Information from Internist, Dr. Zoë Rammelkamp
Creutzfeldt-Jakob Disease is a rare neurodegenerative disease that is devastating and fatal. Symptoms include rapidly progressive memory loss, personality and behavioral changes, and myoclonus (involuntary muscle twitching and jerking). CJD is caused by an abnormal prion protein, which is a defective and destructive protein that replaces the normal prion proteins in the brain. For that reason, it causes rapid neurologic decline.
There are several subtypes of CJD:
The most common subtype is sporadic CJD
This is where the cause of CJD is unknown. Although sporadic CJD is the most common subtype of CJD, it is still very rare: about 1 in a million cases per year. Usually, it is middle-aged people who are affected by sporadic CJD. Sadly, median survival is about 3 to 4 months.
The other subtypes of CJD include variant CJD, genetic CJD, and iatrogenic CJD
Variant CJD is linked to bovine spongiform encephalopathy (BSE), also known as Mad Cow Disease. BSE is also a prion disease, but it affects cattle. It is thought that some cases of variant CJD occurred due to the ingestion of contaminated beef. The number of cases of variant CJD due to contaminated beef is unknown as there is a long incubation period (years) between ingestion of the beef and the development of symptoms. Additionally, a few cases of variant CJD have been linked to blood transfusion. Of note, there have been just about 200 cases of variant CJD since the 1990s, so it is incredibly rare (for reference, there have been about 1500 cases of sporadic CJD in a similar time frame).
Genetic CJD and iatrogenic CJD are also incredibly uncommon, but they do exist. Genetic CJD is caused by a genetic mutation that creates the abnormal prion protein.
Iatrogenic CJD refers to the spread of the abnormal prion protein through medical procedures. This is very rare, and medical professionals go through great efforts to sterilize all medical equipment. Similar to variant CJD, there can be a long incubation period, so all recent cases seem to be linked to surgical procedures from several decades ago.
Overall, CJD is a lethal disease that has no cure. However, it is rare, and numerous policies are in place to prevent the occurrence of variant CJD.
If you are concerned, please reach out to us or your trusted medical provider.