Neuropediatrics 2006; 37 - P124
DOI: 10.1055/s-2006-974135

Posterior Reversible Encephalopathy Syndrome (PRES) in children – a single-institution retrosprective study

S Vlaho 1, V Boda 1, M Baz Bartels 1, S Parbel 1, S Dittrich 1, T Lehrnbecher 2, L Porto 3, M Kieslich 1
  • 1Johann Wolfgang Goethe Universität, Pädiatrische Neurologie, Frankfurt am Main, Germany
  • 2Johann Wolfgang Goethe Universität, Pädiatrische Hämatologie Onkologie, Frankfurt am Main, Germany
  • 3Johann Wolfgang Goethe Universität, Neuroradiologie, Frankfurt am Main, Germany

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiologic phenomenon that is associated with a variety of medical conditions, including renal disease, cancer and cyclosporinA neurotoxicity. Affected patients typically experience seizures, headache, altered mental status, visual problems with concomitant radiographic abnormalities observed predominantly within the posterior cerebrum. This study analyse pediatric patients with PRES in order to indentify likely predisponsing factors, describe the common clinical and radiographic features, and report the clinical outcome.

Methods: We report a single-institution retrosprective study of 12 pediatric patients (5m/7f) who were treated at Goethe University Childrens hospital between 2002–06 and were identified with PRES.

Results: Mean age of PRES onset was 9 y (r:5–18y) The primary diagnoses were ALL (6 patients [pat.]), Glioblastoma, Neuroblastoma (2 pat.) Wilms tu. 1 pat, Fanconi Anemia 1 pat., EVANS syndrome 1 pat. and Fibrodysplasia ossificans progressiva 1 pat.. Hypertension was identified before onset of PRES in 2 pat.. All pat. experienced seizures. 8 pat. visual disturbance (all reversible). 4 pat. had altered mental status before. All pat. got acute EEG studies with patholog. findings. All pat. required AED acute therapy, 3 pat. still need AED therapie after clinical normalisation of PRES. The initial and follow up radiologic findings are described. The significant technique for radiol. detection is not only the T2-wighted MRI but also the diffusion-wighted MRI helping to distinguish vasogemic from cytotoxic edema.

Conclusions: Better clinical recognition and improved radiographic technique leads to an apparent increase of diagnosed pat. with PRES. MRI and EEG abnormalities do not appear to negatively affect clinical outcome. After clinical and radiological recovery we detect pat. with epilepsy. The so far typical characterism of reversible condition is not true in all pat. with PRES. Unlike previous studies our study found patients with PRES to have irreversible sequale.