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. 2019 Jan 3;7:1. doi: 10.1186/s40478-018-0656-4

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© The Author(s). 2019

Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Fig. 5 — Neuropathology of Case 1. The neuropathological analysis showed the presence of severe neuronal loss and spongiform changes in the cerebral cortex (a: frontal cortex, Haematoxylin-Eosin), associated with astrogliosis (b: frontal cortex, GFAP immunostaining). The pattern of PrP^Sc deposition was defined by diffuse, finely granular synaptic-like immunoreactivity (c: 3F4 immunostaining, frontal cortex). In the cerebellum, loss of Purkinje and very mild spongiosis in the molecular layer (d: Haematoxylin-Eosin), astrogliosis (e: GFAP immunostaining) and PrP build up were present: finely granular PrP deposits in the molecular layer and coarser spots in the granular layer (f: 3F4 immunostaining). The PrP deposits were not fluorescent after thioflavin S (not shown). Scale bars: in (a) = 100 μm (a, b, d and f are the same magnification); in (c) = 50 μm (c and e are the same magnification).