Acute necrotizing encephalopathy

Case contributed by Dr Vinay V Belaval

Presentation

History of chicken pox. Presents with fever, multiple episodes of seizures and altered sensorium since 2 days.

Patient Data

Age: 10 years
Gender: Female

Plain and contrast enhanced MRI brain of 10 year old child showed symmetrical T2 & FLAIR hyperintense and T1 hypointense signal lesions in bilateral thalami, posterior ends of bilateral lentiform nuclei, midbrain, pons, bilateral cerebellar hemispheres, bilateral high frontal lobes, bilateral cerebellar vermis and right periventricular temporal white matter. These lesions showed restricted diffusion and thin peripheral enhancement. 

Foci of blooming were seen in thalamic and vermial lesions on gradient sequence.

Mass effect was seen with compression of 3rd and 4th ventricles with mild bilateral lateral ventricular dilatation. Bilateral cerebellar tonsillar herniation was present. 

Overall imaging features were in favor of acute necrotizing encephalopathy of childhood.

Case Discussion

10-year-old child with a recent history of chickenpox presented with fever, multiple episodes of seizures and altered sensorium. Contrast enhanced MRI of the brain showed symmetrical T2 high signal intensity lesions in bilateral thalami, posterior ends of bilateral lentiform nuclei, brainstem, bilateral cerebellar hemispheres, bilateral high frontal and right periventricular white matter with restricted diffusion and peripheral enhancement.  Foci of hemorrhage are seen within thalamic and vermian lesions. 

These imaging features were in favor of acute necrotizing encephalopathy of childhood.

Acute necrotizing encephalopathy is a rare entity characterized by rapid neurological deterioration with typical involvement of bilateral thalami. Involvement of cerebral and cerebellar hemispheres, internal/ external capsules and brainstem is also seen with secondary hemorrhage. 

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