vCJD USA THIRD CASE DOCUMENTED
Wednesday, December 06, 2006 vCJD USA THIRD CASE DOCUMENTED
CJD (NEW VAR.) UPDATE 2006 (12) ******************************* A ProMED-mail post
ProMED-mail is a program of the International Society for Infectious Diseases
[The definition of the designations deaths, definite cases, probable vCJD cases, and the case definitions can be found by accessing the Department of Health website, or by reference to a previous ProMED-mail post in this thread (for example, CJD (new var.) - UK: update March 2002 20020305.3693).
Data on vCJD cases from other parts of the world are now included in these updates whenever available.
Also, data on other forms of CJD (sporadic, iatrogenic, familial and GSS) are now included when they have some relevance to the incidence and etiology of vCJD. - Mod.CP]
In this update:
[1] UK: Department of Health monthly vCJD and CJD statistics, Mon 4 Dec 2006 [2] EUROCJD data as of 31 October 2006 [3] National (US) Prion Disease Pathology Surveillance Center Data: 8 Nov 2006 [4] The Netherlands: 2nd vCJD death [5] USA: 3rd vCJD case (ex Saudi Arabia)
****** [1] UK: vCJD and CJD statistics Date: Mon 4 Dec 2006 From: ProMED-mail Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease Statistics, Mon 4 Dec 2006 [edited]
The Department of Health is today [Mon 4 Dec 2006] issuing the latest information about the numbers of known cases of Creutzfeldt-Jakob disease. This includes cases of variant Creutzfeldt-Jakob disease [abbreviated in ProMED-mail as CJD (new var.) or vCJD], the form of the disease thought to be linked to BSE (bovine spongiform encephalopathy).
Definite and probable CJD cases in the UK, as at Fri 1 Dec 2006
----------------------------------------------------------------
Summary of vCJD cases - deaths ------------------------------
Deaths from definite vCJD (confirmed): 112 Deaths from probable vCJD (without neuropathological confirmation): 46 Deaths from probable vCJD (neuropathological confirmation pending): 0 Number of deaths from definite or probable vCJD (as above): 158
Summary of vCJD cases - alive -----------------------------
Number of probable vCJD cases still alive: 6
Total ----- Number of definite or probable vCJD (dead and alive): 164
(The next table will be published on Mon 8 Jan 2007).
Since the previous monthly statistics were released on Mon 6 Nov 2006, the total number of deaths from definite vCJD remains unchanged and stands at 158. The overall total number of definite or probable vCJD cases (dead and alive) remains unchanged at 164.
These data are consistent with the view that the vCJD outbreak in the UK is in decline. The peak number of deaths was 28 in the year 2000, followed by 20 in 2001, 17 in 2002, 18 in 2003, 9 in 2004, and 5 in 2005. The number of deaths due to definite or probable vCJD in the UK during the first 11 months of 2006 remains at 5.
Totals for all types of CJD cases in the UK in 2005 and 2006
----------------------------------------------------
As of Fri 1 Dec 2006, in the UK in the year 2005, there were 123 referrals of suspected CJD, and there were 65 deaths from sporadic CJD, 7 from familial CJD, 3 from iatrogenic CJD, 6 GSS (Gerstmann-Straussler-Scheinker) syndrome cases, and 5 deaths from vCJD.
The corresponding figures so far for the 1st 11 months of 2006 are: 98 referrals, 53 deaths from sporadic CJD, 5 from vCJD, 5 from familial CJD, 3 from GSS and one from iatrogenic CJD.
During the period from 1995, when vCJD was first diagnosed, up to the present there have been 953 deaths from all forms of CJD including the 158 deaths attributable to definite or probable vCJD.
[These data are accessible via ]
-- ProMED-mail
****** [2] EU - EUROCJD Data Date: Tue 31 Oct 2006 From: ProMED-mail Source: EUROCJD 31 Oct 2006 [edited]
The European And Allied Countries Collaborative Study Group of CJD (EUCJD)
----------------------------------------------------
This web-site includes information from 2 projects funded by the European Commission. The EUROCJD project started in 1993 and compares data from national registries in Australia, Austria, Canada, France, Germany, Italy, the Netherlands, Slovakia, Spain, Switzerland and the UK. The NEUROCJD project started in 1998 after the European Union Council recommended that epidemiological surveillance of CJD should be extended to all member states. The member states involved in this project are Belgium, Denmark, Finland, Greece, Iceland, Ireland, Israel, Norway and Portugal. Both projects are coordinated from the U.K. National CJD Surveillance Unit based in Edinburgh.
Current data as at November 2006*
---------------------------------
Country / Total No. of Primary cases (No. alive) / Cumulative residence in UK (>6 months) / Secondary transmission by blood transfusion
United Kingdom / 162 (6) / 164 / 2 (0) France / 21 (2) / 1 / 0 Republic of Ireland / 4 (1) / 2 / 0 Italy / 1 (0) / 0 / 0 USA / 3 (1*) / 2 / 0 / 0 Canada / 1 (0) / 1 / 0 Saudi Arabia / 1 (1) / 0 / 0 Japan / 1** (0) / 0 / 0 Netherlands*** / 2 (0) / 0 / 0 Portugal / 1 (1) / 0 / 0 Spain / 1 (0) / 0 / 0
Total / 197 (11) / - / 2
Footnotes ----------
* One case probably contracted in Saudi Arabia [see part (5) below], and 2 in U.K. ** Residence in the UK for 24 days *** Data for the Netherlands case (see part [4] below) was omitted in error from the corresponding table for October posted on 31 Oct 2006
-- ProMED-mail
****** [3] USA - CJD National Prion Disease Surveillance Data Date: Wed 8 Nov 2006 From: Terry S. Singeltary Sr Source: CJD Surveillance, National Prion Disease Pathology Surveillance Center, 8 Nov 2006 [edited]
Resources/Case Report ---------------------
Year / Referral / Total / Sporadic / Familial / Iatrogenic / vCJD
1997 / 104 / 60 / 54 / 6 / 0 / 0 1998 / 94 / 51 / 44 / 6 / 1 / 0 1999 / 114 / 74 / 65 / 9 / 0 / 0 2000 / 169 / 111 / 97 / 12 / 2 / 0 2001 / 2247 / 154 / 138 / 16 / 0 / 0 2002 / 265 / 151 / 127 / 22 / 1 / 0 2003 / 284 / 191 / 142 / 45 / 1 / 0 2004 / 360 / 202 / 167 / 21 / 0 / 1* 2005 / 357 / 201 / 144 / 39 / 0 / 0 2006 / 208 / 86 / 46 / 8 / 0 /1**
Total / 2202 / 1281 / 1024 / 184 / 5 / 2*
* Acquired in the United Kingdom ** Acquired in Saudi Arabia [see part (5) below]
[The gradual increase in sporadic and other forms of CJD in the USA throughout this period may merely reflect improvements in surveillance and diagnosis rather than a real increase in incidence. For example, according to the tables above, there were approximately 1 per million cases of sporadic CJD in the USA during 2005, compared with 2 cases per million in the UK during the same period. This difference could be indicative of a real difference in incidence or a difference in efficiency of diagnosis. - Mod.CP]
-- Terry S. Singeltary Sr
****** [4] The Netherlands - 2nd death Date: Thu 16 Nov 2006 From: Mary Marshall Source: iol.co.za Medical, Reuters report, Thu 16 Nov 2006 [edited]
A 2nd Dutch person has died from the human variant of mad cow disease following the death of a woman last year, health authorities said on Thu 16 Nov 2006. The Dutch Institute for Health and Environment (RIVM) give no details about the victim, but Dutch television stations said he was a 16-year-old boy.
A spokeswoman for the RIVM said the victim died from the brain-wasting [variant] Creutzfeldt-Jakob Disease (vCJD), the human form of Bovine Spongiform Encephalopathy (BSE) about 2 weeks ago. She did not explain why the RIVM had waited 2 weeks to confirm the death, which was reported in Dutch media on Thursday. The RIVM diagnosed the person with the human variant of mad cow [disease] in June and said at the time that the patient had most probably become infected by eating contaminated meat products. It was the 2nd Dutch death from the human variant of mad cow disease after a 26-year-old woman died in May 2005.
The disease is fatal and incurable. It is thought to be caused by eating food tainted with material from cattle with BSE, a progressive neurological disorder. More than 150 cases of vCJD have been reported around the world, mostly in Britain, but also in France, Ireland, Italy, Japan, Canada and the United States. Mad cow disease first emerged in Britain in the 1980s and has forced the destruction of millions of cattle.
-- ProMED-mail
****** [5] Date: Wed 29 Nov 2006 From: Terry S. Singeltary Sr. Source: CDC, National Center for Infectious Diseases, Wed 29 Nov 2006 [edited]
Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the Middle East
---------------------------------------------------
The Virginia Department of Health [] and the Centers for Disease Control and Prevention announced the recent confirmation of a vCJD case in a U.S. resident. This is the 3rd vCJD case identified in a U.S. resident. This latest U.S. case occurred in a young adult who was born and raised in Saudi Arabia and has lived in the United States since late 2005. The patient occasionally stayed in the United States for up to 3 months at a time since 2001 and there was a shorter visit in 1989. In late November 2006, the Clinical Prion Research Team at the University of California San Francisco Memory and Aging Center confirmed the vCJD clinical diagnosis by pathologic study of adenoid and brain biopsy tissues. The 2 previously reported vCJD case-patients in U.S. residents were each born and raised in the United Kingdom (U.K.), where they were believed to have been infected by the agent responsible for their disease. There is strong scient! ific evidence that the agent causing vCJD is the same agent that causes bovine spongiform encephalopathy (BSE, commonly known as mad cow disease).
Variant CJD is a rare, degenerative, fatal brain disorder that emerged in the United Kingdom in the mid-1990s. Although experience with this new disease is limited, evidence to date indicates that there has never been a case transmitted from person-to-person except through blood transfusion. Instead, the disease is thought to result primarily from consumption of cattle products contaminated with the BSE agent. Although no cases of BSE in cattle have been reported in Saudi Arabia, potentially contaminated cattle products from the United Kingdom may have been exported to Saudi Arabia for many years during the large U.K. BSE outbreak.
The current case-patient has no history of receipt of blood, a past neurological procedure, or residing in or visiting countries of Europe. Based on the patient's history, the occurrence of a previously reported Saudi case of vCJD attributed to likely consumption of BSE-contaminated cattle products in Saudi Arabia, and the expected greater than 7 year incubation period for food-related vCJD, this U.S. case-patient was most likely infected from contaminated cattle products consumed as a child when living in Saudi Arabia (1). The current patient has no history of donating blood and the public health investigation has identified no risk of transmission to U.S. residents from this patient.
As of November 2006, 200 vCJD patients were reported world-wide, including 164 patients identified in the United Kingdom, 21 in France, 4 in the Republic of Ireland, 3 in the United States (including the present case-patient), 2 in the Netherlands and 1 each in Canada, Italy, Japan, Portugal, Saudi Arabia and Spain. Of the 200 reported vCJD patients, all except 10 of them (including the present case-patient) had resided either in the United Kingdom (170 cases) for over 6 months during the 1980-1996 period of the large UK BSE outbreak or alternatively in France (20 cases).
As reported in 2005 (1), the U.S. National Prion Disease Pathology Surveillance Center at Case Western Reserve University confirmed the diagnosis in the one previously identified case of vCJD in a Saudi resident. He was hospitalized in Saudi Arabia and his brain biopsy specimen was shipped to the United States for analysis. This earlier vCJD case-patient was believed to have contracted his fatal disease in Saudi Arabia (1).
Reference --------- (1) Belay ED, Sejvar JJ, Shieh W-J, Wiersma ST, Zou W-Q, Gambetti P, Hunter S, Maddox RA, Crockett L, Zaki SR, Schonberger LB. Variant Creutzfeldt-Jakob disease death, United States. Emerg Infect Dis 2005, 11 (9):1351-1354.
-- Terry S. Singeltary Sr.
[There is inconsistency in the number of vCJD cases attributed to the United States. The Virginia Department of Health/CDC report cited in the above list 3 cases -- 2 cases probably contracted in the U.K. and the young adult described above who was born and raised in Saudi Arabia. The EUROCJD data for October 2006 in part (2) above also lists 2 U.S. cases, both assumed to have been contracted in the U.K. The U.S. National Prion Disease Pathology Surveillance Center Data published on 8 Nov 2006 (part 3 above), on the other hand, list only a single U.S. case believed to have been contracted in the U.K. - Mod.CP]
[see also: CJD (new var.) update 2006 (11) 20061106.3190 CJD (new var.) update 2006 (10) 20061002.2820 CJD (new var.) update 2006 (09) 20060904.2519 CJD (new var.) update 2006 (08) 20060807.2207 CJD (new var.) update 2006 (07) 20060703.1831 CJD (new var.) - Netherlands: 2nd case 20060623.1741 CJD (new var.) update 2006 (06) 20060605.1566 CJD (new var.) update 2006 (05) 20060508.1332 CJD (new var.) update 2006 (04) 20060404.1005 CJD (new var.) update 2006 (03) 20060306.0728 CJD (new var.) - UK: 3rd transfusion-related case 20060209.0432 CJD (new var.) update 2006 (02) 20060206.0386 CJD (new var.) update 2006 (01) 20060111.0101 CJD (new var.) update 2006 20060111.0101 2005 ---- CJD (new var.) update 2005 (12) 20051209.3547 CJD (new var.) update 2005 (11) 20051108.3270 CJD (new var.) update 2005 (10) 20051006.2916 CJD (new var.) update 2005 (09) 20050905.2627 CJD (new var.) update 2005 (08) 20050801.2237 CJD (new var.) update 2005 (07) 20050703.1889 CJD (new var.) update 2005 (06) 20050607.1584 CJD (new var.) update 2005 (05) 20050505.1243 CJD (new var.) update 2005 (04) 20050405.0982 CJD (new var.) update 2005 (03) 20050308.0687 CJD (new var.) update 2005 (02) 20050211.0467 CJD (new var.) - UK: update 2005 (01) 20050111.0095 2004 ---- CJD, genetic susceptibility 20041112.3064 CJD (new var.) - UK: update 2004 (14) 20041206.3242 CJD (new var.) - UK: update 2004 (13) 20041103.2977 CJD (new var.) - UK: update 2004 (12) 20041023.2871 CJD (new var.) - UK: update 2004 (11) 20041008.2758 CJD (new var.) - UK: update 2004 (10) 20040909.2518 CJD (new var.) - UK: update 2004 (09) 20040809.2199 CJD (new var.) - UK: update 2004 (08) 20040806.2150 CJD (new var.) - UK: update 2004 (07) 20040706.1807 CJD (new var.) - UK: update 2004 (06) 20040608.1535 CJD (new var.) - UK: update 2004 (05) 20040510.1262 CJD (new var.) - UK: update 2004 (04) 20040406.0937 CJD (new var.) - UK: update 2004 (03) 20040314.0713 CJD (new var.) - UK: update 2004 (02) 20040202.0400 CJD (new var.) - UK: update 2004 (01) 20040106.0064 CJD (new var.) - France: 8th case 20041022.2864 CJD (new var.) - France: 9th case 20041123.3138 CJD (new var.), blood supply - UK 20040318.0758 CJD (new var.), carrier frequency study - UK 20040521.1365 2003 ---- CJD (new var.) - UK: update 2003 (13) 20031216.3072 CJD (new var.) - UK: update 2003 (01) 20030108.0057 2002 ---- CJD (new var.) - UK: update Dec 2002 20021207.5997 CJD (new var.) - UK: update Jan 2002 20020111.3223 2001 ---- CJD (new var.), incidence & trends - UK (02) 20011124.2875 CJD (new var.), incidence & trends - UK 20011115.2816 CJD (new var.) - UK: reassessment 20011029.2671 CJD (new var.) - UK: update Oct 2001 20011005.2419 CJD (new var.) - UK: regional variation (02) 20010907.2145 CJD (new var.) - UK: update Sep 2001 20010906.2134 CJD (new var.) - UK: update Aug 2001 20010808.1872 CJD (new var.) - UK: 9th Annual Report 20010628.1231 CJD (new var.) - UK: update June 2001 20010622.1188 CJD (new var.) - UK: update 3 Jan 2001 20010104.0025] ................cp/pg/mpp
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Volume 12, Number 12–December 2006
PERSPECTIVE
On the Question of Sporadic
or Atypical Bovine SpongiformEncephalopathy and
Creutzfeldt-Jakob Disease
Paul Brown,* Lisa M. McShane,† Gianluigi Zanusso,‡ and Linda Detwiler§
Strategies to investigate the possible existence of sporadic
bovine spongiform encephalopathy (BSE) require
systematic testing programs to identify cases in countries
considered to have little or no risk for orally acquired disease,
or to detect a stable occurrence of atypical cases in
countries in which orally acquired disease is disappearing.
To achieve 95% statistical confidence that the prevalence
of sporadic BSE is no greater than 1 per million (i.e., the
annual incidence of sporadic Creutzfeldt-Jakob disease
[CJD] in humans) would require negative tests in 3 million
randomly selected older cattle. A link between BSE and
sporadic CJD has been suggested on the basis of laboratory
studies but is unsupported by epidemiologic observation.
Such a link might yet be established by the discovery
of a specific molecular marker or of particular combinations
of trends over time of typical and atypical BSE and various
subtypes of sporadic CJD, as their numbers are influenced
by a continuation of current public health measures that
exclude high-risk bovine tissues from the animal and
human food chains.
SNIP...
Sporadic CJD
The possibility that at least some cases of apparently sporadic CJD might be due to infection by sporadic cases of BSE cannot be dismissed outright. Screening programs needed to identify sporadic BSE have yet to be implemented, and we know from already extant testing programs that at least a proportion of infected animals have no symptoms and thus would never be identified in the absence of systematic testing. Thus, sporadic BSE (or for that matter, sporadic disease in any mammalian species) might be occurring on a regular basis at perhaps the same annual frequency as sporadic CJD in humans, that is, in the range of 1 case per million animals.
Whether humans might be more susceptible to atypical forms of BSE cannot be answered at this time. Experimentally transmitted BASE shows shorter incubation periods than BSE in at least 1 breed of cattle, bovinized transgenic mice, and Cynomolgus monkeys (12,13). In humanized transgenic mice, BASE transmitted, whereas typical BSE did not transmit (13). Paradoxically, the other major phenotype (H) showed an unusually long incubation period in bovinized transgenic mice (12).
The limited experimental evidence bearing on a possible relationship between BSE and sporadic CJD is difficult to interpret. The original atypical BASE strain of BSE had a molecular protein signature very similar to that of 1 subtype (type 2 M/V) of sporadic CJD in humans (5). In another study, a strain of typical BSE injected into humanized mice encoding valine at codon 129 showed a glycopattern indistinguishable from the same subtype of sporadic CJD (15). In a third study, the glycopatterns of both the H and L strains of atypical BSE evidently did not resemble any of the known sporadic CJD subtypes (12).
To these molecular biology observations can be added the epidemiologic data accumulated during the past 30 years. The hypothesis that at least some cases of apparently sporadic CJD are due to unrecognized BSE infections cannot be formally refuted, but if correct, we might expect by now to have some epidemiologic evidence linking BSE to at least 1 cluster of apparently sporadic cases of CJD. Although only a few clusters have been found (and still fewer published), every proposed cluster that has been investigated has failed to show any common exposure to bovines. For that matter, no common exposure has been shown to any environmental vehicles of infection, including the consumption of foodstuffs from bovine, ovine, and porcine sources, the 3 livestock species known to be susceptible to transmissible spongiform encephalopathies. Additional negative evidence comes from several large case-control studies in which no statistically significant dietary differences were observed between patients with sporadic CJD and controls (16,17).
On the other hand, the difficulty of establishing a link between BSE and CJD may be compounded by our ignorance of the infectious parameters of a sporadic form of BSE (e.g., host range, tissue distribution of infectivity, route of transmission, minimum infectious dose for humans, whether single or multiple). Presumably, these parameters would resemble those of variant CJD; that is, high infectivity central nervous system and lymphoreticular tissues of an infected cow find their way into products consumed by humans. Transmissions that might have occurred in the past would be difficult to detect because meat products are generally not distributed in a way that results in detectable geographic clusters.
Barring the discovery of a specific molecular signature (as in variant CJD), the most convincing clue to an association will come from the observation of trends over time of the incidence of typical and atypical BSE and of sporadic and variant CJD. With 4 diseases, each of which could have increasing, unchanging, or decreasing trends, there could be 81 (34) possible different combinations. However, it is highly likely that the trends for typical BSE and variant CJD will both decrease in parallel as feed bans continue to interrupt recycled contamination. The remaining combinations are thus reduced to 9 (32), and some of them could be highly informative.
For example, if the incidence of atypical BSE declines in parallel with that of typical BSE, its candidacy as a sporadic form of disease would be eliminated (because sporadic disease would not be influenced by current measures to prevent oral infection). If, on the other hand, atypical BSE continues to occur as typical BSE disappears, this would be a strong indication that it is indeed sporadic, and if in addition at least 1 form of what is presently considered as sporadic CJD (such as the type 2 M/V subtype shown to have a Western blot signature like BASE) were to increase, this would suggest (although not prove) a causal relationship (Figure 5).
Recognition of the different forms of BSE and CJD depends upon continuing systematic testing for both bovines and humans, but bovine testing will be vulnerable to heavy pressure from industry to dismantle the program as the commercial impact of declining BSE cases ceases to be an issue. Industry should be aware, however, of the implications of sporadic BSE. Its occurrence would necessitate the indefinite retention of all of the public health measures that exclude high-risk bovine tissues from the animal and human food chains, whereas its nonoccurrence would permit tissues that are now destroyed to be used as before, once orally acquired BSE has disappeared.
SNIP...
PLEASE READ FULL TEXT ;
http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm?s_cid=eid06_0965_e
3:00 Afternoon Refreshment Break, Poster and Exhibit Viewing in the Exhibit Hall
3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse
Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University
Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years. ***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.
6:30 Close of Day One
http://www.healthtech.com/2007/tse/day1.asp
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...
http://www.cjdsurveillance.com/resources-casereport.html
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
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?
http://www.neurology.org/cgi/eletters/60/2/176#535
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=dignosing+and+reporting+creutzfeldt+jakob+disease&searchid=1048865596978_1528&stored_search=&FIRSTINDEX=0&journalcode=jama
BRITISH MEDICAL JOURNAL
BMJ
http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2
BMJ
http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
THE SEVEN SCIENTIST REPORT ***
http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-EC244-Attach-1.pdf
PAUL BROWN M.D.
http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000490-vol40.pdf
9 December 2005 Division of Dockets Management (RFA-305)
SEROLOGICALS CORPORATION James J. Kramer, Ph.D. Vice President, Corporate Operations
http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000383-01-vol35.pdf
Embassy of Japan
http://www.fda.gov/ohrms/dockets/dockets/02n0273/02N-0273-EC240.htm
Dockets Entered on December 22, 2005
2005D-0330, Guidance for Industry and FDA Review Staff on Collection of Platelets
by Automated ... EC 203, McDonald's Restaurants Corporation, Vol #:, 34 ...
http://www.fda.gov/ohrms/dockets/dailys/05/Dec05/122205/122205.htm
03-025IF 03-025IF-631 Linda A. Detwiler [PDF] Page 1. 03-025IF 03-025IF-631 Linda A. Detwiler Page 2. Page 3. Page 4. Page 5. Page 6. Page 7. Page 8. Page 9. Page 10. Page 11. Page 12. http://www.fsis.usda.gov/OPPDE/Comments/03-025IF/03-025IF-631.pdf
03-025IF 03-025IF-634 Linda A. Detwiler [PDF] Page 1. 03-025IF 03-025IF-634 Linda A. Detwiler Page 2. Page 3. Page 4. Page 5. Page 6. Page 7. Page 8.
http://www.fsis.usda.gov/OPPDE/Comments/03-025IF/03-025IF-634.pdf
Page 1 of 17 9/13/2005 [PDF] ... 2005 6:17 PM To: fsis.regulationscomments@fsis.usda.gov Subject: [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food ...
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
03-025IFA 03-025IFA-6 Jason Frost [PDF] ... Zealand Embassy COMMENTS ON FEDERAL REGISTER 9 CFR Parts 309 et al [Docket No. 03- 025IF] Prohibition of the Use of Specified Risk Materials for Human Food and ...
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-6.pdf
In its opinion of 7-8 December 2000 (EC 2000), the SSC ... [PDF] Page 1. Linda A. Detwiler, DVM 225 Hwy 35 Red Bank, New Jersey 07701 Phone: 732-741-2290 Cell: 732-580-9391 Fax: 732-741-7751 June 22, 2005 FSIS Docket Clerk US ...
http://www.fsis.usda.gov/OPPDE/Comments/03-025IF/03-025IF-589.pdf
Terry S. Singeltary SR. P.O. Box 42 Bacliff, Texas USA 77518
CJD (NEW VAR.) UPDATE 2006 (12) ******************************* A ProMED-mail post
ProMED-mail is a program of the International Society for Infectious Diseases
[The definition of the designations deaths, definite cases, probable vCJD cases, and the case definitions can be found by accessing the Department of Health website, or by reference to a previous ProMED-mail post in this thread (for example, CJD (new var.) - UK: update March 2002 20020305.3693).
Data on vCJD cases from other parts of the world are now included in these updates whenever available.
Also, data on other forms of CJD (sporadic, iatrogenic, familial and GSS) are now included when they have some relevance to the incidence and etiology of vCJD. - Mod.CP]
In this update:
[1] UK: Department of Health monthly vCJD and CJD statistics, Mon 4 Dec 2006 [2] EUROCJD data as of 31 October 2006 [3] National (US) Prion Disease Pathology Surveillance Center Data: 8 Nov 2006 [4] The Netherlands: 2nd vCJD death [5] USA: 3rd vCJD case (ex Saudi Arabia)
****** [1] UK: vCJD and CJD statistics Date: Mon 4 Dec 2006 From: ProMED-mail Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease Statistics, Mon 4 Dec 2006 [edited]
The Department of Health is today [Mon 4 Dec 2006] issuing the latest information about the numbers of known cases of Creutzfeldt-Jakob disease. This includes cases of variant Creutzfeldt-Jakob disease [abbreviated in ProMED-mail as CJD (new var.) or vCJD], the form of the disease thought to be linked to BSE (bovine spongiform encephalopathy).
Definite and probable CJD cases in the UK, as at Fri 1 Dec 2006
----------------------------------------------------------------
Summary of vCJD cases - deaths ------------------------------
Deaths from definite vCJD (confirmed): 112 Deaths from probable vCJD (without neuropathological confirmation): 46 Deaths from probable vCJD (neuropathological confirmation pending): 0 Number of deaths from definite or probable vCJD (as above): 158
Summary of vCJD cases - alive -----------------------------
Number of probable vCJD cases still alive: 6
Total ----- Number of definite or probable vCJD (dead and alive): 164
(The next table will be published on Mon 8 Jan 2007).
Since the previous monthly statistics were released on Mon 6 Nov 2006, the total number of deaths from definite vCJD remains unchanged and stands at 158. The overall total number of definite or probable vCJD cases (dead and alive) remains unchanged at 164.
These data are consistent with the view that the vCJD outbreak in the UK is in decline. The peak number of deaths was 28 in the year 2000, followed by 20 in 2001, 17 in 2002, 18 in 2003, 9 in 2004, and 5 in 2005. The number of deaths due to definite or probable vCJD in the UK during the first 11 months of 2006 remains at 5.
Totals for all types of CJD cases in the UK in 2005 and 2006
----------------------------------------------------
As of Fri 1 Dec 2006, in the UK in the year 2005, there were 123 referrals of suspected CJD, and there were 65 deaths from sporadic CJD, 7 from familial CJD, 3 from iatrogenic CJD, 6 GSS (Gerstmann-Straussler-Scheinker) syndrome cases, and 5 deaths from vCJD.
The corresponding figures so far for the 1st 11 months of 2006 are: 98 referrals, 53 deaths from sporadic CJD, 5 from vCJD, 5 from familial CJD, 3 from GSS and one from iatrogenic CJD.
During the period from 1995, when vCJD was first diagnosed, up to the present there have been 953 deaths from all forms of CJD including the 158 deaths attributable to definite or probable vCJD.
[These data are accessible via ]
-- ProMED-mail
****** [2] EU - EUROCJD Data Date: Tue 31 Oct 2006 From: ProMED-mail Source: EUROCJD 31 Oct 2006 [edited]
The European And Allied Countries Collaborative Study Group of CJD (EUCJD)
----------------------------------------------------
This web-site includes information from 2 projects funded by the European Commission. The EUROCJD project started in 1993 and compares data from national registries in Australia, Austria, Canada, France, Germany, Italy, the Netherlands, Slovakia, Spain, Switzerland and the UK. The NEUROCJD project started in 1998 after the European Union Council recommended that epidemiological surveillance of CJD should be extended to all member states. The member states involved in this project are Belgium, Denmark, Finland, Greece, Iceland, Ireland, Israel, Norway and Portugal. Both projects are coordinated from the U.K. National CJD Surveillance Unit based in Edinburgh.
Current data as at November 2006*
---------------------------------
Country / Total No. of Primary cases (No. alive) / Cumulative residence in UK (>6 months) / Secondary transmission by blood transfusion
United Kingdom / 162 (6) / 164 / 2 (0) France / 21 (2) / 1 / 0 Republic of Ireland / 4 (1) / 2 / 0 Italy / 1 (0) / 0 / 0 USA / 3 (1*) / 2 / 0 / 0 Canada / 1 (0) / 1 / 0 Saudi Arabia / 1 (1) / 0 / 0 Japan / 1** (0) / 0 / 0 Netherlands*** / 2 (0) / 0 / 0 Portugal / 1 (1) / 0 / 0 Spain / 1 (0) / 0 / 0
Total / 197 (11) / - / 2
Footnotes ----------
* One case probably contracted in Saudi Arabia [see part (5) below], and 2 in U.K. ** Residence in the UK for 24 days *** Data for the Netherlands case (see part [4] below) was omitted in error from the corresponding table for October posted on 31 Oct 2006
-- ProMED-mail
****** [3] USA - CJD National Prion Disease Surveillance Data Date: Wed 8 Nov 2006 From: Terry S. Singeltary Sr Source: CJD Surveillance, National Prion Disease Pathology Surveillance Center, 8 Nov 2006 [edited]
Resources/Case Report ---------------------
Year / Referral / Total / Sporadic / Familial / Iatrogenic / vCJD
1997 / 104 / 60 / 54 / 6 / 0 / 0 1998 / 94 / 51 / 44 / 6 / 1 / 0 1999 / 114 / 74 / 65 / 9 / 0 / 0 2000 / 169 / 111 / 97 / 12 / 2 / 0 2001 / 2247 / 154 / 138 / 16 / 0 / 0 2002 / 265 / 151 / 127 / 22 / 1 / 0 2003 / 284 / 191 / 142 / 45 / 1 / 0 2004 / 360 / 202 / 167 / 21 / 0 / 1* 2005 / 357 / 201 / 144 / 39 / 0 / 0 2006 / 208 / 86 / 46 / 8 / 0 /1**
Total / 2202 / 1281 / 1024 / 184 / 5 / 2*
* Acquired in the United Kingdom ** Acquired in Saudi Arabia [see part (5) below]
[The gradual increase in sporadic and other forms of CJD in the USA throughout this period may merely reflect improvements in surveillance and diagnosis rather than a real increase in incidence. For example, according to the tables above, there were approximately 1 per million cases of sporadic CJD in the USA during 2005, compared with 2 cases per million in the UK during the same period. This difference could be indicative of a real difference in incidence or a difference in efficiency of diagnosis. - Mod.CP]
-- Terry S. Singeltary Sr
****** [4] The Netherlands - 2nd death Date: Thu 16 Nov 2006 From: Mary Marshall Source: iol.co.za Medical, Reuters report, Thu 16 Nov 2006 [edited]
A 2nd Dutch person has died from the human variant of mad cow disease following the death of a woman last year, health authorities said on Thu 16 Nov 2006. The Dutch Institute for Health and Environment (RIVM) give no details about the victim, but Dutch television stations said he was a 16-year-old boy.
A spokeswoman for the RIVM said the victim died from the brain-wasting [variant] Creutzfeldt-Jakob Disease (vCJD), the human form of Bovine Spongiform Encephalopathy (BSE) about 2 weeks ago. She did not explain why the RIVM had waited 2 weeks to confirm the death, which was reported in Dutch media on Thursday. The RIVM diagnosed the person with the human variant of mad cow [disease] in June and said at the time that the patient had most probably become infected by eating contaminated meat products. It was the 2nd Dutch death from the human variant of mad cow disease after a 26-year-old woman died in May 2005.
The disease is fatal and incurable. It is thought to be caused by eating food tainted with material from cattle with BSE, a progressive neurological disorder. More than 150 cases of vCJD have been reported around the world, mostly in Britain, but also in France, Ireland, Italy, Japan, Canada and the United States. Mad cow disease first emerged in Britain in the 1980s and has forced the destruction of millions of cattle.
-- ProMED-mail
****** [5] Date: Wed 29 Nov 2006 From: Terry S. Singeltary Sr. Source: CDC, National Center for Infectious Diseases, Wed 29 Nov 2006 [edited]
Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the Middle East
---------------------------------------------------
The Virginia Department of Health [] and the Centers for Disease Control and Prevention announced the recent confirmation of a vCJD case in a U.S. resident. This is the 3rd vCJD case identified in a U.S. resident. This latest U.S. case occurred in a young adult who was born and raised in Saudi Arabia and has lived in the United States since late 2005. The patient occasionally stayed in the United States for up to 3 months at a time since 2001 and there was a shorter visit in 1989. In late November 2006, the Clinical Prion Research Team at the University of California San Francisco Memory and Aging Center confirmed the vCJD clinical diagnosis by pathologic study of adenoid and brain biopsy tissues. The 2 previously reported vCJD case-patients in U.S. residents were each born and raised in the United Kingdom (U.K.), where they were believed to have been infected by the agent responsible for their disease. There is strong scient! ific evidence that the agent causing vCJD is the same agent that causes bovine spongiform encephalopathy (BSE, commonly known as mad cow disease).
Variant CJD is a rare, degenerative, fatal brain disorder that emerged in the United Kingdom in the mid-1990s. Although experience with this new disease is limited, evidence to date indicates that there has never been a case transmitted from person-to-person except through blood transfusion. Instead, the disease is thought to result primarily from consumption of cattle products contaminated with the BSE agent. Although no cases of BSE in cattle have been reported in Saudi Arabia, potentially contaminated cattle products from the United Kingdom may have been exported to Saudi Arabia for many years during the large U.K. BSE outbreak.
The current case-patient has no history of receipt of blood, a past neurological procedure, or residing in or visiting countries of Europe. Based on the patient's history, the occurrence of a previously reported Saudi case of vCJD attributed to likely consumption of BSE-contaminated cattle products in Saudi Arabia, and the expected greater than 7 year incubation period for food-related vCJD, this U.S. case-patient was most likely infected from contaminated cattle products consumed as a child when living in Saudi Arabia (1). The current patient has no history of donating blood and the public health investigation has identified no risk of transmission to U.S. residents from this patient.
As of November 2006, 200 vCJD patients were reported world-wide, including 164 patients identified in the United Kingdom, 21 in France, 4 in the Republic of Ireland, 3 in the United States (including the present case-patient), 2 in the Netherlands and 1 each in Canada, Italy, Japan, Portugal, Saudi Arabia and Spain. Of the 200 reported vCJD patients, all except 10 of them (including the present case-patient) had resided either in the United Kingdom (170 cases) for over 6 months during the 1980-1996 period of the large UK BSE outbreak or alternatively in France (20 cases).
As reported in 2005 (1), the U.S. National Prion Disease Pathology Surveillance Center at Case Western Reserve University confirmed the diagnosis in the one previously identified case of vCJD in a Saudi resident. He was hospitalized in Saudi Arabia and his brain biopsy specimen was shipped to the United States for analysis. This earlier vCJD case-patient was believed to have contracted his fatal disease in Saudi Arabia (1).
Reference --------- (1) Belay ED, Sejvar JJ, Shieh W-J, Wiersma ST, Zou W-Q, Gambetti P, Hunter S, Maddox RA, Crockett L, Zaki SR, Schonberger LB. Variant Creutzfeldt-Jakob disease death, United States. Emerg Infect Dis 2005, 11 (9):1351-1354.
-- Terry S. Singeltary Sr.
[There is inconsistency in the number of vCJD cases attributed to the United States. The Virginia Department of Health/CDC report cited in the above list 3 cases -- 2 cases probably contracted in the U.K. and the young adult described above who was born and raised in Saudi Arabia. The EUROCJD data for October 2006 in part (2) above also lists 2 U.S. cases, both assumed to have been contracted in the U.K. The U.S. National Prion Disease Pathology Surveillance Center Data published on 8 Nov 2006 (part 3 above), on the other hand, list only a single U.S. case believed to have been contracted in the U.K. - Mod.CP]
[see also: CJD (new var.) update 2006 (11) 20061106.3190 CJD (new var.) update 2006 (10) 20061002.2820 CJD (new var.) update 2006 (09) 20060904.2519 CJD (new var.) update 2006 (08) 20060807.2207 CJD (new var.) update 2006 (07) 20060703.1831 CJD (new var.) - Netherlands: 2nd case 20060623.1741 CJD (new var.) update 2006 (06) 20060605.1566 CJD (new var.) update 2006 (05) 20060508.1332 CJD (new var.) update 2006 (04) 20060404.1005 CJD (new var.) update 2006 (03) 20060306.0728 CJD (new var.) - UK: 3rd transfusion-related case 20060209.0432 CJD (new var.) update 2006 (02) 20060206.0386 CJD (new var.) update 2006 (01) 20060111.0101 CJD (new var.) update 2006 20060111.0101 2005 ---- CJD (new var.) update 2005 (12) 20051209.3547 CJD (new var.) update 2005 (11) 20051108.3270 CJD (new var.) update 2005 (10) 20051006.2916 CJD (new var.) update 2005 (09) 20050905.2627 CJD (new var.) update 2005 (08) 20050801.2237 CJD (new var.) update 2005 (07) 20050703.1889 CJD (new var.) update 2005 (06) 20050607.1584 CJD (new var.) update 2005 (05) 20050505.1243 CJD (new var.) update 2005 (04) 20050405.0982 CJD (new var.) update 2005 (03) 20050308.0687 CJD (new var.) update 2005 (02) 20050211.0467 CJD (new var.) - UK: update 2005 (01) 20050111.0095 2004 ---- CJD, genetic susceptibility 20041112.3064 CJD (new var.) - UK: update 2004 (14) 20041206.3242 CJD (new var.) - UK: update 2004 (13) 20041103.2977 CJD (new var.) - UK: update 2004 (12) 20041023.2871 CJD (new var.) - UK: update 2004 (11) 20041008.2758 CJD (new var.) - UK: update 2004 (10) 20040909.2518 CJD (new var.) - UK: update 2004 (09) 20040809.2199 CJD (new var.) - UK: update 2004 (08) 20040806.2150 CJD (new var.) - UK: update 2004 (07) 20040706.1807 CJD (new var.) - UK: update 2004 (06) 20040608.1535 CJD (new var.) - UK: update 2004 (05) 20040510.1262 CJD (new var.) - UK: update 2004 (04) 20040406.0937 CJD (new var.) - UK: update 2004 (03) 20040314.0713 CJD (new var.) - UK: update 2004 (02) 20040202.0400 CJD (new var.) - UK: update 2004 (01) 20040106.0064 CJD (new var.) - France: 8th case 20041022.2864 CJD (new var.) - France: 9th case 20041123.3138 CJD (new var.), blood supply - UK 20040318.0758 CJD (new var.), carrier frequency study - UK 20040521.1365 2003 ---- CJD (new var.) - UK: update 2003 (13) 20031216.3072 CJD (new var.) - UK: update 2003 (01) 20030108.0057 2002 ---- CJD (new var.) - UK: update Dec 2002 20021207.5997 CJD (new var.) - UK: update Jan 2002 20020111.3223 2001 ---- CJD (new var.), incidence & trends - UK (02) 20011124.2875 CJD (new var.), incidence & trends - UK 20011115.2816 CJD (new var.) - UK: reassessment 20011029.2671 CJD (new var.) - UK: update Oct 2001 20011005.2419 CJD (new var.) - UK: regional variation (02) 20010907.2145 CJD (new var.) - UK: update Sep 2001 20010906.2134 CJD (new var.) - UK: update Aug 2001 20010808.1872 CJD (new var.) - UK: 9th Annual Report 20010628.1231 CJD (new var.) - UK: update June 2001 20010622.1188 CJD (new var.) - UK: update 3 Jan 2001 20010104.0025] ................cp/pg/mpp
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Volume 12, Number 12–December 2006
PERSPECTIVE
On the Question of Sporadic
or Atypical Bovine SpongiformEncephalopathy and
Creutzfeldt-Jakob Disease
Paul Brown,* Lisa M. McShane,† Gianluigi Zanusso,‡ and Linda Detwiler§
Strategies to investigate the possible existence of sporadic
bovine spongiform encephalopathy (BSE) require
systematic testing programs to identify cases in countries
considered to have little or no risk for orally acquired disease,
or to detect a stable occurrence of atypical cases in
countries in which orally acquired disease is disappearing.
To achieve 95% statistical confidence that the prevalence
of sporadic BSE is no greater than 1 per million (i.e., the
annual incidence of sporadic Creutzfeldt-Jakob disease
[CJD] in humans) would require negative tests in 3 million
randomly selected older cattle. A link between BSE and
sporadic CJD has been suggested on the basis of laboratory
studies but is unsupported by epidemiologic observation.
Such a link might yet be established by the discovery
of a specific molecular marker or of particular combinations
of trends over time of typical and atypical BSE and various
subtypes of sporadic CJD, as their numbers are influenced
by a continuation of current public health measures that
exclude high-risk bovine tissues from the animal and
human food chains.
SNIP...
Sporadic CJD
The possibility that at least some cases of apparently sporadic CJD might be due to infection by sporadic cases of BSE cannot be dismissed outright. Screening programs needed to identify sporadic BSE have yet to be implemented, and we know from already extant testing programs that at least a proportion of infected animals have no symptoms and thus would never be identified in the absence of systematic testing. Thus, sporadic BSE (or for that matter, sporadic disease in any mammalian species) might be occurring on a regular basis at perhaps the same annual frequency as sporadic CJD in humans, that is, in the range of 1 case per million animals.
Whether humans might be more susceptible to atypical forms of BSE cannot be answered at this time. Experimentally transmitted BASE shows shorter incubation periods than BSE in at least 1 breed of cattle, bovinized transgenic mice, and Cynomolgus monkeys (12,13). In humanized transgenic mice, BASE transmitted, whereas typical BSE did not transmit (13). Paradoxically, the other major phenotype (H) showed an unusually long incubation period in bovinized transgenic mice (12).
The limited experimental evidence bearing on a possible relationship between BSE and sporadic CJD is difficult to interpret. The original atypical BASE strain of BSE had a molecular protein signature very similar to that of 1 subtype (type 2 M/V) of sporadic CJD in humans (5). In another study, a strain of typical BSE injected into humanized mice encoding valine at codon 129 showed a glycopattern indistinguishable from the same subtype of sporadic CJD (15). In a third study, the glycopatterns of both the H and L strains of atypical BSE evidently did not resemble any of the known sporadic CJD subtypes (12).
To these molecular biology observations can be added the epidemiologic data accumulated during the past 30 years. The hypothesis that at least some cases of apparently sporadic CJD are due to unrecognized BSE infections cannot be formally refuted, but if correct, we might expect by now to have some epidemiologic evidence linking BSE to at least 1 cluster of apparently sporadic cases of CJD. Although only a few clusters have been found (and still fewer published), every proposed cluster that has been investigated has failed to show any common exposure to bovines. For that matter, no common exposure has been shown to any environmental vehicles of infection, including the consumption of foodstuffs from bovine, ovine, and porcine sources, the 3 livestock species known to be susceptible to transmissible spongiform encephalopathies. Additional negative evidence comes from several large case-control studies in which no statistically significant dietary differences were observed between patients with sporadic CJD and controls (16,17).
On the other hand, the difficulty of establishing a link between BSE and CJD may be compounded by our ignorance of the infectious parameters of a sporadic form of BSE (e.g., host range, tissue distribution of infectivity, route of transmission, minimum infectious dose for humans, whether single or multiple). Presumably, these parameters would resemble those of variant CJD; that is, high infectivity central nervous system and lymphoreticular tissues of an infected cow find their way into products consumed by humans. Transmissions that might have occurred in the past would be difficult to detect because meat products are generally not distributed in a way that results in detectable geographic clusters.
Barring the discovery of a specific molecular signature (as in variant CJD), the most convincing clue to an association will come from the observation of trends over time of the incidence of typical and atypical BSE and of sporadic and variant CJD. With 4 diseases, each of which could have increasing, unchanging, or decreasing trends, there could be 81 (34) possible different combinations. However, it is highly likely that the trends for typical BSE and variant CJD will both decrease in parallel as feed bans continue to interrupt recycled contamination. The remaining combinations are thus reduced to 9 (32), and some of them could be highly informative.
For example, if the incidence of atypical BSE declines in parallel with that of typical BSE, its candidacy as a sporadic form of disease would be eliminated (because sporadic disease would not be influenced by current measures to prevent oral infection). If, on the other hand, atypical BSE continues to occur as typical BSE disappears, this would be a strong indication that it is indeed sporadic, and if in addition at least 1 form of what is presently considered as sporadic CJD (such as the type 2 M/V subtype shown to have a Western blot signature like BASE) were to increase, this would suggest (although not prove) a causal relationship (Figure 5).
Recognition of the different forms of BSE and CJD depends upon continuing systematic testing for both bovines and humans, but bovine testing will be vulnerable to heavy pressure from industry to dismantle the program as the commercial impact of declining BSE cases ceases to be an issue. Industry should be aware, however, of the implications of sporadic BSE. Its occurrence would necessitate the indefinite retention of all of the public health measures that exclude high-risk bovine tissues from the animal and human food chains, whereas its nonoccurrence would permit tissues that are now destroyed to be used as before, once orally acquired BSE has disappeared.
SNIP...
PLEASE READ FULL TEXT ;
http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm?s_cid=eid06_0965_e
3:00 Afternoon Refreshment Break, Poster and Exhibit Viewing in the Exhibit Hall
3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse
Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University
Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years. ***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.
6:30 Close of Day One
http://www.healthtech.com/2007/tse/day1.asp
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...
http://www.cjdsurveillance.com/resources-casereport.html
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
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?
http://www.neurology.org/cgi/eletters/60/2/176#535
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=dignosing+and+reporting+creutzfeldt+jakob+disease&searchid=1048865596978_1528&stored_search=&FIRSTINDEX=0&journalcode=jama
BRITISH MEDICAL JOURNAL
BMJ
http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2
BMJ
http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
THE SEVEN SCIENTIST REPORT ***
http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-EC244-Attach-1.pdf
PAUL BROWN M.D.
http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000490-vol40.pdf
9 December 2005 Division of Dockets Management (RFA-305)
SEROLOGICALS CORPORATION James J. Kramer, Ph.D. Vice President, Corporate Operations
http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000383-01-vol35.pdf
Embassy of Japan
http://www.fda.gov/ohrms/dockets/dockets/02n0273/02N-0273-EC240.htm
Dockets Entered on December 22, 2005
2005D-0330, Guidance for Industry and FDA Review Staff on Collection of Platelets
by Automated ... EC 203, McDonald's Restaurants Corporation, Vol #:, 34 ...
http://www.fda.gov/ohrms/dockets/dailys/05/Dec05/122205/122205.htm
03-025IF 03-025IF-631 Linda A. Detwiler [PDF] Page 1. 03-025IF 03-025IF-631 Linda A. Detwiler Page 2. Page 3. Page 4. Page 5. Page 6. Page 7. Page 8. Page 9. Page 10. Page 11. Page 12. http://www.fsis.usda.gov/OPPDE/Comments/03-025IF/03-025IF-631.pdf
03-025IF 03-025IF-634 Linda A. Detwiler [PDF] Page 1. 03-025IF 03-025IF-634 Linda A. Detwiler Page 2. Page 3. Page 4. Page 5. Page 6. Page 7. Page 8.
http://www.fsis.usda.gov/OPPDE/Comments/03-025IF/03-025IF-634.pdf
Page 1 of 17 9/13/2005 [PDF] ... 2005 6:17 PM To: fsis.regulationscomments@fsis.usda.gov Subject: [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food ...
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
03-025IFA 03-025IFA-6 Jason Frost [PDF] ... Zealand Embassy COMMENTS ON FEDERAL REGISTER 9 CFR Parts 309 et al [Docket No. 03- 025IF] Prohibition of the Use of Specified Risk Materials for Human Food and ...
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-6.pdf
In its opinion of 7-8 December 2000 (EC 2000), the SSC ... [PDF] Page 1. Linda A. Detwiler, DVM 225 Hwy 35 Red Bank, New Jersey 07701 Phone: 732-741-2290 Cell: 732-580-9391 Fax: 732-741-7751 June 22, 2005 FSIS Docket Clerk US ...
http://www.fsis.usda.gov/OPPDE/Comments/03-025IF/03-025IF-589.pdf
Terry S. Singeltary SR. P.O. Box 42 Bacliff, Texas USA 77518
Labels: atypical, BSE, sporadic CJD, USA, vCJD
posted by Terry S. Singeltary Sr. | 10:05 AM
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