Posterior reversible encephalopathy syndrome in children with kidney disease

Abstract

Posterior reversible encephalopathy syndrome (PRES) has been described as a neurological condition observed in a variety of clinical settings and is characterized by focal neurological deficits, seizures, headaches, altered mental status, and visual impairment, associated with transient typical lesions on neuroimaging, predominantly in the posterior part of the brain. The most common risk factors for PRES are hypertension, renal diseases, and the use of calcineurin inhibitors. The incidence of PRES in children with renal disorders varies between 4 and 9%, according to different reports. Vasogenic cerebral edema is considered the major pathophysiological mechanism of PRES. There are two main theories regarding the genesis of this edema: (1) hyperperfusion, due to autoregulatory failure of the cerebral vasculature, and (2) hypoperfusion, due to vasoconstriction of the cerebral arteries. In addition, PRES might also be the result of a systemic inflammatory state causing endothelial dysfunction. The management of PRES includes BP control, treatment of seizures, and removal of or reduction in calcineurin inhibitors. Intravenous administration of antihypertensive therapy is preferred, and various drugs have been used in this regard, including nicardipine, labetalol, sodium nitroprusside, and hydralazine. The prognosis of PRES is usually benign, except for rare cases with intracranial hemorrhage.

Focal segmental glomerulosclerosis in children complicated by posterior reversible encephalopathy syndrome

“…In such cases, PRES might occur as a result of a systemic inflammatory state causing endothelial dysfunction. 8 , 25 …”

“… 5 , 6 However, this incidence might be underestimated because some patients may develop PRES without seizures. 7 , 8 …”

Multifactorial Posterior Reversible Encephalopathy Syndrome in Children: Clinical, Laboratory, and Neuroimaging Findings

“…These data are in accordance with prior studies showing a high incidence of hypertension in affected children. [7][8][9][10] Of note, in three nephropathic patients, hypertension was the only trigger since no drugs were administered at the time of the event. Interestingly, since the cerebral blood flow autoregulation threshold is lower in children than in adults, the mean blood pressure at the onset of PRES symptoms is also usually lower.…”

“…In children, hypertensive crises, renal insufficiency, transplantation, and use of immunosuppressants or other medications represent the most common causes of PRES. [1][2][3][4][7][8][9][10][11][12] PRES is usually a reversible condition but long-term sequelae have been reported in the pediatric population, including persistent epilepsy or neurocognitive deficits. 13,14 Although several studies have evaluated different clinical and neuroradiological aspects of PRES, the vast majority have focused on single groups of children affected by specific diseases or predisposing factors (e.g., renal insufficiency, lymphoblastic leukemia, hematopoietic stem cell or solid organ transplantation, or much rarer conditions).…”

“…13,14 Although several studies have evaluated different clinical and neuroradiological aspects of PRES, the vast majority have focused on single groups of children affected by specific diseases or predisposing factors (e.g., renal insufficiency, lymphoblastic leukemia, hematopoietic stem cell or solid organ transplantation, or much rarer conditions). [7][8][9][10][11][12][13][14][15] The aim of this study was to analyze the association among neuroimaging, clinical, and laboratory findings in different pediatric patients affected by PRES.…”