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A quantitative study of the pathological changes in the cerebellum in 15 cases of variant Creutzfeldt–Jakob disease (vCJD)
Author(s) -
Armstrong R. A.,
Ironside J. W.,
Lantos P. L.,
Cairns N. J.
Publication year - 2009
Publication title -
neuropathology and applied neurobiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.538
H-Index - 95
eISSN - 1365-2990
pISSN - 0305-1846
DOI - 10.1111/j.1365-2990.2008.00979.x
Subject(s) - pathology , cerebellum , cerebellar cortex , purkinje cell , vacuole , granular layer , deep cerebellar nuclei , thalamus , pathological , biology , cellular pathology , white matter , anatomy , medicine , neuroscience , biochemistry , magnetic resonance imaging , cytoplasm , radiology
Aims : To determine in the cerebellum in variant Creutzfeldt–Jakob disease (vCJD): (i) whether the pathology affected all laminae; (ii) the spatial topography of the pathology along the folia; (iii) spatial correlations between the pathological changes; and (iv) whether the pathology was similar to that of the common methionine/methionine Type 1 subtype of sporadic CJD. Methods : Sequential cerebellar sections of 15 cases of vCJD were stained with haematoxylin and eosin, or immunolabelled with monoclonal antibody 12F10 against prion protein (PrP) and studied using spatial pattern analysis. Results : Loss of Purkinje cells was evident compared with control cases. Densities of the vacuolation and the protease‐resistant form of prion protein (PrP Sc ) (diffuse and florid plaques) were greater in the granule cell layer (GL) than the molecular layer (ML). In the ML, vacuoles and PrP Sc plaques occurred in clusters regularly distributed along the folia with larger clusters of vacuoles and diffuse plaques in the GL. There was a negative spatial correlation between the vacuoles and the surviving Purkinje cells in the ML. There was a positive spatial correlation between the vacuoles and diffuse PrP Sc plaques in the ML and GL. Conclusions : (i) all laminae were affected by the pathology, the GL more severely than the ML; (ii) the pathology was topographically distributed along the folia especially in the Purkinje cell layer and ML; (iii) pathological spread may occur in relation to the loop of anatomical connections involving the cerebellum, thalamus, cerebral cortex and pons; and (iv) there were pathological differences compared with methionine/methionine Type 1 sporadic CJD.

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