Creutzfeldt-Jakob Disease Rising in the US, Especially Among Older Women

The incidence of Creutzfeldt-Jakob disease (CJD) has risen considerably from 2007 to 2020, particularly among older women, according to a research letter published in JAMA Neurology.

The progressive, universally fatal prion disease CJD has been stable in the United States (US) between 1979 to 2006. The most common subtype of CJD, sporadic CJD, tends to affect older individuals. As the global population ages, the epidemiology of CJD may be evolving.

To evaluate recent trends in CJD in the US, the researchers sourced data for this cross-sectional study from the Wide-Ranging Online Data for Epidemiologic Research multiple cause of death database. Death certificates between 2007 and 2020 for CJD were assessed for volume and decedent demographics.

The incidence of CJD increased consistently between 2007 and 2020, in which there were 5882 total cases and 51.2% occurred among women.

Among men, the incidence increased from 1.05 (95% CI, 0.88-1.21) per million in 2007 to 1.47 (95% CI, 1.28-1.65) per million in 2020 (P =.001). For women, the incidence increased from 1.06 (95% CI, 0.89-1.22) per million to 1.58 (95% CI, 1.39-1.77) per million in 2007 and 2020 (P <.001), respectively.

After adjusting for age, the incidence did not increase significantly over time among men (P =.25) but did among women (P =.045).

The largest increase in incidence was observed among those aged 75 to 84, but the increase over time among this age group did not reach significance among men (P =.18) or women (P =.07).

A jointpoint analysis indicated a significant increase in the crude incidence among both men (average annual percent change [AAPC], 2.9; 95% CI, 2.3-3.6; P <.001) and women (AAPC, 3.5; 95% CI, 2.2-4.7; P <.001) and remained significant after adjusting for age among men (adjusted AAPC, 0.8; 95% CI, 0.1-1.5; P =.02) and women (adjusted AAPC, 1.9; 95% CI, 0.6-3.2; P =.005).

Stratified by age group, the crude AAPC was significant among men aged 55 to 64 (AAPC, 1.7; P =.04) and among women in all age groups (AAPC range, 1.7-2.1; all P £.04).

The major limitation of this analysis was that it was reliant on correct identification of CJD in death certificates.

“Our findings indicate the reported incidence of CJD has risen considerably, disproportionately affecting older and female individuals,” the researchers concluded. “The findings underscore the changing landscape of CJD and suggest a need for monitoring among the aging US population.”