Posterior reversible encephalopathy syndrome in the pediatric renal population

Abstract

Posterior reversible leukoencephalopathy syndrome (PRES) clinically presents with seizures, severe headaches, and mental and visual changes. Our goal was to describe the clinical features, triggering factors, neuro-imaging findings, and electroencephalogram (EEG) findings in a pediatric cohort with renal disease. We retrospectively analyzed the records of 18 children with the diagnosis of PRES between January 2001 and June 2006 at the University of Miami/Holtz Children's Hospital, USA. There were 22 PRES episodes. The most common clinical presentation was generalized tonic-clonic seizures in 59% (13/22). The most common identified trigger of PRES was hypertensive crisis in 59% (13/22). Almost half of the children had no evidence of on-going uncontrolled hypertension; 44% (8/18) had normal funduscopic examination findings, and 50% (9/18) had no or mild left ventricular hypertrophy. Two of the 18 patients had recurrent PRES episodes, three episodes each. Diffuse slowing was the most common finding on the EEGs. Atypical magnetic resonance imaging (MRI) findings were more prevalent in the imaged cases (62% vs 25%, P < 0.05). All the computerized tomography (CT) scans were normal, despite the positive MRI findings in four cases when both types of imaging was used. All the episodes had total clinical resolution. In conclusion, despite the diverse initial trigger, acute hypertension seems to be the common pathogenic pathway for pediatric PRES. MRI seems superior to CT, with better sensitivity due to its high resolution and diffusion-weighted imaging. The lesions do not necessarily have to be in the posterior white matter and may not be totally reversible.

Posterior Reversible Encephalopathy Syndrome (PRES); in a 5 years old, dialysis dependent child with chronic kidney disease.

“…Apart from elevated blood pressure, PRES has been described with; chronic renal failure [2], Chemotherapy [3[, adrenocortical disease, organ transplantation, immunosuppressive drugs [4] and pre-eclampsia or eclampsia.…”

A case of acute post-streptococcal glomerulonephritis that developed posterior reversible encephalopathy syndrome

“…Especially sudden increases in the systemic blood pressure lead to exceeding of the autoregulatory capacity in the brain vessels (8). Consequently, vasoconstriction, subsequently vasodilatation, increase in vascular permeability, disruption in the brain-blood barrier, fluid transduction and petechial hemorrhages occur (6,9). The reason that the findings are mostly observed in the posterior hemispheres is the fact that sympathetic innervation which provides autoregulation in cases of increased blood pressure is found less in the posterior cerebral vessels (6).…”

Encefalopatía reversible posterior en una niña con lupus eritematoso sistémico. Presentación de un caso

“…5,10-13 Para dar cuenta de la rareza de la asociación LES-EPR y sobre todo en pediatría, una recopilación de 3855 pacientes con LES derivada de 4 casuísticas importantes 6-9 describen 22 casos pediátricos con LES-EPR y, teniendo como referencia solo las diferentes patologías renales pediátricas asociadas, en 2 casuísticas de 36 pacientes, 2 la presentaron. 11,12 En nuestro medio, se describieron 7 casos de un centro de referencia nacional, la mayoría con patología renal, de los cuales un paciente tuvo LES-EPR y afectación renal. 13 Nuestra paciente en el debut de un lupus activo desarrolló, en el lapso de 5 días, las manifestaciones típicas de la EPR luego de haber estado expuesta a una multiplicidad de factores patogénicos reconocidos (hipertensión, altas dosis de esteroides, infusión de gammaglobulina, transfusión de eritrocitos, retención hídrica, uremia, hipoalbuminemia, infección), fenómeno comunicado anteriormente en pacientes pediátricos.…”