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From: TSS ()
Date: July 21, 2005 at 1:02 pm PST

Lord Warner: My honourable friend the Parliamentary Under-Secretary of State (Caroline Flint) has made the following Written Ministerial Statement.

Further to the Statements made to the House by the then Secretary of State (John Reid) on 17 December 2003 and 16 March 2004 (and the Written Statements of 22 July 2004 and 9 September 2004) concerning variant Creutzfeldt-Jakob disease (vCJD) and blood, I wish to provide a further update on this subject.

Following cases of possible transmission of vCJD by blood transfusion, we have already put in place a series of precautionary public health measures. These include:

in December 2003 we put in place arrangements for contacting recipients of blood from donors who went on to develop vCJD so that any necessary action could be taken;

since April 2004 we have excluded anyone who has received a blood transfusion since January 1980 from donating blood; and

in September 2004 we announced arrangements to identify and notify patients who had received certain batches of UK manufactured plasma products.

20 Jul 2005 : Column WS114

In the light of further advice I have received from two of my department's expert committees, the CJD Incidents Panel (CJDIP) and the Committee on Microbiological Safety of Blood, Tissues and Organs (MSBTO), I am now announcing further public health precautions in relation to a small group of blood donors whose blood has been transfused to people who later developed vCJD.

The Chief Medical Officer, Sir Liam Donaldson, had asked the expert committees to consider the implications for donors where a recipient of their blood had developed vCJD. The recommendations of the committees are based on an assessment of risk undertaken by the Department of Health's analysts. The risk assessment is being published on the department's website.

There are 110 donors in the UK whose blood was given to three people who later developed vCJD and for whom this blood might be a possible source of their infection. The advice of the committees is that, although we do not know whether these cases of vCJD could be related to the blood that they received, we should take precautionary steps to inform and support the individual blood donors concerned and to safeguard public health.

As an extension to the current precautionary measures, these people are being contacted by the National Blood Service and advised not to donate blood, tissues or organs. Current donors from this group of 110 are being contacted today and offered expert advice and support. The National Blood Service will contact the GPs of lapsed donors, that is those who have not donated blood during the past five years, and make arrangements to contact these people as soon as practicable.

The committees have also advised that the donors in question should be considered at risk of vCJD for wider public health purposes and that the donors and their clinicians should be informed of their risk status and asked to implement the public health precautions currently specified by the CJD Incidents Panel. This means that they should inform doctors, nurses and dentists of their status if they present for surgery or other invasive medical procedures.

These public health precautionary measures are the same as those applied to any patients considered by the CJDIP to be at risk of vCJD, including the individuals notified following the previous Statements to the House.

There is another group of people for whom further public health precautions may need to be considered. This group is all the other recipients of blood from the currently identified group of 110 donors (estimated to be up to 3,000 individuals). At present, these people are already excluded from blood donation themselves by the measures implemented in April 2004. I have asked for additional expert advice on this group and I will take further action if necessary.

20 Jul 2005 : Column WS115

Blood donors should be assured that it is not possible to contract vCJD by giving blood. Blood donors are highly committed to helping others and we greatly value their contribution. The NHS depends on their continued commitment to donating blood which saves lives every day in this country.

The vast majority of the over two million current blood donors will not be involved in this new safety measure and need take no action. However, current and past blood donors who are concerned can contact the National Blood Service helpline on 0845 7711 711.

People who have received blood donations and other members of the public who are concerned should contact NHS Direct on 0845 850 9850.

As with our actions to date on the possible transmission of vCJD, we continue to follow a highly precautionary approach.



Risk Assessment for vCJD and Dentistry
This report analyses the potential risk of vCJD transmission through re-use of instruments in dental surgery. Based on an existing EOR model for hospital surgery, the analysis considers a wide range of scenarios to allow for multiple uncertainties. It covers procedures carried out in "high street" dental practice, rather than more specialised maxillo-facial surgery.

If patients are recognised as being at heightened risk of infection with any form of CJD, instruments used on them are already subject to special precautions against onward infection, being quarantined and if necessary destroyed. However this analysis concerns potential risks of transmission amongst the generality of patients, where instruments might unknowingly be used on someone incubating vCJD. Two potential transmission routes are considered:

a.. Expert consultation suggested an initial focus on the possibility of transmitting infection through accidental abrasion of an infective patient's lingual tonsils, these being the only relevant oral tissue for which there is direct evidence of vCJD infectivity in humans. Most of this analysis focuses on this topic.
b.. However, vCJD infectivity in other tissues encountered in dentistry - e.g. dental pulp - is implied by some animal models. Though such infectivity has not so far been detected in humans, the possibility cannot be ruled out. Furthermore there is evidence that some instruments used in endodontic surgery - e.g. files and reamers - are particularly difficult to clean, and may carry significant residues of material after washing. We therefore also include some illustrative calculations of the transmission risks that could be posed if this residue were to carry vCJD infectivity. It should be stressed that this part of the analysis is purely hypothetical.
Risks to individual patients

On present evidence and advice, the chance of vCJD being transmitted via tonsillar abrasion appears remote. The previous analysis of hospital surgery provides some points of comparison. For example, if similar standards of instrument decontamination are achieved in the two settings:

a.. With assumptions about tissue abrasion as suggested by expert consultation, a single dental procedure on an infective patient would be about 1,000,000,000 times less likely to transmit vCJD than - say - a tonsillectomy. (The latter in turn would be much less likely to do so than a procedure involving the Central Nervous System or the back of the eye.)
b.. Even with very pessimistic assumptions about the chances of tissue abrasion, a differential of about 10,000-fold with tonsillectomy would remain.
c.. If tissues such as dental pulp were to be infective, the risks of transmitting vCJD would obviously increase. However the analysis suggests that even taking a pessimistic scenario, the risks per operation would still be low (at least 10 times lower than for a tonsillectomy).
Risks to Public Health

Any risk of transmission depends critically on the initial prevalence of the disease and the number of invasive dental procedures. As the former is unknown, a range of scenarios is considered. The number of dental procedures in the UK is very large - estimated at about 75 million annually, including both NHS and private treatment. Of these, around 2 million are endodontic procedures. Even so, any risk to public health posed by dental transmission appears small compared to that for hospital surgery in similar scenarios.

Risk Reduction Measures

As for hospital surgery, the key consideration in minimising any risk of transmission is assuring the efficacy of instrument decontamination, even though current methods cannot remove such risks completely. In line with existing SEAC advice, potential risks can be further reduced by introduction of more single-use instruments where appropriate, especially of difficult-to-clean items.

Qualification of Analysis

Almost all the analysis reported here is subject to two major caveats. The first is that decontamination procedures used in "high street" dentistry are not (in general) significantly less effective than has been assumed here. Though the assumptions used are intended to give fairly conservative estimates for the reductions in infectivity achieved, this continues to be an area of uncertainty. It therefore remains important to monitor actual decontamination practice and encourage its improvement.

a.. Download summary report (PDF, 97K)
a.. Download annexes (PDF, 204

Reviewed 20 July 2005

What is the CJD Incidents Panel?
What is a CJD incident?
How to report a CJD incident
CDSC CJD Section support for local incident investigations
Infection control guidance
CJD Incidents Panel terms of reference
Members of the CJD Incidents Panel
Framework document
Public summaries of CJD Incidents Panel meetings
Annual reports of the CJD Incidents Panel

Information about potential surgical exposure to CJD - to be added in July 2005

What is the CJD Incidents Panel?
The CJD Incidents Panel is an expert committee set up in 2000 by the Chief Medical Officer and accountable to the Department of Health. The HPA provides the secetariat for the Panel on behalf of the Department of Health. The CJD Incidents Panel advises hospitals, trusts and public health teams (throughout the UK) on how to manage incidents involving possible transmission of CJD between patients. The CJD Incidents Panel is a sub group of the Advisory Committee on Dangerous Pathogens Working Group on Transmissible Spongiform Encephalopathies.

What is a CJD Incident?
Incidents involve the potential transmission of CJD between patients through invasive clinical procedures.

Incidents occur when patients diagnosed with (or suspected of having) CJD, or patients identified as 'at risk' of CJD, have undergone invasive medical procedures that may have put other patients at risk. Procedures may include surgery, blood donations, and organ and tissue donations.

How to report a CJD Incident
All requests for advice from the Panel should be addressed to:

Dr Kate Soldan
Consultant Scientist
Scientific Secretary to the CJD Incidents Panel
Health Protection Agency Centre for Infections
Communicable Disease Surveillance Centre
61 Colindale Avenue
London NW9 5EQ
Tel: +44 (0)20 8327 6411

Other secretariat contacts are:

Ms Helen Janecek
Senior Administrator
Tel: +44 (0)20 8327 6074
Ms Katie Oakley
CJD Incidents Nurse Investigator
Tel: +44 (0)20 8327 6519

To report an incident to the secretariat, please use the CJDIP reporting form (Version May 2005)

CDSC CJD Team support for local incident investigations
As well as running the CJD Secretariat, the CDSC CJD Team provides additional support for local public health teams, including:

a.. Helping local teams to carry out investigations
b.. Helping local teams to implement Panel advice
Please contact Kate Soldan for further information.

Infection control guidance
Advice on infection control for patients with CJD, suspected of having CJD, or at risk of CJD, is contained in the Joint ACDP/SEAC Working Group on TSE's 2003 guidance 'Transmissible Spongiform Encephalopathies: safe working and the prevention of infection'.

In February 2005 The Chief Dental Officer issued a letter, Information for dentists about the management of patients with or 'at risk' of CJD.

Guidance for local reporting
Clinicians caring for patients with CJD should inform the local Consultant in Communicable Disease Control (or their equivalent in Scotland) of cases classified as possible, probable or definite CJD. Cases should also be reported jointly to the NCJDSU and the National Prion Clinic by fax using the form annexed to the CMO letter dated 9th July 2004.

CJD Incidents Panel terms of reference
The Panel's terms of reference are:

"To assist all those bodies responsible for the provision and delivery of healthcare to decide on the most appropriate action to take to handle incidents involving potential transmission of Creutzfeldt-Jakob Disease (CJD) and variant CJD (vCJD) between patients through clinical interventions, including via surgical instruments, tissues, organs and blood and to keep the relevant devolved administrations informed.

"To consider what information should be collected on patients who may have been exposed; advise on what studies or follow-up may be needed; advise Directors of Public Health on patient tracing and notification exercises where these are indicated; and advise on whether any other measures are needed to protect the wider public health.

"To make regular reports to the Advisory Committee on Dangerous Pathogens Working Group on Transmissible Spongiform Encephalopathies (TSE Working Group).

"To keep the expert guidance under review and make recommendations to the TSE Working Group for further guidelines as necessary, in the light of experience of incidents and research in progress."

Members of the CJD Incidents Panel
Appointments to the Panel are made by the Chief Medical Officer usually for a term of one to three years. Panel members are selected on the basis of their specialist expertise and include people with medical and scientific expertise as well as lay members and ethicists.

Framework Document
The Panel's document Management of possible exposure to CJD through medical procedures sets out a framework for the Panel's advice and seeks to inform health professionals and managers in incidents involving potential transmission of CJD between patients. Both the Framework Document and the interim report of the written consultation on its development are available to download in pdf format as described below:

Framework document - 15.03.04 335 KB
Annexes 1 to 3 - 15.03.04 88 KB
Annex 4 - 15.03.04 88 KB
Glossary - 15.03.04 61 KB

Interim report on written consultation 74 KB
Annex 1 - tables 53 KB
Annex 2 - key responses 260 KB
Annex 3 - organisation type 69 KB

Much of the Panel's work has been based on risk assessments commissioned by the Department of Health which include the following:

Risk Assessment of Exposure to vCJD Infectivity in Blood and Blood Products
Risk Assessment for vCJD and Dentistry
Risk Assessment for transmission of vCJD via surgical instruments: a modelling approach and numerical scenarios.

Public summaries of CJD Incidents Panel meetings
The purpose of these summaries (available below for download in pdf format) is to provide progress reports on the work of the CJD Incidents Panel and to stimulate debate on issues raised. The CJD Incidents Panel wishes to make information on the progress of its work publicly available as rapidly as possible. The attached public summaries are not intended to provide guidance nor to indicate conclusions the Panel has reached but they highlight the areas that are under consideration, particularly those areas where there is a high degree of uncertainty or there are differences of opinion. The Panel hope that these summaries will be of interest to healthcare professionals who may have to cope with an incident in the future. The Panel also hopes that the summaries will stimulate debate, not only among healthcare professionals, but also in the wider public.

January 2005 (added 15.07.05) 35 KB
September 2004 (added 10.02.05) 37 KB
May 2004 31 KB
February 2004 29 KB
October 2003 24 KB
June 2003 24KB
February 2003 23 KB
October 2002 14 KB
June 2002 27 KB
April 2002 29 KB
October 2001 22KB
June 2001 22KB
February 2001 15 KB
November 2000 11 KB

Annual reports of the CJD Incidents Panel
The CJD Incidents Panel reports to the Advisory Committee on Dangerous Pathogens Working Group on Transmissible Spongiform Encephalopathies. The annual reports are available to download in pdf format below:

Third annual report 2002 to 2003 99 KB
Second annual report 2001 to 2002 104 KB
First annual report 2000 to 2001 66 KB

Information about surgical exposure to CJD - to be added in July 2005
Further information about CJD and exposures to potentially contaminated surgical instruments is available in the following documents. These documents are available from the HPA (contacts above), and wil be available to download from this website very shortly (in July 2005).

a.. CJD and Surgical Exposure - Information for Patients
b.. vCJD and Surgical Exposure - Information for Patients
c.. CJD and vCJD surgical exposure - Clinical Information

Information about notification of individuals considered to be 'at-risk' of CJD

Transmission of the 263K scrapie strain by the dental route
Loredana lngrosso1, Flavio Pisani1 and Maurizio Pocchiari1

Laboratory of Virology, lstituto Superiore di Sanità, Viale Regina Elena 299, 00161 Rome, Italy1

Author for correspondence: Maurizio Pocchiari.Fax +39 06 49903012. e- mail


Apart from a few cases of iatrogenic and familial human transmissible spongiform encephalopathies (TSEs) or prion diseases, the cause of Creutzfeldt-Jakob disease (CJD) remains unknown. In this paper we investigated the possibility that dental procedures may represent a potential route of infection. This was assessed by using the experimental model of scrapie in hamster. In the first part of this study we found that after intraperitoneal inoculation, oral tissues commonly involved in dental procedures (gingival and pulp tissues) bore a substantial level of infectivity. We also found high scrapie infectivity in the trigeminal ganglia, suggesting that the scrapie agent had reached the oral tissues through the sensitive terminal endings of the trigeminal nerves. In the second part of the study we inoculated a group of hamsters in the tooth pulp and showed that all of them developed scrapie disease. In these animals, we detected both infectivity and the pathological prion protein (PrPsc) in the trigeminal ganglion homolateral to the site of injection but not in the controlateral one. This finding suggests that the scrapie agent, and likely other TSE agents as well, spreads from the buccal tissues to the central nervous system through trigeminal nerves. Although these findings may not apply to humans affected by TSEs, they do raise concerns about the possible risk of transmitting these disorders through dental procedures. Particular consideration should be taken in regard to new variant CJD patients because they may harbour more infectivity in peripheral tissues than sporadic CJD patients.




In this study we found that gingival and pulp tissues of experimentally scrapie-affected hamsters bear a significant level of infectivity. These findings extend previous data of infectivity in gingival tissues of mice infected with the ME7 (Adams & Edgar, 1978 ) or 139A (Carp, 1982 ) strain of scrapie, and reinforce the notion that oral tissues in scrapie-affected rodents harvest some infectivity. It is likely that the scrapie agent reaches the buccal tissues through the sensitive terminal endings of trigeminal branches. That is supported by the high level of scrapie infectivity (estimated titre, 8·1 log LD50/g) and the great amount of PrP27-30 in the trigeminal ganglia. These results fit with other data reporting infectivity or PrPsc deposit in trigeminal ganglia of cattle affected by BSE (Wells et al., 1998 ) and patients with CJD (Guiroy et al., 1989 ). Altogether these data suggest that similarly to spinal ganglia and other peripheral nervous tissues (Hadlow et al., 1980 , 1982 ; Kimberlin et al., 1983a ; Groschup et al., 1996 ; Wells et al. , 1998 ; McBride & Beekes, 1999 ), trigeminal ganglia can sustain the replication of scrapie and other TSE agents, and as a consequence of that, oral tissues in other TSE-susceptible species, including humans, may harbour some infectivity during the clinical course of the disease.

After i.p. inoculation, the onset of scrapie replication in the trigeminal ganglia occurs about 10 days later than in the brain and 20 days later than in the cervical spinal cord, suggesting a centrifugal spread of the scrapie agent from the sensory nuclei of the trigeminal system [extended from the medulla oblongata up to the mesencephalon and, in i.p. CJD-infected mice, showing early PrPsc deposits (Muramoto et al. 1993 )] to the trigeminal ganglia. These findings support the view that after intraperitoneal inoculation the scrapie agent moves first centripetally to the CNS (Kimberlin & Walker, 1979 , 1982 ; Baldauf et al., 1997 ) and then centrifugally to the peripheral nervous system (Kimberlin et al., 1983a ), including trigeminal ganglia.

The other important result obtained by this study is that the injection of scrapie into the tooth pulp of Syrian hamsters is an efficient route of infection which gives a mean incubation period ranging between those observed after intraocular (about 130 days; Buyukmihci et al., 1983 ; Kimberlin & Walker, 1986 ) and intrasciatic (about 180-190 days; Kimberlin & Walker, 1986 ) inoculations. Temporal differences are probably due to the relative distances between the injection sites and the brain, rather than to different efficiencies of these peripheral routes. The rate of spread of scrapie infection to the CNS, as measured following intraperitoneal inoculation (Kimberlin & Walker, 1979 , 1982 ; Kimberlin et al., 1983a ), oral administration (Beekes et al. , 1996 ) or injection of sciatic (Kimberlin et al., 1983b ) or optic (Kimberlin & Walker, 1986 ; Scott & Fraser, 1989 ) nerves, is always equivalent to about 1 mm/day, consistent with the slowest rate of axonal transport. The same rate of scrapie propagation was estimated between teeth and trigeminal ganglia. Teeth are innervated by bipolar sensory neurons whose cell bodies are in the homolateral trigeminal ganglion. Thirty days after intrapulpal injection there was infectivity and PrP27-30 accumulation in the homolateral trigeminal ganglion but not in the controlateral one, suggesting that the infection had spread through the mandibular branch (about 25 mm long in Syrian hamsters) of the trigeminal nerve at an approximate rate of 1 mm/day.

Although these results cannot be directly applied to humans affected by TSEs, they raise the possibility that surgical instruments used during major dental procedures in CJD patients and inadequately decontaminated may represent a means of man-to-man transmission of TSEs. People incubating nvCJD are of particular concern, because they carry higher level of PrPsc, and likely infectivity, in peripheral tissues than sporadic CJD cases (Hill et al., 1999 ). Concern will further increase if upcoming studies show that trigeminal ganglia of nvCJD patients are more affected than those of sporadic CJD cases.




A spatio-temporal cluster of three cases was discovered in Eastern England. One of the patients had worked as a dentist and used his house as a surgery, ......


Relatives rarely recognised any of the
addresses of known patients and this occurred with equal
frequency in patients and controls. However, detailed
questioning of patients' relatives and subsequent
investigation revealed some remarkable coincidences.
One patient, a dentist, had for many years daily passed the
residence of another patient, the husband of a hairdresser
who worked in the family home. The dentist's wife used
the hairdresser on occasions but the patient himself had
never entered the salon. Interestingly the dentist himself
had possible contact with other cases (vide infra) and a
close acquaintance, another dentist, worked in a small town
in Essex in which two further cases of C.J.D. had occurred.
In a different part of the country a district nurse who
died of possible C.J.D. may have tended a patient dying of
C.J.D. seven years before she herself died of the condition.
The nurse's daughter lived in a nearby city within 30 yards
of the house of another patient. Detailed investigation
of other cases revealed close proximity to other cases but
no definite contact. Intensive questioning of surviving
relatives and other witnesses was limited for fear of
causing unnecessary alarm or distress.


latrogenic Transmission

The high incidence of previous neurosurgical
procedures in the series of patients with C.J.D. described by
Nevin et al. (1960) has been noted previously (Masters et al.,
1979a). Examination of the case notes of the patients suggests
.that iatrogenic transmission occurred on three occasions.

Two patients (cases 5 and 7 in the report of Nevin et al. (i960))


were diagnosed in the same hospital and died in 1956 and 1957

respectively. The operating list for 3rd February 1956 shows
that case 5 was first on the list, having burrholes for
ventriculography. The patient died the next day of pathologically
confirmed C.J.D. Case 7 was third on the list and had a
cortical undercut for longstanding obsessional neurosis.
The operation was a success and the patient remained well until
September 1957 when she was re-admitted with a six-week history
of progressive dementia. Brain biopsy was carried out and C.J.D.
was confirmed histologically. The patient died on l3th
September 1957.

In January 1952 two patients (cases 1 and 2
in the report of Jones and Nevin (1954)) were admitted to
another hospital for investigation of rapidly progressive
dementia and died on 12th January 1952 and 19th January 1952
respectively, of pathologically proven C.J.D. Case 1 had had
burrholes for ventriculography on 31st December 1951 and insertion
of depth electrodes on 10th January 1952. Within two weeks
two other patients in the second series (cases 1 and 2 in the
report of Nevin et al. (I960)) had neurosurgical procedures

in the same theatre, almost certainly using common instruments.
Case I had burrholes for ventriculography on 23rd January 1952
and craniotomy for excision of a meningioma on 30th January 1952.
Case 2 had craniotomy for excision of an abscess on 16th
January 1952. Case 1 was readmitted 19 months later with a
four-week history of progressive dementia and died in October
1953 of pathologically proven C.J.D. Case 2 was readmitted
on l7th November 1953 with a twelve-week history of progressive
dementia and died on 25th November 2953 of pathologically

proven C.J.D.


Four other cases with a previous history of
neurosurgical procedures and subsequent development of C.J.D.
were discovered but in none of the cases was a link with other
patients found. The first patient had burrholes for ventricu-
lography in August 1952 in the course of investigation of a
suspected cerebrovascular accident, and remained well until
October 1954 when she was readmitted with rapidly progressive
dementia. She died on 15th November 1954 of pathologically
confirmed C.J.D. The other three cases are described in the
section on the epidemiology of the retrospective study.

The possibility that iatrogenic transmission
might have occurred to other patients was prompted by the
discovery of likely transmission by neurosurgery. In the
first case described above the third patient on an operating
list developed C.J.D. 19 months after a neurosurgical
procedure. The case notes of two other patients on the same
operating list and of all patients operated on in the same
theatre over the subsequent two weeks were examined, but there
is no evidence that any of these patients subsequently died
of C.J.D. In the second group of patients described above,

Case 1 in Jones and Nevin's report of 1954 had burrholes for
ventriculography and insertion of depth electrodes in the course
of investigation of a dementing illness subsequently proven
to be C.J.D. The iatrogenic transmission of C.J.D. by
depth electrodes, 'sterilised' with alcohol and formalin
vapour, has been described (Bernouilli et al., 1977). The
depth electrodes inserted in the case described in this study
were similarly 'sterilised' in formalin and were used in over
200 patients over the subsequent 20 years. However, none
of these patients was readmitted with dementia and neither
are they recognised in the list of known cases of C.J.D. which
includes cases from the 1960's.

Spatio-temporal cluster

In 1965 a patient died of C.J.D., confirmed at
necropsy. In 1968 a patient who lived within 250 metres
also died of the disease. These two patients shared the
same general practitioner but unfortunately it has been impossible
to examine the medical records which have been destroyed.
In 1980 a patient who lived midway between the two previous
ones and within sight of both houses died of pathologically
proven C.J.D. This last patient worked as a dentist from
1950 to 1977 and used his house as a surgery. The dental
records have been examined but are incomplete and do not
Include either of the two previous patients. It has been
impossible to obtain details of dental history by other
means, and the possibility that the patients were treated by
the dentist cannot be further explored.

Contact with familial cases

A patient who died in 1980 of pathological]
confirmed C.J.D. had previous social contact with a group of
three cases representing a familial cluster. Clinical
details of two of the familial cases are scanty, and in all
three cases the duration of illness is more prolonged than
usual in this disease, but in the third case pathological
confirmation of the diagnosis was obtained (Figure 10).


Case III.4 The patient presented in January 1969
with a four week history of forgetfulness and
bizarre behaviour. She was found to be demented
but neurological examination was not carried out.
Investigation revealed no abnormality although
EEG was not obtained. The patient became
progressively mentally impaired and by December
1969 was mute and incontinent. She remained
inaccessible with 'tremulous movements of the
limbs' until she died of hypostatic pneumonia
on l5th August 1970. Necropsy was not performed.

Case III.5 The patient presented in 1965 with a
three month history of drowsiness, forgetfulness
and frontal headache. On examination she was
demented and pneumoencephalography showed mild
cortical atrophy. In July 1966 the patient was
admitted for permanent care. She was severely
demented, inaccessible and incontinent with rigidity
of the limbs. She deteriorated and died in
December 1966 of bronchopneumonia. Necropsy was
not performed.

Case III.6 The patient presented in 1966 with
intellectual deterioration and progressive dysphasia.
By July 1968 she was Incontinent and unable to dress
or feed herself- By August she was unable to respond
.bo simple commands and there was generalised hypotonia
and hyper-reflexia, an extensor left plantar response
and bilateral grasp reflexes. She deteriorated and
died on 9th February 1969 of pneumonia. Necropsy was
carried out at the Maudsley Hospital. Histology
showed cortical atrophy with loss of nerve cells,
spongy change and some astrocytic proliferation,
extensive in the frontal lobe and patchy in the
temporal lobe. Spongy change and gliosis were also
present in the caudate lobe and medial part of the
thalamus, and gliosis in the putamen. There was
pallor and wasting of the white matter in the anterior
parts of the hemispheres. The medial parts of the
basis pedunculi showed wasting and gliosis as did the
cortico-spinal tracts in the pons and the pyramids.
A diagnosis of Creutzfeldt-Jakob disease was made.

Case 11.24 The patient was admitted on 7th February
1980 with a two month history of progressive confusion
. and forgetfulness. On examination she was demented,
dysphasic and unco-operative. On 13th February 1980
she developed myoclonus and rigidity of the limbs.
Her condition rapidly deteriorated with the development
of akinetic mutism and decorticate posturing. EEG
was suggestive of C.J.D. The patient died of
pneumonia on 29th April 1980. Post mortem
examination revealed generalised spongifonn change
and gliosis typical of C.J.D.

The figure shows the relationship between the patients.
Cases III.5 and III.6 were sisters and Case III.4 their
first cousin. The sisters lived in the same house until
their early twenties and case III.6 had close social contact

with her cousin III.4 in her teens and early twenties,
meeting regularly, holidaying together and working in the
same factory. III.6 visited her cousin regularly during
her last illness. Case 11.24, who was related by marriage
but with no consanguinity, knew III.6 socially for 20 years,
meeting about twice a year for afternoon tea and at family
gatherings. They continued to meet during 111.6's last
illness, and 11.24 visited her during her final admission,
the last occasion in August 1968, five months prior to death.



10. Arakawa K, Nagara H, Itoyama Y et al. Clustering of three cases of Creutzfeldt-Jakob disease near Fukuoka City, Japan. Acta Neurol Scand 1991; 84: 445-447.

11. Adams D H, Edgar W M. Transmission of agent of Creutzfeldt-Jakob disease. BMJ 1978; 1: 987.

12. Will R G, Matthews W B. Evidence for case-to-case transmission of Creutzfeldt-Jakob disease. J Neurol Neurosurg Psychiatry 1982; 45: 235-238.


i noticed from the NPDPSC figures, which by the way on there 2005 key goals states they have married the CJD Foundation in there surveillance attempt, no mention of CJD Voice there either, even though there moles are pulling names off the CJD Voice list. that would be o.k. if as bev put it;

When and how can we expect to reap benefits from the information collected?

The link below is taken from the National CJD Surveillance website (headed
by Dr. Gambetti). Now, please note at the top of the page the notation that
this is funded by the NIH and the CDC.

Now look at this link for the Foundation: This page says:
"HISTORY OF THE FOUNDATION (some text removed from the History section)
* Foundation moved to Akron, Ohio September 2002
* Collaboration with the National Prion Disease Pathology Surveillance
Center, 2002

IF the federal gov. is willing to cover-up mad cow disease (any strain) or any strain of TSE from consumption of a species of animals in the USA tainted with a TSE, as they have in TEXAS and other states, better documented in TEXAS though, the federal gov via the back door of the npdpsc/cjdfINC are probably doing the same thing with any atypical TSE in humans. my gosh, it's a nobrainer to make cjd reportable in every state mandatory with a WRITTEN CJD QUESTIONNAIRE WITH COPIES STAYING WITH FAMILY MEMBERS.

I find it appauling that the NPDPSC and the CJD Foundation have stooped so low as to ONLY have a phone call CJD surveillance system set up. JUST like the Texas mad cows, atypical TSEs will never be documented this way. UNLESS someone overseas, that you can trust to the utmost, performs the pathology testing. THERE hiding mad cows in TEXAS, they only want a CJD QUESTIONNAIRE with only phone in questions and answers, as to no record for families or public. then you don't even have cjd reportable in all states and in some of the ones you do, it's under 50 or 55. all strains of mad cow aside, would it not be prudent to have all human TSEs reportable Nationally and Internationally, if only for the Iatrogenic CJD/TSE reasons. we know that sporadic CJDs spreads with ease via the surgical/medical arena. ALSO, the NPDPSC/CJDFINC figures on there attempt to track this disease with any accuracy shows that indeed sporadic CJD has been rising, from 54 cases in 1997 to 163 cases in 2004. something that i find interesting is the familial cases of CJD, where it shows that in 1997 the familial cases were 6, increased gradually to 22 cases in 2002, then over doubled in 2003 to 45 cases, then back to 21 cases in 2004. why the sudden increase in 2003 of familial CJD to 45 cases? course we all know what there story will be, better surveillance. right!

WE are the majority, WE are 85%+ of all cases, whether the NPDPSC/CJDFINC likes it or not, were here to stay, were not going to stop asking questions, we are NOT going to accept this so called PHONE IN ONLY CJD QUESTIONNAIRE with no records. YOU can alienate us, call us what ever you want, do what ever, were not going away, as you can see from the figures. you may as well accept us as one of the gang, and put us on the front page of your site and stop acting like we don't exist, or wishing we did not. AND if anyone believes that 85%+ of all sporadic CJD cases just happen due to the synthetic mammalian prion study that prusiner did or the in vitro generation of infectious scrapie-prions by soto et al, well, there dreaming. there is simply to much science and transmission studies to prove other wise...

and the old going over death certificates as a means of a CJD surveillance program routine has already proven to be very inadequate;


Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...

full text;



One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys bu 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...


i am still disgusted in Bacliff, Texas USA.........TSS


----- Original Message -----
From: Terry S. Singeltary Sr.
Sent: Saturday, July 16, 2005 11:48 AM
Subject: Re: [CJDVoice] Transcript of Tele-News Conference with Agriculture Secretary Mike Johann Calling in from Madagascar more Johann's BSe

cjd voice group;

some of this is a repeat, some is not. i just re-did it and please note the editorial by Froma Harrop about the 'mad cow rhetoric' and blaming it on democrats. i have posted two emails for anyone wanting to write them.
skroll down to;

note to Dems: Give the mad cow rhetoric a rest

CJD VOICE has been around a long time. i know some from other organizationswish we would just dry up and go away. NOT going to happen! we must stick together. while i am on a roll here, i have a question. not trying to start any BSe here, but i am curious about something.
why is CJD VOICE NOT listed on CJD Foundation site;



The Cruetzfeldt-Jakob Disease Foundation consists of members who are
concerned about the complexity of issues surrounding this fatal brain
disease. Our mission is to support families and loved ones touched by CJD.

We carry out this mission by means of:

* A national toll free HelpLine, (800) 659-1991
* Publication of CJD Information pamphlet, September 2003
* Political and public policy advocacy
* A Family Conference
* Collaboration with the National Prion Disease Pathology Surveillance
Center and the Centers for Disease Control and Prevention
* Monitoring and communicating by newsletter all of the current research
and Foundation activities
* Collaboration with international support groups involved in similar
work including:
* The CJD Support Network, U.K.
* The Human BSE Foundation, U.K.
* The CJD Support Network, Japan
* The Fatal Familial Insomnia Foundation, Italy
* The Human Growth Hormone Foundation, Australia
* Collaboration with Dr. Pierluigi Gambetti, director of the NPDPSC, the
CDC and Dr. Nick Baird, director of the Ohio State Department of Health, we
are developing a template in the state of Ohio to increase physician
awareness and reporting of CJD. When this has been successfully implemented,
we will work with the Health Departments of the other states to duplicate
this program.
* Our website

WE have been here and fighting to get CJD reportable in all states, and have succeeded in getting it done. NO mention of CJD Voice here? Was CJD Voice even mentioned at the CJD Foundation conference? if not, why not? someone mentioned about bonding together with other groups across the big pond, but what about bonding right here. the human bse foundation has never wanted anything to do with sporadic CJD, i have tried for years to get them involved with sporadic CJD, have it in my files. but this dusturbs me and thought i should bring it up to some of the old ones still lurking here. CJD VOICE has been the founding fighters for our loved ones, we have been ignored all along. oh heck seems we do make it to the CJD Foundations LINKS page, lucky us;

and i don't care if they ever link up CJD Watch or where i post what others don't at mad cow site, but why CJD Voice after all we have done over the past 8 or 9 years??? no free-for-all intended here, but i want to know what is going on, and so do others???

SO HERE IS MY HEAD, and there is the ax, go for it...


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