Stephen J. Schueler, M.D.

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Jakob-Creutzfeldt Disease Underlying Cause

Prion proteins occur in both a normal form, which is a harmless protein found in the body's cells, and in an infectious form, which causes disease. The infectious form of the protein has a different folded shape than the normal form. Once the abnormal prions appear, they clump together within cells and somehow trigger cell death.

There are three major categories of CJD:

  • Sporadic Creutzfeldt-Jakob disease:
    • Most common type
    • Appears with no known risk factors
  • Hereditary Creutzfeldt-Jakob disease:
    • Transferred genetically
    • Family history of the disease
  • Acquired Creutzfeldt-Jakob disease:
    • Rare
    • When acquired it is usually transmitted through certain medical procedures

Continue to Jakob-Creutzfeldt Disease Transmission

Last Updated: Jan 21, 2009 References
Authors: Stephen J. Schueler, MD; John H. Beckett, MD; D. Scott Gettings, MD
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PubMed Jakob-Creutzfeldt Disease References
  1. Beghi E, Gandolfo C, Ferrarese C, Rizzuto N, Poli G, Tonini MC, Vita G, Leone M, Logroscino G, Granieri E, Salemi G, Savettieri G, Frattola L, Ru G, Mancardi GL, Messina C. Bovine spongiform encephalopathy and Creutzfeldt-Jakob disease: facts and uncertainties underlying the causal link between animal and human diseases. Neurol Sci. 2004 Jul;25(3):122-9. [15300459]
  2. Glatzel M, Stoeck K, Seeger H, Luhrs T, Aguzzi A. Human prion diseases: molecular and clinical aspects. Arch Neurol. 2005 Apr;62(4):545-52. [15824251]
  3. Mallucci G, Collinge J. Update on Creutzfeldt-Jakob disease. Curr Opin Neurol. 2004 Dec;17(6):641-7. [15542971]
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