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Keywords = acute necrotising encephalopathy

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1 pages, 117 KB  
Comment
Comment on Hoppe et al. Bilateral Symmetrical Brain MRI Findings in Acute Necrotising Encephalopathy Type 1. Children 2025, 12, 974
by Memik Teke
Children 2025, 12(9), 1182; https://doi.org/10.3390/children12091182 - 4 Sep 2025
Viewed by 385
Abstract
I read with great interest the recent case report by Alexander T. Hoppe et al. [...] Full article
(This article belongs to the Special Issue Genetic Rare Diseases in Children)
7 pages, 1733 KB  
Case Report
Bilateral Symmetrical Brain MRI Findings in Acute Necrotising Encephalopathy Type 1
by Alexander T. Hoppe, Twinkle Ghia, Richard Warne, Peter Shipman and Rahul Lakshmanan
Children 2025, 12(8), 974; https://doi.org/10.3390/children12080974 - 24 Jul 2025
Cited by 1 | Viewed by 1218
Abstract
Background: Acute necrotising encephalopathy (ANE) is a rare and severe type of encephalopathy with bilateral symmetrical brain lesions, often following a viral prodrome. ANE type 1 (ANE1) is a disease subtype with a predisposing mutation in the gene encoding RAN binding protein 2 [...] Read more.
Background: Acute necrotising encephalopathy (ANE) is a rare and severe type of encephalopathy with bilateral symmetrical brain lesions, often following a viral prodrome. ANE type 1 (ANE1) is a disease subtype with a predisposing mutation in the gene encoding RAN binding protein 2 (RANBP2). Methods: We report a case of a 3-year-old girl with clinical symptoms of ANE and brain MRI findings suggesting ANE1, which was subsequently confirmed by genetic analysis. Results: MRI of the brain demonstrated symmetrical high T2/FLAIR signal changes in the lateral geniculate bodies, claustrum, ventromedial thalami, subthalamic nuclei, mamillary bodies, and brainstem, with partly corresponding diffusion restriction, as well as additional haemorrhagic changes in the lateral geniculate bodies on susceptibility weighted imaging. Genetic analysis revealed a heterozygous pathogenic variant of the RANBP2 gene. With immunosuppressive and supportive treatment, the patient fully recovered and was discharged after 10 days in the hospital with no residual symptoms. Conclusions: Recognition of the characteristic MRI findings in ANE1 can facilitate a timely diagnosis and enhance the clinical management of the patient and their relatives, especially given the high risk of disease recurrence. Full article
(This article belongs to the Special Issue Genetic Rare Diseases in Children)
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