Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease

The deadly Creutzfeldt-Jakob disease — human form of the ‘mad cow’ disease — may spread through touch, say scientists who found that an abnormal protein linked to the fatal infection can be detected from the skin of patients. Scientists from National Institutes of Health (NIH) and Case Western Reserve University in the United States have detected abnormal prion protein in the skin of nearly two dozen people who died from Creutzfeldt-Jakob disease (CJD).
The scientists also exposed a dozen healthy mice to skin extracts from two of the CJD patients, and all developed prion disease.

CJD is an incurable and ultimately fatal transmissible, neuro-degenerative disorder in the family of prion diseases.
Prion diseases originate when normally harmless prion protein molecules become abnormal and gather in clusters and filaments in the human body and brain.
The reasons for this process are not fully understood.
The accumulation of these clusters has been associated with tissue damage that leaves sponge-like holes in the brain.
Human prion diseases include fatal insomnia; kuru; Gerstmann-Straussler-Scheinker syndrome; and variant, familial and sporadic CJD.
Sporadic CJD is the most common human prion disease, affecting about one in one million people annually worldwide.
Other prion diseases include scrapie in sheep; chronic wasting disease in deer, elk and moose; and bovine spongiform encephalopathy, or mad cow disease, in cattle.

Most people associate prion diseases with the brain, although scientists have found abnormal infectious prion protein in other organs, including the spleen, kidney, lungs and liver.

“There is no evidence that transmission can occur in real-world situations via casual skin contact.
The study also raises the possibility of using skin tissue samples to test for human and animal prion diseases.
The test is widely used with samples of brain and spinal-fluid for the diagnosis of CJD, but such samples are not always available.

Source:TH