Acute necrotizing encephalopathy associated with enterovirus infection

Acute Necrotizing Encephalopathy: An Underrecognized Clinicoradiologic Disorder

“…Although polymerase chain reaction (PCR) for some viruses was positive in cerebrospinal fluid (CSF) in some reports, there was no clue for encephalitis through autopsic pathological examinations, which showed notably minimal brain inflammation in relation to the marked parenchymal abnormality [ 3 , 4 , 20 , 22 , 25 , 35 ]. Moreover, CSF pleocytosis is usually absent in patients with ANE.…”

“…These cells produced a high level of cytokines, suggesting that NK cells might be associated with the pathogenesis of ANE [ 28 ]. In addition, several lines of evidence showed that levels of cytokines were elevated in the serum and/or CSF in different virus-associated ANE including interleukin- (IL-) 6, tumor necrosis factor-alpha (TNF- α ), IL-10, IL-15, IL-1 β , soluble TNF receptor (sTNFR), and interferon-gamma (IFN- γ ) [ 18 , 20 , 23 , 26 , 28 , 38 , 40 – 44 ]. IL-6 and TNF- α were critical among these cytokines, because the former was neurotoxic at high concentrations, whereas the latter could damage the endothelium of the central nervous system.…”

“…The missense mutations in the gene encoding the nuclear pore protein Ran Binding Protein 2 ( RANBP2 ) were found to be the susceptibility alleles for familial and recurrent cases of ANE, and they proposed naming this type of ANE as “ANE1” [ 50 ]. Since the relationship between ANE and the RANBP2 gene was identified, there have been several reports on recurrent or familial cases, while the mechanism of this mutation leading to pathology remains unclear [ 20 , 22 , 51 ]. RANBP2 is located on the cytoplasmic surface of the nuclear pore, and its numerous functions throughout the cell cycle have been found, which include facilitation of the protein transportation and sumoylation of protein cargoes during interphase, intracellular trafficking, or energy maintenance in certain type of cells, including neurons, and contribution to the nuclear envelop breakdown as well as facilitation of sister chromatid resolution during mitosis [ 50 ].…”

Pediatric Hemorrhagic Brainstem Encephalitis Associated With HHV-7 Infection

“…5 However, there are many individual case reports of good recovery with immunosuppressive therapies. 6 Acute necrotizing encephalopathy (ANE) of childhood, on the other hand, initially reported in Asian children following influenza A infection, tends to cause bilateral, sometimes hemorrhagic, injury to the thalami, but further reports have shown that this illness affects children worldwide and can also be associated with enterovirus infection 7 , 8 . A familial form of ANE has been reported in association with mutations in the nuclear pore protein RANBP2.…”

Efficacy of Pulse Steroid Therapy in Acute Viral Encephalopathy (Case Report)

“…Okumura et al [11] concluded that administration of steroid within 24 hours after the onset was related to better outcome of the patients with acute encephalopathy Tabarki et al [12] showed that in spite of the late administration of the steroids the outcome of the patients was relatively good. Kimura et al [13] pointed in their study that after administration of 3 days steroid therapy (1000 mg/day) the patient was responded quickly.…”