case_images: PMC5523543_01
case_images: Overview of main histopathological findings. (a) and (b) Placental histopathological results showing a rather loose villous stroma with numerous foamy/vacuolated Hofbauer cells. Periodic Acid-Schiff (PAS) stain; original magnifications: x200 (a) and x1000 (b).
case_images: CRIPE2017-9523427.001.jpg
case_images: PMC5523543_01_CRIPE2017-9523427.001.jpg
case_images: fig1
case_images:  Microscopic examination showed that the main histopathological finding was that placental villous stroma was rather loose and, remarkably, presented numerous foamy/vacuolated Hofbauer cells (Figures 1(a) and 1(b)).
case_images: Major neuropathological findings in our case of Sly disease. ((a) and (b)) Histopathological sections from the brain showing numerous neuronal cells with remarkably foamy/vacuolated perikaryonal cytoplasm (arrow-heads; yellow) in the striatum of this 25-year-old fetus. Note also the presence of PGBs (arrows in (b)) in the neuropil. (b), (c), (d), (e), (f), and (g) illustrate the histomorphological appearance and staining characteristics of the many PGBs (some of which indicated by arrows) detected in various brain structures of this fetus, namely, the incipient cerebral white matter ((c) and (d)), the developing cortical plate of the neocortex (e), the telencephalic periventricular germinal zone (f), the striatum (b), and the developing cerebellum (g). This figure, besides, shows that at all levels of the brain, all PGBs appear to be deposited in the neuropil, and none actually look clearly intracellular with any of the various histopathological techniques we employed ((c), (d), (e), (f), and (g)). ((a) through (g)) Light microscopic photographs, Cresyl-violet (Nissl) staining ((a), (b), (e), and (f)), Hematoxylin-Eosin staining (g), and Periodic Acid-Schiff/D-amylase (PAS-D) staining ((c) and (d)). Original magnification: x400 ((a), (b), (d) and (f)), x250 (c), x500 (e), and x320 (g).
case_images: CRIPE2017-9523427.002.jpg
case_images: PMC5523543_01_CRIPE2017-9523427.002.jpg
case_images: fig2
case_images: Remarkably, most neuronal cells in the cerebral deep gray matter, notably the striatum, presented a foamy perikaryonal cytoplasm (Figures 2(a) and 2(b)). These structures thus presented staining and histomorphological characteristics typical of PGBs (Figures 2(b), 2(c), 2(d), 2(e), 2(f), and 2(g)). Topographically, PGBs were detected in the different layers of the developing cerebral wall: they were more abundant in the cerebral WM (Figures 2(c) and 2(d)). Many PGBs were, however, also seen in the cortical plate of the developing neocortex (Figure 2(e)), in the deep cerebral gray matter (Figure 2(b)), and in the telencephalic periventricular germinal zone (Figure 2(f)), though in lesser numbers than in the WM. A few were also found in the allocortex/hippocampal formation and in the cerebellum (Figure 2(g)). It is noteworthy that at all levels of the brain examined in this unique case, practically all these PGBs, appeared to be deposited in the neuropil of the various sites described, and none actually looked clearly intracellular as portrayed with the various histopathological techniques we employed (Figures 2(c), 2(d), 2(e), 2(f), and 2(g)).