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Fig 1. Case 1: Coronal T2-weighted MR images obtained 1 year after the onset of ALSD (A–C). AtNOphy is seen in the anteromedial part of the bilateral temporal lobes, especially on the right side, but not apparent in the frontal lobes. Postmortem coronal T2-weighted MR images (D–F) show asymmetric atrophy of the temporal lobes, more severe on the right side. T2 hyperintensities are also seen in the anteromedial part of the right temporal white matter. G, The right temporal lobe in (D) shows linear hyperintensity in the subcortical white matter (arrowheads). H, A Klüver-Barrera-stained section corresponding to boxed area in (G) shows laminar pallor of the subcortical white matter (arrowheads). I, Boxed area in (H) shows spongiform changes in the cortex (arrows). J, Histologic examination of the area in (I) shows spongiform changes with neuronal loss and gliosis. H, Klüver-Barrera stain x 20. I, hematoxylin-eosin, x 100. J, GFAP, x 200. K, Right temporal lobe in (E) shows hyperintensities in the medial part of the temporal white matter, especially in the underlying U-fibers (arrowheads). L, A Klüver-Barrera-stained section corresponding to (K) reveals pallor in the medial part of the temporal white matter, especially in the underlying U-fibers (arrowheads). M, A Holzer-stained section corresponding to (K) reveals gliosis in the medial part of the temporal white matter, especially in the underlying U-fibers (arrowheads). N, O, Histologic examination of normal signal intensities on MR image (K–M, circles) shows no apparent degenerative changes. P–R, Histologic examination of the U-fibers (arrows) in (K–M) shows loss of myelin (P) and axons (Q) with moderate gliosis (R). N, P, Klüver-Barrera stain. O, Q, Bielschowsky stain. R, GFAP stain. N, O, P, Q, R, x 200.
