Almanac of Clinical MedicineAlmanac of Clinical Medicine2072-05052587-9294Moscow Regional Research and Clinical Institute (MONIKI)133210.18786/2072-0505-2020-48-033Acute necrotizing encephalopathy of childhood. Diagnostic and treatment challenges in COVID-19 pandemicKitaevaV. E.<p>Varvara E. Kitaeva - Fifth Year Student, Faculty of General Medicine.</p><p>20-1 Delegatskaya ul., Moscow, 127473, Tel.: +7 (906) 086 68 70</p>varvara-kitaeva@mail.ruhttps://orcid.org/0000-0002-9334-8246KotovA. S.<p>Alexey S. Kotov - MD, PhD, Head of Department of Pediatric Neurology; Professor, Chair of Neurology, Postgraduate Training Faculty.</p><p>61/2 Shchepkina ul., Moscow, 129110</p>alexeykotov1980@gmail.comhttps://orcid.org/0000-0003-2988-5706A.I. Yevdokimov Moscow State University of Medicine and DentistryMoscow Regional Research and Clinical Institute (MONIKI)190920204832361408202014082020Copyright © 2020, Kitaeva V.E., Kotov A.S.2020<p>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.</p>acute necrotizing encephalopathy of childhoodbilateral lesions of the thalamusstatus epilepticuselectroencephalographymagnetic resonance imagingCOVID-19острая некротизирующая энцефалопатия детского возрастадвустороннее поражение таламусовэпилептический статусэлектроэнцефалографиямагнитно-резонансная томографияCOVID-19[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. 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