Posterior reversible encephalopathy syndrome: A neuropsychiatric manifestation of systemic lupus erythematosus

Posterior Reversible Encephalopathy Syndrome (PRES) is an acute neurological syndrome clinically characterized by seizures, altered mental status, headache, and visual disturbances. It is caused by a variety of abnormalities in the endothelial function that ultimately result in vasogenic edema in the circulation of the central nervous system. This is reflected by the neuroimaging findings, that most often show reversible parieto-occipital edema. An important proportion of patients with PRES present with Systemic Lupus Erythematosus (SLE), and its complications, as their sole risk factors. This review describes the relationship between these two clinical entities and explains the pathophysiological models that have been proposed to describe the development of PRES. We explain how SLE can cause alterations in every pathway implicated in the development of PRES. Given the relatively high frequency and the distinct clinical course, PRES in the setting of SLE might be best described as a distinct neuropsychiatric syndrome associated with SLE.

Introduction

Posterior reversible encephalopathy syndrome (PRES) is a heterogeneous neurological syndrome characterized by reversible subcortical vasogenic edema, predominantly of the posterior brain (predominantly parietal and occipital lobes). Clinical manifestations include seizures, encephalopathy, headache, and visual disturbances (including cortical blindness). PRES has been attributed to many etiologies, including systemic lupus erythematosus (SLE). Throughout this review, we discuss PRES on its own and in the setting of SLE, and give our rationale as to why it should be considered as part of the neuropsychiatric systemic lupus erythematosus (NPSLE) syndromes.