The spatial pattern of the vacuolation (?spongiform change?) in the cerebral cortex in variant Creutzfeldt-Jakob disease (vCJD)

2002 ◽  
Vol 30 (1) ◽  
pp. 43-50 ◽  
Author(s):  
R. A. Armstrong ◽  
N. J. Cairns ◽  
J. W. Ironside ◽  
P. L. Lantos
Keyword(s):  
Cerebral Cortex ◽  
Spatial Pattern ◽  
Spongiform Change ◽  
Jakob Disease

scholarly journals Laminar Distribution of the Pathological Changes in Sporadic and Variant Creutzfeldt-Jakob Disease

2011 ◽  
Vol 2011 ◽  
pp. 1-7
Author(s):  
R. A. Armstrong
Keyword(s):  
Cerebral Cortex ◽  
Prion Diseases ◽  
Neuronal Loss ◽  
Pathological Changes ◽  
Spongiform Change ◽  
Protein Deposits ◽  
Laminar Distribution ◽  
Jakob Disease ◽  
Cortical Regions ◽  
The Brain

The laminar distributions of the pathological changes in the cerebral cortex were compared in the prion diseases sporadic Creutzfeldt-Jakob disease (sCJD) and variant CJD (vCJD). First, in some cortical regions, the vacuolation (“spongiform change”) was more generally distributed across the cortex in sCJD. Second, there was greater neuronal loss in the upper cortex in vCJD and in the lower cortex in sCJD. Third, the “diffuse” and “florid” prion protein () deposits were more frequently distributed in the upper cortex in vCJD and the “synaptic” deposits in the lower cortex in sCJD. Fourth, there was a significant gliosis mainly affecting the lower cortex of both disorders. The data suggest that the pattern of cortical degeneration is different in sCJD and vCJD which may reflect differences in aetiology and the subsequent spread of prion pathology within the brain.

scholarly journals Teaching NeuroImages: Cerebral cortex swelling in Creutzfeldt-Jakob disease with V180I mutation

Neurology ◽  
2018 ◽  
Vol 91 (2) ◽  
pp. e185-e186
Author(s):  
Atsuhiko Sugiyama ◽  
Minako Beppu ◽  
Satoshi Kuwabara
Keyword(s):  
Cerebral Cortex ◽  
Jakob Disease

Changes in N-acetylaspartate and myo-inositol detected in the cerebral cortex of hamsters with Creutzfeldt-Jakob disease

1998 ◽  
Vol 16 (8) ◽  
pp. 963-968 ◽  
Author(s):  
K.L. Behar ◽  
R. Boucher ◽  
W. Fritch ◽  
L. Manuelidis
Keyword(s):  
Cerebral Cortex ◽  
Jakob Disease

scholarly journals Network mediation of pathology pattern in sporadic Creutzfeldt–Jakob disease

2020 ◽  
Vol 2 (1) ◽  
Author(s):  
Benjamin Freeze ◽  
Pedro Maia ◽  
Sneha Pandya ◽  
Ashish Raj
Keyword(s):  
Spatial Pattern ◽  
Regional Distribution ◽  
Structural Connectivity ◽  
Brain Regions ◽  
Diffusion Weighted Mri ◽  
Progressive Dementia ◽  
Regional Patterns ◽  
Diffusion Weighted ◽  
Jakob Disease

Abstract Sporadic Creutzfeldt–Jakob disease is a rare fatal rapidly progressive dementia caused by the accumulation and spread of pathologically misfolded prions. Evidence from animal models and in vitro experiments suggests that prion pathology propagates along neural connectivity pathways, with the transmission of misfolded prions initiating a corruptive templating process in newly encountered brain regions. Although particular regional patterns of disease have been recognized in humans, the underlying mechanistic basis of these patterns remains poorly understood. Here, we demonstrate that the spatial pattern of disease derived from publicly available human diffusion-weighted MRI data demonstrates stereotypical features across patient cohorts and can be largely explained by intrinsic connectivity properties of the human structural brain network. Regional diffusion-weighted MRI signal abnormalities are predicted by graph theoretical measures of centrality, with highly affected regions such as cingulate gyrus demonstrating strong structural connectivity to other brain regions. We employ network diffusion modelling to demonstrate that the spatial pattern of disease can be predicted by a diffusion process originating from a single regional pathology seed and operating on the structural connectome. The most likely seeds correspond to the most highly affected brain regions, suggesting that pathological prions could originate in a single brain region and spread throughout the brain to produce the regional distribution of pathology observed on MRI. Further investigation of top seed regions and associated connectivity pathways may be a useful strategy for developing therapeutic approaches.

scholarly journals Unilateral Creutzfeldt-Jakob Disease Presenting as Rapidly Progressive Aphasia

1994 ◽  
Vol 21 (4) ◽  
pp. 350-352 ◽  
Author(s):  
Andrew Kirk ◽  
L.C. Ang
Keyword(s):  
Left Hemisphere ◽  
Right Hemisphere ◽  
Molecular Layer ◽  
Neuronal Loss ◽  
Occipital Cortex ◽  
Spongiform Change ◽  
History Of ◽  
Jakob Disease ◽  
The Right ◽  
Ct And Mri

Abstract:A 64-year-old man presented with a three day history of progressive Broca’s aphasia, followed within 3 weeks by exclusively right-sided myoclonus, rigidity, and dystonia. Within 4 weeks he was globally aphasie. He died within 7 weeks of onset. In the final week, rigidity and myoclonus became bilateral. CT and MRI were normal. SPECT showed diminished perfusion of the left hemisphere. EEG showed periodic discharges on the left. At autopsy, there were marked cortical spongiform change, neuronal loss, and gliosis throughout the left hemisphere and in the right occipital cortex. Elsewhere in the right hemisphere, spongiform change was non-existent to minimal. There was moderate spongiform change in the molecular layer of the cerebellar cortex, much more marked on the left. Clinical and pathological unilateral cerebral predominance extended to the ipsilateral cerebellum. Creutzfeldt-Jakob disease is an important consideration in patients with rapidly progressive unilateral cerebral signs associated with a movement disorder.

scholarly journals Quantitative Study of Spongiform Change in Putamen of 24 Cases of Creutzfeldt-Jakob Disease

2004 ◽  
Vol 63 (3) ◽  
pp. 193-198 ◽  
Author(s):  
Lydie Truchot ◽  
Anna Bencsik ◽  
Armand Perret-Liaudet ◽  
Anne-Gaëlle Biacabe ◽  
Marlène Richard ◽  
...  
Keyword(s):  
Quantitative Study ◽  
Spongiform Change ◽  
Jakob Disease
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