PMC9755544_01
MRI brain findings of acute necrotizing encephalopathy caused by a RANBP2 mutation (ANE1) at the time of presentation (top row) and at 3 month follow up (bottom row). Symmetric T2 hyperintensity and swelling of the external capsules (A- white arrows), thalami (A-white arrowheads), mammillary bodies (B- white arrows), mesial temporal lobes (B- white arrowheads) and midbrain (B-black arrowhead) are seen on axial T2 weighted images on the brain (A and B). Sagittal (C) and axial (D) T2 weighted MRI of the cervical spine show expansile T2 hyperintensity extending from C3 to C6 levels (C- white arrow) primarily involving the central gray matter (D- white arrow). 3 month follow up axial T2 weighted images of the brain (E and F) and sagittal (G) and axial (H) T2 weighted MRI of the cervical spine show resolution of areas of edema and swelling with small areas of gliosis and volume loss.
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PMC9755544_01_10.1177_2329048X221143689-fig1.jpg
fig1-2329048X221143689
Brain magnetic resonance imaging (MRI) with and without IV contrast was performed which showed characteristic symmetric T2 prolongation and restricted diffusion within the thalami, external capsule, mammillary bodies, mesial temporal lobes and the brainstem (Figure 1). C3-C6 consistent with myelitis (Figure 1). A three month follow up MRI of the brain and cervical spine showed resolution of the previously seen areas of abnormal signal intensity with residual volume loss and gliosis (Figure 1).