Objective: Our objective is to report  image findings of an acute necrotizing encephalopathy of childhood (ANEC) in a 15 years-old girl, completely normal until 18 months of life. 

Background: Acute necrotizing encephalopathy of childhood is a rare form of acute encephalopathy originally described in East Asia. There are less than 150 cases reported in literature. Rarely was reported outskirts of Asia-Countries. Typically is manifested after nonspecific infectious illness. The clinical manifestations range from mild forms of ANEC to a fatal outcome. The diagnosis is based on the topography of lesions findings, typically with bilateral and symmetric in the thalami. 

Methods: We report a case of non-progressive coreoatethosis following acute necrotizing encephalopathy of childhood at 18 months of life.

Results: The patient is a 15-year –old girl that had had a normal neurologic development until 18^th month of life. After a febrile seizure, that lasted more or less 30 minutes she developed a non-progressive severe form of coreoatethosis. She is mentally normal, writing using a computer.

The spinal MRI was normal while the Brain MRI showed bilateral and exactly symmetric areas at the basal ganglia (caudate and putamen) hyperintense in FLAIR [Figue1] and hypointense in T1 [Figue2] with marked atrophy of both.

The images were compatible with ANEC, but showed some features typically found in Leigh’s syndrome. 

Conclusions: The patient reported presented with non-progressive coreoatethosis following acute necrotizing encephalopathy of childhood at 18 months of life. The spectrum of ANEC ranges from mild forms of ANEC to a fatal outcome. The diagnosis is based on the topography of lesions findings, typically with bilateral and symmetric in the thalami. Our case presented with atypical images findings, as the lesions affected mainly the striatum. 

References: 1) Mastroyianni SD, Gionnis D, Voudris K, Skardoutsou A, Mizuguchi M. Acute necrotizing encephalopathy of childhood in non-Asian patients: report of three cases and literature review. J Child Neurol. 2006 Oct;21(10):872-9. 

2) Wang KY, Singer HS, Crain B, Gujar S, Lin DD. Hypoxic-ischemic encephalopathy mimicking acute necrotizing encephalopathy. Pediatr Neurol. 2015 Jan;52(1):110-4.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/non-progressive-coreoatethosis-secondary-to-infantile-necrotizing-encephalopathy-mimicking-leigh-disease/