30 The CNS is a major organ system affected in PRES. Hypertension is also a common presentation pattern in pediatric PRES and it is the most common identifiable trigger.…”

“…Notable co-morbidities in children linked to PRES thus include acute kidney injury/chronic kidney disease, autonomic disturbances such as Guillain-Barré syndrome, thrombotic thrombocytopenic purpura, systemic lupus erythematosus (SLE), use of illicit drugs like cocaine, acetaminophen-induced hepatorenal failure, exposure to immunosuppressive drugs such as cyclosporine, tacrolimus, or chemotherapeutic agents like tacrolimus, and interferon alfa. 37,38,[39][40][41][42] Recurrent episodes of PRES in same individuals have been reported 30,43 especially in patients undergoing dialysis. 44,45 It is therefore imperative to closely monitor blood pressure in such patients and avoid further precipitants.…”

Etiologies, Cerebral Vasomotion, and Endothelial Dysfunction in the Pathophysiology of Posterior Reversible Encephalopathy Syndrome in Pediatric Patients

“…[260][261][262][263][264][265][266][267][268] In patients with sickle cell disease, missense mutations of the sixth codon of the hemoglobin β chain generate replacement of the acidic amino acid glutamate with the neutral amino acid valine, 208,[276][277][278] generating differential hemoglobin electrophoresis migration patterns and pathologic protein conformations augmenting the propensity of hemoglobin to polymerize under conditions of acidosis, hypoxia, and dehydration and thus effectively amplifying red blood cell microvascular aggregability and increases of the blood viscosity. [260][261][262][263][264][265][266][267][268] These events accelerate the rate of formation of thrombotic red blood cell aggregates within the cerebral microvasculature and generate decelerations of microvascular cerebral blood flow velocity. [279][280][281][282][283] Risk factors augmenting the propensity to a patient suffering from sickle cell disease to develop PRES include renal disease, hypertension, blood transfusions, HSCT, and direct perturbations of the microvasculature consequent to hemoglobin polymerization generating nondeformable red blood cells obstructing the cerebral capillaries.…”

“…HSCT generates an exquisitely risk-factor modifiable prevalence of PRES, varying from approximately 5% to a third of patients. 209,222,[256][257][258][259] Accordingly, the risk of developing PRES in patients undergoing HSCT is significantly increased in those patients with hemoglobinopathy, 22,202,209,[260][261][262][263][264][265][266][267][268] graft versus host disease, 232 and hypertension. 232 Thavamani and Umapathi 32 reported a significantly lower overall prevalence of developing PRES in pediatric patients receiving HSCT of 0.5%.…”