Is reversible posterior leukoencephalopathy with severe hypertension completely reversible in all patients?

Abstract

Leukoencephalopathy with severe hypertension is a recently described entity in nephrology, with only a few case reports to date in children. We prospectively studied 18 children with severe hypertension to evaluate the clinical features, severity, reversibility, and prognosis. All were subjected to clinical and biochemical tests, magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA). Headache was reported in 16 children, 13 had confusion and drowsiness, 12 had nausea and vomiting, and 9 had visual disturbances, seizure, and dyspnea. Only 2 had focal neurological deficit (1 with right facial palsy and another with right lateral rectus palsy). Of these 18 children, 14 patients had hypertensive retinopathy and 4 had normal fundus. MRI revealed leukoencephalopathic changes in 16 of 18 patients. These changes were bilateral occipito-parietal in 9 patients, diffuse white/gray matter lesion in 2, brain stem hyperintensity in 2, and hemorrhagic lesion in 3. On MRA, 11 of 18 patients had attenuation of cerebral arteries of different degree. On follow-up, MRI findings resolved in all except 3 patients and all patients had normal MRA, except for 1 with persistent minimal attenuation and another with spasm in all vessels. We conclude that leukoencephalopathy with severe hypertension is reversible both clinically and radiologically in the majority of children after the control of hypertension. However, a few patients may have residual damage and may need psychometric analysis and follow-up for neurodevelopmental sequelae.

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.…”

“…[1][2][3]6,7,10,12,14 En conclusión, la EPR debe considerarse una manifestación aguda del LES. El uso de la resonancia magnética en sus diferentes secuencias permite hacer el diagnóstico y descartar otras patologías que engloban el LNPS.…”

Reversible Posterior Leukoencephalopathy Syndrome in a Young Adult Patient Receiving Peritoneal Dialysis

“…The clinical reversibility of this syndrome has been extensively described (1,2,(4)(5)(6)8). However, the functional vasogenic brain edema may convert to cytotoxic brain edema if proper intervention is not applied.…”

Studying the evolution and the magnetic resonance findings of reversible posterior leukoencephalopathy in children

“…Recently, several studies have been trying to predict the evolution of RPLS 8,11,12 . Increased signal inten- 720 Arq Neuropsiquiatr 2006;64(3-B)…”