Posterior Reversible Encephalopathy Syndrome (PRES) in an Anuric Patient With a WT1 Mutation and Chronic Renal Graft Failure: A Case Report.

Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological entity frequently observed in solid organ transplant recipients, where it is often attributed to the neurotoxicity of calcineurin inhibitors (CNIs). However, its occurrence in anuric patients who had bilateral nephrectomy and chronic kidney disease of the graft, independent of any immunosuppressive treatment, is very rare. This case highlights the crucial and isolated role of extreme fluid overload and hemodynamic instability in triggering PRES. A 23-year-old woman, carrying a known mutation of the Wilms tumor 1 (WT1) gene and having undergone bilateral nephrectomy in childhood followed by kidney transplantation, presented to the emergency department with altered mental status, generalized seizures, and severe hypertension. She was on intensive home hemodialysis due to chronic kidney disease of the graft. Brain magnetic resonance imaging (MRI) revealed bilateral and symmetrical T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities in the insular regions, temporal lobes, and cerebellar tonsils, consistent with vasogenic edema. After ruling out infectious, ischemic, and autoimmune etiologies, a diagnosis of PRES was made. Intensive blood pressure control and optimized fluid management resulted in rapid neurological recovery and near-complete resolution of the radiological lesions. In this case, significant volumetric and hemodynamic fluctuations alone can disrupt cerebral autoregulation and trigger PRES in anuric patients, even years after immunosuppressant discontinuation. Clinicians must remain vigilant for the possibility of PRES in dialysis patients with acute neurological symptoms, as rapid management of blood pressure and fluid balance is essential to ensure reversibility and prevent permanent neurological sequelae.