Acute necrotizing encephalopathy of childhood. Diagnostic and treatment challenges in COVID-19 pandemic

Cover Page

Cite item

Abstract

Acute necrotizing encephalopathy of childhood (ANEC) can occur in previously healthy children during a respiratory infection with fever and can manifest by epileptic seizures. Magnetic resonance imaging (MRI) typically shows bilateral lesions of the brainstem, cerebellum, thalamuses, basal neclei, and hemispheral white matter. We describe three clinical cases with an initial diagnosis of ANEC. In the first case, a 12-year old patient developed headache, leg weakness and high blood pressure during treatment of hepatitis C virus infection with PEG-interferon alfa2b. Later on she had myoclonic seizures with subsequent epileptic status, tetraparesis, confusion, and hyperthermia. Her clinical chemistry parameters showed a non-significant increase in liver enzymes levels. Cerebrospinal fluid was remarkable for increased protein level. The patient's brain MRI showed typical for ANEC bilateral thalamic lesions. The second case manifested with myoclonic seizures and subsequent epileptic status in a 17-months' old patient with a respiratory infection (vomiting, rhinitis, fever, and hyperthermia). His brain MRI showed bilateral lesions in the brainstem (dorsal part of the pons), thalamus, subcortical nuclei, white matter of the cerebral hemispheres, as well as lesions in the left hippocampus. The patient had increased urine levels of malic, 2-hydroxyisovalerianic, 3-hydroxy-isovalerianic, N-acetylaspartic, 3-hydroxybutyric, and lactic acids and increased blood levels of alanine, glutamic acid, glycine, and ornithine. By the time of the study, no hereditary metabolic disease was identified. In the third case, a 3-year old patient with a respiratory infection with vomiting and fever developed left-sided hemiparesis after she had fallen out of bed. The brain MRI revealed acute ischemic damage. Her cerebrospinal fluid was remarkable for a decreased protein level. Currently, ANEC is a diagnosis of exclusion. In the third patient, ANEC was obviously misdiagnosed. It is necessary to clarify the diagnostic criteria for the syndrome and to develop a management protocol for patients with ANEC.

About the authors

V. E. Kitaeva

A.I. Yevdokimov Moscow State University of Medicine and Dentistry

Author for correspondence.
Email: varvara-kitaeva@mail.ru
ORCID iD: 0000-0002-9334-8246

Varvara E. Kitaeva - Fifth Year Student, Faculty of General Medicine.

20-1 Delegatskaya ul., Moscow, 127473, Tel.: +7 (906) 086 68 70

Russian Federation

A. S. Kotov

Moscow Regional Research and Clinical Institute (MONIKI)

Email: alexeykotov1980@gmail.com
ORCID iD: 0000-0003-2988-5706

Alexey S. Kotov - MD, PhD, Head of Department of Pediatric Neurology; Professor, Chair of Neurology, Postgraduate Training Faculty.

61/2 Shchepkina ul., Moscow, 129110

Russian Federation

References

  1. Mizuguchi M, Abe J, Mikkaichi K, Noma S, Yoshida K, Yamanaka T, Kamoshita S. Acute necrotising encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions. J Neurol Neurosurg Psychiatry. 1995;58(5):555-61. doi: 10.1136/jnnp.58.5.555.
  2. Mizuguchi M. Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain Dev. 1997;19(2):81-92. doi: 10.1016/s0387-7604(96)00063-0.
  3. Campistol J, Gassio R, Pineda M, Fernandez-Al-varez E. Acute necrotizing encephalopathy of childhood (infantile bilateral thalamic necrosis): two non-Japanese cases. Dev Med Child Neurol. 1998;40(11):771-4. doi: 10.1111/j.1469-8749.1998.tb12346x
  4. Ravid S, Topper L, Eviatar L. Acute necrotizing encephalopathy presenting as a basal ganglia syndrome. J Child Neurol. 2001;16(6):461-2. doi: 10.1177/088307380101600618.
  5. Kim KJ, Park ES, Chang HJ, Suh M, Rha D-W. Novel Influenza A (H1N1)-Associated Acute Necrotizing Encephalopathy: A Case Report. Ann Rehabil Med. 2013;37(2):286-90. doi: 10.5535/arm.2013.37.2.286.
  6. Ergul AB, Altug U, Aydin K, Guven AS, Altuner To-run Y. Acute necrotizing encephalopathy causing human bocavirus. Neuroradiol J. 2017;30(2): 164-7. doi: 10.1177/1971400916687586.
  7. Чучин МЮ. Острая некротическая энцефалопатия при вирусной инфекции. Детская больница. 2012;1(47):23-8. [Chuchin MYu.
  8. Горелик ЕЮ, Скрипченко НВ, Иванова МВ, Вишневецкая ЕМ, Глебовская ОИ, Попов ПА. Острая некротизирующая энцефалопатия -клинические наблюдения у детей раннего возраста. Инфекционные болезни. 2016;14(2):65-70. doi: 10.20953/1729-9225-2016-2-65-70.
  9. Denier C, Balu L, Husson B, Nasser G, Bur-glen L, Rodriguez D, Labauge P, Chevret L. Familial acute necrotizing encephalopathy due to mutation in the RANBP2 gene. J Neurol Sci. 2014;345(1-2):236-8. doi: 10.1016/j.jns.2014.07.025.
  10. Poyiadji N, Shahin G, Noujaim D, Stone M, Patel S, Griffith B. COVID-19-associated Acute Hemorrhagic Necrotizing Encephalopathy: Imaging Features. Radiology. 2020;296(2):E119-20. doi: 10.1148/radiol.2020201187.

Supplementary files

There are no supplementary files to display.


Copyright (c) 2020 Kitaeva V.E., Kotov A.S.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies