PMC10365096_01
PMC10365096_02
Difference in the signal intensity of a transmantle lesion across magnetic resonance imaging sequences. Axial T2-weighted (A) and fluid attenuated inversion recovery (B) images show high-intensity areas, while spoiled gradient recalled echo image (C) reveals low-intensity areas (arrowhead).
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F1
A 3.0 Tesla MRI revealed a high-intensity area on T2-weighted/fluid attenuated inversion recovery (T2W/FLAIR) images (Figures 1A,B) and a low intensity area on spoiled gradient recalled echo images (SPGR) (Figure 1C) in the caudal portion of the left postcentral gyrus.
Coronal spoiled gradient recalled echo (STIR) image (A) and fluid attenuated inversion recovery (FLAIR) image (B) show an abnormal hyperintense signal band extending from the ventricle to the subcortical area (arrowhead). The signal is difficult to discern on the T1-weighted image; STIR or FLAIR images are most useful in detecting a transmantle lesion. (C) The magnified axial STIR image reveals cortical thickening and blurred gray/white matter junction of structurally abnormal lesion (arrowhead).
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F2
Additionally, a high-intensity area on coronal FLAIR/short tau inversion recovery (STIR) images extended from the body of the lateral ventricle to the cortex (Figures 2A,B). STIR images showed cortical thickening and blurred gray/white matter junctions (Figure 2C).
Axial (A) and coronal (B) images of technetium-99m ethyl cysteinate dimer single-photon emission computed tomography show hypoperfusion in the left frontoparietal portion (arrowhead). The area of hypoperfusion appears to be concordant with the structurally abnormal gyrus shown in Figures 1, 2.
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F3
Technetium-99m ethyl cysteinate dimer single-photon emission computed tomography showed hypoperfusion in the left frontoparietal lobe, which was consistent with the MRI lesion (Figures 3A,B).
PMC10365096_03
(A) Corpora amylacea accumulate in the subpial and the perivascular spaces in the superficial cerebral cortices (hematoxylin and eosin stain). (B) Corpora amylacea also accumulated in the perivascular spaces of the subcortical white matter (hematoxylin and eosin stain). Note the density of reactive astrocytes are in the background tissue. (C) Manlow staining highlights gliosis in the subpial cortex and the white matter in purple. (D) Dysplastic neurons, some of which contain neurofibrillary tangles (arrow), and a balloon cell (arrowheads) are present in the focal area of distal aspect of the resected specimen (hematoxylin and eosin stain). (E) The dotted square indicates the area of focal cortical dysplasia. Outside the dotted square, the cortex and subcortical white matter are abnormal, demonstrating accumulation of corpora amylacea in perivascular space (hematoxylin and eosin stain). Note that corpora amylacea does not exist within the FCD. The black square corresponds to (D).
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F4
Histological examination with the H&E and KB stains revealed thickening of the superficial cortical layer of the cerebrum with abundant corpora amylacea, which accumulated in the subpial space extending along with the perivascular, Virchow-Robin space (Figure 4A). Moreover, such accumulation of corpora amylacea was also extensive in the broad areas of the subcortical white matter (Figure 4B) where the dens fibrillary gliosis highlighted by the Manlow stain (Figure 4C) and GFAP shown by immunohistochemistry was present, obscuring the cortico-medullary junction. In addition, at the very end of the resected specimen, a focal cortical area with disorganized cortical architecture exhibited scattered dysplastic neurons and balloon cells (Figure 4D) positive for NeuN, NFP, and synaptophysin. Moreover, there were no corpora amylacea in the subpial zone, and the perivascular space within the cortex with FCD, whereas in the subcortical white matter beneath the FCD, the corpora amylacea accumulation around the perivascular spaces were observed (Figure 4E).