Fig. 2

Neuropathologic findings of patient II-2. On a coronal section of the left hemisphere, the basal ganglia and the frontotemporal cortex are not atrophied (a), whereas the subiculum shows mild atrophy (b). Severe discoloration is observed in the substantia nigra (c). The medullary reticular formation is atrophied (d, arrow). The fasciculus anterolateralis of the spinal cord shows tract degeneration, whereas the lateral column is spared (e, arrow). For comparison, photomicroscopies from a healthy control (60 years old, female) are also shown (f, g). The superficial layers of the temporal tip (h) demonstrate loss of neurons, astrogliosis, and spongiotic changes. The melanin-containing neurons are severely depleted in the pars reticulate of the substantia nigra (i). Ballooned neurons in the temporal cortices (j) and the anterior horn of spinal cord (k, arrow) are shown. d–g Klüver–Barrera staining and h–k hematoxylin–eosin staining were used. Scale bars: a–g 1 cm, h, i 100 μm, and j, k 20 μm