Decompressive Craniectomy for Posterior Reversible Encephalopathy Syndrome (PRES): Case Report

Amit Azriel,Micky Gidon,Avi Cohen,Vladimir Merkin,Alexandr Shtreizent,Israel Melamed,Yuval Sufaro,Ilan Shelef

doi:10.4236/crcm.2015.45034

Abstract

Background: Posterior reversible encephalopathy syndrome (PRES) is an uncommon clinical-neuroradiological syndrome with an unclear pathophysiology. Correlation between PRES and the use of immunosuppressant drugs have previously been described, as well as correlation between elevated blood pressure and PRES. Characteristic brain MRI manifestations include hyperintense lesions on T2 and FLAIR (fluid-attenuated inversion recovery) images. PRES is usually reversible within a short period of time after discontinuation of the presumably offending drug. Some cases of PRES might complicate with intracranial hemorrhage, refractory status epilepticus or expansive vasogenic brain edema (also regarded as “tumefactive” PRES). Methods: We present a case of a young man diagnosed with Hodgkin’s lymphoma, following a laparotomy due to cecal volvulus. The patient received glucocorticoids and elevated blood pressure values were recorded. Brain imaging studies were performed due to generalized epileptic seizures, demonstrating neuroradiological findings consistent with PRES. Neurological and neuroradiological deterioration was noted, necessitating urgent neurosurgical intervention. A complete neurological and functional rehabilitation was achieved. Conclusion: The uncommon cases of complicated PRES should be taken under consideration whenever clinical deterioration is noted following the diagnosis of PRES. Early neuroradiological evaluation should be sought, together with aggressive medical and surgical treatment in cases of life threatening mass effect.

Highlights

Posterior reversible encephalopathy syndrome (PRES) is an uncommon clinicalneuroradiological syndrome with an unclear pathophysiology
Acute volume overload by blood transfusion may exceed the capacity of autoregulation resulting in vasogenic edema and development of Posterior Reversible Encephalopathy Syndrome (PRES) [9]
Second laparotomy was performed following general and hemodynamic deterioration, requiring vasopressors support for a short period of time

Summary

Background

Reversible posterior leukoencephalopathy syndrome was first described in 1996 by Hinchey et al as a reversible clinical-neuroradilogical syndrome [1]. Casey et al proposed the name Posterior Reversible Encephalopathy Syndrome (PRES) in 2000 after demonstrating substantial cortical involvement on MRI FLAIR images, mainly in milder cases [2]. The endothelial damage alters the normal blood-brain barrier function, resulting in fluid leakage and vasogenic edema [4]. Acute volume overload by blood transfusion may exceed the capacity of autoregulation resulting in vasogenic edema and development of PRES [9]. The typical neuroraiological MRI manifestations of PRES include T2 and fluid-attenuated inversion recovery (FLAIR) hyperintense vasogenic edema involving cortical and/or subcortical white matter of the affected regions, almost always involving the parieto-occipital lobes [13]. Expansive vasogenic edema with intracranial mass effect might occur in less than 5% of cases, regarded as “tumefactive” PRES [12]. We report a case of a young man who diagnosed with Hodgkin’s lymphoma that developed tumefactive PRES and required lifesaving decompressive craniectomy

Case Report

Discussion

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Conclusion