What is spongiform encephalopathy?

Mad cow (prion) dis. The brain in prion disease shows large distended vacuoles (bubbles) to the extent that under the microscope, the vacuoles are empty, giving the brain an appearance that is reminiscent of a sponge (or swiss cheese, for that matter).

Related Questions

What causes spongiform encephalopathy in a healthy person?

Several types. There are types thought to be transmissible via a protein known as a prion and others that are inherited or by a gene mutation. There is some thought that there may be infectious agent other than a prion. The most known via media exposure has been mad cow disease which is thought to be transmitted to humans if contaminated beef is consumed. Read more...

What causes the development of spongiform encephalopathy?

Prions. Prions are small abnormal proteins that can be transmitted by eating certain types of foods which usually contain parts of the central nervous system (i.e. The brain). The prions cause a reaction in the brain of the consumer which affect other normal proteins which leads to brain damage and progressive neuro-degeneration. Read more...

What is transmissible spongiform encephalopathies what are the treatments?

Prion diseases. . Transmissible spongiform encephalopathies (tses) are disease that affect the brain and nervous system. They are caused by unique pathogens called prions, which are abnormally folded proteins. Scrapie, bovine spongiform encephalopathy ("mad cow disease"), and kuru are examples. There is no approved treatments, but vaccines, gene therapies, and custom peptides are being investigated. Read more...

What is a brain with spongiform encephalopathy?

Prion disease. There are a group of degenerative brain disease that start as a subacute loss of brain function, with dementia, progressing to coma and death. These disease show by a loss of cells in the brain, but no evidence of inflammation. The brain has a "swiss cheese " under the microscope, they are called spongiform encephalopathies because of this. They are caused by infectious particles called prions. Read more...
Protein disease. Spongiform encephalopathies are diseases of the brain caused by abnormal proteins that cause holes to form in the brain, making it appear like a sponge (hence the name spongiform). Causes can be infectious, hereditary or sporadic. Symptoms include problems with personality changes, psychiatric issues, movement, gait, and memory. These diseases are degenerative and to date there is no cure. Read more...

How do you diagnose spongiform encephalopathy?

Brain biopsy. There are new CSF markers that can be tested if the fluid is sent to the national prion surveillance lab at case western (e.g., 14-3-3 and tau) but biopsy is still more likely to be definitive. Neither 14-3-3 nor tau is completely specific for bse. Read more...

Can transmissible spongiform encephalopathy be contracted?

Yes. Transmissible spongiform encephalopathy is caused by an infectious protein called prion. It can be transmitted through blood products or eating contaminated food or inflected organs such as brains. Read more...

Why is transmissible spongiform encephalopathy such a devastating thing?

Destroys most cortex. Tses cause rapidly progressive cerebrocortical destruction, leaving patients in a persistent vegetative state or dead. There is no effective intervention. Read more...

How is bovine spongiform encephalopathy cured?

It isn't. This is a disease of cattle (mad cow disease) that is fatal to them and may cause a variant of creutzfeldt-jakob disease in humans which has no cure and is also fatal. Read more...

How common is bovine spongiform encephalopathy?

< 1 per million cows. There are ~42 million head of cattle in the usa, and the best estimate is that there are between 4 and 7 infected animals. Read more...