Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan

1997 ◽  
Vol 19 (2) ◽  
pp. 81-92 ◽  
Author(s):  
Masashi Mizuguchi
Keyword(s):  
Acute Encephalopathy ◽  
Acute Necrotizing Encephalopathy ◽  
Acute Necrotizing

scholarly journals Only a Touch of the Flu? The Simultaneous Manifestation of Acute Necrotizing Encephalopathy in Two Consanguineous Patients

2015 ◽  
Vol 2 (2) ◽  
Author(s):  
C. Bloch ◽  
B. Suter ◽  
A. Fischmann ◽  
H. Gensicke ◽  
S. Rüegg ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Missense Mutation ◽  
Autosomal Dominant ◽  
Treatment Strategies ◽  
Influenza B ◽  
Acute Encephalopathy ◽  
Acute Necrotizing Encephalopathy ◽  
Acute Necrotizing

Abstract This case report describes the simultaneous manifestation of acute necrotizing encephalopathy in 2 consanguineous patients after infection with influenza B based on the autosomal dominant missense mutation of the RANBP2-gene. Differential diagnosis of acute encephalopathy, clinical and radiological clues, and treatment strategies are outlined.

scholarly journals The Clinical and Imaging Characteristics Associated With Neurological Sequelae of Pediatric Patients With Acute Necrotizing Encephalopathy

2021 ◽  
Vol 9 ◽  
Author(s):  
Hong-Min Zhu ◽  
Si-Min Zhang ◽  
Cong Yao ◽  
Meng-Qing Luo ◽  
Hui-Jing Ma ◽  
...  
Keyword(s):  
Steroid Therapy ◽  
Pediatric Patients ◽  
Brain Lesion ◽  
Acute Encephalopathy ◽  
Neurological Sequelae ◽  
Acute Necrotizing Encephalopathy ◽  
Lesion Location ◽  
Imaging Characteristics ◽  
Acute Necrotizing

Background: Acute necrotizing encephalopathy of childhood (ANE) is a rare but rapidly progressing encephalopathy. Importantly, the exact pathogenesis and evidence-based treatment is scarce. Thus, we aimed to identify the clinical, imaging, and therapeutic characteristics that associated with prognosis of pediatric ANE patients.Methods: A retrospective study was conducted on pediatric patients with ANE who were admitted to Wuhan Children's Hospital between January 2014 and September 2019. All cases met the diagnostic criteria for ANE proposed by Mizuguchi in 1997. The clinical information and follow-up data were collected. The prognostic factors were analyzed by trend chi-square test and Goodman–Kruskal gamma test.Results: A total of 41 ANE patients ranging in age from 8.9 to 142 months were included in this study. Seven cases (17%) died, and the other 34 survivors had different degrees of neurological sequelae. Factors tested to be significantly correlated with the severity of neurological sequelae were the intervals from prodromal infection to acute encephalopathy (G = −0.553), conscious disturbance (r = 0.58), endotracheal intubation (r = 0.423), elevation of alanine aminotransferase (r = 0.345), aspartate aminotransferase (r = 0.393), and cerebrospinal fluid protein (r = 0.490). In addition, dynamic magnetic resonance imaging (MRI) evaluation on follow-up revealed that the total numbers of brain lesion location (χ2 = 6.29, P < 0.05), hemorrhage (r = 0.580), cavitation (r = 0.410), and atrophy (r = 0.602) status were significantly correlated with the severity of neurological sequelae, while early steroid therapy (r = −0.127 and 0.212, respectively) and intravenous immunoglobulin (IVIG) (r = 0.111 and −0.023, respectively) within 24 h or within 72 h after onset showed no association.Conclusions: Intervals from prodromal infection to acute encephalopathy (≤1 day), total numbers of brain lesion location (≥3), the recovery duration of hemorrhage and atrophy (>3 months), and the presence of cavitation predict severe neurological sequelae in pediatric patients with ANE. Early treatments, including steroid therapy and IVIG, had no correlation with better outcomes. Further studies are needed to establish a consensus guideline for the management of ANE.

Excellent Outcome of Acute Necrotizing Encephalopathy in an Adult With Bacterial Infections, Case Report

2021 ◽  
pp. 194187442199137
Author(s):  
Yan Wang ◽  
John R. Younce ◽  
Joel S. Perlmutter ◽  
Soe S. Mar
Keyword(s):  
Differential Diagnosis ◽  
Bacterial Infections ◽  
Vasogenic Edema ◽  
Brain Magnetic Resonance Imaging ◽  
Acute Necrotizing Encephalopathy ◽  
Excellent Outcome ◽  
Intravenous Antibiotics ◽  
Acute Necrotizing ◽  
Supportive Management

Acute necrotizing encephalopathy (ANE) is a rare para-infectious encephalopathy that classically occurs in children. However, ANE should be considered in the differential diagnosis of adults with symmetric brain lesions after a prodromal illness given recent reports of coronavirus disease of 2019 (COVID-19) to presumably cause ANE in adults. We report a case of a 29-year-old male presenting with fever, malaise, and rapid deterioration into coma. Brain magnetic resonance imaging revealed multifocal symmetric areas of diffusion restriction and surrounding vasogenic edema involving bilateral thalami, pons and cerebellar hemispheres with a core of susceptibility artifact, and minimal thalamic contrast enhancement, most consistent with ANE. Extensive infectious workup revealed isolated Escherichia coli and Neisseria gonorrhoeae in his urine. Despite the severe encephalopathy on initial presentation, the patient improved with intravenous antibiotics and supportive management with minimal residual deficits at 9 months follow-up. We aim to provide an overview of the radiological features, differential diagnosis, treatment and prognosis of ANE. Becoming familiarized with this rare but devastating disease will improve detection, treatment, and ultimately prognosis, especially in the era of a new pandemic.

Acute necrotizing encephalopathy of childhood associated with human herpes virus 6 in Croatia

2018 ◽  
Vol 40 (3) ◽  
pp. 639-641 ◽  
Author(s):  
Vedran Stevanović ◽  
Zoran Barušić ◽  
Klaudija Višković ◽  
Oktavija Đaković Rode ◽  
Goran Tešović
Keyword(s):  
Herpes Virus ◽  
Human Herpes ◽  
Acute Necrotizing Encephalopathy ◽  
Human Herpes Virus ◽  
Human Herpes Virus 6 ◽  
Acute Necrotizing ◽  
Herpès Virus

Pandemic Influenza A-Associated Acute Necrotizing Encephalopathy Without Neurologic Sequelae

2011 ◽  
Vol 45 (5) ◽  
pp. 344-346 ◽  
Author(s):  
Akira Kumakura ◽  
Chihiro Iida ◽  
Makiko Saito ◽  
Masashi Mizuguchi ◽  
Daisuke Hata
Keyword(s):  
Pandemic Influenza ◽  
Influenza A ◽  
Acute Necrotizing Encephalopathy ◽  
Neurologic Sequelae ◽  
Acute Necrotizing

scholarly journals Acute Necrotizing Encephalopathy

2019 ◽  
Vol 29 (07) ◽  
pp. 649-653 ◽  
Author(s):  
Farida Jan ◽  
Sidra Jafri ◽  
Shahnaz Ibrahim
Keyword(s):  
Acute Necrotizing Encephalopathy ◽  
Acute Necrotizing

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.

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