Life-threatening complications of posterior reversible encephalopathy syndrome in children

• DOI: 10.1016/j.ejpn.2014.04.014
• Publication Date: 2014-04-25T14:17:28Z
• Authors: Duccio M. Cordelli; Riccardo Masetti; Emilia Ricci; Francesco Toni; Daniele Zama; Monica Maffei; Andrea Gentili; Antonia Parmeggiani; Andrea Pession; Emilio Franzoni
• Keywords: Male; Adolescent; Electroencephalography; PRES; Hypertensive encephalopathy; Intracerebral hemorrhage; Cerebellar herniation; Status epilepticus; Magnetic Resonance Imaging; Severity of Illness Index; 3. Good health; 03 medical and health sciences; Status Epilepticus; 0302 clinical medicine; Child, Preschool; Cerebellar herniation; Hypertensive encephalopathy; Intracerebral hemorrhage; Life-threatening complications; PRES; Status epilepticus; Adolescent; Cerebral Hemorrhage; Child; Child, Preschool; Electroencephalography; Encephalocele; Female; Humans; Magnetic Resonance Imaging; Male; Posterior Leukoencephalopathy Syndrome; Retrospective Studies; Severity of Illness Index; Status Epilepticus; Tomography, X-Ray Computed; Humans; Female; Posterior Leukoencephalopathy Syndrome; Child; Tomography, X-Ray Computed; Cerebral Hemorrhage; Encephalocele; Retrospective Studies

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.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.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).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.