NR AENH
AU Gibbons,R.V.; Holman,R.C.; Belay,E.D.; Schonberger,L.B.
TI Creutzfeldt-Jakob disease in the United States: 1979-1998.
QU JAMA. Journal of the American Medical Association 2000 Nov 8; 284(18): 2322-3
IA http://jama.ama-assn.org/issues/v284n18/ffull/jlt1108-6.html
KI JAMA. 2001 Feb 14;285(6):733-4. PMID: 11176901 JAMA. 2001 Feb 14;285(6):733; discussion 733-4. PMID: 11176900
PT research letter
VT
To the Editor: Creutzfeldt-Jakob disease (CJD) is the most common transmissible spongiform encephalopathy in humans. In response to concerns about the emergence of new variant CJD (nvCJD) in the United Kingdom, the Centers for Disease Control and Prevention (CDC) enhanced its ongoing CJD surveillance.[1,2] We describe results of mortality surveillance for CJD in the United States from 1979 through 1998.
Methods
We analyzed multiple cause-of-death data[3] for CJD (International Classification of Diseases, Ninth Revision, code 046.1). We excluded 8 deaths because of coding errors, clear alternative diagnoses, or pathological findings indicating the absence of CJD; we included an additional 5 deaths reported to the CDC by other surveillance methods. Age-specific and age-adjusted annual CJD death rates were calculated. Age-adjusted rates were standardized by the direct method, using the 1990 census population.
Results
From 1979 through 1998, 4751 deaths due to CJD were reported in the United States (Table 1). The average annual age-adjusted death rate was 0.97 deaths per million persons, ranging from 0.78 in 1980 to 1.13 in 1997 (Figure 1). The overall annual rates have been relatively stable since 1985 (P = .64, linear regression analysis).
The median age at death was 68 years. Ten CJD decedents were younger than 30 years, including 3 who died during the period of the nvCJD epidemic in the United Kingdom (1995-1998). Neuropathologic evaluation of 2 of these 3 patients ruled out nvCJD; the third patient had iatrogenic CJD associated with receipt of human growth hormone.
Comment
Because CJD is invariably fatal, more than 85% of patients die within 1 year, and the diagnosis is best ascertained at or near the time of death, mortality data analysis is an efficient way of monitoring CJD incidence in the United States. Two studies have indicated that mortality data analyses identify at least 80% of CJD deaths in the United States.[1,2] The relatively high sensitivity of CJD mortality surveillance is supported by the fact that no marked increase in the CJD death rate was seen in recent data despite the extensive attention nvCJD, CJD, and bovine spongiform encephalopathy have received since 1996.
As of October 2, 2000, a total of 84 nvCJD cases had been reported in the United Kingdom.[4] The median age at death of patients with nvCJD was 27.5 years (unpublished data, National Creutzfeldt-Jakob Disease Surveillance Unit, Edinburgh, Scotland, October 16, 2000). In the United States, the CDC enhanced CJD mortality surveillance by focusing on the striking difference in age distribution of nvCJD cases from that of US sporadic CJD cases. This enhancement included follow-up investigation of patients with CJD who were younger than 55 years and the establishment of the National Prion Disease Pathology Surveillance Center (NPDPSC) in collaboration with the American Association of Neuropathologists (AANP). The clinical and neuropathologic record reviews of 101 patients with CJD who died before age 55 years from 1994 through 1997 have been completed; 45 of the 101 patients had neuropathologic confirmation of the CJD diagnosis (CDC, authors' unpublished data). The NPDPSC alerted US members of the AANP and the US and Canadian Academy of Pathologists about nvCJD pathology and periodically requested reports of CJD and suspected nvCJD cases. None of the CJD surveillance efforts, including analysis through September 20, 2000, of brain tissues of suspected and confirmed CJD cases at the NPDPSC,[5] detected any evidence of nvCJD in the United States (Pierluigi Gambetti, MD, Case Western Reserve University, written communication, September 27, 2000).
Robert V. Gibbons, MD, MPH
Robert C. Holman, MS
Ermias D. Belay, MD
Lawrence B. Schonberger, MD, MPH
Division of Viral and Rickettsial Diseases, National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Ga
1. Holman RC, Khan AS, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States, 1979-1994. Emerg Infect Dis. 1996;2:333-337. MEDLINE
2. Tan L, Williams MA, Khan MK, et al. Risk of transmission of bovine spongiform encephalopathy to humans in the United States. JAMA. 1999;281:2330-2339.
3. US Dept of Health and Human Services. Vital Statistics Mortality Data, Multiple Cause Detail, 1979-1998, Public Use Data Tape Contents and Documentation Package. Hyattsville, Md: National Center for Health Statistics; 2000.
4. Department of Health, United Kingdom. Monthly Creutzfeldt-Jakob disease statistics. Available at: http://www.doh.gov.uk/cjd/stats/oct00.htm. Accessed October 16, 2000.
5. Will RG, Zeidler M, Stewart GE, et al. Diagnosis of new variant Creutzfeldt-Jakob disease. Ann Neurol. 2000;47:575-582. MEDLINE
6. National Prion Disease Pathology Surveillance Center.. Available at: http://www.cjdsurveillance.com. Accessed June 22, 2000.
MH Adult; Age Distribution; Creutzfeldt-Jakob Syndrome/*mortality; Female; Human; Male; Middle Age; United States/epidemiology
AD
Robert V. Gibbons, MD, MPH
Robert C. Holman, MS
Ermias D. Belay, MD
Lawrence B. Schonberger, MD, MPH
Division of Viral and Rickettsial Diseases, National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Ga
SP englisch
PO USA
EA pdf-Datei und HTML-Version