Reversible Posterior Leukoencephalopathy Syndrome Preceding End Stage Renal Failure as the Initial Presentation of Connective Tissue Disease

Abstract

Introduction: Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare clinico-radiological entity that has been infrequently reported in patients with connective tissue diseases, particularly in those with established renal involvement.
Case report: A 51 year old female presented with headache, confusion, transient loss of vision and seizures. She was found to have elevated blood pressure. Her level of consciousness deteriorated rapidly necessitating mechanical ventilation. Hematological and biochemical investigations were essentially normal. Brain CT scan revealed widespread subcortical white matter changes which were more prominent in the posterior parietal lobes, raising the possibility of RPLS. Vasculitic screen revealed elevated ANA, anti-Ro and anti-La antibody titers. Her blood pressure was controlled with medications and this was followed by improvement in her neurological condition. Two weeks later, she had a brain MRI scan which was essentially normal, confirming the diagnosis of RPLS. Her renal function, however, deteriorated rapidly during her hospital stay and a renal biopsy showed features consistent with the microangiopathic hemolytic uremic syndrome. She progressed to end stage renal failure and was maintained on regular dialysis. Eight months later she successfully received a kidney transplant.
Conclusion: RPLS can be the initial presentation in patients with connective tissue diseases, and this possibility should not be overlooked by the physician.

Keywords: connective tissue disease, renal failure, reversible posterior leukoencephalopathy, RPLES