Cerebral Cortical and White Matter Lesions in Amyotrophic Lateral Sclerosis with Dementia: Correlation with MR and Pathologic Examinations
E. Matsusuea,
S. Sugiharaa,
S. Fujiia,
T. Kinoshitad,
T. Nakanob,
E. Ohamac and
T. Ogawaa
a From the Division of Radiology, Department of Pathophysiological and Therapeutic Science
b Department of Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Tottori, Japan
c Department Neuropathology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, Tottori, Japan
d Department of Radiology, St. Luke's International Hospital, Tokyo, Japan
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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.
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Fig 2. Case 2: Axial (A–C) and coronal (D–F) T2-weighted MR images obtained 4 months before death. Asymmetric cerebral atrophy is seen in the frontal and temporal lobes, more marked on the right side. Atrophy is more prominent in the anteromedial part of the temporal lobes. Focal and faint hyperintensities are seen in the subcortical white matter in the anteromedial part of the right temporal lobe (arrowheads in E). No definite T2-hyperintensities are seen in the corticospinal tracts in the internal capsules, cerebral peduncles, and pontine base. Change in signal intensity of the substantia nigra is also not seen.
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Fig 3. Case 3: Postmortem axial (A–C) and coronal (D–F) T2-weighted MR images. Cerebral atrophy is seen in the frontal and temporal lobes, especially in the anteromedial part of the temporal lobes. Confluent and diffuse hyperintensities are seen in the frontal and temporal white matter. No definite T2-hyperintensities are seen in the corticospinal tracts and substantia nigra. G, The left temporal lobe in (D) shows hyperintensity in the subcortical and deep white matter. H, A Klüver-Barrera-stained section corresponding to (G) reveals pallor in the temporal subcortical and deep white matter. Histologic examination of hyperintensity on MR image (G, circle) shows severe loss of myelin (I) and axons (J), which reveals tissue rarefaction. I, Klüver-Barrera stain. K, Bielschowsky stain. I, K, x 200.
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