Reversible posterior leukoencephalopathy in a patient with systemic sclerosis/systemic lupus erythematosus overlap syndrome

Reversible posterior leukoencephalopathy is a recently recognized neurological syndrome, first characterized 7 years ago [1]. In this article, we present such a case with images typical of the syndrome. This is followed by discussion of the typical presentation, its associations, hypothetical aetiology and treatment modalities. A 39-year-old woman with systemic sclerosis/systemic lupus erythematosus overlap syndrome was admitted with fever. This followed a second course of pulsed intravenous cyclophosphamide for relapse of lupus nephritis WHO class IV with crescents. Her past history included Raynaud’s phenomenon, pulmonary fibrosis, oesophageal dysmotility and membranous glomerulonephritis causing nephrotic syndrome 8 years previously. She had also had seizures in pregnancy associated with pre-eclamptic toxaemia. During the admission, her blood pressure rose to 170/100 mmHg. She developed cortical blindness followed by status epilepticus. A head computed tomography (CT) scan showed bilateral subcortical white matter changes in the posterior lobes, suggestive of posterior leukoencephalopathy. A magnetic resonance imaging (MRI) scan showed high T2 signal in the left cerebellar white matter and both occipital poles involving grey and white matter (Figure 1). A magnetic resonance venogram (MRV) was normal (Figure 2). Her blood pressure was controlled with intravenous nitrates and prostacyclin. Her symptoms resolved rapidly. A repeat MRI scan 2 weeks later showed resolution of some of the changes (Figure 3). A repeat renal biopsy showed crescent formation in two glomeruli with no evidence of malignant hypertension.