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FIG 1. Case 1.
A–C, Axial T1-weighted MR images obtained 2 days after admission. T1-weighted image (A) shows hyperintensities in the globus pallidus on both sides (arrows). T1-weighted images show no definite signal intensity changes in the cerebral cortices (A–C).
D–F, T2-weighted MR images obtained 2 days after admission. Signal intensity abnormalities are difficult to discern on T2-weighted image (D). T2-weighted images (E and F) show focal, faint hyperintensities in the cerebral cortices (arrows). Confluent and diffuse T2 hyperintensities are seen in the deep and subcortical cerebral white matters (E).
G and H, Postmortem axial T2-weighted MR images show deep cortical and subcortical laminar hyperintensities in the entire cerebral hemisphere. Diffuse T2 hypertensities are seen in the deep and subcortical cerebral white matters (G).
I and J, Coronal T2-weighted MR images show deep cortical and subcortical laminar hyperintensities in the entire cerebral hemisphere. Hyperintensities are also seen in the deep and subcortical white matters (J). Boxed area in panel I shows hyperintensities in the deep layers of the cerebral cortices and subcortical white matters (open arrows) as well as deep cerebral white matter. The boundary between deep layers of the cerebral cortex and subcortical white matter is not clear.
K, The hematoxylin and eosin stained section corresponding to panel J shows laminar pallor of the deep layers of the cerebral cortex (arrowheads) and laminar pallor of the underlying U-fibers (arrows), as well as moderate pallor of the deep cerebral white matter. (Hematoxylin and eosin stain x100.)
L, Histologic findings of the arrowhead area in panel K shows spongy changes of the neuropil, pyknotic, and shrunken neurons (arrowheads) and Alzheimer type II astrocytes (arrows), which have large pale nuclei with marginated chromatin and scanty cytoplasms. (Hematoxylin and eosin stain x100.)
M and N, Histologic findings of the U-fibers (K, arrows) show tissue rarefaction and severe loss of myelin (M) and axons (N). (M, Klüver-Barrera stain x100; N, Bielschowsky stain x100.)
O and P, Histologic findings of the circled area in panels J and K reveals moderate loss of myelin (O) and axons (P). (O, Klüver-Barrera stain x100; P, Bielschowsky stain x100.)
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