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From: TSS ()
Subject: Monthly Creutzfeldt Jakob Disease statistics Monday 8 January 2007
Date: January 8, 2007 at 7:33 am PST

Monday 8 January 2007 10:16
Department of Health (National)

Monthly Creutzfeldt Jakob Disease statistics


The Department of Health is today issuing the latest information about the numbers of known cases of Creutzfeldt Jakob disease. This includes cases of variant Creutzfeldt Jakob disease (vCJD) - the form of the disease thought to be linked to BSE. The position is as follows:

Definite and probable CJD cases in the UK: As at 5 January 2007

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

Alive
Number of probable vCJD cases still alive: 7
Total number of definite or probable vCJD (dead and alive): 165
The next table will be published on Monday 5th February 2007

Referrals: a simple count of all the cases which have been referred to the National CJD Surveillance Unit for further investigation in the year in question. CJD may be no more than suspected; about half the cases referred in the past have turned out not to be CJD. Cases are notified to the Unit from a variety of sources including neurologists, neuropathologists, neurophysiologists, general physicians, psychiatrists, electroencephalogram (EEG) departments etc. As a safety net, death certificates coded under the specific rubrics 046.1 and 331.9 in the 9th ICD Revisions are obtained from the Office for National Statistics in England and Wales, the General Register Office for Scotland and the General Register Office for Northern Ireland.

Deaths: All columns show the number of deaths that have occurred in definite and probable cases of all types of CJD and GSS in the year shown. The figures include both cases referred to the Unit for investigation while the patient was still alive and those where CJD was only discovered post mortem (including a few cases picked up by the Unit from death certificates). There is therefore no read across from these columns to the referrals column. The figures will be subject to retrospective adjustment as diagnoses are confirmed.

Definite cases: this refers to the diagnostic status of cases. In definite cases the diagnosis will have been pathologically confirmed, in most cases by post mortem examination of brain tissue (rarely it may be possible to establish a definite diagnosis by brain biopsy while the patient is still alive).

Probable vCJD cases: are those who fulfil the 'probable' criteria set out in the Annex and are either still alive, or have died and await post mortem pathological confirmation. Those still alive will always be shown within the current year's figures.

Sporadic: Classic CJD cases with typical EEG and brain pathology. Sporadic cases appear to occur spontaneously with no identifiable cause and account for 85% of all cases.

Probable sporadic: Cases with a history of rapidly progressive dementia, typical EEG and at least two of the following clinical features; myoclonus, visual or cerebellar signs, pyramidal/extrapyramidalsigns or akinetic mutism.

Iatrogenic: where infection with classic CJD has occurred accidentally as the result of a medical procedure. All UK cases have resulted from treatment with human derived pituitary growth hormones or from grafts using dura mater (a membrane lining the skull).

Familial: cases occurring in families associated with mutations in the PrP gene (10 - 15% of cases).

GSS: Gerstmann-Straussler-Scheinker syndrome - an exceedingly rare inherited autosomal dominant disease, typified by chronic progressive ataxia and terminal dementia. The clinical duration is from 2 to 10 years, much longer than for CJD.

vCJD: Variant CJD, the hitherto unrecognised variant of CJD discovered by the National CJD Surveillance Unit and reported in The Lancet on 6 April 1996. This is characterised clinically by a progressive neuropsychiatric disorder leading to ataxia, dementia and myoclonus (or chorea) without the typical EEG appearance of CJD. Neuropathology shows marked spongiform change and extensive florid plaques throughout the brain.

Definite vCJD cases still alive: These will be cases where the diagnosis has been pathologically confirmed (by brain biopsy).


REFERRALS OF DEATHS OF DEFINITE AND PROBABLE CJD
SUSPECT CJD


Year Referrals Year Sporadic Iatrogenic Familial GSS vCJD Total
Deaths
1990 [53] 1990 28 5 0 0 - 33
1991 75 1991 32 1 3 0 - 36
1992 96 1992 45 2 5 1 - 53
1993 78 1993 37 4 3 2 - 46
1994 118 1994 53 1 4 3 - 61
1995 87 1995 35 4 2 3 3 47
1996 133 1996 40 4 2 4 10 60
1997 162 1997 60 6 4 1 10 81
1998 154 1998 63 3 3 2 18 89
1999 170 1999 62 6 2 0 15 85
2000 178 2000 50 1 2 1 28 82
2001 179 2001 58 4 3 2 20 87
2002 163 2002 72 0 4 1 17 94
2003 162 2003 79 5 4 2 18 108
2004 114 2004 51 2 4 1 9 67
2005 123 2005 65 3 7 6 5 86
2006 106 2006 53 1 5 3 5 67
2007 0 2007 0 0 0 0 0 0
Total 2151 Total 883 52 57 32 158 1182
Ref's Deaths

* As at 5th January 2007
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
Alive
Number of definite/probable vCJD cases still alive: 7

Total number of definite or probable vCJD (dead and alive): 165

Notes to editor

ANNEX
DIAGNOSTIC CRITERIA FOR VARIANT CJD

I
A) PROGRESSIVE NEUROPSYCHIATRIC DISORDER
B) DURATION OF ILLNESS > 6 MONTHS
C) ROUTINE INVESTIGATIONS DO NOT SUGGEST AN ALTERNATIVE DIAGNOSIS
D) NO HISTORY OF POTENTIAL IATROGENIC EXPOSURE

II
A) EARLY PSYCHIATRIC SYMPTOMS *
B) PERSISTENT PAINFUL SENSORY SYMPTOMS **
C) ATAXIA
D) MYOCLONUS OR CHOREA OR DYSTONIA
E) DEMENTIA

III
A) EEG DOES NOT SHOW THE TYPICAL APPEARANCE OF SPORADIC CJD *** (OR NO EEG PERFORMED)
B) BILATERAL PULVINAR HIGH SIGNAL ON MRI SCAN

IV
A) POSITIVE TONSIL BIOPSY
DEFINITE: IA (PROGRESSIVE NEUROPSYCHIATRIC DISORDER) and NEUROPATHOLOGICAL CONFIRMATION OF vCJD ****
PROBABLE: I and 4/5 OF II and III A and III B or I and IV A
* depression, anxiety, apathy, withdrawal, delusions.
** this includes both frank pain and/ or unpleasant dysaesthesia
*** generalised triphasic periodic complexes at approximately one per second
****spongiform change and extensive PrP deposition with florid plaques, throughout the cerebrum and cerebellum.


[ENDS]

Client ref 2007/0005

GNN ref 142643P

https://www.gnn.gov.uk/Content/Detail.asp?ReleaseID=254733&NewsAreaID=2

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§

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

Atypical features of dementia in a patient with

Creutzfeldt-Jakob disease


Maria Pąchalska1ABCDEFG, Henryk Kurzbauer2ABDE, Maria Formińska-Kapuścik3ABDE,

Andrzej Urbanik4ABDE, Grażyna Bierzyńska-Macyszyn5BDE, Paweł Właszczuk5BDE

1 Institute of Psychology, University of Gdańsk, Gdańsk, Poland

2 Department of Neurology, Ministerial Hospital, Ministry of Internal Affairs and Administration, Cracow, Poland

3 Eye Surgery Clinic, Specialized Ophthalmological Hospital no. 5, Katowice, Poland

4 Department of Radiology, Collegium Medicum, Jagiellonian University, Cracow, Poland

5 Histopathology Section, Department of Morphology, Medical University of Silesia, Katowice, Poland

Summary

Background: This article describes a Polish patient (female, right-handed, age 68 at onset) diagnosed with the

Heidenhain variant of Creutzfeldt-Jakob Disease (HvCJD), characterized clinically by isolated visual

disturbances with no ocular dysfunction prior to the development of myoclonus and other

symptoms of CJD.

Case Report: Nothing in the history pointed to iatrogenic or acquired CJD, and genetic testing ruled out familial

CJD. The neuroradiological picture (MRI) showed non-specifi c features of cerebral atrophy (cortical

and subcortical). An EEG revealed periodic triphasic sharp waves, particularly in the occipital

lobes, and myoclonus occurring synchronically with generalized periodic epileptiform discharges.

Comprehensive neuropsychological testing documented rapidly progressive dementia, with dysgraphia

and aphasia deteriorating to organic mutism. Post-mortem neuropathological examination

confi rmed spongiform encephalopathy, especially in occipital cortex, with amyloid plaques

but without neurofi brillary tangles.

Conclusions: Over the crucial 6-week period the patient went from “Mild Cognitive Impairment” to a status resembling

the fi nal stages of Alzheimer’s disease, without any evidence of a CVA. The only aspect

of this case that does not fi t the usual criteria for the Heidenhain variant is the fact that the patient

survived over a year in a persistent vegetative state. Ophthalmologists and family physicians

should be aware of the possibility of HvCJD in any patient over 60 presenting with otherwise inexplicable

visual disturbances in the absence of signifi cant ocular pathology, even when other symptoms

of dementia may not be immediately noticeable.

snip...

CONCLUSIONS

In the case reported here, a clinical diagnosis of the Heidenhain

variant of Creuzfeldt-Jakob Disease, initially suggested by the

early and prominent presentation of visual symptoms (ranging

from microopsia and macropsia to visual hallucinations),

and backed by EEG and neuroadiological fi ndings, was con-

fi rmed by neuropathological results. Neuropsychological testing

documented the characteristically rapid course of the disease.

The course of development of clinical symptoms and

their relationship to the ophthalmological and neuroradiological

picture are of considerable theoretical interest.

Ophthalmologists and family physicians should be aware of

the possibility of HvCJD in any patient over 60 presenting

with otherwise inexplicable visual disturbances in the absence

of signifi cant ocular pathology, even when other symptoms

of dementia may not be immediately noticeable. ...

http://www.medscimonit.com/pub/vol_13/no_1/9923.pdf

Text and figures of the latest annual report of the CJD unit (published 4th December 2006).

FOURTEENTH ANNUAL REPORT 2005

CREUTZFELDT-JAKOB DISEASE

SURVEILLANCE IN THE UK

SUMMARY

The national surveillance programme for Creutzfeldt-Jakob disease (CJD) in the UK was

initiated in May 1990. In 1999, the National CJD Surveillance Unit (NCJDSU) became a

WHO Collaborative Centre for Reference and Research on the surveillance and epidemiology

of human transmissible spongiform encephalopathies (TSEs). In September 2001 the

National Care Team was formed, which currently comprises a care coordinator and a secretary. It

is based within the NCJDSU and was formed in response to concerns regarding the care of CJD

patients.

The information provided in this fourteenth report continues to provide evidence of a high level

of case ascertainment. In last year’s report, a decrease in the number of referrals was noted, along

with a drop in the number of recorded sporadic CJD deaths in 2004. This raised concerns about

the completeness of case ascertainment. Further data are now available. While the decline in the

number of referrals has been maintained in 2005, analysis suggests that much, if not all of the

decline is due to changes in the number of referrals who turn out not to be CJD cases. The

number of sporadic cases in 2005 was higher than in 2004 and the data for 2005 may still be

incomplete; thus the present data do not suggest a significant consistent decline in the number of

recorded sporadic CJD deaths (discussed in more detail in Section 2). Detailed clinical and

epidemiological information has been obtained for the great majority of patients. The case-control

study for risk factors of CJD has continued recruitment and initial analysis has been undertaken,

with the results with respect to vCJD published in early 2006. Although there is ongoing evidence

that the post mortem rate for patients with suspected CJD has declined, in line with general

autopsy rates in the UK, it remains high (around 60%). The decline is reflected in the reduced

number of brain specimens examined in the neuropathology laboratory; sporadic CJD numbers

being 52 in 2003, 32 in 2004 and 32 in 2005.

In 1990-2005 mortality rates from sporadic CJD in England, Wales, Scotland and Northern

Ireland were, respectively, 0.89, 1.01, 0.88 and 0.49/million/year. The differences between these

rates are not statistically significant (p>0.5). The variation in the observed mortality rates between

the different regions within the UK is not statistically significant (p>0.3). The highest and lowest

mortality rates from sporadic CJD were observed in the South West (SMR=131) and Northern

Ireland (SMR=67). The mortality rates from sporadic CJD in the UK are comparable to those

observed in most other European countries and elsewhere in the world, including countries that

are free of BSE.

Up to 31 December 2005, there were 153 deaths from definite or probable variant CJD (vCJD) in

the UK. Of these, 110 were confirmed by neuropathology. A further 6 probable cases were alive

as at 31st December 2005. The clinical, neuropathological and epidemiological features of these

cases of vCJD are remarkably uniform and consistent with our previous descriptions. Risk factors

for the development of vCJD include age, residence in the UK and methionine homozygosity at

Section 1

T

Fourteenth Annual Report 2005 4

codon 129 of the prion protein gene - all 139 cases (87%) of vCJD with available genetic analysis

have been methionine homozygotes. The incidence of vCJD is higher in the north of the UK

than in the south. Analysis of the incidence of vCJD onsets and deaths from January 1994 to

December 2005 indicates that a peak has been passed. While this is an encouraging finding, the

incidence of vCJD may increase again, particularly if different genetic subgroups are found but

with longer incubation periods. The identification of disease-related PrP in the spleen of a blood

recipient of PRNP-129 MVgenotype is not inconsistent with such an hypothesis. In addition, this

case along with the report of the prevalence of abnormal PrP in the large study of appendix and

tonsil tissues suggests a possibility of a greater number of preclinical or subclinical cases in the

population than might be indicated by the present numbers of confirmed clinical cases.

The only statistically significant geographic cluster of vCJD cases in the UK was in Leicestershire.

All geographically associated cases of vCJD are considered for investigation according to a

protocol which involves the NCJDSU, colleagues at the HPA, HPS and local public health

physicians.

The activities of the NCJDSU are strengthened by collaboration in other surveillance projects,

including the Transfusion Medicine Epidemiology Review and the study of Progressive

Intellectual and Neurological Deterioration in Children. The collaboration of our colleagues in

these projects is greatly appreciated; the effectiveness of this collaboration allowed the

identification in 2003 of a case of variant CJD associated with blood transfusion and the

identification in 2004 of PrPres in the spleen of a recipient of blood donated by someone

incubating vCJD. In early 2006 a further case of variant CJD associated with blood transfusion

was identified.

The success of the National CJD Surveillance Project continues to depend on the extraordinary

level of co-operation from the neuroscience community and other medical and paramedical staff

throughout the UK. We are particularly grateful to the relatives of patients for their help with this

study.

snip...full text some 40 pages or so ;

http://www.cjd.ed.ac.uk/report14.pdf

Reporting CJD cases to public health departments - Guidance Document. - (updated November 2006)

http://www.cjd.ed.ac.uk/guidance.htm


Incident Reporting Form New version December 2006 (34kB)


http://www.hpa.org.uk/infections/topics_az/cjd/frameworkannex1-Aug2005.doc


VARIANT CREUTZFELDT-JAKOB DISEASE

CURRENT DATA (DECEMBER 2006)

http://www.cjd.ed.ac.uk/vcjdworld.htm


POTENTIAL TREATMENTS FOR CREUTZFELDT-JAKOB DISEASE


Dr RSG Knight, NCJDSU
updated July 2006

http://www.cjd.ed.ac.uk/TREAT.htm


European CJD stats


http://www.eurocjd.ed.ac.uk/allcjd.htm

http://www.eurocjd.ed.ac.uk/sporadic.htm

http://www.eurocjd.ed.ac.uk/genetic.htm

http://www.eurocjd.ed.ac.uk/nvcjd.html

[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





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