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12b

12b. Immunocytochemistry for prion protein in the cerebellum in kuru shows intense labelling of the kuru plaques in the molecular layer and granular layer, and in addition shows multiple smaller plaque-like structures within the granular layer and a fine background granular/synaptic pattern of accumulation.

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12a

12a. The cerebellum in kuru shows moderate spongiform change in the molecular layer with a large kuru plaque composed of a fibrillary amyloid structure with a dense eosinophilic core and a paler periphery within the molecular layer (lower centre). H&E stain.

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11d

11d. Immunocytochemistry for prion protein in the cerebral cortex in a case of iCJD in a
human dura mater graft recipient shows a widespread granular/synaptic pattern of accumulation, with no plaque-like structures identified.

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11b

11b. Immunocytochemistry for prion protein in the cerebellum in a case of iCJD in a human growth hormone recipient shows intense labelling of kuru plaques in the granular layer and molecular layer, with smaller plaque-like aggregates also present.

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11a

11a. The cerebellum in a case of iCJD in a human growth hormone recipient shows occasional small kuru plaques (centre) within the granular layer and mild spongiform change in the
molecular layer (right) H&E stain.

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10b

10b. Immunocytochemistry for glial fibrillary acidic protein in fatal familial insomnia shows intense labelling of the reactive astrocytes within the affected regions of the thalamus. No significant spongiform change is present and Immunohistochemistry for prion protein
in this region of the brain gives a negative reaction.

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10a

10a. The thalamus in fatal familial insomnia shows intense neuronal loss and gliosis, particularly in the medial and dorsal nuclei. There is no spongiform change present and no amyloid plaques are evident. H&E stain.

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9b

9b. Immunocytochemistry for prion protein in GSS (P102L) shows intense labelling of the large multicentric plaques which can be identified the molecular layer, the granular layer and even in the subcortical white matter.

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7h

7h. Immunocytochemistry for prion protein in the putamen in variant CJD shows numerous  small rounded aggregates often arranged in a linear distribution, apparently  representing peri-axonal accumulation. No florid plaques are present in this field.

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7f

H&E stain. 7f. Immunocytochemistry for glial fibrillary acidic protein in the pulvinar in variant CJD shows intense labelling of the large reactive astrocytes. A minor degree of vacuolation is present in the upper of this image, but no florid plaques are present.

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7e

7e. The thalamus in variant CJD shows severe neuronal loss and gliosis, particularly in the pulvinar and in the dorsomedial nucleus. There is very little spongiform change and florid plaques are not present.

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7d

7d. Immunocytochemistry for prion protein in the cerebellar cortex in variant CJD shows intense staining of the florid plaques and the smaller plaques in the granular layer, with some amorphous accumulation in the molecular layer.

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7c

7c. The cerebellar cortex in variant CJD contains florid plaques in the molecular (centre), surrounded by spongiform change. There is widespread vacuolation in the molecular layer, and numerous amyloid plaques are also present in the granular layer. H&E stain.

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7b

7b. Immunocytochemistry for PrP in the cerebral cortex in variant CJD shows intense labelling of the large florid plaques, but also demonstrates multiple smaller plaque-like structures not evident on the H&E stain. In addition there is a widespread amorphous accumulation of PrP around smaller neurons and occasional blood vessels.

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7a

7a. The characteristic pathological feature in variant CJD is the florid plaque (centre) composed of large radiating fibrils of amyloid with a dense core and paler periphery, surrounded by a halo of spongiform change. Multiple smaller plaques are present elsewhere in this image and there is severe neuronal loss with accompanying astrocytosis. H&E stain.

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6b

6b. Immunocytochemistry for prion protein in the cerebral cortex of the VV2 subtype of sCJD shows a characteristic perineuronal and granular/synaptic pattern of accumulation, with decoration of large pyramidal neurons and their apical ascending dendrites (centre) in layer 5 of the cortex.

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4b

4b Immunocytochemistry for prion protein in the MV2 subtype of sCJD shows intense labelling of the kuru plaques in the granular layer of the cerebellum (centre). Numerous smaller plaque-like structures not evident on the H&E stain are also visualised by this technique.

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4a

4a The cerebellum in the MV2 subtype of sCJD shows characteristic small kuru plaques (centre and bottom) in the granular layer. Spongiform change is present to a mild degree in the molecular layer (right). H&E stain.

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3b

3b. Immunocytochemistry for prion protein in the MM2 (cortical) subtype of sCJD shows widespread perivacuolar accumulation. A fine background granular/synaptic pattern of abnormal prion protein accumulation is also evident.

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3a

3a. The MM2 (cortical) subtype of sCJD is characterised by widespread confluent spongiform change, where large-cyst like structures are present in the neuropil in an irregular distribution.
The size of these vacuoles is much larger that in the MM1 subtype of sCJD (see figure 1A). H&E stain.

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2b

2b. Immunohistochemistry for glial fibrillary acidic protein shows the widespread reactive
astrocytosis present in the affected thalamic nuclei in the MM2 (thalamic) subtype of sCJD. There is relatively little spongiform change present in this tissue.

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2a

2a. Severe neuronal loss and gliosis in the MM2 (thalamic) subtype of sCJD is a typical neuropathological finding, most evident in the medial and anterior thalamic nuclei. There is relatively little spongiform change present in the affected areas. H&E stain.