scholarly journals Acute Necrotizing Encephalopathy in an Adult

2015 ◽  
Vol 5 ◽  
pp. 20 ◽  
Author(s):  
Ramakrishna Narra ◽  
Adinarayana Mandapalli ◽  
Suseel Kumar Kamaraju
Keyword(s):  
Poor Prognosis ◽  
Viral Infections ◽  
Mortality Rates ◽  
Magnetic Resonance Imaging Examination ◽  
High Morbidity ◽  
Resonance Imaging ◽  
Neurologic Disorder ◽  
Acute Necrotizing Encephalopathy ◽  
Healthy Infants ◽  
Acute Necrotizing

Acute necrotizing encephalopathy (ANE) is a rapidly progressing neurologic disorder that occurs in children after common viral infections of the respiratory or gastrointestinal systems. This disease is commonly seen in East Asia. Normal healthy infants and children can get affected. The condition carries a poor prognosis with high morbidity and mortality rates. We report here a case of a 23-year-old female with ANE and describe its neuroimaging findings. Magnetic resonance imaging examination performed showed symmetric lesions involving the thalami, brainstem, and cerebellum.

scholarly journals RanBP2/Nup358 enhances miRNA activity by sumoylating and stabilizing Argonaute 1

2019 ◽  
Author(s):  
Qingtang Shen ◽  
Yifan E. Wang ◽  
Mathew Truong ◽  
Kohila Mahadevan ◽  
Jing Ze Wu ◽  
...  
Keyword(s):  
Cytokine Production ◽  
Viral Infections ◽  
Nuclear Pore ◽  
Missense Mutations ◽  
Acute Necrotizing Encephalopathy ◽  
Messenger Ribonucleoprotein ◽  
Cytoplasmic Filaments ◽  
Acute Necrotizing ◽  
Main Components ◽  
Ultimate Fate

SUMMARYRanBP2/Nup358 is one of the main components of the cytoplasmic filaments of the nuclear pore complex. It has been speculated that RanBP2, which has an E3 SUMO-ligase domain, may alter the composition of messenger ribonucleoprotein (mRNP) complexes as they emerge from the nuclear pore and thus regulate the ultimate fate of the mRNA in the cytoplasm. Four separate missense mutations in RanBP2 cause Acute Necrotizing Encephalopathy 1 (ANE1), which manifests as a sharp rise in cytokine production after common viral infections such as influenza and parainfluenza. However, how RanBP2 and its ANE1-associated mutations affect cytokine production is not well understood. Here we report that RanBP2 represses the translation of the interleukin-6 (IL6) mRNA, which encodes a cytokine that is aberrantly up-regulated in ANE1. Our data indicates that soon after its production, the IL6 mRNP recruits the RNA-induced silencing complex (RISC) bound to Let7 miRNA. After this mRNP is exported, RanBP2 sumoylates the RISC-component AGO1, thereby stabilizing it and enforcing mRNA silencing. Collectively, these results support a model whereby RanBP2 promotes an mRNP remodelling event that is critical for the miRNA-mediated suppression of clinically relevant mRNAs, such as IL6.

scholarly journals COVID-19 Associated Acute Necrotizing Encephalopathy in a Hospitalized Cohort in Oregon

2020 ◽  
Author(s):  
Holly Vanni ◽  
Austin D Jou ◽  
Christine E Choo ◽  
Eric C. Walter ◽  
Richard A. Mularksi
Keyword(s):  
Observational Studies ◽  
Viral Infections ◽  
Case Reports ◽  
Health Maintenance Organization ◽  
Time Frame ◽  
Health Maintenance ◽  
Acute Necrotizing Encephalopathy ◽  
Acute Necrotizing ◽  
The Central Nervous System ◽  
Novel Coronavirus

Abstract Background/Objective: Severe Acute Respiratory Syndrome Coronavirus-2 (SARSCoV-2) is a novel coronavirus strain that most commonly affects the respiratory system with observational studies and case reports suggesting this virus may target the central nervous system. To date there has been one case report of COVID-19 related acute necrotizing encephalopathy.Methods: Observational study of COVID-19 patients admitted to two hospitals of a large metropolitan health maintenance organization serving over 600,000 members using retrospective electronic and radiographic medical record evaluation across 4 months (March – June of 2020) after multiple cases of acute necrotizing encephalopathy were diagnosed.Results: During this time frame 216 patients were diagnosed with COVID-19, 106 (49%) required hospitalization, 21 (20%) required admission to the intensive care unit (ICU) and 18 (17%) required intubation. Of the 18, 4 (22%) had clinicoradiologic evidence of acute necrotizing encephalopathy (ANE) diagnosed, two with associated areas of hemorrhage.Conclusions: Acute necrotizing encephalopathy (ANE) with or without hemorrhage, is a rare CNS disease, usually seen in childhood as a complication of viral infections. We identified a high percentage of COVID-19 patients with clinicoradiologic evidence of acute necrotizing encephalopathy in our cohort. To our knowledge, ANE has not been reported in patients with SARS or MERS. Understanding the pathogenesis, neurotropism and effects of the SARS-CoV-2 virus is important in developing treatments and improving morbidity and mortality.

Brain magnetic resonance imaging of an adult case of acute necrotizing encephalopathy

2007 ◽  
Vol 254 (8) ◽  
pp. 1135-1137 ◽  
Author(s):  
Haruhisa Kato ◽  
Hideo Hasegawa ◽  
Mutsumi Iijima ◽  
Masanobu Uchigata ◽  
Taizo Terada ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Brain Magnetic Resonance Imaging ◽  
Resonance Imaging ◽  
Adult Case ◽  
Acute Necrotizing Encephalopathy ◽  
Acute Necrotizing

scholarly journals Case Report of RANBP2 Mutation and Familial Acute Necrotizing Encephalopathy

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Mohamad Paktinat ◽  
Kamran Hessami ◽  
Soroor Inaloo ◽  
Hamid Nemati ◽  
Pegah Katibeh ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Late Presentation ◽  
Resonance Imaging ◽  
Case Presentation ◽  
Delay In Diagnosis ◽  
Acute Necrotizing Encephalopathy ◽  
High Clinical Suspicion ◽  
Level Of Consciousness ◽  
Acute Necrotizing ◽  
Magnetic Resonance Imaging Mri

Introduction. Acute necrotizing encephalopathy (ANE), a rare entity with unique clinical presentation, can be associated significant morbidity and mortality. The majority of ANE reported cases are sporadic. However, reports of extremely rare familial cases are scarce. Case Presentation. We described three cases, two siblings and their cousin, affected by ANE, all of them exhibiting RAN-binding protein 2 (RANBP2) gene mutation. They all presented with seizure and decreased level of consciousness. Unlike the siblings, the cousin eventually expired mainly due to the delay in diagnosis, resulting from late presentation of typical brain involvements of ANE in magnetic resonance imaging (MRI). Conclusion. The presented cases are the first reports of familial ANE in Iran. Attempt was made to raise awareness on this disease, because high clinical suspicion plays an important role in the early diagnosis and proper management of these patients.

scholarly journals COVID-19 Associated Acute Necrotizing Encephalopathy in a Hospitalized Cohort in Oregon

2020 ◽  
Author(s):  
Holly Vanni ◽  
Austin D Jou ◽  
Christine E Choo ◽  
Eric C. Walter ◽  
Richard A. Mularksi
Keyword(s):  
Observational Studies ◽  
Viral Infections ◽  
Case Reports ◽  
Health Maintenance Organization ◽  
Time Frame ◽  
Health Maintenance ◽  
Acute Necrotizing Encephalopathy ◽  
Acute Necrotizing ◽  
The Central Nervous System ◽  
Novel Coronavirus

Abstract Background/Objective: Severe Acute Respiratory Syndrome Coronavirus-2 (SARSCoV-2) is a novel coronavirus strain that most commonly affects the respiratory system with observational studies and case reports suggesting this virus may target the central nervous system. To date there has been one case report of COVID-19 related acute necrotizing encephalopathy.Methods: Observational study of COVID-19 patients admitted to two hospitals of a large metropolitan health maintenance organization serving over 600,000 members using retrospective electronic and radiographic medical record evaluation across 4 months (March – June of 2020) after multiple cases of acute necrotizing encephalopathy were diagnosed.Results: During this time frame 216 patients were diagnosed with COVID-19, 106 (49%) required hospitalization, 21 (20%) required admission to the intensive care unit (ICU) and 18 (17%) required intubation. Of the 18, 4 (22%) had clinicoradiologic evidence of acute necrotizing encephalopathy (ANE) diagnosed, two with associated areas of hemorrhage.Conclusions: Acute necrotizing encephalopathy (ANE) with or without hemorrhage, is a rare CNS disease, usually seen in childhood as a complication of viral infections. We identified a high percentage of COVID-19 patients with clinicoradiologic evidence of acute necrotizing encephalopathy in our cohort. To our knowledge, ANE has not been reported in patients with SARS or MERS. Understanding the pathogenesis, neurotropism and effects of the SARS-CoV-2 virus is important in developing treatments and improving morbidity and mortality.

scholarly journals Extensive cerebellar involvement and cognitive impairment in COVID-19-associated acute necrotizing encephalopathy

2021 ◽  
Vol 14 ◽  
pp. 175628642098517
Author(s):  
Dumitru Ciolac ◽  
Igor Crivorucica ◽  
Eremei Zota ◽  
Nadejda Gorincioi ◽  
Daniela Efremova ◽  
...  
Keyword(s):  
Young Male ◽  
Neurological Complications ◽  
Distinct Pattern ◽  
Imaging Findings ◽  
Resonance Imaging ◽  
Behavioral Impairment ◽  
Acute Necrotizing Encephalopathy ◽  
Acute Necrotizing ◽  
Cerebellar Involvement ◽  
Extensive Involvement

Neurological complications of the newly appeared severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are increasingly recognized. Here, we report a case of a young male presenting with a clinical and neuroimaging scenario of an acute necrotizing encephalopathy related to the coronavirus disease 2019 (COVID-19). This case is notable by its distinct pattern of magnetic resonance imaging findings of an extensive involvement of the cerebellum, and emergence of cognitive and behavioral impairment.

Presentation and Outcome of Acute Necrotizing Encephalopathy of Childhood: A 10-Year Single-Center Retrospective Study From Hong Kong

2020 ◽  
Vol 35 (10) ◽  
pp. 674-680 ◽  
Author(s):  
Chit Kwong Chow ◽  
Che Kwan Louis Ma
Keyword(s):  
Clinical Laboratory ◽  
Resonance Imaging ◽  
Factors Affecting ◽  
Acute Necrotizing Encephalopathy ◽  
Acute Necrotizing ◽  
Alanine Aminotransferase Level ◽  
Prognostic Outcome ◽  
Potential Factors ◽  
Magnetic Resonance Imaging Mri ◽  
The Brain

Acute necrotizing encephalopathy (ANE) is a rare disease in childhood. We reviewed the 10-year data from a local pediatric department, reported the clinical characteristics, laboratory tests, neuroimaging findings, and outcome of the acute necrotizing encephalopathy cases and identified the potential factors affecting the outcome. Eight episodes of acute necrotizing encephalopathy among 7 patients were recorded, in which all of them had an initial presentation of fever and seizure. We identified that acute necrotizing encephalopathy patients with a severe score of Glasgow Coma Scale (GCS) on presentation, brainstem involvement in magnetic resonance imaging (MRI) of the brain, and higher MR imaging scores were associated with worse outcome. Association of outcome with acute necrotizing encephalopathy severity score, platelet count, and serum alanine aminotransferase level did not reach a statistically significant level. These results highlight the importance of combined clinical, laboratory, and neuroimaging findings in determining the prognostic outcome of acute necrotizing encephalopathy patients.

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