- —Creutzfeldt-Jakob Disease
- human spongiform encephalopathy that results from an infection by a prion
- transmission has been traced to inoculations by injections of human growth hormone, transplantation of corneas, and implantation of cerebral electrodes
- butchers and meat handlers are at greater risk of contracting the disease
- variant ;bovine spongiform encephalopathy (so-called mad cow disease).
- The infective prion is a proteinaceous particle that contains little or no nucleic acid.
- The disease occurs in adults in their late 50s.
- —Cortical gray matter involvement without cerebral atrophy may represent an early phase of the disease.
- —[In contrast to Creutzfeldt-Jakob disease, wherein bilateral involvement of the corpus striata and thalami is seen on the imaging studies, the bovine spongiform encephalopathy is characteristically demonstrated by bilateral thalamic pulvinar hyperintensity on T2-weighted and FLAIR images
abnormal mri brain Images > A. FLAIR image demonstrates hyperintensity in both thalami, in the periatrial white matter, and in the anterior cingulated gyri. B, Diffusion-weighted image shows restricted diffusion in both thalami and cingulated gyri