PMC3420586_01
(A-C) Macroscopic findings. Severe atrophy of the paleostriatum together with dilatation of the lateral ventricle is evident in the coronal sections, while the thickness of the cerebral cortex is relatively intact (A). The brainstem, the gray matter of the cerebellar hemisphere (B), and the spinal cord (C) also show marked atrophy.
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Gross examination of the brain (1300 g) revealed a highly atrophic brainstem, cerebellum, and spinal cord (Figures 1(B) and 1(C)), while the thickness of the cerebral cortex was almost intact (Figure 1(A)). Severe atrophy of the paleostriatum together with dilatation of the lateral ventricle was observed in coronal sections (Figure 1(A)). Additionally, the cerebellar hemisphere demonstrated fading in the gray matter (Figure 1(B)). On the other hand, no depigmentation of the substantia nigra or of the locus coeruleus was observed in sections of the brainstem (Figures 1(B) and 1(C)). 
(A-H) Histological and immunohistochemical findings. Interfascicular oligodendroglia with halos in the cytoplasm and astrocytes are observed along with nerve fibers (A, HE staining). alpha-synuclein staining reveals numerous alpha-synuclein-positive glial cytoplasmic inclusions (GCI) in the oligodendroglia in the cerebral cortex (B). In the midbrain, the oligodendroglia and astrocytes show severe cytogenic edema (C, HE staining). alpha-synuclein-positive GCI in the oligodendroglia is also seen in the midbrain (D). The medulla oblongata shows extensive neuronal loss with scant oligodendroglial cells (E, HE staining), where alpha-synuclein is positive in the cytoplasma (F). Neuronal loss and gliosis are observed in the spinal cord (G, HE staining). Some alpha-synuclein-positive GCI in the oligodendroglia and astrocytes (arrows) are seen in the spinal cord (H).
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The anti-alpha-synuclein antibody immunostained the glial and neuronal inclusions extensively, especially the cytoplasmic and nuclear inclusions in the oligodendroglia. alpha-synuclein-positive GCI in the oligodendroglia was occurred in the cerebral cortex, the midbrain, the medulla oblongata, and the spinal cord (Figures 2(B), 2(D), 2(F), and 2(H)).