What is CJD?

CJD is a disease caused by a misfolding protein, called a prion. (PREE-on) Prion diseases are a group of rare and fatal brain diseases which occur in both humans and animals. In humans, it is known as CJD.

Cows get BSE, which stands for bovine spongiform encephalopathy. Deer and elk contract CWD, or chronic wasting disease. There is no cure or clinical diagnosis for CJD. There is no cure for any of the diseases in the prion family.

According the CJD Foundation, “The sporadic form accounts for approximately 85% of the cases, the familial form approximately 15%.” The Foundation also says, “Most of the cases are "classical" or "sporadic" CJD (sCJD), occurring for no, as yet, known reason.” When you hear people talking about vCJD, or variant CJD, they are talking about the form that affects typically younger patients and is, in fact, caused by eating beef tainted with BSE.

There is no treatment for CJD. Once it is determined that is what a patient has, doctors can only make every effort to keep the patient comfortable until death, which can come in a matter of days, weeks or months. The length of the disease depends on the mutation.

All prion disease are considered spongiform encephalopathies. Encephalopathy means “brain disease,” and “spongiform” means sponge-like. In other words, the brain becomes filled with holes and looks like a sponge under the microscope.

As the brain is being eaten by prions, the patient will shake, experience mycolonus, hallucinate, become incontinent, lose the ability to walk, lose the ability to speak, experience aphasia, and other symptoms that are very difficult for a family to watch.

My mother died one month and two days after being taken to the emergency room for what we thought were the symptoms of Parkinson’s disease or multiple sclerosis, maybe early-onset Alzheimer’s Disease. Never did we think she had a fatal disease that would kill her in weeks.