CDC Surveillance in the US

[Guest guest]

I hope this is not the same thing that Terry posted to the group a few days

ago - if I have repeated something, my apologies to the group! Don't let the

date at the top of the page fool you. This page was last updated by the CDC

on Feb. 28, 1999. Here's the link:

http://www.cdc.gov/epo/mmwr/preview/mmwrhtml/00043220.htm " >

Surveillance for Creutzfeldt-Jakob Disease -- United States or read the

text below.

August 09, 1996 / 45(31);665-8

Surveillance for Creutzfeldt-Jakob Disease -- United States

Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy

(BSE) in cattle are subacute degenerative diseases of the brain classified as

transmissible spongiform encephalopathies. BSE was first identified in 1986 in

the United Kingdom (UK), where an epizootic involving greater than 155,000

cattle appeared to have been greatly amplified by exposure of calves to

contaminated rendered cattle carcasses in the form of meat and bone meal

nutritional supplements (1). On March 20, 1996, an expert advisory committee

to the government of the UK (1995 estimated population: 58.3 million)

announced its conclusion that the agent responsible for BSE might have spread

to humans, based on recognition of 10 persons with onset of a reportedly new

variant form of CJD * during February 1994-October 1995. The 10 persons ranged

in age from 16 to 39 years (median age at illness onset: 28 years); of the

eight persons who had died, five were aged less than 30 years (2). In

comparison, in the United States, deaths associated with CJD among persons

aged less than 30 years have been extremely rare (median age at death: 68

years) (3). As a result of the newly recognized variant of CJD described in

the UK, CDC updated its previous review of national CJD mortality (3) and

began conducting active CJD surveillance in five sites in the United States.

These reviews did not detect evidence of the occurrence of the newly described

variant form of CJD in the United States. National CJD Mortality Data

Based on multiple cause-of-death data obtained from CDC's National Center for

Health Statistics, the annual death rates for CJD (International

Classification of Diseases, Ninth Revision, code 046.1) during 1979-1994 were

stable at approximately 1 case per million population (Figure_1). Data for

1979-1993 are final; 1994 data are provisional.

The number of deaths attributed to CJD among persons aged less than 45 years

ranged from zero in 1984 to eight in 1981 and 1993. In most years no CJD-

associated deaths were reported among persons aged less than 30 years; no year

had more than one. During 1990-1994, CJD was coded as a cause of death on the

death certificate for two persons aged less than 30 years. One of these two

died in 1993 and had been previously identified as part of ongoing

surveillance for CJD among recipients of pituitary-derived human-growth

hormone; the other died in 1994, but was excluded from analysis because

follow-up investigation revealed a postmortem examination that did not confirm

the initial CJD diagnosis but indicated a diffuse T-cell proliferative

disease. Active CJD Surveillance

In early April 1996, active surveillance for the newly reported variant of CJD

and physician-diagnosed CJD cases was conducted in four Emerging Infections

Program ** sites (Connecticut, Minnesota, Oregon, and the San Francisco Bay

area of California) and the Division of Public Health, Georgia Department of

Human Resources, along with the Atlanta Metropolitan Active Surveillance

Project (total 1993 population for these areas: 16.3 million). CJD deaths were

defined as any deaths that the surveillance teams in each of these five sites

identified as having been attributed to CJD by a physician. Surveillance

efforts included review of available death certificate data during 1991-1995

and contact by phone, mail, or fax with neurologists, neuropathologists, and

pathologists to identify patients who died from CJD during 1991-1995.

Approximately 800 neurologists and neuropathologists, constituting 92%-100% of

these specialists in these surveillance areas, and greater than 90% of

pathologists in three areas were contacted. In addition, clinical and

neuropathologic records for each CJD patient aged less than 55 years were

sought for review.

A total of 94 deaths attributed to CJD were identified in the active

surveillance areas during 1991-1995. The annual number of CJD deaths was

stable (mean: 19; range: 18-19), and the average annual CJD death rate was 1.2

(range by site: 0.7-1.7) per million population (Table_1). Consistent with the

national CJD mortality pattern, nine (10%) of the 94 patients were aged less

than 55 years; one of the nine was aged less than 45 years, and none were aged

less than 30 years.

The clinical and neuropathologic record review of the nine patients aged less

than 55 years did not identify any with the variant form of CJD. A brain

biopsy was performed for the one decedent who was aged less than 45 years, and

an autopsy was performed for four of the other eight. One decedent for whom

there was no brain biopsy or autopsy was a familial case of CJD from a family

that had a known genetic abnormality associated with CJD.

One additional CJD patient aged less than 45 years who died in early 1996 was

identified by the surveillance teams. This decedent's clinical history was

similar to the description of the new variant of CJD, but brain pathology at

autopsy was inconsistent with that diagnosis.

Of the 94 CJD deaths, 81 (86%) were identified from death certificate review.

For the 13 deaths that were identified only through survey of neurologists,

neuropathologists, or pathologists, the death certificate either was not coded

as CJD or had not yet been filed.

Reported by: A Reingold, MD, Univ of California at Berkeley, G Rothrock, MPH,

California Emerging Infections Program, M Starr, DVM, K Reilly, DVM, D Vugia,

MD, S Waterman, MD, State Epidemiologist, California State Dept of Health

Svcs. R Marcus, School of Medicine, Yale Univ, New Haven; M Cartter, MD, J

Hadler, MD, State Epidemiologist, Connecticut State Dept of Public Health. M

Farley, MD, M Bardsley, MPH, W Baughman, MSPH, Atlanta Metropolitan Active

Surveillance Project, J Koehler, DVM, K Toomey, MD, State Epidemiologist, Div

of Public Health, Georgia Dept of Human Resources. R Danila, PhD, K Mac,

MD, M Osterholm, PhD, State Epidemiologist, Minnesota Dept of Health. E

DeBess, DVM, S Ladd-, MS, P Cieslak, MD, D Fleming, MD, State

Epidemiologist, Oregon Health Div. State Br, Div of Applied Public Health

Training (proposed), Epidemiology Program Office; Office of the Director,

National Center for Infectious Diseases; Special Pathogens Br and Office of

the Director, Div of Viral and Rickettsial Diseases, National Center for

Infectious Diseases, CDC.

Editorial Note

Editorial Note: This analysis did not detect evidence of a recent outbreak of

the newly described variant of CJD in the United States. Limitations of the

surveillance data include the absence of neuropathologic examinations of brain

tissue for many patients with CJD and the limited size of the population under

active surveillance. Nonetheless, the conclusions also are supported by a

review of 67 brain specimens from confirmed CJD patients in the United States

submitted during 1991-1996 to the University of California at San Francisco, a

CJD neuropathology center; none of these specimens had the neuropathologic

features of the variant form of CJD (S. DeArmond, and S. Prusiner, University

of California at San Francisco, personal communication, 1996).

The active surveillance efforts also confirmed the findings of an earlier

study indicating that death certificate reviews identify greater than or equal

to 80% of CJD deaths in the United States (4). To broaden surveillance for the

variant form of CJD in the United States, CDC is encouraging physicians to

increase their index of suspicion for this illness and, with state and

territorial epidemiologists, is investigating CJD deaths among persons aged

less than 55 years identified through routinely reported mortality data. CDC

also is working with the American Association of Neuropathologists to improve

surveillance for CJD in all age groups. Recent experimental evidence involving

intracerebral inoculation of cynomolgus macaque monkeys with brain tissue

obtained from cattle with BSE supports a possible causal link between BSE and

the variant CJD (5). Therefore, ongoing CJD and BSE surveillance in many

countries of the world, including the United States and especially in the UK,

will be critical for determining whether and to what extent the agent of BSE

is causing disease in humans. This need is underscored by the report during

March 20-June 26, 1996, of two additional confirmed cases of the newly

recognized variant of CJD in persons with onset at age less than 30 years, one

in France and one in the UK (6).

References

CDC. World Health Organization consultation on public health issues related to

bovine spongiform encephalopathy and the emergence of a new variant of

Creutzfeldt-Jakob disease. MMWR 1996;45:295-6,303.

Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob

disease in the UK. Lancet 1996;347:921-5.

Holman RC, Khan AS, Kent J, Strine TW, Schonberger LB. Epidemiology of

Creutzfeldt-Jakob disease in the United States, 1979-1990: analysis of

national mortality data. Neuroepidemiology 1995;14:174-81.

Davanipour Z, Smoak C, Bohr T, Sobel E, Liwnicz B, Chang S. Death

certificates: an efficient source for ascertainment of Creutzfeldt-Jakob

disease cases. Neuroepidemiology 1995;14:1-6.

Lasmezas CI, Deslys JP, Demaimay R, et al. BSE transmission to macaques.

Nature 1996;381: 743-4.

Chazot G, Broussolle E, Lapras C, Blattler T, Aguzzi A, Kopp N. New variant of

Creutzfeldt-Jakob disease in a 26-year-old French man {Letter}. Lancet

1996;347:1181.

This newly recognized variant of CJD has been characterized by a specific,

uniform brain pathologic profile and the classical, pathognomonic spongiform

changes of CJD found on histologic examination of brain tissue. This profile

includes, in both the cerebellum and cerebrum, numerous kuru-type amyloid

plaques surrounded by vacuoles and prion protein accumulation at high

concentration, indicated by immunocytochemical analysis. Atypical clinical

features include prominent behavior changes at the time of clinical

presentation with subsequent onset of neurologic abnormalities, including

ataxia within weeks or months, dementia and myoclonus late in the illness, a

duration of illness of at least 6 months, and nondiagnostic

electroencephalographic changes (2). ** Emerging infections programs were

established in 1994 through cooperative agreements between CDC and state

health departments to conduct special surveillance and

laboratory/epidemiologic projects and to pilot and evaluate prevention

programs.

Figure_1

Return to top.

Table_1

Note: To print large tables and graphs users may have to change their printer

settings to landscape and use a small font size.

TABLE 1. Number of deaths from Creutzfeldt-Jakob disease,

by year and age group, and average annual death rate, *

by age group -- active surveillance sites, + 1991-1995

============================================================

Age group (yrs)

----------------------------

Year <55 Yrs>=55 Yrs Total

-------------------------------------

1991 2 17 19

1992 2 & 17 19

1993 1 17 18

1994 1 18 19

1995 3 16 19

Total 9 85 94

Rate & 0.1 5.3 1.2

------------------------------------------------------------

* Per million population.

+ Emerging Infections Program sites (Connecticut, Minnesota,

Oregon, and the San Francisco Bay area of California) and

the Division of Public Health, Georgia Department of

Human Resources, along with the Atlanta Metropolitan

Active Surveillance Project (total 1993 population for

these areas: 16.3 million).

& One case occurred in a person aged <45

years.= " =========================================================== "

Return to top.

Disclaimer All MMWR HTML versions of articles are electronic conversions

from ASCII text into HTML. This conversion may have resulted in character

translation or format errors in the HTML version. Users should not rely on

this HTML document, but are referred to the electronic PDF version and/or the

original MMWR paper copy for the official text, figures, and tables. An

original paper copy of this issue can be obtained from the Superintendent of

Documents, U.S. Government Printing Office (GPO), Washington, DC 20402-9371;

telephone: . Contact GPO for current prices.

Return To: MMWR MMWR Home Page CDC Home Page

**Questions or messages regarding errors in formatting should be addressed to

mmwrq@....

Page converted: 09/19/98