Fig. 3

Autopsy findings of amyotrophic lateral sclerosis (ALS) pathology. Top view of the brain before fixation. Cloudiness of the pia mater was notable, especially on the surface of the central region, suggesting cortical atrophy around the central sulci (red arrows) (a). The primary motor cortex (red arrowheads) exhibits a dark-brownish color, with the border’s fuzziness at the corticomedullary junction (b). The primary motor cortex’s low-power view shows cortical thinning and vacuolar formation in the white matter just beneath the cortex (c). Cortical degeneration associated with severe neuronal loss and gliosis (d). There were many vacuoles in the subcortical white matter of the primary motor cortex. The vacuoles appear to be isolated from each other (e). Klüver-Barrera (KB) staining at the level of the internal capsule (f) and the pyramid of the medulla oblongata (g) demonstrate microvacuolar formation with infiltration of macrophages. KB staining in the thoracic spinal cord revealed corticospinal tract degeneration (h). Bunina body (thin black arrow) (i) and transactivation reaction DNA-binding protein 43 kDa (TDP-43)-positive cytoplasmic inclusions (black arrowheads) in the remaining motor neurons in the anterior spinal horn (j, k) and in the hypoglossal nuclei (l) are shown. Native TDP-43 was preserved in the nuclei of normal motor neurons (k, black arrows). GM: gray matter, WM: white matter. Scale bars = a 3 cm, b 1 cm, c, h 500 μm, d-g 100 μm, i, j 10 μm