What is the rare brain disease which causes sponge-like holes in the brain?

Two people are believed to have died from a rare and incurable brain disease within just a few months of each other in the United States.

According to health officials, three cases of Creutzfeldt-Jakob Disease (CJD) have been reported in Hood River County, a region in the northwestern state of Oregon, over the last eight months. This rare condition causes the brain to rapidly deteriorate, forming sponge-like holes that lead to severe neurological damage and, ultimately, death.

While only a handful of cases are reported each year in the US , the sudden appearance of multiple infections in a single county has raised red flags.

More from Health

In big relief to Chinese e-commerce firms, US slashes tariff on small parcels Parents deported, toddler held in US: How 2-year-old Maikelys was reunited with mother in Venezuela

With a third case still under investigation, experts are now scrambling to understand the possible answers to and whether others may be at risk.

“We’re trying to look at any common risk factors that might link these cases,” Hood River County Health Department director Trish Elliot told The Oregonian. “But it’s pretty hard in some cases to come up with what the real cause is.”

So, what exactly is Creutzfeldt-Jakob disease? How does it spread? And what’s the current situation in Hood River County? Here’s a closer look.

What is Creutzfeldt-Jakob disease?

According to the Mayo Clinic, Creutzfeldt-Jakob Disease (pronounced KROITS-felt YAH-kobe) is a rare but fatal brain disorder that causes a rapid form of dementia.

The disease is triggered by abnormal proteins known as prions. These prions build up in the brain over time and damage nerve cells, creating small sponge-like holes visible under a microscope. As the brain tissue breaks down, it leads to severe cognitive decline, movement problems, and eventually, death.

Editor’s Picks

1

The Maneka Gandhi column: We've pretended that Mad Cow Disease does not exist in India, it's time we acknowledge its fatality 2

UK economy beats expectations to grow 0.6% in February quarter

While CJD shares some similarities with Alzheimer’s disease , it progresses much faster and is almost always fatal within a year of diagnosis.

The condition gained widespread attention in the 1990s during the outbreak of variant CJD (vCJD) in the United Kingdom, which was linked to people eating beef from cattle infected with “mad cow disease.” However, the majority of CJD cases have no connection to beef consumption.

According to the US’ Centers for Disease Control and Prevention, approximately 500 to 600 new cases occur in the country each year.

How does it spread?

Unlike viruses or bacteria, CJD isn’t contagious in the usual sense. You can’t catch it through coughing, sneezing, or close contact with someone who has it. Instead, it can develop in three ways:

Sporadically: This is the most common form, occurring without any clear cause. In these cases, the disease seems to strike out of nowhere, which is why it’s called sporadic CJD. Around 85 per cent of all CJD cases fall under this category.

By inheritance: Fewer than 15 per cent of people with CJD have a family history. This form is linked to mutations in the PRNP gene, which is responsible for producing the prion protein. If the gene is altered, it can lead to the misfolding of prions that trigger the disease.

By contamination: Though extremely rare, CJD can also be transmitted through medical procedures involving contaminated instruments or tissue. For example, a few cases have been linked to the use of surgical tools or transplants involving infected tissue. However, modern protocols now require any potentially exposed instruments to be destroyed, drastically reducing such risks.

What are the symptoms?

According to the Mayo Clinic, Creutzfeldt-Jakob disease is characterised by changes in neurological abilities. Symptoms get worse quickly, usually within several weeks to a few months. Early symptoms include:

• Personality changes.

• Memory loss.

• Impaired thinking.

• Blurry vision or blindness.

• Insomnia.

• Problems with coordination.

• Trouble speaking.

• Trouble swallowing.

• Sudden, jerky movements.

What is the treatment?

Sadly, there is no cure for Creutzfeldt-Jakob Disease, and according to the CDC, classic CJD is always fatal—often within just a few months of the first symptoms appearing.

There’s currently no treatment that can slow or stop the disease’s rapid progression. Because of this, the focus is entirely on supportive care, aimed at making patients as comfortable as possible during the course of their illness.

This care can include medications to help ease symptoms like anxiety, depression, or physical pain. Antidepressants are sometimes used to manage emotional distress, while painkillers may be given to relieve discomfort.

As the disease progresses, many patients require full-time nursing care and help with basic tasks like feeding and mobility.

With input from agencies