This 10-year-old female has a history of systemic lupus erythematosus and has suddenly developed right-side blindness. She is dealing with an undiagnosed clinical syndrome, as well as a complication. Identifying both the underlying syndrome and its complication will be critical to the treatment plan.
Answer: She is dealing with an intracranial hemorrhage as a complication of posterior reversible encephalopathy syndrome (PRES). Always consider an underlying disease in young stroke patients, including brain tumor with intra-tumoral bleeding, vascular malformation, venous thrombosis, vascular dissection or septic emboli, etc. The presence of characteristic FLAIR/DWI hyperintensities in bilateral parieto-occipital cortex suggests PRES. However, in classic PRES the DWI is normal, near normal or has less parenchymal volumetric involvement than seen on FLAIR. PRES is due to loss of vascular autoregulatory function and is associated with hypertension and conditions linked to hypertension. Some drugs are linked to PRES, the classic being cyclosporine.
Note: This case was shared by Dr. Chih-Chun Wu, who was recently selected as a scholarship winner for ProScan’s NYC Neuro Case Review. Congratulations to Dr. Wu.