Figure 2.

Neuropathological autopsy findings of the patient with frontotemporal dementia (1‐III‐9). (A) Frontal and temporal lobe atrophy was observed. (B) The bilateral internal segments of the globus pallidus exhibited atrophy and pigmentation. (C) Midbrain atrophy and dyspigmentation were observed. (D) The cytoplasm of the remaining neurons had numerous basophilic inclusions in the nuclei of the brainstem. (E) The cytoplasmic basophilic inclusions were also scattered in the globus pallidus. (F) Gallyas–Braak silver staining revealed numerous neuronal cytoplasmic inclusions and oligodendrocytic coiled bodies. However, few neurofibrillary tangles and senile plaques were observed. (G) The cytoplasmic inclusions stained positively with phosphorylated tau (AT8). Immunostaining also revealed AT8‐positive cytoplasmic vacuoles around inclusions. (H) The inclusions were positive for four‐repeat tau antibody. (I) The inclusions did not have three‐repeat tau immunoreactivity. (J) The inclusions of four‐repeat tau were also found in the anterior horn of the spinal cord. (K) Electron microscopy showed that neurons had many cytoplasmic vacuoles. (L) Neurofibrillary tangles were scattered in between cytoplasmic vacuoles.