Multifactorial Posterior Reversible Encephalopathy Syndrome in Children: Clinical, Laboratory, and Neuroimaging Findings

The aim of this study was to investigate the association of neuroimaging, clinical, and laboratory findings in children with different underlying diseases who developed posterior reversible encephalopathy syndrome (PRES). All consecutive pediatric patients referred to our institute between 2000 and 2017 were retrospectively evaluated for newly diagnosed PRES. Clinical data, medical history, and therapies administered at the time of PRES and laboratory findings were reviewed as the magnetic resonance imaging (MRI) obtained at PRES presentation and during follow-up. Statistics included Mann-Whitney U, Chi-square, and Fisher's exact tests. A total of 39 pediatric patients (25 males, median age = 8.8 years) with a confirmed diagnosis of PRES were included. The patients were divided into four groups based on the underlying disease: (1) patients with hemato-oncological diseases not transplanted (n = 15, 38.5%), (2) who underwent hematopoietic stem cell transplantation (HSCT; n = 12, 30.8%), (3) affected by renal (n = 8, 20.5%), and (4) by autoimmune diseases (n = 4, 10.2%). Hemato-oncological patients and those undergoing HSCT presented a higher incidence of involvement of deep gray matter structures and hemorrhagic lesions when compared with patients with renal and autoimmune disorders (p = 0.036 and p = 0.036, respectively). No differences emerged among different groups of patients regarding presenting PRES symptoms, arterial blood pressure, laboratory findings, and extent of MRI abnormalities and these parameters did not show association with outcome. In conclusion, in pediatric patients with different diseases who develop PRES, involvement of deep gray matter structures and hemorrhagic lesions are more common in hemato-oncological patients and those undergoing HSCT. Clinical and radiologic outcome is favorable independently of clinical data and extent of MRI abnormalities.