| 1 | 69/M | 2/1 | | 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.
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| 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.
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| 3 | 69/M | 5 | | 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.
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