Reversible posterior leukoencephalopathy syndrome in systemic lupus erythematosus

Reversible posterior leukoencephalopathy syndrome (RPLS) is an acute form of cerebrovascular injury that has been described recently in the setting of uncontrolled hypertension, puerperal eclampsia, or treatment with certain immunosuppressive drugs, including cyclosporine. It is reversible if treated promptly. Two patients with systemic lupus erythematosus (SLE), renal failure, and uncontrolled hypertension developed acute cerebrovascular symptoms; one had seizures and the other had headache and blurred vision. Both patients showed abnormal predominantly posterior lobe findings on neuroimaging films. The patients' symptoms and imaging abnormalities resolved completely with prompt correction of their hypertension and concomitant treatment with corticosteroids. RPLS should be recognized in SLE patients with uncontrolled hypertension and renal failure who present with headaches, seizures, cortical blindness, and other visual abnormalities. Prompt treatment with control of hypertension and withdrawal of precipitating drugs may be most important and can prevent permanent neurologic damage.