Presentation of reversible posterior leukoencephalopathy syndrome in patients on calcineurin inhibitors

Abstract

Background: Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinico-radiologic diagnosis associated with numerous medical conditions including hypertension, immunosuppressant medications, and eclampsia. It is characterized by headache, altered mental status, seizures, visual disturbance, and neuroimaging consistent with posterior-predominant vasogenic edema. The objective of this study was to characterize the clinical spectrum and outcomes in a large series of RPLS patients, and to compare the presentation of patients taking calcineurin inhibitors (CNIs) to that of other RPLS patients.

Methods: We reviewed records of patients seen by the neurology and transplant services over an 18-year period. Comorbid conditions, medications, blood pressure, laboratory testing, clinical outcomes, and radiographic findings were collected.

Results: 84 episodes of RPLS were identified in 79 patients. Etiologies included CNIs (43%), hypertension (29%), renal disease (12%), preeclampsia/eclampsia (7%), and chemotherapy (5%). Patients on CNIs had lower blood pressures (p=0.002) and a lower prevalence of headache (p=0.02) compared to RPLS patients with other etiologies. Clinical recovery occurred in 65% of episodes, and radiographic resolution occurred in 67%.

Conclusions: Patients with CNI-induced RPLS have lower blood pressure than other RPLS patients, but otherwise present similarly. RPLS typically occurs within days to weeks of CNI initiation in patients without elevated medication levels. Clinical and radiographic recovery occurred in the majority of patients in this series, but one-third suffered residual neurologic deficits or death. These findings highlight the importance of prompt recognition and treatment of RPLS triggers to prevent permanent sequelae.