Acute necrotizing encephalopathy

Abstract

Acute necrotizing encephalopathy (ANEC) is a rare disease well recognized in Japan but has not yet been reported from Indian subcontinent. We describe here a case of ANEC with the neuroimaging findings. P. vivax infection was detected as an associated finding and the treatment given.

Acute Necrotizing Encephalopathy: An Underrecognized Clinicoradiologic Disorder

“…Both environmental factors, which may contribute to the antecedent infections, and host factors such as individual susceptibility or alterations of genes might be involved [ 16 ]. Usually, ANE develops secondary to viral infections, including influenza A and influenza B, novel influenza A (H1N1), parainfluenza, varicella, human herpesvirus 6 and herpesvirus 7 (HHV-6 and HHV-7), enterovirus, novel reovirus train (MRV2Tou05), rotavirus, herpes simplex virus, rubella, coxsackie A9, and measles, among which the influenza virus and HHV-6 are most common [ 5 , 9 – 13 , 17 – 34 ]. Prodromal viral infections appear to play a critical role in the initiation of ANE.…”

“…The clinical course of ANE is fulminant and diverse, from a mild form with completely recovery or mild sequelae to a severe form with a high mortality [ 25 , 31 , 37 , 59 , 60 ]. Usually, survivors of ANE go through three phases during the clinical course including prodromal stage, period of acute encephalopathy, and recovery stage as shown in Figure 1 [ 5 , 7 , 9 , 10 , 18 – 20 , 23 , 29 , 31 , 35 , 61 – 63 ]. Due to the decreased incidence of autopsies, the diagnosis of ANE was mainly based on characteristic neuroradiologic findings [ 3 , 64 , 65 ].…”

“…Gradually, with the resolution of edema and mass effect, the feature of petechial hemorrhage and necrosis appears, and hypodense areas on CT become mottled because of the irregular hyperdense spots at the center which result from the extravasation of blood vessels or petechial hemorrhage [ 35 , 59 ]. On the corresponding T 1 -weighted imaging (T 1 WI), increased signal intensities in the center surrounded by the decreased signals are detected, while T 2 -weighted imaging (T 2 WI) may reveal decreased signal intensities that are surrounded by increased or homogeneous increased signal intensities [ 5 , 10 , 26 , 67 ]. Small petechial hemorrhage is usually obscure.…”

“…Due to the pathogenesis of ANE, mainly the hypercytokinemia secondary to variable viral infections through immune-mediated mechanism, the immunomodulatory therapy, particularly the therapy that suppressed the cytokine production, has the potential to improve the outcome of ANE. Intravenous glucocorticoids, immunoglobulin, and plasmapheresis should be effective on the basis of the pathogenesis of ANE [ 5 , 20 , 24 , 25 , 30 , 57 , 60 , 63 , 68 , 69 ]. Among these therapies, intravenous glucocorticoids, including methylprednisolone and dexamethasone, were the most mentioned and studied, although empirically without systematically determined.…”

First case of acute haemorrhagic leucoencephalitis following Plasmodium vivax infection: Comment

“…[2] A proper literature search using Medical Subject Headings terms can minimise errors of this sort.…”