Reversible posterior encephalopathy syndrome in children with nephrotic syndrome

Aim To investigate the clinical features and prognoses of children who develop reversible posterior encephalopathy syndrome (RPES) during treatment for nephrotic syndrome (NS). Methods The clinicoradiological characteristics and prognoses of 51 patients with NS, including 21 with RPES and 30 without, were analyzed. Results Compared with the controls, the RPES patients exhibited a higher rate of tacrolimus (P = 0.01) and cyclosporine (P = 0.02) treatment; higher-dose prednisolone (P = 0.01) treatment; higher systolic blood pressure (P = 0.04), serum cholesterol (P = 0.03), and proteinuria (P < 0.01); and lower serum albumin levels (P = 0.03). Hypertension was present in 85.7% of RPES patients. The clinical manifestations of RPES included an altered mental status, seizures, headaches, nausea and vomiting, and visual impairment. Electroencephalography findings included slow waves and focal sharp or/and spiked waves; magnetic resonance imaging showed lesions localized in the occipital, parietal, frontal, temporal lobes and the cerebellum and brainstem; and magnetic resonance angiography revealed vertebral artery narrowing. All RPES patients recovered completely with timely and appropriate therapy. Conclusion Hypertension, calcineurin inhibitor and high-dose steroid treatments, high serum cholesterol and proteinuria levels, and low serum albumin levels can predispose children with NS to RPES, although both the clinical and imaging outcomes are satisfactory.