A severity score for acute necrotizing encephalopathy

Yamamoto, Hiroyuki; Okumura, Akihisa; Natsume, Jun; Kojima, Seiji; Mizuguchi, Masashi

doi:10.1016/j.braindev.2014.05.007

Cited by 65 publications

(64 citation statements)

References 18 publications

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“…In addition, whereas elevated CSF protein above 60 mg/dL has been reported as a poor prognostic factor, temporal course of CSF protein has not been reported. 2 Our patient had a drastically elevated protein around the time of her initial decompensation and decline 12 hours later, suggesting an acute and fulminant inflammatory response, consistent with the hypothesized cytokine storm and correlating with our observed findings on CT. Aminotransaminase elevation was a somewhat delayed finding in our patient, following elevation in CSF protein.…”

Section: Discussionsupporting

confidence: 87%

“…1 The exact etiology is unknown, but it is hypothesized that a "cytokine storm" is involved in its pathogenesis. 2,3 We describe a child with fulminant and rapid ANEC whose computed tomography (CT) was normal on arrival but showed typical findings of ANEC on repeat CT just three hours later, along with the temporal course of imaging and laboratory findings.…”

Section: Introductionmentioning

confidence: 99%

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Temporal Course of Imaging and Laboratory Findings in a Child with Fulminant Acute Necrotizing Encephalopathy

Larsh

Hsich

2020

Pediatric Neurology

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Section: Discussionsupporting

confidence: 87%

Section: Introductionmentioning

confidence: 99%

Temporal Course of Imaging and Laboratory Findings in a Child with Fulminant Acute Necrotizing Encephalopathy

Larsh

Hsich

2020

Pediatric Neurology

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“…Another ANE severity scale (ANE-SS) which ranges from 0 to 9 points has been developed, and presence of shock, brain stem lesions, an age over 48 months, a platelet count below 100,000/ μ L, and an elevated level of CSF protein correspond to 3, 2, 2, 1, and 1 points, respectively [ 73 ]. The ANE-SS could be a predictor for the outcome or prognosis of children with ANE [ 73 ]. Nevertheless, some extended or novel imaging technologies, as mentioned above, might also be a predictor of the prognosis.…”

Section: Prognosis and Its Predictors In Patients With Anementioning

confidence: 99%

Acute Necrotizing Encephalopathy: An Underrecognized Clinicoradiologic Disorder

Pan

et al. 2015

Mediators of Inflammation

178

281

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Acute necrotizing encephalopathy (ANE) is a rare but distinctive type of acute encephalopathy with global distribution. Occurrence of ANE is usually preceded by a virus-associated febrile illness and ensued by rapid deterioration. However, the causal relationship between viral infections and ANE and the exact pathogenesis of ANE remain unclear; both environmental and host factors might be involved. Most cases of ANE are sporadic and nonrecurrent, namely, isolated or sporadic ANE; however, few cases are recurrent and with familial episodes. The recurrent and familial forms of ANE were found to be incompletely autosomal-dominant. Further the missense mutations in the gene encoding the nuclear pore protein Ran Binding Protein 2 (RANBP2) were identified. Although the clinical course and the prognosis of ANE are diverse, the hallmark of neuroradiologic manifestation of ANE is multifocal symmetric brain lesions which are demonstrated by computed tomography (CT) or magnetic resonance imaging (MRI). The treatment of ANE is still under investigation. We summarize the up-to-date knowledge on ANE, with emphasis on prompt diagnosis and better treatment of this rare but fatal disease.

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“…Treatment of ANEC with antiviral drugs, immunoglobulin, methylprednisolone, dexamethasone, plasmapheresis, antithrombin III and therapeutic hypothermia has been tried 12 22. Our patient was treated with supportive measures, mechanical ventilation, IVIG in the acute phase and oseltamivir for influenza A infection.…”

Section: Discussionmentioning

confidence: 99%

Acute necrotising encephalopathy in a child with H1N1 influenza infection: a clinicoradiological diagnosis and follow-up

et al. 2016

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Acute necrotising encephalopathy of childhood (ANEC) is a fulminant disorder with rapid progressive encephalopathy, seizures and poor outcome. It has been reported in association with various viral infections. We describe the clinicoradiological findings and short-term follow-up in a child with H1N1 influenza-associated ANEC. Laminar, target or tricolour pattern of involvement of the thalami was seen on apparent diffusion coefficient images. Our patient had significant morbidity at discharge despite early diagnosis and management with oseltamivir and immunoglobulin. Repeat imaging after 3 months had shown significant resolution of thalamic swelling, but there was persistence of cytotoxic oedema involving bilateral thalami. She was pulsed with intravenous steroids and maintained on a tapering schedule of oral steroids. This report emphasises the need for a high index of suspicion to establish early diagnosis, promotion of widespread immunisation strategies to prevent influenza outbreak, and more research to establish standard treatment protocols for this under-recognised entity.

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A severity score for acute necrotizing encephalopathy

Cited by 65 publications

References 18 publications

Temporal Course of Imaging and Laboratory Findings in a Child with Fulminant Acute Necrotizing Encephalopathy

Temporal Course of Imaging and Laboratory Findings in a Child with Fulminant Acute Necrotizing Encephalopathy

Acute Necrotizing Encephalopathy: An Underrecognized Clinicoradiologic Disorder

Acute necrotising encephalopathy in a child with H1N1 influenza infection: a clinicoradiological diagnosis and follow-up

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