Posterior Reversible Encephalopathy Syndrome (PRES) in Adult Multi-Visceral Transplant patients- Cambridge University Hospital experience

Abstract

Introduction: Neurological dysfunction after intestinal or multivisceral transplant (MVT) can arise due to drug toxicity, delerium due to sepsis, renal failure, stroke or central nervous system (CNS) infection. Less well known is posterior reversible encephalopathy syndrome (PRES).[1] We report here cases of PRES at a UK adult intestinal transplant centre. Methods: Data is collected prospectively on a database which was interrogated retrospectively to identify cases. Following this, case notes and radiology reports were reviewed. Results: We report three cases of PRES in our MVT cohort. Two females and one male with a median age of 42 years old. All patients were diagnosed by consistent imaging findings on magnestic resonance imaging (MRI) and exclusion of other causes. The time to diagnosis of PRES post transplant was 44 days, 9 days and 7 days (delayed in the first case as the initial MRI was normal but a further scan 7 days later showed consistent features of PRES). Patients presented with headache and confusion, partial status epilepticus and headache and visual hallucinations, resectively. All patients responded to withdrawal of tacrolimus and all were successfully rechallenged with tacrolimus at a later date. Two patients required temporary anti-hypertensive treatment. Interestingly, all patients had pre-transplant encephalopathy (2 cases of hepatic encephalopathy and one case of D-lactic encephalopathy). Conclusion: PRES was first Described by Hinchey et al in 1996.[2] There are variety of presenting symptoms including headache, visual disturbace, confusion, and hemianopia. The diagnosis of this condition in the setting of MVT can be challenging, characteristic features are best seen on T2-weighted FLAIR MRI sequences. The mechanism of PRES is unknown, however, there are proposed theories including cerebral autoregulation failure and hypoperfusion, immunologic theory through T cell/Endothelial cell interaction and circulating cytotoxic hypothesis.[3] PRES is uncommon but the prognosis of this reversable neurologic condition is good if recognised in a timley manner. The diagnosis of PRES can be challenging. Our data would suggest that there should be a particularly high level of suspicion in patients who were encephalopathic pre-transplant. Complete recovery with immunosuppression switching is possible and once there has been complete neurologica recovery, tacrolimus can be successfully reintroduced.