Occurrence and Transmission

Classic CJD has been recognized since the early 1920s. The most common form of classic CJD is believed to occur sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions. This sporadic disease occurs worldwide, including the United States, at a rate of  roughly 1 to 1.5 cases per 1 million population per year, although rates of up to two cases per million are not unusual. The risk of CJD increases with age, and in persons aged over 50 years of age, the annual rate is approximately 3.4 cases per million.

Whereas the majority of cases of CJD (about 85%) occur as sporadic disease, a smaller proportion of patients (5-15%) develop CJD because of inherited mutations of the prion protein gene. These inherited forms include Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia.

Creutzfeldt-Jakob Disease Deaths and Age-Adjusted Death Rate, United States, 1979-2016*

Creutzfeldt-Jakob disease deaths an age-adjusted death rate, United States, 1979-2016. See details in table below.

* Deaths obtained from the multiple cause-of-death data for 1979-1998 are based on ICD-9 codes, and those beginning in 1999 are based on ICD-10 codes with available computerized literal death certificate data.  Death information was also obtained from other surveillance mechanisms; data include familial prion diseases. Rates are adjusted to the US standard 2000 projected population.

Creutzfeldt-Jakob Disease Deaths and Age-Adjusted Death Rate, United States, 1979-2016*
Year Deaths (approximate) Age-adjusted death rate
1979 179 0.850
1980 172 0.787
1981 214 0.988
1982 201 0.901
1983 183 0.825
1984 221 0.985
1985 235 1.033
1986 247 1.073
1987 268 1.129
1988 243 1.020
1989 242 1.011
1990 210 0.859
1991 238 0.982
1992 255 1.018
1993 256 1.012
1994 278 1.079
1995 265 1.023
1996 261 0.984
1997 305 1.152
1998 281 1.038
1999 272 0.990
2000 238 0.853
2001 259 0.917
2002 260 0.904
2003 284 0.967
2004 279 0.921
2005 296 0.972
2006 290 0.925
2007 330 1.019
2008 352 1.086
2009 353 1.067
2010 396 1.149
2011 409 1.160
2012 380 1.053
2013 478 1.299
2014 441 1.167
2015 481 1.225
2016 492 1.224