Paediatric posterior reversible encephalopathy syndrome: a review of aetiologies, clinical and radiological spectrum, pathophysiology, diagnostic criteria and management

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a rare clinico-radiological syndrome postulated to be due to disturbed cerebral autoregulation characterized by acute onset seizures, altered sensorium, headache, visual disturbances, and typical neuroimaging findings of vasogenic oedema in parietooccipital white matter. Although originally described in adults, PRES is increasingly recognized in children, particularly in association with renal disease, hypertension, autoimmune disorders, malignancy, chemotherapy, transplantation, infections, and immune-mediated conditions. Paediatric PRES differs from adult PRES in having a higher frequency of atypical imaging findings involving brainstem, spinal cord, basal ganglia; and broader etiological diversity. Advances in neuroimaging, molecular biology, and endothelial dysfunction research have improved understanding of disease mechanisms. However, diagnostic criteria remain largely clinico-radiological. This review summarizes current evidence regarding diagnosis, typical and atypical radiological findings, aetiologies, tertiary-care experiences, pathophysiological mechanisms, emerging genetic associations, and an algorithmic approach to management.