Acute Necrotizing Encephalopathy in Children: a Long Way to Go

Lee, Yun Jeong; Hwang, Seung-Hyun; Kwon, Soonhak

doi:10.3346/jkms.2019.34.e143

Cited by 32 publications

(32 citation statements)

References 30 publications

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“…Also, hemorrhage and necrosis were detected in MRI findings in five (55.5%) patients, hypertransanemia in seven (77.7%) patients, and thrombocytopenia in two (22.2%) patients. While hypertransanemia was detected in 62% of the patients in a study, it was found at a rate close to our study [27]. Our study showed that two cases (cases 7 and 8) with recurrence died.…”

Section: Discussionsupporting

confidence: 85%

Acute necrotizing encephalopathy of childhood: a single-center experience

Aksoy¹,

Öztoprak²,

Çelik³

et al. 2021

Turk J Med Sci

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Background/aim: Acute necrotizing encephalopathy is a rare type of acute encephalopathy characterized by multi-ocal brain lesions and associated severe neurological findings and various organ dysfunctions may accompany it. Materials and Methods: Patients with acute necrotizing encephalopathy of childhood diagnosed by pediatric neurology and pediatric intensive care at Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital between 2007 and 2020 were included in this study. Results: Nine patients (six females, three males) with a mean age of 4.05 ± 1.94 years (age range 1-6.5) were included in this study. The interval range between fever and encephalopathy in patients was 1-4 days. Influenza A (3H1N1, one untyped) was detected in four patients, influenza B in three patients, and no cause was found in two patients. Major clinical findings other than febrile encephalopathy in all patients were a hemodynamic shock in seven patients, seizures in six patients, vomiting in five patients, dystonia in three patients, and flaccid paralysis in the upper extremity in one patient. Despite all our treatment approaches, including plasmapheresis, moderate to severe neurological sequelae was observed in all of our patients, who survived even with significant radiological improvement. Three patients for whom we could not perform plasmapheresis died. Conclusion:Our study revealed that thalamic involvement increased as the interval shortened, and brainstem involvement increased in patients over four years of age. The presence of persistent vomiting accompanying encephalopathy during the parainfectious period and plasmapheresis treatment being a treatment option that could prevent mortality were cautionary for our study.

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Section: Discussionsupporting

confidence: 85%

Acute necrotizing encephalopathy of childhood: a single-center experience

Aksoy¹,

Öztoprak²,

Çelik³

et al. 2021

Turk J Med Sci

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“…MRI showed characteristic hemorrhagic rim enhancing lesions within the thalami, medial temporal lobes, and subinsular regions [ 8 ]. ANE is a rare but well-recognized complication of viral illnesses, particularly in children [ 13 , 14 ] and was reported in adults during the novel influenza A H1N1 pandemic [ 15 ]. Although the pathogenesis of ANE is not known, it is hypothesized to be immune-mediated, with more than 90% of cases preceded by fever and upper respiratory infection.…”

Section: Clinical Observationsmentioning

confidence: 99%

Neurological Involvement in COVID-19 and Potential Mechanisms: A Review

et al. 2020

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As the current understanding of COVID-19 continues to evolve, a synthesis of the literature on the neurological impact of this novel virus may help inform clinical management and highlight potentially important avenues of investigation. Additionally, understanding the potential mechanisms of neurologic injury may guide efforts to better detect and ameliorate these complications. In this review, we synthesize a range of clinical observations and initial case series describing potential neurologic manifestations of COVID-19 and place these observations in the context of coronavirus neuro-pathophysiology as it may relate to SARS-CoV-2 infection. Reported nervous system manifestations range from anosmia and ageusia, to cerebral hemorrhage and infarction. While the volume of COVID-19-related case studies continues to grow, previous work examining related viruses suggests potential mechanisms through which the novel coronavirus may impact the CNS and result in neurological complications. Namely, animal studies examining the SARS-CoV have implicated the angiotensin-converting-enzyme-2 receptor as a mediator of coronavirus-related neuronal damage and have shown that SARS-CoV can infect cerebrovascular endothelium and brain parenchyma, the latter predominantly in the medial temporal lobe, resulting in apoptosis and necrosis. Human postmortem brain studies indicate that human coronavirus variants and SARS-CoV can infect neurons and glia, implying SARS-CoV-2 may have similar neurovirulence. Additionally, studies have demonstrated an increase in cytokine serum levels as a result of SARS-CoV infection, consistent with the notion that cytokine overproduction and toxicity may be a relevant potential mechanism of neurologic injury, paralleling a known pathway of pulmonary injury. We also discuss evidence that suggests that SARS-CoV-2 may be a vasculotropic and neurotropic virus. Early reports suggest COVID-19 may be associated with severe neurologic complications, and several plausible mechanisms exist to account for these observations. A heightened awareness of the potential for neurologic involvement and further investigation into the relevant pathophysiology will be necessary to understand and ultimately mitigate SARS-CoV-2-associated neurologic injury.

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“…Temporal lobe (1/1) Occipital cortices (1/1) External capsule (1/1) Brainstem (1/1) Howard et al, 2018 (2) (Howard et al, 2018) 17 mo, 5y Kelly et al, 2019 (1) (Kelly et al, 2019) 22y (1 st event at 15 mo) elevated transaminases and 12% with findings suggestive of disseminated intravascular coagulation (DIC) (Lee et al, 2019, Seo et al, 2010. In patients with liver dysfunction, transaminitis is seen in the absence of hyperammonemia (Lee et al, 2019, Seo et al, 2010. ANE1 is a subtype of ANE that is distinguished by its genetic origins and potentially relapsing disease course and is the focus of this study.…”

Section: Clinical Disease Coursementioning

confidence: 99%

Genetic Acute Necrotizing Encephalopathy Associated with RANBP2: Clinical and Therapeutic Implications in Pediatrics

Levine

Ahsan

et al. 2020

Multiple Sclerosis and Related Disorders

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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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Acute Necrotizing Encephalopathy in Children: a Long Way to Go

Cited by 32 publications

References 30 publications

Acute necrotizing encephalopathy of childhood: a single-center experience

Acute necrotizing encephalopathy of childhood: a single-center experience

Neurological Involvement in COVID-19 and Potential Mechanisms: A Review

Genetic Acute Necrotizing Encephalopathy Associated with RANBP2: Clinical and Therapeutic Implications in Pediatrics

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