ReviewMultiple sclerosis and related disorders2020
Genetic Acute Necrotizing Encephalopathy Associated with RANBP2: Clinical and Therapeutic Implications in Pediatrics.
Review in Multiple sclerosis and related disorders, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 2 registered trials, which are not on this map. Cited by 51 papers, 2 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Clinical and Radiological Prognostic Factors of Acute Necrotizing Encephalopathy in Sohag University Hospital
Role of the Nuclear Pore Component RANBP2 in Inflammatory Responses to Viral Infections
Who cites it
51 citing papers in PubMed, 2 syntheses or guidelines pooled it, 82 citations in OpenAlex.
- Initiation of tocilizumab within 24 hours in acute necrotizing encephalopathy is related to a good outcome: a systematic review.Journal of neurology · 2025Pooled it
- Clinical and genetic characteristics of RANBP2 mutations in children with acute necrotizing encephalopathy.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Pooled it
- Infection-triggered encephalopathy syndromes: a meta-analysis of clinical characteristics and outcomes in 1946 cases.EClinicalMedicine · 2026Article
- Neurological findings may be initial presenting symptom in children with inborn errors of immunity.Acta neurologica Belgica · 2026Article
- Early laboratory-based differentiation of severe influenza pneumonia and influenza-associated encephalopathy in children using a multi-model, stability-aware machine-learning workflow: a single-center retrospective cohort study.BMC infectious diseases · 2026Article
- The genetic driver of Acute Necrotizing Encephalopathy, RANBP2, regulates the inflammatory response to Influenza A virus infection.Nature communications · 2026Article
- Fatal hemorrhagic leukoencephalitis following VZV reactivation in an immunocompetent host: a case report.Journal of neurology · 2025Article
- Observational
- Acute haemorrhagic necrotizing encephalopathy and inflammatory demyelinating encephalopathy associated with COVID-19 in adults in Southern China.BMC infectious diseases · 2025Article
- Bilateral Symmetrical Brain MRI Findings in Acute Necrotising Encephalopathy Type 1.Children (Basel, Switzerland) · 2025Article
- The Genetic Landscape of Acute Necrotizing Encephalopathy: Insights Into the Possible Pathogenesis.Journal of clinical neurology (Seoul, Korea) · 2025Article
- International consensus definitions for infection-triggered encephalopathy syndromes.Developmental medicine and child neurology · 2025Article
- Advances in the Study of Acute Necrotizing Encephalopathy.Infection and drug resistance · 2025Review
- Case Report: long-term clinical outcomes inFrontiers in pharmacology · 2025Article
- Acute necrotizing encephalopathy caused by bacterial infection.BMC infectious diseases · 2024Article
- Highly beneficial outcome in severe acute necrotizing encephalopathy with tocilizumab treatment.Journal of neurology · 2024Article
- Nup358 restricts ER-mitochondria connectivity by modulating mTORC2/Akt/GSK3β signalling.EMBO reports · 2024Article
- Acute necrotizing encephalopathy in adult patients with influenza: a case report and review of the literature.BMC infectious diseases · 2024Review
- A rare and devastating etiology of febrile seizure.Annals of the Child Neurology Society · 2024Article
- Association of RAN and RANBP2 Gene Polymorphisms With Glioma Susceptibility in Chinese Children.Cancer reports (Hoboken, N.J.) · 2024Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Genetic (also known as familial) acute necrotizing encephalopathy (ANE1) is a rare disease presenting with encephalopathy often following preceding viral febrile illness in patients with a genetic predisposition resulting from a missense mutation in the gene encoding RAN Binding Protein 2 (RANBP2). The acute episode is characterized by deterioration in consciousness, often with focal neurologic deficits and seizures. Additionally, symmetric multifocal brain lesions are seen in the bilateral thalami as well as other characteristic regions, involving both gray and white matter. Prognosis is variable, with a high mortality rate and most surviving patients having persistent neurologic deficits. Early treatment with high dose steroids is associated with a more favorable outcome, however the diagnosis is often overlooked resulting in delayed treatment. The RANBP2 mutation associated with ANE1 causes an incompletely penetrant predisposition to encephalopathy in the setting of febrile illness through a mechanism that remains elusive. There are several non-mutually exclusive hypotheses suggesting possible etiologies for this phenotype based on the many functions of RANBP2 within the cell. These include dysfunctions in nucleocytoplasmic trafficking and intracellular metabolic regulation, as well as cytokine storm, and abnormal distribution of mitochondria. This narrative review explores these key concepts of the RANBP2 mutation and its clinical and therapeutic implications in pediatric populations.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.