Fig. 4

Autopsy findings of neuronal intranuclear inclusion disease (NIID) pathology. Small hyaline-like inclusions were observed in the nucleus of the posterior ganglion cell (black arrow) (a). The motor neurons harboring intranuclear (red arrows) (b) and intracytoplasmic (c) inclusions that were immunopositive for p62 are shown. p62-positive intranuclear inclusions in astrocytes, around vacuole formation in the white matter of precentral gyrus (black arrowheads) (d). Periventricular ependymal cells have high prevalence of p62-positive intranuclear inclusions (e). Intranuclear inclusions immunopositive for p62 were observed in the astrocytes (black arrowheads) and oligodendrocytes (red arrowhead) in the spinal cord (f). The photograph (g) shows p62-positive intranuclear inclusions in glial cells of the cerebellar cortex (black arrowhead) and Purkinje cells (thin red arrows). In addition to the intranuclear inclusions (black arrowhead), intracytoplasmic glial inclusions immunopositive for p62 (thin black arrow) were occasionally found in the severely affected white matter lesions in the cerebellum (h). Normal (i) and demyelinated (j) white matter of the cerebellum are shown in KB stains, square areas in (k), which are a schematic representation at the level of the dentate nucleus. Scale bars = a-d, f–h 20 μm, e 10 μm, i, j 200 μm