Clinical outcome of acute necrotizing encephalopathy in related to involving the brain stem of single institution in Korea

Abstract

PurposeAcute necrotizing encephalopathy (ANE) is a fulminant disease of the brain characterized by bilateral thalamic lesions, and is prevalent among children in East Asia. The prognosis of ANE is usually poor with a high mortality rate and neurological sequelae. This study aimed to delineate the clinical characteristics and prognostic factors of ANE.MethodsWe retrospectively analyzed clinical data of 399 pediatric patients with encephalitis who were admitted to Samsung Medical Center from December 1998 to March 2011. We enrolled ten patients (11 cases) with ANE and analyzed their demographic, clinical, and neuroimaging data. The location and extent of the brain regions were checked based on fluid-attenuated inversion recovery, T1-, and T2-weighted imaging findings; the presence of contrast enhancement, restricted diffusion, and hemorrhage.ResultsTen patients were identified, including one patient with two episodes. The median age of onset was 1.5 years (0.4-8.4 years). The mortality rate was 40%, and only 30% of patients survived without neurological sequelae. The definite involvement of the brainstem on brain magnetic resonance imaging was significantly correlated with mortality (P=0.04).ConclusionBroad and extensive brainstem involvement suggested the fulminant course of ANE. Early diagnosis of ANE before brainstem involvement, through careful identification of symptoms of brain dysfunction, may be the best way to achieve better neurological outcomes.

Acute necrotizing encephalopathy of childhood secondary to dengue infection: A case report from Pakistan

“…[ 6 ] Our patient had brainstem involvement, and it leads to brain death while a report from Korea showed that 40% of their patient with brainstem involvement expired during the acute illness and another 30% developed severe neurological impairment. [ 10 ]…”

Fatal Acute Necrotizing Encephalopathy: Clinical Presentation and Imaging Findings

“…Lee et al examined the thalamus in six acute necrotizing encephalopathy patients, finding hyperintensity on DWI and a low ADC in four patients, hypointensity on DWI and a high ADC in one patient, and no change in DWI or ADC in one patient. 3 Brainstem involvement, rather than findings for DWI and ADC, suggests poor outcome. 3 Influenza and human herpesvirus-6 are representative pathogens for acute necrotizing encephalopathy.…”

“…3 Brainstem involvement, rather than findings for DWI and ADC, suggests poor outcome. 3 Influenza and human herpesvirus-6 are representative pathogens for acute necrotizing encephalopathy. 4 Our clinical observations suggested human herpesvirus-6 as the most likely pathogen for the fulminant encephalopathy in this patient.…”

Magnetic Resonance Imaging to Predict Neurological Outcome in Children with Acute Encephalitis

“…24 In children with acute necrotising encephalitis, extensive and marked T2-weighted hyperintense signal changes in the brainstem with restricted diffusion or haemorrhage are associated with mortality. 25 Extensive damage in the medulla and pons is a sign of profound progression of disease, as severe brainstem dysfunction may lead to neurogenic oedema, respiratory insufficiency, cardiopulmonary collapse, and mortality. 25 Nonetheless, the lack of association between cerebellar lesions and adverse neurological outcome may indicate that MRI has no prognostic value in acute cerebellitis.…”

“…25 Extensive damage in the medulla and pons is a sign of profound progression of disease, as severe brainstem dysfunction may lead to neurogenic oedema, respiratory insufficiency, cardiopulmonary collapse, and mortality. 25 Nonetheless, the lack of association between cerebellar lesions and adverse neurological outcome may indicate that MRI has no prognostic value in acute cerebellitis. 26 Further studies are needed to elucidate the patterns of association.…”