MRI scan of a CJD brain, showing its spongy appearance.
Look at the picture carefully!!
Are you able to spot those tiny winy holes in the brain??
Classic Creutzfeldt-Jakob disease (CJD) is a human prion disease and also a neurodegenerative disorder with characteristic clinical and diagnostic features. This disease is rapidly progressive and always deadly. Most importantly, we would like to state out a point that classic CJD is not related to "mad cow" disease. Infection with this disease leads to death usually within 1 year of onset of illness.
*Important Note: Classic CJD also is distinct from "variant CJD", another prion disease that is related to BSE.Four types of CJD
Cause of CDJ
- a specific protein called a prion, which lacks nucleic acids, resists proteolytic digestion, and spontaneously aggregates in the brain.
The tissue slide above displays sponge-like lesions in the brain tissue of a classic CJD patient. This lesion is typical of many prion diseases. Most cases are sporadic; 5% to 15% are familial, with an autosomal dominant pattern of inheritance.
In the present, there is only treatment for relieving symptoms. Current clinical trials are testing promising antiviral drugs such as amantadine, which has proven helpful in treating Parkinson's disease. Some patients taking these antivirals have shown brief periods of improvement, with no harmful side effects. However, no treatment has been discovered that stops CJD completely. It is invariably fatal, often within a year of the onset of symptoms.
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