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Case Reports
. 2011 Aug;37(5):554-9.
doi: 10.1111/j.1365-2990.2011.01174.x.

A case of Gerstmann-Sträussler-Scheinker disease with a novel six octapeptide repeat insertion

A Vital, J-L Laplanche, J-R Bastard, X Xiao, W-Q Zou, C Vital
Case Reports

A case of Gerstmann-Sträussler-Scheinker disease with a novel six octapeptide repeat insertion

A Vital et al. Neuropathol Appl Neurobiol. 2011 Aug.
No abstract available

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Figures

Fig. 1
Fig. 1
a Haematoxylin-eosin stained section of the cerebellum showing multicentric amyloid plaques in the molecular layer, each surrounded by radially arranged bundles (insert); b these deposits are fluorescent under UV light after thioflavine S staining; c they are stained by Congo red, d with a yellow-green birefringence; e PrP-immunostaining is strongly positive on the periphery of amyloid plaques; f GFAP-positive astrocytic gliosis is marked in the molecular layer of the cerebellum (bar a, e, f 80 μm; b 65 μm; a insert, c, d 40μm)
Fig. 2
Fig. 2
A: Western blot analysis of insoluble PrP on brain homogenates labeled as 1 (frontal cortex), 2 (temporal cortex), 3 (occipital cortex), and 8 (cerebellum). A 5 μl brain homogenate each was detected by Western blotting with 3F4 after treated with or without proteinase K (PK) at 50 μg/ml at 37°C for 1 h. T1: PrPSc type 1 and T2: PrPSc type 2 from sporadic Creutzfeldt-Jakob disease. B: Less exposed film showing a better gel profile of PrP from the cerebellum (8) treated with or without PK.
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