title: Life-threatening complications of posterior reversible encephalopathy syndrome in children.
abstract: BACKGROUND
  Although the posterior reversible encephalopathy syndrome (PRES) is considered to have a benign clinical outcome, the presentation of PRES can be associated with life-threatening complications such as severe cerebral hemorrhage, cerebellar herniation and refractory status epilepticus (SE). The aim of this paper is to report incidence, clinical features and outcome of life-threatening complications related to PRES in children.
  
  
  METHODS
  Patients who suffered from life-threatening complications were retrospectively identified from a group composed by 27 consecutive children diagnosed with PRES in our hospital between 2000 and 2012. The clinical, radiological and EEG features and the outcome of these patients were evaluated and compared to the characteristics of patients with no complications.
  
  
  RESULTS
  Five patients (18%) presented life-threatening complications: 2 cerebral hemorrhages with mass effect and midline shift (1 massive intraparenchymal hemorrhage and 1 subdural hemorrhage and intraparenchymal hemorrhage), 2 transforaminal cerebellar herniations and 1 refractory SE. Two children died because of complications and 2 children required urgent neurosurgical intervention. The infratentorial involvement at onset of PRES and the observation of focal neurological deficits other than visual disturbances were significantly more frequent in children with life-threatening complications (p < 0.01).
  
  
  CONCLUSIONS
  PRES is associated with a non-negligible incidence of life-threatening complications. A careful clinical, neuroradiological and EEG monitoring is necessary in order to improve the outcome especially in the case of focal neurological deficits and infratentorial involvement.
authors.affiliation: Child Neurology Unit, University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy. Electronic address: ducciomaria.cordelli@aosp.bo.it.
authors.forename: Duccio M
authors.initials: DM
authors.lastname: Cordelli
authors.affiliation: Pediatric Oncology and Hematology Unit "Lalla Seràgnoli", University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy.
authors.forename: Riccardo
authors.initials: R
authors.lastname: Masetti
authors.affiliation: Child Neurology Unit, University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy.
authors.forename: Emilia
authors.initials: E
authors.lastname: Ricci
authors.affiliation: Neuroradiology Department, IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Toni
authors.affiliation: Pediatric Oncology and Hematology Unit "Lalla Seràgnoli", University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy.
authors.forename: Daniele
authors.initials: D
authors.lastname: Zama
authors.affiliation: Neuroradiology Department, IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna, Italy.
authors.forename: Monica
authors.initials: M
authors.lastname: Maffei
authors.affiliation: Pediatric Intensive Care Unit, University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Gentili
authors.affiliation: Child Neurology Unit, University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy.
authors.forename: Antonia
authors.initials: A
authors.lastname: Parmeggiani
authors.affiliation: Pediatric Oncology and Hematology Unit "Lalla Seràgnoli", University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Pession
authors.affiliation: Child Neurology Unit, University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy.
authors.forename: Emilio
authors.initials: E
authors.lastname: Franzoni
mesh_terms: D000293:Adolescent; D002543:Cerebral Hemorrhage / Q000175:diagnosis / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004677:Encephalocele / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D054038:Posterior Leukoencephalopathy Syndrome / Q000150:complications*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013226:Status Epilepticus / Q000175:diagnosis / Q000209:etiology*; D014057:Tomography, X-Ray Computed
keywords: Cerebellar herniation; Hypertensive encephalopathy; Intracerebral hemorrhage; Life-threatening complications; PRES; Status epilepticus