Prion Disease on the Rise in the U.S.


Seattle — The incidence of the spongiform brain disease Creutzfeldt-Jakob disease (CJD) increased by more than half in the U.S. between 2000 and 2014, according to a new study reported here, although the reasons for the increase are unclear.

People were hospitalized for CJD at a rate of 1.6 per million individuals in 2000 (95% CI 1.2–2.0), said Alison Seitz, MD, of New York-Presbyterian Hospital in New York City, during a recorded platform presentation at the American Academy of Sciences. Reported. Neurology Annual Meeting.

By 2014, she said, the rate had risen to 2.7 per million (95% CI 2.2–3.2), for an average annual increase of 4.6 percent.

Importantly, her analysis did not adjust for the changing age distribution of the US population, which certainly became older on average during the study period. Since “sporadic” CJD, accounting for 80% to 95% of all cases, is most common in older people, some increase in the incidence of crude CJD would be expected in the aging population.

But growth in the older demographic has been much less at 4.6% annually — like 2% to 3%, a . According to 2021 report From the Federal Administration for Community Life. This suggests that something other than the increasingly top-heavy age pyramid is going on.

Setz notes that one possibility is simply to increase attention to the presence of CJD. As it happens, 1993 was the year of “mad cow disease” before it hit the world headlines. CJD cases involving beef cattle In 1994, a bovine was found infected with spongiform encephalopathy in the UK, causing global panic. Some cases also occurred in the US, although against the background of naturally occurring CJD, they Hardly registered in CDC data,

Yet those same figures suggested a substantial increase in overall CJD mortality starting around 2000. The CDC gets its data mainly from death certificates. Setz and his colleagues decided to draw on a different data source, national patient sample (NIS), which may be more accurate given all the known issues with death certificates. For one thing, NIS has complete information about registered patients, including a full account of the ICD diagnostic codes entered for each individual. For the current study, the researchers searched for codes 046.1, 046.11, and 046.19 to capture CJD cases.

Another nice feature of the NIS is that, while it doesn’t cover all U.S. hospitals—”this is a sample,” Setz observed, with about 8 million patient encounters per year—the summary data must be matched to overall population characteristics. Loaded to eat.

Thus, the 1,837 CJD hospitalizations identified in the 2000-2014 NIS data increased to 8,778 overall in the US during the period, Seitz said.

The overall upward trend was not stable over time. For example, a spike in 2008 was bracketed by years of sharp declines. Furthermore, these year-to-year changes did not exactly match the CDC’s mortality data, although CJD is equally fatal and usually occurs within a year of diagnosis.

Seitz also cited the possibility that hospital registrars came free with codes for CJD, “appropriately or not,” as another possible explanation for the increased incidence in the NIS data.

One potentially relevant factor Seitz hasn’t mentioned chronic wasting disease, prion-causing deformities affecting deer, elk and related animals. The CDC says that no confirmed cases of in utero-to-human transmission are known. Still, the situation has been spreading in wild deer and elk over the past two decades, which is now spread to “at least 27 states” in the Midwest, South and East Coast, according to the agency. latest statement,

In 2021, more than 6.3 million deer They are estimated to have been killed and likely handled by poachers, so exposure to infected animals is likely, and likely to increase, over the period covered by the new study. (Seitz could not be reached for comment.)

  • Jon Geever was managing editor from 2014 to 2021; He is now a regular contributor.

Disclosures

Seitz reported holding stock in several health-related companies, but has no financial interests relevant to the CJD study.

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