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

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, a...

Full description

Bibliographic Details
Main Authors: V. E. Kitaeva, A. S. Kotov
Format: Article
Language:Russian
Published: MONIKI 2020-09-01
Series:Alʹmanah Kliničeskoj Mediciny
Subjects:
Online Access:https://www.almclinmed.ru/jour/article/view/1332
_version_ 1818595375798288384
author V. E. Kitaeva
A. S. Kotov
author_facet V. E. Kitaeva
A. S. Kotov
author_sort V. E. Kitaeva
collection DOAJ
description 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.
first_indexed 2024-12-16T11:15:01Z
format Article
id doaj.art-5ad9d214507e4f50acda25729fb98c69
institution Directory Open Access Journal
issn 2072-0505
2587-9294
language Russian
last_indexed 2024-12-16T11:15:01Z
publishDate 2020-09-01
publisher MONIKI
record_format Article
series Alʹmanah Kliničeskoj Mediciny
spelling doaj.art-5ad9d214507e4f50acda25729fb98c692022-12-21T22:33:37ZrusMONIKIAlʹmanah Kliničeskoj Mediciny2072-05052587-92942020-09-01480323610.18786/2072-0505-2020-48-033733Acute necrotizing encephalopathy of childhood. Diagnostic and treatment challenges in COVID-19 pandemicV. E. Kitaeva0A. S. Kotov1A.I. Yevdokimov Moscow State University of Medicine and DentistryMoscow Regional Research and Clinical Institute (MONIKI)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.https://www.almclinmed.ru/jour/article/view/1332acute necrotizing encephalopathy of childhoodbilateral lesions of the thalamusstatus epilepticuselectroencephalographymagnetic resonance imagingcovid-19
spellingShingle V. E. Kitaeva
A. S. Kotov
Acute necrotizing encephalopathy of childhood. Diagnostic and treatment challenges in COVID-19 pandemic
Alʹmanah Kliničeskoj Mediciny
acute necrotizing encephalopathy of childhood
bilateral lesions of the thalamus
status epilepticus
electroencephalography
magnetic resonance imaging
covid-19
title Acute necrotizing encephalopathy of childhood. Diagnostic and treatment challenges in COVID-19 pandemic
title_full Acute necrotizing encephalopathy of childhood. Diagnostic and treatment challenges in COVID-19 pandemic
title_fullStr Acute necrotizing encephalopathy of childhood. Diagnostic and treatment challenges in COVID-19 pandemic
title_full_unstemmed Acute necrotizing encephalopathy of childhood. Diagnostic and treatment challenges in COVID-19 pandemic
title_short Acute necrotizing encephalopathy of childhood. Diagnostic and treatment challenges in COVID-19 pandemic
title_sort acute necrotizing encephalopathy of childhood diagnostic and treatment challenges in covid 19 pandemic
topic acute necrotizing encephalopathy of childhood
bilateral lesions of the thalamus
status epilepticus
electroencephalography
magnetic resonance imaging
covid-19
url https://www.almclinmed.ru/jour/article/view/1332
work_keys_str_mv AT vekitaeva acutenecrotizingencephalopathyofchildhooddiagnosticandtreatmentchallengesincovid19pandemic
AT askotov acutenecrotizingencephalopathyofchildhooddiagnosticandtreatmentchallengesincovid19pandemic