Creutzfeldt-Jakob disease (CJD)
spongiform encephalopathy (TSE)
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.
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 which
causes cell death and the resulting brain lesions.
TSE fact sheet (PDF, 80 KB)
for Disease Control and Prevention Prion diseases - CDC (Exit DHS)
Information for health professionals
This is a Wisconsin disease surveillance category II disease:
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 or by other means within 72 hours
upon recognition of a case.
Information on communicable disease reporting
Wisconsin case reporting
and public health follow-up guidelines: CJD,
Prion dementia information for Wisconsin medical providers Contacts
Local Health Departments - Regional offices - Tribal agencies
November 19, 2014