Acute Necrotizing Encephalopathy of Childhood: A Multicenter Experience in Saudi Arabia
Background: Acute necrotizing encephalopathy of childhood (ANEC) is a rapidly progressing encephalopathy characterized by fever, depressed level of consciousness, and seizures. Diagnosis depends on clinical presentation and characteristic neuroimaging findings of abnormal signal intensity involving...
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Frontiers Media S.A.
2020-10-01
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Online Access: | https://www.frontiersin.org/article/10.3389/fped.2020.00526/full |
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DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Fahad A. Bashiri Sultan Al Johani Muddathir H. Hamad Amal Y. Kentab Ali H. Alwadei Khalid Hundallah Hamdi H. Hasan Walaa Alshuaibi Lamyaa Jad Muhammad Talal Alrifai Abrar Hudairi Rana Al Sheikh Asma'a Alenizi Nawaf A. Alharthi Tayseer A. Abdelmagid Duaa Ba-Armah Mustafa A. Salih Brahim Tabarki |
spellingShingle |
Fahad A. Bashiri Sultan Al Johani Muddathir H. Hamad Amal Y. Kentab Ali H. Alwadei Khalid Hundallah Hamdi H. Hasan Walaa Alshuaibi Lamyaa Jad Muhammad Talal Alrifai Abrar Hudairi Rana Al Sheikh Asma'a Alenizi Nawaf A. Alharthi Tayseer A. Abdelmagid Duaa Ba-Armah Mustafa A. Salih Brahim Tabarki Acute Necrotizing Encephalopathy of Childhood: A Multicenter Experience in Saudi Arabia Frontiers in Pediatrics necrotizing encephalopathy children seizure influenza virus RANBP2 gene |
author_facet |
Fahad A. Bashiri Sultan Al Johani Muddathir H. Hamad Amal Y. Kentab Ali H. Alwadei Khalid Hundallah Hamdi H. Hasan Walaa Alshuaibi Lamyaa Jad Muhammad Talal Alrifai Abrar Hudairi Rana Al Sheikh Asma'a Alenizi Nawaf A. Alharthi Tayseer A. Abdelmagid Duaa Ba-Armah Mustafa A. Salih Brahim Tabarki |
author_sort |
Fahad A. Bashiri |
title |
Acute Necrotizing Encephalopathy of Childhood: A Multicenter Experience in Saudi Arabia |
title_short |
Acute Necrotizing Encephalopathy of Childhood: A Multicenter Experience in Saudi Arabia |
title_full |
Acute Necrotizing Encephalopathy of Childhood: A Multicenter Experience in Saudi Arabia |
title_fullStr |
Acute Necrotizing Encephalopathy of Childhood: A Multicenter Experience in Saudi Arabia |
title_full_unstemmed |
Acute Necrotizing Encephalopathy of Childhood: A Multicenter Experience in Saudi Arabia |
title_sort |
acute necrotizing encephalopathy of childhood: a multicenter experience in saudi arabia |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Pediatrics |
issn |
2296-2360 |
publishDate |
2020-10-01 |
description |
Background: Acute necrotizing encephalopathy of childhood (ANEC) is a rapidly progressing encephalopathy characterized by fever, depressed level of consciousness, and seizures. Diagnosis depends on clinical presentation and characteristic neuroimaging findings of abnormal signal intensity involving the thalami as well as the supra and infra-tentorial areas. Treatment modalities are not well-established; empirical treatment with antibiotics and antiviral agents is the initial step, followed by steroids and immunoglobulin, as well as supportive care. Patients with ANEC have a variable prognosis, but mortality is very high.Methods: A retrospective chart review of patients diagnosed with ANEC in five tertiary centers from January 2015 to October 2018 was performed. Clinical and radiological findings, as well as the therapeutic approach and outcomes, were described.Results: Twelve children were included ranging in age from 10 months to 6 years. All patients presented with preceding febrile illness, altered level of consciousness, and seizure. Radiological features showed abnormal signals in the thalami, and five patients (41.7%) had brainstem involvement. All patients received empirical treatment with antibiotics and antiviral agents. Ten patients (83.3%) received intravenous immunoglobulin (IVIG) and IV Methylprednisolone therapy. Outcomes were variable ranging from good outcomes with minimal neurological deficits to poor outcomes and death in 25% of cases.Conclusion: ANEC is a rare fulminant disease in children. The treatment is challenging. Early interventions with the use of IVIG and IV Methylprednisolone may change the outcome; however, further studies are needed to establish a consensus guideline for the management. |
topic |
necrotizing encephalopathy children seizure influenza virus RANBP2 gene |
url |
https://www.frontiersin.org/article/10.3389/fped.2020.00526/full |
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doaj-f60dfa80ad47449f84dc3275ddfebaba2020-11-25T03:57:22ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602020-10-01810.3389/fped.2020.00526564934Acute Necrotizing Encephalopathy of Childhood: A Multicenter Experience in Saudi ArabiaFahad A. Bashiri0Sultan Al Johani1Muddathir H. Hamad2Amal Y. Kentab3Ali H. Alwadei4Khalid Hundallah5Hamdi H. Hasan6Walaa Alshuaibi7Lamyaa Jad8Muhammad Talal Alrifai9Abrar Hudairi10Rana Al Sheikh11Asma'a Alenizi12Nawaf A. Alharthi13Tayseer A. Abdelmagid14Duaa Ba-Armah15Mustafa A. Salih16Brahim Tabarki17Division of Pediatric Neurology, Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi ArabiaDivision of Pediatric Neurology, Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi ArabiaDivision of Pediatric Neurology, Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi ArabiaDivision of Pediatric Neurology, Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi ArabiaPediatric Neurology Department, National Neuroscience Institute, King Fahd Medical City, Riyadh, Saudi ArabiaDivision of Pediatric Neurology, Department of Pediatrics, Prince Sultan Military Medical City, Riyadh, Saudi ArabiaNeuroradiology Division, Department of Radiology, College of Medicine, King Saud University, Riyadh, Saudi ArabiaMedical Genetics Division, Department of Pediatrics, King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Saudi ArabiaPediatric Neurology Department, National Neuroscience Institute, King Fahd Medical City, Riyadh, Saudi ArabiaDivision of Neurology, Pediatric Department, King Abdullah Children Hospital, King Abdulaziz Medical City, Ministry of National Guard, Riyadh, Saudi ArabiaDivision of Pediatric Neurology, Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi ArabiaDivision of Pediatric Neurology, Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi ArabiaDivision of Pediatric Neurology, Department of Pediatrics, Prince Sultan Military Medical City, Riyadh, Saudi ArabiaPediatric Department, King Abdullah bin Abdulaziz University Hospital, College of Medicine, Princess Nourah Bint Abdulrahman University, Riyadh, Saudi ArabiaDepartment of Pediatrics, Armed Forces Hospital Southern Region, Khamis Mushayt, Saudi ArabiaDivision of Neurology, Pediatric Department, King Abdullah Children Hospital, King Abdulaziz Medical City, Ministry of National Guard, Riyadh, Saudi ArabiaDivision of Pediatric Neurology, Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi ArabiaDivision of Pediatric Neurology, Department of Pediatrics, Prince Sultan Military Medical City, Riyadh, Saudi ArabiaBackground: Acute necrotizing encephalopathy of childhood (ANEC) is a rapidly progressing encephalopathy characterized by fever, depressed level of consciousness, and seizures. Diagnosis depends on clinical presentation and characteristic neuroimaging findings of abnormal signal intensity involving the thalami as well as the supra and infra-tentorial areas. Treatment modalities are not well-established; empirical treatment with antibiotics and antiviral agents is the initial step, followed by steroids and immunoglobulin, as well as supportive care. Patients with ANEC have a variable prognosis, but mortality is very high.Methods: A retrospective chart review of patients diagnosed with ANEC in five tertiary centers from January 2015 to October 2018 was performed. Clinical and radiological findings, as well as the therapeutic approach and outcomes, were described.Results: Twelve children were included ranging in age from 10 months to 6 years. All patients presented with preceding febrile illness, altered level of consciousness, and seizure. Radiological features showed abnormal signals in the thalami, and five patients (41.7%) had brainstem involvement. All patients received empirical treatment with antibiotics and antiviral agents. Ten patients (83.3%) received intravenous immunoglobulin (IVIG) and IV Methylprednisolone therapy. Outcomes were variable ranging from good outcomes with minimal neurological deficits to poor outcomes and death in 25% of cases.Conclusion: ANEC is a rare fulminant disease in children. The treatment is challenging. Early interventions with the use of IVIG and IV Methylprednisolone may change the outcome; however, further studies are needed to establish a consensus guideline for the management.https://www.frontiersin.org/article/10.3389/fped.2020.00526/fullnecrotizing encephalopathychildrenseizureinfluenza virusRANBP2 gene |