nvCJD TSE BLOOD UPDATE

P04.49

Case Report of Variant Creutzfeldt-Jakob Disease in a Macaque after BloodTransfusion

Lescoutra-Etchegaray, N1; Ruchoux, MM1; Correia, E1; Jolit, A1; Freire, S1;Lasmezas,CI2; Deslys, JP1; Comoy, E1 1CEA/DSV/IMETI/SEPIA, France; 2Scripps Florida,USA



A fourth human case of probable transmission of vCJD through transfusion hasnow been reported but a number of features affecting transfusion-relatedinfection remain imprecise, including infectious dose, length of incubation period andcritical infectious window of blood donors.

We report here the first case of experimental transmission of vCJD in primates byblood transfusion. Experimental infection of Cynomolgus macaque has beendemonstrated to be a sensitive model for the investigation of human prion diseases,inducing similar distribution of infectivity in peripheral lymphoid tissues and equivalentbrain pathology. In our study, transfusion was performed with 40 ml of whole blooddrawn from a vCJD-infected macaque at the terminal stage of the disease. Clinicalsymptoms of vCJD appeared in the recipient animal after five years of incubation. Thetotal amount of infectivity in the transfused blood was approximately 106 fold lowerthan in the brain (titration still in progress). In several animals infected intravenouslywith brain homogenate, the presence of PrPres in serial lymph nodes biopsiesand in other organs at autopsy was examined and results will be presented.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf



P04.51

Atypical Presentation of Variant Creutzfeldt-Jakob Disease in a 73 Year OldBlood Transfusion Recipient

Wroe, S1; Pal, S1; Webb, T1; Alner, K2; Hewitt, P3; Brander, S4; Wadsworth,JD5; Collinge, J1 1National Hospital for Neurology and Neurosurgery, National Prion Clinic,UK; 2National Hospital for Neurology and Neurosurgery, Department of Neuropsychology, UK;3Health Protection Agency, UK; 4National Hospital for Neurology and Neurosurgery,Department of Neuropathology, UK; 5Institute of Neurology, UCL, UK



We report atypical presentation of variant Creutzfeldt-Jakob Disease (vCJD) identifiedante-mortem in a 73 year-old recipient of blood products. This patient was transfusedfollowing orthopaedic surgery in December 1997. Tracing of blood products identifieda single unit of non-leucodepleted red cells from an individual who developedneuropathologically confirmed vCJD eleven months after donation. Nine years posttransfusion, this individual was referred to the National Prion Clinic for specialistinvestigation. Six years post transfusion the recipient complained of fluctuating fatigueand impaired concentration. At this time neurological examination and MRI brain(T1/T2 weighted/DWI) were normal. Progressive symptoms emerged six months laterwith imbalance and deteriorating cognition. Examination two months after onset ofneurological symptoms demonstrated cognitive deficits, dyspraxia or visuospatialdysfunction and normal motor, sensory and gait examination. Six weeks later cognitiveimpairment was identified alongside tremulousness, impaired manual dexterity andlimb ataxia. Serological investigations were normal. MRI (T1/T2 weighted/FLAIR/DWI)demonstrated prominent signal change throughout the dorsal thalamus, consistentwith vCJD. PRNP genotyping revealed no mutations and homozygosity for methionineat codon 129. The prolonged incubation period of vCJD and possibility ofasymptomatic carrier states pose major public health concerns. This case highlightsthe significant risk encountered by recipients of contaminated blood products and thenecessity for their specialist monitoring.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf



P04.36

Enhanced Surveillance of Persons Identified as at Increased Risk of CJD Dueto Blood Transfusion or Healthcare Procedures

Brookes, D1; Chow, Y1; Ward, HJT2; Will, RG2; Hewitt, P3; Gill, ON11HPA, CJD, UK; 2National CJD Surveillance Unit, UK; 3Colindale, NHS Bloodand Tissue, UK

Introduction:

Reports of four iatrogenic transmissions of variant-CJD (vCJD) infectionin the UK (all due to transfusion of blood from donors who later developed vCJD),evidence from iatrogenic transmissions of sporadic CJD and experimental workon CJD infectivity in tissues and on healthcare instruments have given rise toconcern about the risks of iatrogenic transmission of CJD. This risk warrants a)certain public health precautions, and b) follow-up of individuals with identified risks inorder to gain evidence about their risks and ensure appropriate management of these risks.Evidence of transmission via iatrogenic routes is important to inform public healthmeasures and so prevent ongoing transmission of CJD.

Methods:

The Health Protection Agency and Health Protection Scotland holds detailsof persons identified as ‘at-risk’ of vCJD due to blood transfusion and ofpersons identified as ‘at-risk’ of CJD (of any type) from other healthcareprocedures. The GPs/clinicians of all persons identified as ‘at-risk’ for public healthpurposes are provided with: information; risk assessment updates; advice on

public health precautions and advice on referral to specialist care. Procedures are beingestablished to obtain enhanced surveillance data on these individuals,including:clinical status updates, date and cause of death, surplus tissue and bloodspecimens, and postmortem investigations.

Results:

Persons ‘at-risk’ of CJD have experienced a range of exposures. Estimatedrisks are uncertain and overlapping. Some individuals - recipients of vCJDimplicated blood components - are considered to be at a clearly higher riskof infection: active follow-up is currently conducted for these individuals. Intime, the enhanced surveillance of persons at increased risk of CJD will provideestimates of transmission risks and of the impact of iatrogenic exposures on mortality.Conclusion: Knowledge about iatrogenic transmission of CJD is being gainedby the follow-up of individuals who have been identified as ‘at-risk’ of CJDin the UK. This enhanced surveillance may need to be sustained for many years.

http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf



Prions in Plasma of Patients with Sporadic Creutzfeldt-Jakob DiseaseSafar, J.G1,3, Geschwindm M.D2,3, Letessier, F1,Kuo, A1, Pomeroy, K1, Deering, C1, Serban, A1,Groth, D1, Miller, B.L2,3, DeArmond, S.J1,4,Prusiner, S.B1,3,51Institute for Neurodegenerative Diseases,2Memory and Aging Center, Departments of3Neurology, 4Pathology, and 5Biochemistryand Biophysics, University of California,San Francisco, California 94143

http://www.prion2007.com/pdf/Prion%20Friday.pdf

http://www.prion2007.com/pdf/Prion%20Thursday.pdf

http://www.prion2007.com/pdf/Prion%20Wednesday.pdf



http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf




ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007

Date: Mon, 24 Sep 2007 21:31:55 -0500



I suggest that you all read the data out about h-BASE and sporadic CJD, GSS, blood, and some of the other abstracts from the PRION2007. ...



http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&F=&S=&P=19744





*** PLEASE READ AND UNDERSTAND THE RAMIFICATIONS OF THIS !!! THE PRICE OF POKER INDEED GOES UP. ...TSS

USA BASE CASE, (ATYPICAL BSE), AND OR TSE (whatever they are calling it today), please note that both the ALABAMA COW, AND THE TEXAS COW, both were ''H-TYPE'', personal communication Detwiler et al Wednesday, August 22, 2007 11:52 PM. ...TSS



http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=19779



Prions in Plasma of Patients with Sporadic Creutzfeldt-Jakob DiseaseSafar, J.G1,3, Geschwindm M.D2,3, Letessier, F1,Kuo, A1, Pomeroy, K1, Deering, C1, Serban, A1,Groth, D1, Miller, B.L2,3, DeArmond, S.J1,4,Prusiner, S.B1,3,51Institute for Neurodegenerative Diseases,2Memory and Aging Center, Departments of3Neurology, 4Pathology, and 5Biochemistryand Biophysics, University of California,San Francisco, California 94143

http://www.prion2007.com/pdf/Prion%20Friday.pdf

http://www.prion2007.com/pdf/Prion%20Thursday.pdf

http://www.prion2007.com/pdf/Prion%20Wednesday.pdf



P04.33

Diagnostic Utility of CSF Biomarkers and General CSF Findings in a U.SSporadic CJD Cohort

Geschwind, MD; Haman, A; Torres-Chae, C; Raudabaugh, BJ; Devereux, G;Miller, BL University of California, San Francisco, USA

Background:

The diagnostic utility of CSF biomarkers, including 14-3-3 protein,neuron-specific enolase (NSE), AB42, total Tau (T-Tau), and phosphorylatedTau (PTau)/ T-Tau ratio are often are used for sporadic CJD (sCJD) diagnosisis controversial. We have previously reported the CSF 14-3-3 protein to havepoor sensitivity for CJD diagnosis. In this study, now report the sensitivity and specificityof several CSF biomarkers and general characteristics in a U.S. cohort of sCJDand non-prion rapidly progressive dementia (RPD) controls (Non-CJD cohort)subjects.



Design/Methods:

Clinical diagnoses are made through review of medical records, clinical evaluation,
and in many cases pathology. Data is stored in a secure clinical relational database,
which was queried for various CSF findings, including cell count, protein, IgG index,
oligoclonal bands (OCBs), 14-3-3, NSE, T-Tau in patients with probable or definite
sporadic CJD and non-prion rapidly progressive dementias (RPD),most of whom were referred to our center with a potential diagnosis of CJD.For probable sCJD diagnosis, we used UCSF criteria that are modified from WHO tosubstitute MRI for 14-3-3. T-Tau and NSE were considered positive if > 1300pg/ml and > 35 ng/ml, respectively. For this analysis, ambiguous 14-3-3 results wereconsidered as negative.

Results:

14-3-3 protein (n=149) sensitivity was only 48% (47% for definite; 50% forprobable sCJD). NSE (n=49) sensitivity was 63% (66% for definite; 59% for probablesCJD). T-Tau (n=28) was the most sensitive at 68% (69% for definite; 67% forprobable sCJD). The specificity of these biomarkers among our CJD and RPD controls(n=72) was 66% for 14-3-3 (n=47), 81% for NSE (n=21), and 100% for T-Tau (n=7). The14-3-3 had the lowest sensitivity and specificity. Mildly elevated CSFprotein (<100>20, is uncommon in sCJD.

Conclusions/Relevance:

In a cohort from a major U.S. CJD referral center, the 14-3-3is neither sensitive nor specific for sCJD. NSE and T-Tau also show poor sensitivity forsCJD. T-Tau may be more specific than 14-3-3 and NSE, but our “n” is small.WHO sCJD criteria should be revised; by eliminating 14-3-3 and including brainMRI into the criteria. We are currently analyzing the effects of disease duration andcodon 129 polymorphism on these CSF biomarker results.


http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


USA NVCJD BLOOD RECALLS ONLY ;

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CREUTZFELDT JAKOB DISEASE MAD COW BASE, CWD, SCRAPIE UPDATE OCT 2007

http://cjdmadcowbaseoct2007.blogspot.com/



CJD TSE NEWS

http://disc.server.com/Indices/236650.html


CJD VOICE (voice for _all_ victims of human TSE)

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