Duccio Maria Cordelli1, Chiara Marra2, Lara Ciampoli3, Davide Barbon4, Francesco Toni5, Daniele Zama6, Lucio Giordano7, Giuseppe Milito8, Stefano Sartori9, Laura Sainati10, Thomas Foiadelli11, Tommaso Mina12, Lucia Fusco13, Marta Santarone14, Chiara Iurato15, Alessandro Orsini16, Giovanni Farello17, Alberto Verrotti18, Arianna Aceti19, Riccardo Masetti20. 1. Unit of Child Neurology and Psychiatry, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Italy. Electronic address: ducciomaria.cordelli@unibo.it. 2. Unit of Child Neurology and Psychiatry, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Italy. Electronic address: chiara.marra3@studio.unibo.it. 3. Unit of Child Neurology and Psychiatry, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Italy. Electronic address: lara.ciampoli@studio.unibo.it. 4. Unit of Child Neurology and Psychiatry, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Italy. Electronic address: davide.barbon@studio.unibo.it. 5. IRCCS Istituto Delle Scienze Neurologiche di Bologna, UOC Neuroradiologia, Bologna, Italy. Electronic address: francesco.toni@isnb.it. 6. Unit of Pediatric Oncology and Haematology "Lalla Seràgnoli", Department of Pediatrics, Sant'Orsola Hospital, University of Bologna, Bologna, Italy. Electronic address: daniele.zama@aosp.bo.it. 7. Child Neurology and Psychiatry Unit, Spedali Civili, Brescia, Italy. Electronic address: lucio.giordano@asst-spedalicivili.it. 8. Child Neurology and Psychiatry Unit, Spedali Civili, Brescia, Italy. Electronic address: gmilito78@gmail.com. 9. Pediatric Neurology and Neurophysiology Unit, Department of Women's and Children's Health, University Hospital of Padua, Italy; Neuroimmunology Group, Pediatric Research Institute "Città Della Speranza", Padova, Italy. Electronic address: stefano.sartori@unipd.it. 10. Clinic of Pediatric Hematology Oncology, Department of Woman's and Child Health, Azienda Ospedale Università di Padova, Padova, Italy. Electronic address: laura.sainati@unipd.it. 11. Pediatric Clinic, Fondazione IRCCS Policlinico San Matteo, Pavia, University of Pavia, Italy. Electronic address: t.foiadelli@smatteo.pv.it. 12. Oncoematologia Pediatrica, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy. Electronic address: t.mina@smatteo.pv.it. 13. Department of Neuroscience, Bambino Gesù Children's Hospital, Rome, Italy. Electronic address: lucia.fusco@opbg.net. 14. Department of Neuroscience, Bambino Gesù Children's Hospital, Rome, Italy. Electronic address: marta.santarone@opbg.net. 15. University of Pisa, Department of Clinical and Experimental Medicine, Paediatric Unit, Pisa, Italy. Electronic address: chiara.iurato@gmail.com. 16. Paediatric Neurology, Department of Paediatrics, University Hospital of Pisa, Pisa, Italy. Electronic address: aorsini.mg@gmail.com. 17. Department of Pediatrics, University of Perugia, Italy. Electronic address: giuseppe.farello@univaq.it. 18. Department of Pediatrics, University of Perugia, Italy. Electronic address: alberto.verrottidipianella@univaq.it. 19. Department of Medical and Surgical Sciences, Neonatal Intensive Care Unit, AOU Bologna, University of Bologna, Italy. Electronic address: arianna.aceti2@unibo.it. 20. Unit of Pediatric Oncology and Haematology "Lalla Seràgnoli", Department of Pediatrics, Sant'Orsola Hospital, University of Bologna, Bologna, Italy. Electronic address: riccardo.masetti5@unibo.it.
Abstract
AIM: The aim of this study was to describe the characteristics of Posterior Reversible Encephalopathy Syndrome (PRES) in infants and young children (<6 years) and to compare them with the older pediatric population affected by PRES. METHODS: we retrospectively reviewed records of 111 children (0-17 years) diagnosed with PRES from 2000 to 2018 in 6 referral pediatric hospitals in Italy. The clinical, radiological and EEG features, as well as intensive care unit (ICU) admission rate and outcome of children aged <6 years were compared to those of older children (6-17 years). Factors associated with ICU admission in the whole pediatric cohort with PRES were also evaluated. RESULTS: Twenty-nine patients younger than 6 years (26%) were enrolled with a median age at onset of PRES of 4 years (range: 6 months-5 years). Epileptic seizures were the most frequent presentation at the disease onset (27/29 patients). Status epilepticus (SE) was observed in 21/29 patients: in detail, 11 developed convulsive SE and 10 presented nonconvulsive SE (NCSE). SE was more frequent in children <6 years compared with older children (72% vs 45%) as well as NCSE (35% vs 10%). Seventeen children aged <6 years required ICU admission. Prevalence of ICU admissions was higher within younger population compared to older (59% vs 37%). In the whole study population SE was significantly associated with ICU admission (p = 0.001). CONCLUSIONS: PRES in children < 6 years differs from older children in clinical presentation suggesting a more severe presentation at younger age.
AIM: The aim of this study was to describe the characteristics of Posterior Reversible Encephalopathy Syndrome (PRES) in infants and young children (<6 years) and to compare them with the older pediatric population affected by PRES. METHODS: we retrospectively reviewed records of 111 children (0-17 years) diagnosed with PRES from 2000 to 2018 in 6 referral pediatric hospitals in Italy. The clinical, radiological and EEG features, as well as intensive care unit (ICU) admission rate and outcome of children aged <6 years were compared to those of older children (6-17 years). Factors associated with ICU admission in the whole pediatric cohort with PRES were also evaluated. RESULTS: Twenty-nine patients younger than 6 years (26%) were enrolled with a median age at onset of PRES of 4 years (range: 6 months-5 years). Epileptic seizures were the most frequent presentation at the disease onset (27/29 patients). Status epilepticus (SE) was observed in 21/29 patients: in detail, 11 developed convulsive SE and 10 presented nonconvulsive SE (NCSE). SE was more frequent in children <6 years compared with older children (72% vs 45%) as well as NCSE (35% vs 10%). Seventeen children aged <6 years required ICU admission. Prevalence of ICU admissions was higher within younger population compared to older (59% vs 37%). In the whole study population SE was significantly associated with ICU admission (p = 0.001). CONCLUSIONS: PRES in children < 6 years differs from older children in clinical presentation suggesting a more severe presentation at younger age.