Fig. 2

Neuropathologic findings (a) Coronal section showing atrophy of the caudate nucleus. Klüver-Barrera staining (KB). b, c Neuronal loss with gliosis in the basal ganglia: moderate in the caudate nucleus b and mild in the putamen (c). d Diffuse myelin pallor in the frontal white matter. KB. e Mild neuronal loss in the frontal cortex, accentuated in the deep cortical layer. KB. f Loss of Purkinje cells with Bergmann gliosis (arrows) in the cerebellar cortex. HE staining. g Depleted immunoreactivity of calbindin-D28k in the cerebellar cortex. The cell body and dendrites of Purkinje cells are strongly stained in the control brain. Calbindin-D28k immunohistochemistry (IHC). h Numerous neurons possessing 1C2-positive diffuse staining in nuclei and the magnified image (inset). Sector CA1 of Ammon’s horn. 1C2-IHC. Ctrl, control; Pt, patient. Bar in a = 1 cm for a; 350 μm for b, c; 1.5 cm for d; 400 μm for e; 150 μm for f, h; and 200 μm for g