Fig. 5

Autopsy findings. Autopsy findings of Case 1 and Case 2. The parts surrounded by the black squares in A and B are enlarged and shown in the upper left corner. A H&E stain of hippocampal CA1 of Case 1. The volume of the hippocampus was preserved. Eosinophilic intranuclear inclusions were observed chiefly in astrocytes. B Klüver-Barrera stain of Case 1 frontal lobe. Marked degeneration in the white matter was observed. C–H, J, and L–M Immunohistochemical staining with anti-p-62 antibody. I and K immunohistochemical staining with anti-ubiquitin antibody. C Neuron and astrocyte in the frontal lobe (Case 1). D Neuron in the frontal lobe (Case 2). E Purkinje cell and Bergmann glial cells (Case 1). F Neuron in Edinger–Westphal nucleus (Case 1). G Neuron in the spinal cord anterior horn (Case 1). H Dorsal root ganglion neuron (Case 1). I Dorsal root ganglion neuron (Case 2). J Sympathetic ganglion neurons (Case 1). K Schwann cells in the dorsal root of the spinal cord (Case 2). L Renal tubule cells in the kidney (Case 1). M Skeletal muscle cells in the iliopsoas (Case 1). p-62 positive or ubiquitin-positive intranuclear inclusion was observed in the neuron and astrocyte in the frontal lobe, Purkinje cells, spinal motor neurons, dorsal root ganglion (DRG), sympathetic ganglion neurons, Schwann cells, renal tubule cells, and skeletal muscle cells, but not in the neuron in the Edinger–Westphal nucleus. Scale Bars, A and C–M: 10 μm; B: 200 μm