Fig. 2

Neuropathological findings. a–c Macroscopic findings of the brain. a Severe atrophic changes in the temporal lobe are visible in the convex external view of the cerebrum. b Sections reveal atrophy of the temporal cortex. c The substantia nigra in the midbrain appears brownish at each level. d Semi-macroscopic findings of the temporal pole show severe atrophic changes and myelin pallor. e–m Microscopic findings of the temporal pole (e–h), amygdala (i), substantia nigra (j), locus coeruleus (k), cervical anterior horn (l), and cervical anterior root (m). Severe neuronal loss with gliosis is visible in the temporal pole (e), amygdala (i), and substantia nigra (j). Prominent accumulation of p-tau is observed in the cortex (f) as well as the white matter (g) of the temporal pole. A glial cytoplasmic inclusion with high magnification is shown in (h). Neuronal/glial inclusions and threads positive for p-tau are frequently visible in the locus coeruleus (k). Anterior horn cells (l) and anterior roots (m) in the cervical cord show positive staining for p-tau. KB staining (d); HE staining (e, i, j); immunohistochemistry for p-tau (f–h, k–m). Scale bars: 5 mm in (d), 500 µm in (e), and 100 µm in (f, g, i–l), 50 µm in (m), and 25 µm in (h)