Reversible posterior leucoencephalopathy syndrome in systemic lupus and vasculitis

Objectives-Reversible posterior leucoencephalopathy syndrome (RPLS) may dev elop in patients with renal insufficiency, hypertension, and immunosuppress ion, and is managed by prompt antihypertensive and anticonvulsant treatment . Four patients with renal insufficiency and fluid overload associated with Wegener's granulomatosis tone patient) and systemic lupus erythematosus (S LE) (three patients) are described, whose clinical picture and neuroimaging indicated RPLS. Case reports-All. patients had headache, seizures, visual abnormalities, an d transient motor deficit, and were hypertensive at the onset of the sympto ms. Head computed tomography (CT) scan and magnetic resonance imaging showe d predominantly posterior signal abnormalities, which were more conspicuous on T-2 weighted spin echo images than on CT scan. All patients had some fo rm of cytotoxic treatment shortly before the syndrome developed, and dramat ically responded to blood pressure control and anticonvulsant treatment. In two patients with SLE, dialysis was required for renal insufficiency. Discussion-Follow up neuroimaging studies showed almost complete resolution of signal abnormalities, and suggested that RPLS was associated with cereb ral oedema without concomitant infarction. The treatment of hypertension an d neurotoxic condition such as uraemia appears of primary importance, while immunosuppressive treatment may cause further damage of the blood-brain ba rrier.