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From: TSS ()
Subject: The public health impact of prion diseases (1)
Date: March 13, 2005 at 6:55 pm PST

-------- Original Message --------
Subject: The public health impact of prion diseases (1)
Date: Sun, 13 Mar 2005 20:56:35 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

##################### Bovine Spongiform Encephalopathy #####################

Annu Rev Public Health. 2005;26:191-212.

The public health impact of prion diseases (1).

Belay ED, Schonberger LB.

Division of Viral and Rickettsial Diseases, National Center for
Infectious Diseases, Centers for Disease Control and Prevention,
Atlanta, Georgia 30333; email:

Several prion disease-related human health risks from an exogenous
source can be identified in the United States, including the
iatrogenic transmission of Creutzfeldt-Jakob disease (CJD), the
possible occurrence of variant CJD (vCJD), and potential zoonotic
transmission of chronic wasting disease (CWD). Although
cross-species transmission of prion diseases seems to be limited by
an apparent "species barrier," the occurrence of bovine spongiform
encephalopathy (BSE) and its transmission to humans indicate that
animal prion diseases can pose a significant public health risk.
Recent reports of secondary person-to-person spread of vCJD via
blood products and detection of vCJD transmission in a patient
heterozygous at codon 129 further illustrate the potential public
health impacts of BSE.

PMID: 15760286 [PubMed - in process]


Reply to Singletary 26 March 2003

Previous Correspondence

Ryan A. Maddox, MPH
Centers for Disease Control and Prevention Atlanta GA,
Ermias D. Belay, MD, Lawrence B. Schonberger, MD

Send Correspondence to journal:
Re: Reply to Singletary

Email Ryan A.
Maddox, MPH, et al.

Mr. Singletary raises several issues related to current Creutzfeldt-
Jakob disease (CJD) surveillance activities. Although CJD is not a
notifiable disease in most states, its unique characteristics,
particularly its invariably fatal outcome within usually a year of
onset, make routine mortality surveillance a useful surrogate for
ongoing CJD surveillance.[1] In addition, because CJD is least
accurately diagnosed early in the course of illness, notifiable-disease
surveillance could be less accurate than, if not duplicative of, current
mortality surveillance.[1] However, in states where making CJD
officially notifiable would meaningfully facilitate the collection of
data to monitor for variant CJD (vCJD) or other emerging prion diseases,
CDC encourages the designation of CJD as a notifiable disease.[1]
Moreover, CDC encourages physicians to report any diagnosed or suspected
CJD cases that may be of special public health importance (e.g., vCJD,
iatrogenic CJD, unusual CJD clusters).

As noted in our article, strong evidence is lacking for a causal link
between chronic wasting disease (CWD) of deer and elk and human
disease,[2] but only limited data seeking such evidence exist. Overall,
the previously published case-control studies that have evaluated
environmental sources of infection for sporadic CJD have not
consistently identified strong evidence for a common risk factor.[3]
However, the power of a case-control study to detect a rare cause of CJD
is limited, particularly given the relatively small number of subjects
generally involved and its long incubation period, which may last for
decades. Because only a very small proportion of the US population has
been exposed to CWD, a targeted surveillance and investigation of
unusual cases or case clusters of prion diseases among persons at
increased risk of exposure to CWD is a more efficient approach to
detecting the possible transmission of CWD to humans. In collaboration
with appropriate local and state health departments and the National
Prion Disease Pathology Surveillance Center, CDC is facilitating or
conducting such surveillance and case- investigations, including related
laboratory studies to characterize CJD and CWD prions.

Mr. Singletary also expresses concern over a recent publication by
Asante and colleagues indicating the possibility that some sporadic CJD
cases may be attributable to bovine spongiform encephalopathy (BSE).[4]
The authors reported that transgenic mice expressing human prion protein
homozygous for methionine at codon 129, when inoculated with BSE prions,
developed a molecular phenotype consistent with a subtype of sporadic
CJD. Although the authors implied that BSE might cause a sporadic
CJD-like illness among persons homozygous for methionine, the results of
their research with mice do not necessarily directly apply to the
transmission of BSE to humans. If BSE causes a sporadic CJD-like illness
in humans, an increase in sporadic CJD cases would be expected to first
occur in the United Kingdom, where the vast majority of vCJD cases have
been reported. In the United Kingdom during 1997 through 2002, however,
the overall average annual mortality rate for sporadic CJD was not
elevated; it was about 1 case per million population per year. In
addition, during this most recent 6-year period following the first
published description of vCJD in 1996, there was no increasing trend in
the reported annual number of UK sporadic CJD deaths.[3, 5] Furthermore,
surveillance in the UK has shown no increase in the proportion of
sporadic CJD cases that are homozygous for methionine (Will RG, National
CJD Surveillance Unit, United Kingdom, 2003; personal communication).


1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Diagnosis and
reporting of Creutzfeldt-Jakob disease. JAMA 2001;285:733-734.

2. Belay ED, Maddox RA, Gambetti P, Schonberger LB. Monitoring the
occurrence of emerging forms of Creutzfeldt-Jakob disease in the United
States. Neurology 2003;60:176-181.

3. Belay ED. Transmissible spongiform encephalopathies in humans. Annu
Rev Microbiol 1999;53:283-314.

4. Asante EA, Linehan JM, Desbruslais M, et al. BSE prions propagate as
either variant CJD-like or sporadic CJD-like prion strains in transgenic
mice expressing human prion protein. EMBO J 2002;21:6358-6366.

5. The UK Creutzfeldt-Jakob Disease Surveillance Unit. CJD statistics.
Available at: Accessed February 18,

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States 26 March 2003

Next Correspondence
Terry S. Singeltary,
retired (medically)

Send Correspondence to journal:
Re: RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States


Terry S. Singeltary

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to
comment on the CDC's attempts to monitor the occurrence of emerging
forms of CJD. Asante, Collinge et al [1] have reported that BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype that is indistinguishable from type 2 PrPSc, the commonest
sporadic CJD. However, CJD and all human TSEs are not reportable
nationally. CJD and all human TSEs must be made reportable in every
state and internationally. I hope that the CDC does not continue to
expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in
the USA in both animal and man. CWD in deer/elk is spreading rapidly and
CWD does transmit to mink, ferret, cattle, and squirrel monkey by
intracerebral inoculation. With the known incubation periods in other
TSEs, oral transmission studies of CWD may take much longer. Every
victim/family of CJD/TSEs should be asked about route and source of this
agent. To prolong this will only spread the agent and needlessly expose
others. In light of the findings of Asante and Collinge et al, there
should be drastic measures to safeguard the medical and surgical arena
from sporadic CJDs and all human TSEs. I only ponder how many sporadic
CJDs in the USA are type 2 PrPSc?


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