Eating is firmly entrenched in many cultures, but sometimes, these traditions can have disturbing consequences.

The number of deaths and cases of CJD are both going up around the world right now. For instance, from 1970 to 2018, the number of sCJD cases in Australia went up. Classic CJD is known as sCJD, and the variant, usually develops because of eating meat from an animal infected with bovine spongiform encephalopathy (sometimes called Mad Cow Disease or BSE), is noted as vCJD.

In 2003, a cow brought in from Canada was the only animal in the United States that was found to have typical BSE. In 2023, six cases of unusual BSE in a cow were discovered.

The number of deaths in the US because of CJD is going up, with an average of 3.5 per million people over 50 years old dying each year, according to the Centers for Disease Control and Prevention. In the same way, the age-adjusted death rate from sCJD in Canada rose from 0.84 per million in 1998 to 1.30 per million in 2013.

The name for the deadly neurologic disease is Creutzfeldt-Jakob, and in its histologic presentations on a slide, it can and is often mistaken for Alzheimer's disease. I know this firsthand from working in a research center at a major hospital. We were doing work on Alzheimer's and had an agreement with several hospitals and nursing homes to provide brains from people who would donate them for research.

The man chosen for the job kept a white plastic bucket with a tight cover next to his desk and a beeper hooked on his belt. When the beeper went off, he had to jump into his car with the bucket and collect whatever was offered, no matter what time of day or night it might be.

On one collection journey, once he returned to the center, an expert in neuropathology noted something they hadn't realized: this brain didn't have Alzheimer's; the person had CJD, which is highly communicable. And we now had three CJD brains.

I learned that, in the operating room, if they were performing some brain procedure on a patient with CJD, all the instruments were to be incinerated, not just put into an autoclave. This is truly a dangerous virus, and no treatment exists for it.

Symptoms of CJD

Usually, the symptoms get worse in a few weeks to a few months. Some early signs are:

Changes in personality
Loss of memory
Having trouble thinking
Vision problems or going blind
Not sleeping
Having trouble coordinating
Have trouble talking
Not able to swallow
Moves quickly and jerkily
Most people die within a year

A look at death records showed that the number of people in the US dying from a prion disease, such as CJD, which spreads quickly and kills everyone, rose steadily from 2007 to 2020.

In that time period, there were 5,882 CJD codes on death records, and 51.2% of those cases were women. The study found that the recorded cases of CJD have gone up and that older people and women are more likely to get it. Research from Japan found similar trends. The changes may be due to changing demographics, but they could also be because new screening tools like MRI are better at finding CJD.

Papua New Guinea’s Fore and nearby language groups had a high incidence of the prion disease Kuru, similar to CJD. It reached its peak in the late 1950s. Transumption, or eating dead family members, happened at funeral feasts, where it was passed on.

From the village of Uwami in New Guinea, the disease spread through marriage with women from surrounding tribes. Through love and respect for the dead person and their family, it was a religious practice with rituals that went along with it. Since transumption was mostly engaged in by women and children, it makes sense these groups had a high level of disease.

You probably will not get CJD. It is not possible for coughing or sneezing to spread the disease. Touching or sexual contact is also not a way to spread it.

Three things can cause CJD to happen:

On occasion, Creutzfeldt-Jakob disease seems to happen to most people for no clear reason. This kind, also known as random or sporadic CJD, makes up most of the cases.

Genetic inheritance. Not even 15% of people who have CJD have a family history of it. But, they might show changes in their genes linked to the disease.

By spreading virus. A very small number of people have gotten CJD after medical treatments. As part of these procedures, infected pituitary human growth hormone was injected into the patient. There were also skin and cornea grafts from people who had CJD.

Also, some people have gotten Creutzfeldt-Jakob disease after having brain surgery with an instrument that was contaminated.

For the most part, medical practices and improvements in medical diagnostic instrumentation have prevented the spread of CJD, and the careful inspection of meat is also one means of eradicating it. However, although it is unlikely that you will get it, there is a small instance of persons who develop CJD without any apparent source for the viral infection.