Unilateral posterior reversible encephalopathy syndrome characterized with a long and gradually exacerbating course over 3 years and that presented propofol infusion syndrome - A case report

Posterior reversible encephalopathy syndrome (PRES) is characterized by acute neurological symptoms and vasogenic edema, and most patients wholly recover. We report a unilateral PRES patient characterized by a gradual onset followed by propofol infusion syndrome (PRIS) due to general anesthesia therapy. A 32-year-old woman had ovarian dysfunction treated by Kaufmann's treatment for 17 years. Three years ago, she developed seizures, and photophobia and myoclonus sometimes occurred. This time, she had strong photophobia and nausea for 3 months and then developed tonic-clonic seizures for 3 min. Her blood pressure and laboratory test on admission were all within normal limits. She presented no neurological deficits at admission, but the T2-weighted image (T2WI) showed a high-intensity area (HIA), and arterial spin labeling (ASL) image described cerebral blood flow (CBF) increase in the left parieto-occipital region. We diagnosed PRES and started anticonvulsants, antihypertensive, and steroid pulse therapy. However, her aphasia and neuroimaging findings worsened, so we started general anesthesia treatment with propofol on day 29. On day 32, she suddenly developed multiple organ dysfunctions due to PRIS. After intensive care with other sedatives over 2 months, the systemic status and neurological symptoms gradually improved almost as before the onset. On day 90, HIA in the T2WI in the lesion became small, and CBF was severely downregulated in the ASL image. Unilateral PRES's pathophysiology and the association with the female hormone remain unknown. Some patients undergo gradual onset and long-term courses, and we should care for PRIS during PRES treatment.