Posterior reversible encephalopathy syndrome in pediatric acute leukemia: Case series and literature review

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state coupled with a unique radio imaging appearance. We describe this rare, mostly reversible condition in five cases undergoing similar treatment under preset protocol (MCP-841) for acute lymphoblastic leukemia (ALL) at our centre. Hypertension is a well-known adverse effect of high-dose corticosteroid therapy primarily mediated by its effects on the mineralocorticoid receptor especially in pediatric population and we hypothesize that this may be the etiology of PRES in two of these patients.

Posterior reversible encephalopathy syndrome after intrathecal methotrexate infusion: a case report and literature update

“…PRES reflects a cerebral vasogenic oedema and the main pathophysiology is that of neurotoxicity. It is speculated that PRES is due to a breakdown in the blood brain barrier leading to a leakage of fluid to the intermediate space of the cerebral parenchyma which in turn generates vasogenic oedema (18). Immunosuppressant drugs have also a cytotoxic effect on the vascular endothelium.…”

“…Whether vasospasm per se contributes to ischemia it remains unclear. Alterations in systemic blood pressure may affect the vessels of the brain, however sympathetic auto-regulatory mechanisms can control and reverse this effect (18,19).…”

“…The findings from the physical examination, in the majority of cases, are non-specific for the disease. With the exception of brisk deep tendon reflexes, particularly of the lower extremities, and possibly extensor plantar responses, the rest of the clinical examination is relatively unimpressive (18).…”

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

“…The spectrum of etiologic precipitants correlating with, or causative of and generating, PRES is quite broad in both adults and children (►Table 1). 7,10,20,34,47,50,54,69,70,116,[165][166][167][186][187][188][189] The impressive, varied, and myriad conditions and diseases in patients developing PRES have both enhanced and complicated our theories on the pathogenesis of the condition. In general, the most common precipitants giving rise to PRES in the adult and pediatric populations include hypertension, renal disease, solid organ and hematologic malignancy, 9,15,39,42,190 chemotherapy, [17][18][19][20][21][22][23][24][25][26] solid organ transplantation, 16,[35][36][37][191][192][193] and immunosuppressive therapy.…”

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

“…54 Stroke mimic not recognized as PRES leads to incorrect diagnostic pathway with potential for iatrogenic harm through improper administration of thrombolytic therapy. 66 When compared to the high income countries also, poverty and poor clinical settings in LMIC contributes to the diagnostic challenges and may contribute to limited research interest in pediatric PRES with consequent underreporting of PRES [67][68][69] Most of the reports of pediatric PRES have been from developed countries 2,4,[10][11][12][13][14][15][16][17][18][19][20][21] and a few from Africa. Nandi and colleagues 70 in South Africa were able to diagnose PRES in 4 children with novel aetiological associations: hypoxia following accidental strangulation, near-drowning episode, a child with thalassaemia receiving routine blood transfusions, and a fourth child who had PRES while recovering from toxic epidermal necrolysis syndrome (TENS).…”