Figure 3.
TEM in cerebellum. (A) Granular layer neurons in a 3 year-old male. A few, vacuolar (*) spaces are seen between neurons. (B) One Purkinje cell is surrounded by glial and neuronal bodies. There is a significant number of vaculated spaces (*), particularly towards the molecular layer (upper third) where axons are identified. Same 3 year-old as (A). (C) Close-up of Golgi apparatus and dilated endoplasmic reticulum (ER) in a MMC 11 year-old child. Notice the lysosomal structure with nanoparticles (arrow). (D) Purkinje neuron cytoplasm exhibiting mitochondria with abnormal cristae and two distinct spherical NPs (arrows). The ER is markedly dilated. (E) Fourteen-year-old Purkinje cell nucleus with three spherical NPs (arrows) in the midst of heterochromatin. The double nuclear membrane is apparently intact along the white arrow heads but disappears under the black arrow heads. (F) Granular cell layer in a 36 year-old male shows a significant number of neuropil vacuolated areas (*) and a marked contrast between an apparently intact nucleus (arrow) and two nuclei with chromatin condensation, nuclear shrinkage, and the formation of apoptotic bodies (opened arrows). (G) A close-up of (F) shows the empty spaces between neurons (*) and the large lysosomal bodies (arrow heads) in the granular cells, seen also in (H). (H) These two neurons have already marked cytoplasmic changes with fragmented organelles and ill-defined cell membranes. (I) A conglomerate of lysosomal structures (arrow) with NPs (arrow heads). (J) In the same sample, two mitochondria with ill-defined membranes (black arrow heads) and apparently intact double membranes (white arrow heads). (K) Cerebellar blood vessel in a 45 year-old male with significant vacuolization and fragmentation of the perivascular neuropil (*). Notice the endothelial cell (EC) and the vessel lumen. (L) A close-up of the same vessel to show the erythrophagocytosis of the endothelium. The arrow points to a NP trapped between the erythrocyte engulfed by the endothelial cell and the endothelial coverture. The multiple *s point to the NVU breakdown.