Pathological changes in the primary visual cortex (area V1) in sporadic Creutzfeldt-Jakob disease.
OBJECTIVE
To determine the degree of pathological change in the primary visual cortex (area V1) in patients with Creutzfeldt-Jakob disease.


METHODS
The vacuolation, surviving neurons, glial cells, and deposits of prion protein were quantified in area V1 obtained postmortem in nine cases of the sporadic type of Creutzfeldt-Jakob disease.


RESULTS
Variations in the density of glial cells and in prion protein deposition were particularly evident between patients. In the upper and lower cortical laminae, vacuoles and prion protein deposits were regularly distributed in clusters with a mean dimensions of 450 to 1000 microm. Vacuolation in area V1 was most severe in lamina III and the glial cell reaction in lamina V or VI. Surviving neurons were most abundant in lamina II or III, whereas prion protein deposition either affected all laminae equally or was maximal in lamina II or III.


CONCLUSIONS
The data suggest that pathological changes in area V1 in sporadic type of Creutzfeldt-Jakob disease may affect the transmission of visual information from area V1 to V2 and to subcortical visual areas. In addition, the data suggest an association between the developing pathology and the functional domains of area V1.
Armstrong
R A
RA
Vision Sciences, Aston University, Birmingham, United Kingdom. R.A.Armstrong@aston.ac.uk
D000368:Aged; D002452:Cell Count; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology*; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009457:Neuroglia / Q000378:metabolism / Q000473:pathology; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D011328:Prions / Q000378:metabolism; D014617:Vacuoles / Q000473:pathology; D014793:Visual Cortex / Q000473:pathology*
D011328:Prions