Reversible posterior leucoencephalopathy syndrome in systemic lupus and vasculitis

Abstract

OBJECTIVES—Reversible posterior leucoencephalopathy syndrome (RPLS) may develop in patients with renal insufficiency, hypertension, and immunosuppression, and is managed by prompt antihypertensive and anticonvulsant treatment. Four patients with renal insufficiency and fluid overload associated with Wegener's granulomatosis (one patient) and systemic lupus erythematosus (SLE) (three patients) are described, whose clinical picture and neuroimaging indicated RPLS.
CASE REPORTS—All patients had headache, seizures, visual abnormalities, and transient motor deficit, and were hypertensive at the onset of the symptoms. Head computed tomography (CT) scan and magnetic resonance imaging showed predominantly posterior signal abnormalities, which were more conspicuous on T₂ weighted spin echo images than on CT scan. All patients had some form of cytotoxic treatment shortly before the syndrome developed, and dramatically 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 cerebral oedema without concomitant infarction. The treatment of hypertension and neurotoxic condition such as uraemia appears of primary importance, while immunosuppressive treatment may cause further damage of the blood-brain barrier.



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Figure 1  .

Brain MR scan of patient 2 in the acute stage of the disease: T₂ weighted images show widespread white matter signal abnormalities, which particularly involve frontal, occipital (A) and parietal (B) lobes.

Figure 2  .

Follow up brain MR scan of patient 2, showing the disappearance of the white matter signal abnormalities.