Article Figures & Data
Figures
- 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.
- 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.
- 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.
Tables
Case No. Age/Sex Duration of Illness (yrs/mo) Clinical Findings Brain Weight (grams) Histologic Findings 1 69/M 2/1 Mild dementia
Aphasia
Dysphagia
Muscular atrophy of tongue and shoulder muscle
1,280 Mild degeneration of corticospinal tracts in brain stem and spinal cord.
Neuronal loss with gliosis in lower motor neurons.
Bunina bodies in remaining motor neurons in spinal cord.
Microvacuoles, mild neuronal loss, and gliosis in second and third layers of frontal and temporal cortices.
Spongiform changes and mild gliosis in frontal and temporal subcortical white matter.
Moderate loss of pigmented neurons with gliosis in substantia nigra.
Ubiquitin-positive cytoplasmic inclusions in hippocampal dentate granule cells and small neurons in parahippocampal cortex.
2 50/M 5/4 Dementia
Dysphagia
Fasciculation and atrophy of tongue
Atrophy of upper and lower extremities, particularly distal part of upper extremities
1,400 Mild degeneration of corticospinal tracts in brain stem and spinal cord.
Neuronal loss with gliosis in lower motor neurons.
Bunina bodies in remaining motor neurons in spinal cord.
Microvacuoles, mild neuronal loss, and gliosis in second and third layers of frontal and temporal cortices.
Spongiform changes and mild gliosis in frontal and temporal subcortical white matter.
Moderate loss of pigmented neurons with gliosis in substantia nigra.
Ubiquitin-positive cytoplasmic inclusions in hippocampal dentate granule cells and small neurons in parahippocampal cortex.
3 69/M 5 Apathy
Depression
Change of character
Dementia
Atrophy of upper and lower extremities
1,200 Mild degeneration of corticospinal tracts in brain stem and spinal cord.
Neuronal loss with gliosis in lower motor neurons.
Bunina bodies in remaining motor neurons in spinal cord.
Microvacuoles, mild neuronal loss, and gliosis in second and third layers of frontal and temporal cortices.
Tissue rarefaction in subcortical and deep white matters of frontal and temporal lobes.
Moderate loss of pigmented neurons with gliosis in substantia nigra.
No ubiquitin-positive cytoplasmic inclusions in hippocampal dentate granule cells and other cortical neurons.
Note:—ALSD indicates amyotrophic lateral sclerosis with dementia.
Case No. Age/Sex Duration of Illness (yrs/mo) Antemortem T2-Weighted Imaging Findings Postmortem T2-Weighted Imaging Findings 1 69/M 2/1 Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.
Mild atrophy of frontal lobes.
Atrophy of cerebral cortices, but no signal changes in cerebral cortices.
No signal changes in cerebral white matter.
No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra (obtained 1 year before death).
Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.
Mild atrophy of frontal lobes.
Atrophy of cerebral cortices, but no signal changes in cerebral cortices.
Hyperintensities in anteromedial white matter of temporal lobes. Linear subcortical hyperintensities in temporal lobes, more marked on right side.
No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra.
2 50/M 5/4 Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.
Mild atrophy of frontal lobes.
Atrophy of cerebral cortices, but no signal changes in cerebral cortices.
Faint hyperintensities in subcortical white matter in anteromedial part of right temporal lobe.
No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra (obtained 4 months before death)
Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.
Mild atrophy of frontal lobes.
Atrophy of cerebral cortices, but no signal changes in cerebral cortices.
Hyperintensities in subcortical white matter of frontal and temporal lobes.
No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra.
3 69/M 5 Not available Symmetric atrophy of frontal and temporal lobes, more marked in anteromedial part of temporal lobes.
Atrophy of cerebral cortices, but no signal changes in cerebral cortices.
Hyperintensities in subcortical and deep white matter of frontal and temporal lobes.
No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra.
Note:—ALSD indicates amyotrophic lateral sclerosis with dementia.
