Acute necrotizing encephalopathy of childhood (ANEC)

Case contributed by Khalid Alhusseiny
Diagnosis almost certain

Presentation

Fever followed by disturbed level of consciousness and seizures.

Patient Data

Age: 5 years
Gender: Male

There are bilateral symmetrical signal abnormalities involving both thalami, brainstem tegmentum and cerebellar white matter. They appear hyperintense on T2, FLAIR, and DWI while hypointense on T1. Other smaller foci of diffusion restriction are also seen involving the cerebral white matter. The thalamic abnormalities show blooming on SWI. No post-contrast enhancement is seen.

Spectroscopy shows abnormally elevated lipid/lactate and glutamine/glutamate peaks.

There is a remarkable regression in the size and intensity of the previously seen signal abnormalities with evolution on T1 consistent with aging blood products.

Case Discussion

The described findings are highly suggestive of acute necrotizing encephalopathy of childhood. Follow-up MRI revealed disease regression following medical treatment.

Case courtesy: Prof. Dr. Yasser Ragab

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.