Associated Diseases

[Home] [Intro] [Updates]

Prions are the controversial causes of the Transmissible Spongiform Encephalopathies (TSE) that effect both humans and animals as described below. The characteristic pathology of TSEs is a vacuolization of the brain causing holes such that the brain has a sponge-like appearance. Proliferation of astrocytes, destruction of neurons, and the formation of plaques are other common features. Dementia and other neurological abnormalities are the most common manifestations in all of these TSEs, always resulting in death.

Human Diseases

Creutzfeldt-Jakob disease

CJD effects 1-2 million people globally, most of whom are between 50-70 years old. Progressive dementia and loss of coordination are the most recognizable features of the disease. It is most often sporadic often by horizontal transmission, but is inherted in 10-15% of the cases. A new variant (vCJD) was identified in 1994, as infecting adolescents in Britain. Plaques and spongiform regions were found on their brains.

For more details, check out the following websites:

The link between Bovine Spongiform Encephalopathy and Creutzfeldt-Jakob Disease
Clinical Identification of CJD
Comprehensive definitions and articles for CJD (from Medline)

Kuru

One of the more well known prion diseases, Kuru, was most likely caused by the ritual of eating the brains of the dead in New Guinea. When this practice ended, so did the occurance of the disease. Kuru caused behavioral problems, loss of coordination and dementia.

For more details, check out the following websites:

Brief description of Kuru

Fatal Familial Insomnia

FFI is initially characterized by a difficulty sleeping then by dementia.

For more details, check out the following websites:

Brief description of Fatal Familial Insomnia

Gerstmann-Straussler syndrome

GSS mostly strikes 40-50 year olds at an extremely low rate. Loss of motor control is more common in GSS than is dementia. Just as CJD, GSS can be sporadic or inherited.

For more details, check out the following websites:

Brief description of GSS

Bovine Spongiform Encephalopathy (BSE)

BSE was the agent of Mad Cow Disease outbreak that peaked in Britain in 1992, and infected one million cattle. The source of the epidemic was prion infected meat and bone meal fed to the cattle. Since the outbreak, massive preventive measures have been established and strengthened. A strain thought to be a CJD strain was later found to be a BSE variant in humans. It is referred to as new variant CJD or variant CJD (vCJD).

For more details, check out the following websites:

More Information on Mad Cow Disease
Additional information on Mad Cow Disease

[Home] [Intro] [Updates]