Posterior reversible encephalopathy syndrome in children with kidney diseases

Abstract

Posterior reversible encephalopathy syndrome (PRES) was originally used to describe a reversible, predominantly posterior leukoencephalopathy in patients who had renal insufficiency, hypertension, or who received immunosuppressive therapy. Since PRES is prevalent in children with kidney diseases, awareness and understanding of it is important for practicing pediatric nephrologists. A comprehensive approach to the diagnosis of PRES includes thorough determination of predisposing factors, clinical symptoms, and mandatory appropriate imaging. Unfortunately, the pathophysiology of PRES is still obscure and specificity of radiological examination has not yet been established. Two major predisposing factors, namely hypertension and calcineurin inhibitors, are well recognized. In addition, nephrotic syndrome is a common underlying condition for development of PRES. Frequent symptoms include altered consciousness (coma, stupor, lethargy, confusion), seizure, headache, and visual disturbance. Most of these symptoms usually develop abruptly and resolve within a few weeks after proper management. Cranial magnetic resonance (MR) imaging is the first-line modality of imaging studies for detecting PRES. Diffusion-weighted imaging with quantification of apparent diffusion coefficient (ADC) values by ADC mapping may provide more accurate and specific images in the future.

Pediatric posterior reversible encephalopathy syndrome presenting with isolated cerebellar edema and obstructive hydrocephalus

“…It is thought to be due to diffuse vasogenic edema in white matter tracts, most often secondary to severe acute hypertension. 9 First characterized by Hinchey et al 4 in children, PRES has primarily been described in association with cancer chemotherapy, 10 primary renal disease, 5 and auto-immune disease. Occasionally PRES is severe enough to trigger ICU admission.…”

Posterior Reversible Encephalopathy Syndrome: A Noteworthy Syndrome in End-Stage Renal Disease Patients

“…In 70-80% of patients, PRES is accompanied by moderate-to-severe hypertension, while in another 20-30% the BP is normal or minimally elevated. However, PRES can occur without significant hypertension [7]. The symptoms may have a gradual or acute onset, are not specific, and can mimic a variety of neurological conditions, which should be excluded [3,14].…”

“…The syndrome has been observed in numerous clinical conditions such as nephrotic syndrome, acute poststreptoccocal glomerulonephritis, solid organ transplantation, bone marrow and stem cell transplantation, eclampsia, systemic lupus erythematosus, hemolytic uremic syndrome, and calcineurin inhibitors treatment [3,4,5]. The syndrome is well described in children [6], especially in those with kidney diseases [7]. Two cases of children on peritoneal dialysis (PD) presenting with PRES have also been described [8,9].…”

Posterior Reversible Encephalopathy Syndrome in Two Omani Children with Underlying Renal Diseases

“…According to the second theory, hypertension causes activation of the autoregulatory system, which results in vasoconstriction of the brain vessels with hypoperfusion, ischaemia and subsequent fluid leakage. 3,4 However, a large number of patients with PRES do not have hypertension. 3,4 Consequently, Marra et al recently described the endothelial hypothesis; this theory differs from those earlier as it asserts that hypertension does not necessarily have to be present for PRES to develop.…”

“…3,4 However, a large number of patients with PRES do not have hypertension. 3,4 Consequently, Marra et al recently described the endothelial hypothesis; this theory differs from those earlier as it asserts that hypertension does not necessarily have to be present for PRES to develop. 4 The hypothesis suggests that an immune-related cascade can cause PRES through the weakening of brain vessels, leading to fluid leakage and oedema.…”