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An expert notes several reasons why this could be happening.
By: George Citroner
In a medical mystery that has left researchers puzzled, an unexpected surge in cases of Creutzfeldt-Jakob disease (CJD), a rare and fatal degenerative condition, has emerged as a cause for concern.
The alarming trend is taking a disproportionate toll on seniors, particularly older women.
What Is CJD?
CJD is a rare brain disorder marked by distinct alterations and rapid deterioration of brain tissue that affect muscle coordination, cognition, and memory. It is caused by a transmissible pathogen known as a proteinaceous infectious particle, or prion. This pathogen induces abnormal folding of prions abundant in the brain, resulting in swift and severe brain damage, ultimately leading to death. Between 70 percent and 95 percent of individuals diagnosed with the condition die within a year.
Prions resist conventional methods used to treat other pathogens. They withstand extreme heat and radiation that are typically effective against bacteria and viruses and remain impervious to antibiotics and antivirals.
CJD is the predominant form of human prion disease, with approximately 85 percent of cases categorized as sporadic (spontaneous type) and 15 percent stemming from inherited mutations of the prion protein gene.
Unprecedented Increase in CJD Cases
Researchers analyzed death certificate data from 2007 to 2020, revealing an elevated incidence of CJD. Out of 5,882 CJD deaths during this period, slightly over 50 percent occurred among women, according to the research letter published in JAMA Neurology.
There was an increase in incidence from 1.06 to 1.58 per million cases in women and from 1.05 to 1.47 per million in men. Adjusting for age, the rise was significant for women but not men.
“Our findings indicate the reported incidence of CJD has risen considerably, disproportionately affecting older and female individuals,” the authors wrote.
The research appears to be well-conducted, but the brevity of the research letter format is an issue, Dr. Jessica Robinson-Papp, professor and vice chair for clinical research at the Department of Neurology at the Icahn School of Medicine at Mount Sinai, told The Epoch Times. It is “very short, which gives the authors less opportunity to thoroughly explain the work,” she noted.
Factors such as an aging population and improved detection methods might contribute to the observed rise, Dr. Robinson-Papp added. Notably, similar 2020 findings from Japanese data revealed increased CJD incidence, especially in those over 50.
Behind the Rise in Cases
There are three explanations for the study findings, Dr. Marc L. Gordon, chief of neurology at Northwell Zucker Hillside Hospital in New York, told The Epoch Times. They are as follows:
People are living longer, and people’s longer lifespans alone contribute to an increased likelihood of developing CJD.
We’ve developed much better ways to identify the disease.
We’ve gotten better at recognizing the MRI findings suggestive of CJD.
“There are [also] techniques we have to definitively make the diagnosis in ways that might not have been … able to do, let’s say, 20 or 30 years ago,” he added.
Dr. Gordon highlighted modern techniques, like the cerebrospinal fluid real-time quaking-induced conversion (CSF RT-QuIC) test, enabling precise diagnosis by detecting signs of abnormal prions in patients’ spinal fluid. “That was a test that only became available relatively recently,” he noted.
There is a possibility that “something else going on that we don’t recognize” that is contributing to the surge, Dr. Gordon said.
