Creutzfeldt-Jakob disease

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[edit] Discussion of Creutzfeldt-Jakob disease

  • Creutzfeldt-Jakob disease (CJD) is a transmissible spongiform encephalopathies (a prion disease) characterized by rapidly progressive dementia, cerebral atrophy, myoclonus and death.
  • CJD leads to spongiform degeneration of the brain, which is thought to be caused by the conversion of normal prion protein to proteinaceous infectious scrapie particles that accumulate in and around neurons and lead to cell death.
  • There are three different forms of CJD including the sporadic form, variant CJD and familial CJD. Sporadic CJD is the most common (85%). The clinical diagnosis of sCJD is based on clinical signs, characteristic results on electroencephalography (EEG), and detection of the protein 14-3-3 in the CSF.(2)

[edit] Imaging Findings for Creutzfeldt-Jakob disease

  • MR imaging findings of Creutzfeldt-Jakob disease that were reported previously are abnormal high-signal-intensity areas in the cerebral cortex and basal ganglia and rapidly progressive cerebral atrophy on T2-weighted images.
  • Diffusion-weighted imaging abnormalities for Creutzfeldt-Jakob disease include regions of abnormal high signal intensity in the cortex, caudate nucleus, putamen, and thalamus with a distribution pattern that does not correspond to that of the arterial circulation.
  • The cortical abnormalities may be unilateral or bilateral, diffuse or focal, and symmetric or asymmetric.
  • In early-stage Creutzfeldt-Jakob disease, high-signal-intensity abnormalities are most frequently observed in the cortex.

[edit] Images

Patient #1

[edit] See Also

[edit] External Links

[edit] References for Creutzfeldt-Jakob disease

1. Ryutarou Ukisu, Tamio Kushihashi, Eriko Tanaka, Maiko Baba, Nobutaka Usui, Hidefumi Fujisawa, and Hiroki Takenaka. Diffusion-weighted MR Imaging of Early-Stage Creutzfeldt-Jakob Disease: Typical and Atypical Manifestations. RadioGraphics 2006 26: S191-204S.

2. AJNR Am J Neuroradiol 28:1114 –18