Creutzfeldt-Jakob Disease (CJD)

Transmissible Spongiform Encephalopathy (TSE)

General Information

CJD is a rare incurable disease of humans that affects the nervous system and results in rapidly progressive dementia, loss of motor control, paralysis and death. It is one of several related diseases called transmissible spongiform encephalopathies or TSEs for short. The term "encephalopathy" means the brain is affected and the term "spongiform" refers to the microscopic holes seen in the brain, giving it a sponge-like appearance.

Various animal species have distinct types of TSEs. In addition to CJD which affects humans, other TSEs include bovine spongiform encephalopathy (BSE, also known as "mad cow disease"), scrapie in sheep and chronic wasting disease (CWD) in deer and elk.

• Venison and CWD - What Hunters should Know – Wisconsin Department of Natural Resources
• Recommendations for reducing the spread of CWD – Wisconsin Department of Natural Resources

CJD is caused by an agent, called a prion, that is a self-replicating protein. The current theory is that the normal form of the prion, found in all people, is converted into an abnormal form that causes cell death and the resulting brain lesions.

• CJD, TSE fact sheet, P-42183
• Centers for Disease Control and Prevention (CDC): Prion diseases

Information for Providers

This is a Wisconsin disease surveillance category II disease:
Report to the patient's local public health department electronically, through the Wisconsin Electronic Disease Surveillance System (WEDSS), by mail or fax using an Acute and Communicable Disease case report, F-44151 (Word) or by other means within 72 hours upon recognition of a case.

Information on communicable disease reporting

Case Reporting and Investigation Protocol (EpiNet): P-01913, TSE, CJD (PDF)

Prion disease information for Wisconsin medical providers, P-01262 (PDF)

Reporting Prion Diseases for Infection Preventionists, P-01610 (PDF)