Ignacio Delgado MD, Presenter: Nothing to Disclose

Chae Bong Whang, Abstract Co-Author: Nothing to Disclose

Angel Sanchez-Montanez, Abstract Co-Author: Nothing to Disclose

Miquel Raspall, Abstract Co-Author: Nothing to Disclose

Alfons Macaya, Abstract Co-Author: Nothing to Disclose

Elida Vazquez MD, Abstract Co-Author: Nothing to Disclose

Posterior reversible encephalopathy syndrome (PRES) consists of  vasogenic edema in the posterior circulation territories. Although usually considered benign and reversible, characteristics of this syndrome in pediatric patients remain obscure.The purpose of this study is to evaluate which clinical and magnetic resonance imaging (MRI) findings can help to predict the prognosis of PRES and whether or not there is difference between PRES in children and adults.

We retrospectively evaluated MRI and clinical findings from children with PRES between 2007 and 2013. All patients were studied by MRI. Follow-up MRI was performed on most of the children. We assessed the MRI features, clinical data, and the patients' outcomes. 

A total of 20 pediatric patients with PRES were included into this study. Mean age was 7.7 years and 55% were males. Recurrence of PRES was observed in one patient. Most common predisposing causes were renal and hemato-oncologic diseases, frequently associated with endotheliotoxic medication (cyclosporine A 10/20, tacrolimus 5/20, mycophenolate 3/20, corticoids 3/20). Presenting symptoms were seizures in 85%, altered mental status in 65% , visual disturbance 25% and headache in 15% . Arterial hypertension was present in 16/20 of patients. The parieto-occipital regions were the most commonly involved in MRI (19/20), followed by the frontal and temporal lobes. 9/20 showed restriction of diffusion (DWI) on initial MRI, 2 patients showed hemorrhagic changes. Follow-up MRI was performed on 14 patients and in 6/14 residual lesions were found. Six patients developed epilepsy, two patients ataxia, one patient had a persistent mydriasis, 1 hypotonia and 1 learning disability. Six of the patients with sequelae had hemorrhage or DWI lesions on initial MRI.

PRES should be recognized and trigger agents must be discontinued to prevent long-term sequelae. Recurrence is infrequent. PRES has not a so good long-term prognosis in children. Epilepsy is the most frequent sequela. DWI restriction is a marker of non reversible edema and indicates poor prognosis. Presence of hemorrhagic changes on initial MRI is also linked with a worst patient’s outcome.

Chemotherapy, immunosupressors and other endotheliotoxic medication play and important role in the pathophysiology of pediatric PRES. MRI based on DWI and GRE T2 or SWI may help to predict the prognosis.

Delgado, I, Whang, C, Sanchez-Montanez, A, Raspall, M, Macaya, A, Vazquez, E, Posterior Reversible Encephalopathy Syndrome in Children: Is It Really So Reversible?.  Radiological Society of North America 2014 Scientific Assembly and Annual Meeting, - ,Chicago IL. http://archive.rsna.org/2014/14045474.html