Atypical Reversible Posterior Leukoencephalopathy Syndrome in Thrombotic Thrombocytopenic Purpura

Abstract

A 32-year-old man with an atypical form of reversible leukoencephalopathy syndrome (RPLS) caused by thrombotic thrombocytopenic purpura (TTP) is reported. In this particular case, a timely diagnosis of TTP was established primarily on the clinical findings, which led to the early initiation of plasmapheresis and resulted in excellent clinical recovery. The pathophysiological aspects of the relationship between TTP and RPLS are discussed in light of the clinical and radiological features (including diffusion-and perfusion-weighted magnetic resonance imaging studies) of this case. The mechanism for TTP-associated, or TTP-induced, leukoencephalopathy is suggested to be independent of hypertension and vasoconstriction. TTP-associated endothelial injury can play a major role as the inciting mechanism for the development of RPLS.