Acute necrotizing encephalopathy of childhood: typical findings in an atypical disease

Abstract

Acute necrotizing encephalopathy of childhood (ANEC) is a disease entity seen nearly exclusively in East Asian children that is characterized by multifocal, symmetric lesions involving the thalami, brainstem, cerebellum, and white matter. We present a child who developed dramatic neurologic symptoms following a viral prodrome. Serial MRI examinations demonstrated characteristic lesions of ANEC, while laboratory analyses revealed evidence of acute infection with human herpesvirus-6 (HHV-6). We highlight the MRI findings in both the acute and convalescent phases of ANEC, discuss the implications of neuroimaging on the child's clinical course, and emphasize the integral role of the radiologist in correctly diagnosing this rare disease.

Acute Necrotizing Encephalopathy in a Child during the 2009 Influenza A(H1N1) Pandemia: MR Imaging in Diagnosis and Follow-Up

“…9 At the present time, many authors have speculated that ANE may be an immune-mediated or metabolic disease entity. 5 Nonetheless, the etiology and pathogenesis of this disease still remain unknown.…”

Acute Necrotizing Encephalopathy in 3 Brothers

“…5 Influenza A (H3N2) and B are the most commonly identified viral etiologies; however, there have been other etiologies reported, such as human herpes virus 6. 13,17,18 Although brainstem lesions appear more commonly in influenza-related cases, documentation of influenza does not seem to predict outcome. 19 In our series, patient 2 was documented to have an acute infection with influenza A, but we were unable to determine a pathogen for the upper respiratory infection symptoms in his brothers.…”

Acute Necrotizing Encephalopathy: A Rare Case

“…Familial cases may be due to RANBP2 gene mutations, and are known as "infectioninduced acute encephalopathy 3 (IIAE3) [6,7]. It is believed that virus or its variant increases intracranial cytokine production (TNF alpha, IL 1 and 6), which progresses disease rapidly [8].…”