Successful re-initiation of calcineurin inhibitors in patients with posterior reversible encephalopathy syndrome (PRES)

Abstract

18014 Background: Posterior Reversible Encephalopathy Syndrome (PRES) is characterized by headache, altered mental status, seizure or visual loss and is associated with white matter vasogenic edema predominantly affecting the posterior occipital and parietal lobes of the brain. It often occurs after abrupt increases in blood pressure in the background of calcineurin inhibitor therapy. Methods: Retrospective study of 10 patients (pts) diagnosed with PRES while receiving cyclosporine (CSA). Results: Six children, (mean age 10.7 y; range 8–14 y), and 4 adults (mean age 28 y, range 20–35 y) were diagnosed with PRES. Seven had severe aplastic anemia as a primary diagnosis while one pt each had MDS, AML- M4 and Pre B-ALL. Seven had undergone an allogeneic stem cell transplant and all 10 pts were on CSA when PRES occurred. Besides CSA use, potential inciting risk factors for PRES include: concomitant infections (7pts), grade 3–4 GVHD (4 pts), use of ATG (5 pts), high dose steroids (5 pts), meropenem (4 pts) and hypomagnesemia (5 pts). Eight pts experienced seizures and 5 had visual disturbances. The average Mean Arterial Pressure (MAP) when PRES was diagnosed (132 mmHg; range 108–169) was significantly higher than baseline (mean rise 36.94 mmHg; range 12–85). MRI findings of increased T2 fluid attenuated inversion recovery signal predominantly involved the posterior brain regions (8 pts), but atypical findings of global involvement with frontal preponderance were seen in 2 pts. Nine pts required anti-epileptic drugs (AED) acutely; the 8 pts who seized continued AED (single agent levetiracetam in 7/8 pts). Continuous infusional antihypertensives were required for acute BP management in 4 pts; 7 pts required maintenance antihypertensives. CSA was never discontinued in 2 pts and restarted in all pts (median 2 days; range 0–79 days, from diagnosis of PRES to reinitiating CSA) without recurrence of PRES. Conclusions: The “typical” characteristics of specific posterior and white matter involvement is not always observed in PRES. Single agent levetiracetam can effectively prevent recurrent PRES-associated seizures. Prompt BP management and seizure prophylaxis can safely allow for continuing or promptly restarting calcineurin inhibitors critical to pt management. No significant financial relationships to disclose.