Duccio M Cordelli1, Riccardo Masetti2, Emilia Ricci3, Francesco Toni4, Daniele Zama2, Monica Maffei4, Andrea Gentili5, Antonia Parmeggiani3, Andrea Pession2, Emilio Franzoni3. 1. Child Neurology Unit, University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy. Electronic address: ducciomaria.cordelli@aosp.bo.it. 2. Pediatric Oncology and Hematology Unit "Lalla Seràgnoli", University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy. 3. Child Neurology Unit, University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy. 4. Neuroradiology Department, IRCCS Institute of Neurological Sciences, Bellaria Hospital, Bologna, Italy. 5. Pediatric Intensive Care Unit, University of Bologna, S. Orsola Malpighi Hospital, Via Massarenti 11, Bologna, Italy.
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.
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: Femke Molema; Monique Williams; Janneke Langendonk; Sarwa Darwish-Murad; Jacqueline van de Wetering; Ed Jacobs; Willem Onkenhout; Esther Brusse; Anke van der Eerden; Margreet Wagenmakers Journal: JIMD Rep Date: 2020-01-22
Authors: Abbas Alshami; Asseel Al-Bayati; Steven Douedi; Mohammad A Hossain; Swapnil Patel; Arif Asif Journal: BMC Neurol Date: 2021-03-09 Impact factor: 2.474