Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rare disease that causes fast deterioration of an affected person’s brain. As this condition worsens and damages your brain, it causes dementia-like symptoms.

With CJD, faulty proteins, known as prions, build up in your brain cells, damaging and destroying those cells. The condition is very severe, and its effects develop and worsen quickly. It’s ultimately fatal, and unfortunately, there’s no way to cure, treat or even slow down the progress of this disease.

Who does it affect?

Creutzfeldt-Jakob (pronounced “croy-tz-felt ya-cob”) disease typically affects people between ages 50 and 80, becoming more common as you get older. However, the genetic subtype of CJD usually happens earlier, most often between ages 30 and 50. It affects men and women at equal rates overall.

Variant CJD

One of the subtypes of CJD, known as variant CJD (vCJD), affects people who ate beef that came from cattle infected with bovine spongiform encephalopathy (BSE).

How common is this condition?

CJD is very rare, with 1 to 2 cases happening in every 1 million people worldwide. About 350 people receive a diagnosis of CJD in the U.S. each year.

What happens when you have Creutzfeldt-Jakob disease?

CJD is a degenerative brain disease, meaning it causes damage to your brain that worsens over time. Experts classify it as a “transmissible spongiform encephalopathy” (TSE). The incubation period, which is the time it takes from when you first get the disease to when it starts causing symptoms, can range from months to years.

TSE conditions cause small pockets, or clusters, of damage throughout your brain. This damage causes your brain to look like a sea sponge or to have many holes or hollows like Swiss cheese.

As this brain damage happens, you lose the abilities controlled in the affected area. Overall, people with CJD develop a wide range of symptoms, including memory loss, problems thinking, uncontrolled muscle spasms or movement difficulties, and more. CJD is ultimately fatal because of how much damage it causes.