Monday, February 25, 2013

Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia

Observation|Feb 2013

 

Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia

 

David Y. Johnson, MD; Diana L. Dunkelberger, MA; Maya Henry, PhD; Aissatou Haman, MD; Michael D. Greicius, MD, PhD; Katherine Wong, BA; Stephen J. DeArmond, MD, PhD; Bruce L. Miller, MD; Maria Luisa Gorno-Tempini, MD, PhD; Michael D. Geschwind, MD, PhD


JAMA Neurol. 2013;70(2):254-257. doi:10.1001/2013.jamaneurol.139.

 

ABSTRACT
 
 
 
Objective
 
 
To report the clinical, neuropsychological, linguistic, imaging, and neuropathological features of a unique case of sporadic Jakob-Creutzfeldt disease in which the patient presented with a logopenic variant of primary progressive aphasia.


Design Case report.


Setting
 
 
Large referral center for atypical memory and aging disorders, particularly Jakob-Creutzfeldt disease.


Patient
 
 
Patient presenting with logopenic variant primary progressive aphasia initially thought to be due to Alzheimer disease.


Results
 
 
Despite the long, slow 3.5-year course, the patient was shown to have pathology-proven sporadic Jakob-Creutzfeldt disease.


Conclusions
 
 
 
 
These findings expand the differential of primary progressive aphasia to include prion disease.








Saturday, January 22, 2011


Alzheimer's, Prion, and Neurological disease, and the misdiagnosis there of, a review 2011






Monday, August 20, 2012


CASE REPORTS CREUTZFELDT-JAKOB DISEASE: AN UNDER-RECOGNIZED CAUSE OF DEMENTIA




see the Duke, Pa, Yale, and Mexican study here, showing the misdiagnosis of CJD TSE prion disease as Alzheimers ;






Saturday, October 13, 2012


On the issue of transmissibility of Alzheimer disease: A critical review






Friday, February 15, 2013


Scottish TSE Network November Symposium Announcement Event: 12 November 2012 Title: Is Alzheimer’s Disease a transmissible disease? SUMMARY







Thursday, February 21, 2013


National Prion Disease Pathology Surveillance Center Cases Examined January 16, 2013





 
 
Letters|February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214.
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
 



TSS

Thursday, February 14, 2013

Cleveland County NC Deaths linked to rare TSE PRION brain disease ?

Greetings TSE family,




more BSe $$$




God, if they only knew.




please see as follows ;




Deaths linked to rare brain disease?




Two possible cases of Creutzfeldt-Jakob Disease were identified in Cleveland County that possibly claimed the lives of two individuals in Dec. 2012 and January of this year. CJD comes in various forms and could have a relation to mad cow disease which has never been identified or found in the county or the state. CJD can be contracted through genetics, sporadically or through contact with contaminated human tissues.




By Alicia Banks




Published: Wednesday, February 13, 2013 at 18:49 PM.




Alicia Banks











A type of rare, degenerative brain disease - not unlike mad cow disease - may have claimed the lives of two county residents within the span of two months, according to local health officials.




Deaths of two people - one in January of this year and another in December 2012 - were labeled “probable cases” of Creutzfeldt-Jakob’s disease (CJD), according to Kim Crane, communicable disease and prevention supervisor for the Cleveland County Health Department.




Diseases like CJD are marked by abnormal proteins called prions that can develop in the brains of both humans and animals, according to the Centers for Disease Control and Prevention.




About 350 deaths related to CJD were reported in the U.S. during 2009, according to the CDC’s website.




Thirteen CJD-related deaths were reported by the NC Department of Health and Human Services in 2010, the most recent year in which stats for disease cases were available. None of the cases reported were related to mad cow disease.




Crane said she wasn't allowed to release any additional information about the individuals involved in both cases, such as age, gender, residency in the county and additional medical information.




Varying forms of the disease




CJD comes in many forms, but two are most prominent: variant and classic.




Variant, or vCJD, has been linked to outbreaks of people eating infected beef from animals with bovine spongiform encephalopathy (BSE), also known as “mad cow disease.” Cases of variant CJD and BSE appeared almost simultaneously in the United Kingdom during the early 1990s, according to the Mayo Clinic’s website.




In 1996, three cases of CJD were identified in the US, but they were contracted in the United Kingdom and Saudi Arabia, according to Marilyn Haskell, a public health veterinarian and epidemiologist with the N.C. Department of Health and Human Services.




Classic CJD occurs spontaneously in humans from abnormal brain prions. It is not related to mad cow disease, Haskell said.




Classic CJD usually develops later in life around age 60, the CDC reports, while the average age of people contacting CJD linked to mad cow disease is about 28 years old.




“Having a probable case does not mean the person had CJD,” Haskell said.




No other probable or confirmed cases of CJD were reported on record in the county, Crane said.




Confirming presence of the disease




Generally, CJD carries symptoms similar to other brain disorders such as advanced Alzheimer’s, including dementia, memory loss, difficulty speaking and personality changes.




A person can have classic CJD for years before symptoms surface, and most classic forms are fatal.




CJD can only be confirmed by a human brain autopsy or biopsy after death, according to health officials.




An autopsy wasn’t performed on either of the two probable cases in Cleveland County, Crane said.




Autopsies to confirm CJD in North Carolina have to be arranged by the hospital handling the case, Haskell said.




There is no known prevention or cure for classic CJD.




The disease can also be inherited through genetics or contaminated brain tissue. Passing contaminated tissues can occur in medical procedures, including cornea, skin transplants and even surgeries with contaminated instruments, the Mayo Clinic reports.




The National Institute of Neurological Disorders and Stroke says forms of CJD can’t be passed on through the air or by touching.




Reach reporter Alicia Banks at 704-669-3338, email abanks@shelbystar.com and follow on Twitter @TheStarAlicia.




What is Creutzfeldt-Jakob Disease?




A rare, degenerative fatal brain disorder that affects one person in every 1 million people per year worldwide. About 200 cases are reported in the US each year.




Symptoms of CJD




Signs of CJD can sometimes mirror Alzheimer’s Disease, including:




-Failing memory




-Behavioral changes




-Lack of coordination




-Behavioral changes




-Blindness




-Coma may occur




Most individuals with CJD die within one year after symptoms surface






What are the different forms of CJD?




- Classic or sporadic : the disease appears even though the person has no known risk factors for the disease. This accounts for 85 percent of cases.




- Hereditary : Marked by a family history of the disease that can be tested for a genetic mutation associated with CJD.




- Variant : Linked to people eating infected beef with bovine spongiform encephalopathy (BSE) found in cows, also known as “mad cow disease.”




- CJD can also be transmitted through contact with contaminated brain tissue from surgeries or medical procedures




How many cases of Creutzfeldt-Jakob Disease were reported in N.C.?




Since 2011, 13 cases of confirmed, suspected or probable types of CJD cases were reported in the state.




*Note: No cases of variant CJD, suspected to be related to mad cow disease, have ever been reported or identified in North Carolina.




*Source: The National Institute for Neurological Disorders and Stroke, and The North Carolina Department of Health and Human Services






How can you get help?




Contact the Cleveland County Health Department at 704-484-5100 for any questions about CJD or if you're experiencing any of the above symptoms. Also contact your local healthcare provider.














1 in a million ???





or really, what officials don’t publish, 1 in 9,000 CJD cases per year in the population group age 55 and older.





big difference...






>>> A type of rare, degenerative brain disease - not unlike mad cow disease - may have claimed the lives of two county residents within the span of two months, according to local health officials.





POPULATION CLEVELAND COUNTY N.C. 97,489 - Jul 2011






o.k., someone explain to me how you can have a sporadic FFI, but with no family genetic connection, and it still be a familial type prion disease, yet be similar to the atypical h-g-BSE in the USA, but no human TSE there from in USA?


sporadic cjd, sporadic ffi, sporadic gss, now VPSPr?, all these human TSE are simply a human TSE prion disease of _unknown_ route and source, and officials have no clue if they are zoonosis or not.


consumption, medical, surgical, dental, friendly fire from sub-clinical TSE consumption, to iCJD in someone else ???


all iatrogenic CJD is, is sporadic CJD until route and source of the TSE prion agent is confirmed, and this is not done very often.


and remember, iCJD, is friendly fire from another person, i.e. the pass it forward mode of mad cow disease.


NOW, there is evidence that some sporadic CJD is linked to the atypical BSE and atypical Scrapie.


the UKBSEnvCJD only theory should be put to bed once and for all.


the USA and North America have more _documented_ TSE prion disease in the wild and livestock and different species (other than zoo animals, canine and feline TSE prion history), than any other country in the world, or continent.


consumption, sub-clinical there from, and from that, what about second hand transmission via medical surgical, iatrogenic routes ???


I don’t buy the star trek like cloaking device for humans, the usda cdc fda et al claim we have from the many different strains of mad cow disease, mad sheep and goat disease, and mad deer and elk disease in the USA. ..tss







Saturday, December 29, 2012



MAD COW USA HUMAN TSE PRION DISEASE DECEMBER 29 2012 CJD CASE LAB REPORT










Monday, December 31, 2012



Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State, 2006–2011-2012










16 YEAR OLD TSE MAD COW TYPE PRION DISEASE DEATH IN USA





Monday, January 14, 2013



Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe










MAD COW USDA ATYPICAL L-TYPE BASE BSE, the rest of the story...










Tuesday, November 6, 2012



Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and Sporadic CJD, November-December 2012 update










Tuesday, June 26, 2012



Creutzfeldt Jakob Disease Human TSE report update North America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012



type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the rise in Canada and the USA










Saturday, March 5, 2011



MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA










Sunday, February 12, 2012



National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas










Monday, August 9, 2010



Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more Prionbaloney ?










Wednesday, March 28, 2012



VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion poker goes up again $



OR-10 15:25 - 15:40 VARIABLY PROTEASE-SENSITIVE PRIONOPATHY IS TRANSMISSIBLE IN BANK VOLES Nonno










Sunday, August 09, 2009



CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009










Tuesday, August 18, 2009



BSE-The Untold Story - joe gibbs and singeltary 1999 – 2009










Sunday, February 10, 2013



Creutzfeldt-Jakob disease (CJD) biannual update (February 2013) Infection report/CJD










Monday, October 10, 2011



EFSA Journal 2011 The European Response to BSE: A Success Story



snip...



EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.



snip...











see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;








Thursday, August 12, 2010



Seven main threats for the future linked to prions



First threat



The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.



***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.



Second threat



snip...










Saturday, June 25, 2011



Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque



"BSE-L in North America may have existed for decades"








Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.



snip...



The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...










Monday, September 26, 2011



L-BSE BASE prion and atypical sporadic CJD










Wednesday, March 28, 2012


VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $









*** The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.





AS OF AUGUST 2012 ;




CJD UPDATE USA




1 Listed based on the year of death or, if not available, on year of referral;


2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted;


3 Disease acquired in the United Kingdom;


4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case;



*** 5 Includes 8 cases in which the diagnosis is pending, and 18 inconclusive cases;



*** 6 Includes 10 (9 from 2012) cases with type determination pending in which the diagnosis of vCJD has been excluded.



*** The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI) and 42 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2224 cases of sporadic Creutzfeldt-Jakob disease (sCJD).












Tuesday, November 6, 2012


Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and Sporadic CJD, November-December 2012 update










Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis













full text with source references ;










Sunday, August 21, 2011



The British disease, or a disease gone global, The TSE Prion Disease (SEE VIDEO)










U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? (see video at bottom)










Wednesday, April 25, 2012



USA MAD COW DISEASE AND CJD THERE FROM SINGELTARY ET AL 1999 – 2012










Tuesday, July 29, 2008



Heidenhain Variant Creutzfeldt Jakob Disease Case Report



FINAL AUTOPSY DIAGNOSIS



I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.



SKROLL down a bit for Mom's autopsy of hvCJD. ...










Monday, February 11, 2013



APHIS USDA Letter to Stakeholders: Trade Accomplishments and failures (BSE, SCRAPIE, TSE, PRION, AKA MAD COW TYPE DISEASE)










Wednesday, May 16, 2012



Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?



Proposal ID: 29403










PLEASE REMEMBER ;






The Akron, Ohio-based CJD Foundation said the Center for Disease Control revised that number in October of 2004 to about one in 9,000 CJD cases per year in the population group age 55 and older. HAVE YOU GOT YOUR CJD QUESTIONNAIRE ASKING REAL QUESTIONS PERTAINING TO ROUTE AND SOURCE OF THE TSE AGENT THAT KILLED YOUR LOVED ONE ???





if not, why not...






Friday, November 30, 2007



CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION













layperson


Terry S. Singeltary Sr.






Sunday, February 10, 2013

Creutzfeldt-Jakob disease with unusually extensive neuropathology in a child treated with native human growth hormone

Clin Neuropathol. 2012 May-Jun;31(3):127-34.





Creutzfeldt-Jakob disease with unusually extensive neuropathology in a child treated with native human growth hormone.





Mikol J, Deslys JP, Zou WQ, Xiao W, Brown P, Budka H, Goutieres F.





Source





Denis Diderot University. Jacqueline.mikol@wanadoo.fr






Abstract





We report a case of iatrogenic Creutzfeldt-Jakob disease(iCJD) in a child with a neonatal growth hormone (GH) deficiency that was treated with native human growth hormone (hGH) between the ages of 9 months and 7 years. Three years after the end of treatment a progressive neurological syndrome consistent with Creutzfeldt-Jakob disease (CJD) developed, leading to death within a year, at age 11. Neuropathological examination showed an unusual widespread form of CJD, notably characterized by (i) involvement of the cerebellar white matter, (ii) cortico-spinal degeneration and (iii) ballooned neurons. A transitional form of the disease between common iatrogenic and panencephalopathic CJD is suggested.
















SHORT REPORT





Creutzfeldt-Jakob disease 38 years after diagnostic use of human growth hormone





E A Croes, G Roks, G H Jansen, P C G Nijssen, C M van Duijn ...............................................................





J Neurol Neurosurg Psychiatry 2002;72:792-793






A 47 year old man is described who developed pathology proven Creutzfeldt-Jakob disease (CJD) 38 years after receiving a low dose of human derived growth hormone (hGH) as part of a diagnostic procedure. The patient presented with a cerebellar syndrome, which is compatible with iatrogenic CJD. This is the longest incubation period described so far for iatrogenic CJD. Furthermore, this is the first report of CJD after diagnostic use of hGH. Since the patient was one of the first in the world to receive hGH, other cases of iatrogenic CJD can be expected in the coming years.





Prion diseases are potentially transmissible. Human to human transmission was first reported in 1974, when a 55 year old woman was described who developed symptoms of Creutzfeldt-Jakob disease (CJD) 18 months after a corneal transplant.1 Since then, transmission has been reported after stereotactic electroencephalographic (EEG) depth recording, human growth hormone (hGH) and gonadotrophin treatment, and dura mater transplantation.2-5 More than 267 patients with iatrogenic CJD are known today and their number is growing.6 The most important iatrogenic cause of CJD is still contaminated cadaveric hGH. Exposure to contaminated hGH occurred before 1985, when recombinant growth hormone became available. In a recent study, incubation periods in 139 patients with hGH associated CJD were found to range from 5-30 years, with a median of 12 years.6 One of the factors influencing incubation time is genotype on polymorphic codon 129 of the prion protein gene.7 The incubation time is significantly shorter in people who are homozygous for either methionine or valine on this polymorphism.7





We describe the second patient with hGH related CJD in the Netherlands. The patient developed the disease 38 years after hGH injections. To our knowledge, this is the longest incubation period described for any form of iatrogenic CJD. Further-more, our patient was not treated with hGH but only received a low dose as part of a diagnostic procedure.





CASE REPORT





This patient presented at the age of 47 years with paraesthesia in both arms for six months, difficulty with walking for four weeks, and involuntary movements of mainly the upper extremities of two weeks' duration. He did not notice any change in cognitive function, although his twin sister had noticed minor memory disturbances. There was no family history of neurological disease. During childhood the patient had experienced a growth delay compared with his twin sister and with the average in the Netherlands. When he was 9 years old, a nitrogen retention test with 6 IU hGH over five days was performed to exclude growth hormone deficiency. Since the result was not decisive, a quantitative amino acid test was performed, which measures 30 amino acids during fasting and one, two, and three hours after growth hormone injection. No abnormal amino acid concentrations were found making the diagnosis of primordial dwarfism most likely. Therefore, no treatment with hGH was given.





On neurological examination we found a slight dysarthria without aphasia. Cranial nerve function was normal. Walking was unstable and wide based. During movements of the upper extremities myoclonic jerks were present. Sensation, muscle tone, and strength were normal. Co-ordination was impaired in all four limbs with a disturbed balance. Tendon reflexes were brisk at the arms and increased at the legs with a clonus in the ankle reflex. Plantar responses were both normal. On the mini mental state examination, the patient scored 30/30. Routine laboratory investigation, thyroid function, vitamin concentrations (B-1, B-6, B-12, and E), and copper metabolism were normal. Admission EEG examination showed generalised arrhythmic slow activity with diffuse spikes and spike waves. EEG examination two months later showed a further slowing of the rhythm with bilateral diphasic sharp waves but was not typical for CJD. Cerebral magnetic resonance imaging was normal. Cerebrospinal fluid examination showed 1 cell/3 µl, normal glucose and protein concentrations, and a strongly positive 14-3-3 protein test. The patient was homozygous for methionine on the PRNP codon 129 polymorphism. On clinical grounds, CJD was diagnosed. Within one month the patient's condition deteriorated rapidly and because of severe disturbances in coordination and progressive myoclonus he became bedridden. An eye movement disorder developed with slow saccadic and dysmetric eye movements. Temperature became unstable with peaks of 39°C without an infectious focus, for which a disorder of autoregulation was presumed. Until a very advanced stage, cognitive function was intact. The patient died five months after admission. The diagnosis of CJD was confirmed at necropsy. The brain weighed 990 g and showed clear cortical and cerebellar atrophy. Spongiosis, neuronal loss, and gliosis were found predominantly in the putamen, caudate nucleus, and basotemporal and cerebellar cortex; the cerebellum was the most severely affected of these. Vacuoles ranged from 2-12 µm. No amyloid or Kuru plaques were found. Immunohistochemical staining (3F4 antibody 1:1000, Senetek, USA) was clearly positive for prion protein accumulation in a "synaptic" distribution. Most deposition was found in the stratum moleculare of the cerebellum.





DISCUSSION





We describe a 47 year old patient who developed pathology proven CJD 38 years after hGH injections. The patient was never treated with hGH but received a small dose as part of a diagnostic procedure. The onset of CJD was signalled by prodromal symptoms of paraesthesia followed by a rapidly progressive ataxia. The disease presentation and course with predominantly cerebellar and eye movement disorders are compatible with iatrogenic CJD caused by hGH treatment.6 8





Growth hormone treatment was first described in 1958 but hGH was not produced on a larger scale from human pituitary glands until the beginning of the 1960s. In the Netherlands growth hormone extraction started in 1963 and was soon centrally coordinated. Until 1979 growth hormone was extracted non-commercially from pituitaries by a pharmaceutical company. In 1971 commercial products also became available. Our patient was one of the first to receive hGH in the Netherlands but the origin of this product was not recorded. A causal relation can therefore not be established with full certainty, but coincidentally receiving growth hormone and developing this very rare disease is unlikely. Since the clinical course in this relatively young patient is in accordance with an iatrogenic cause, we think the probability is high that the hGH injections explain the development of CJD in this patient.





The first Dutch patient with hGH related CJD died in 1990. 9 During several periods from 1963 to 1969 she received intramuscular injections of hGH. During an unknown period the hGH was derived from South America. At age 39, 27 years after starting the treatment, she developed an ataxic gait, slurred speech, sensory disorders, and myoclonus, but her cognitive function remained normal. Postmortem examination of the brain confirmed the diagnosis of CJD.9 Following the identification of this patient, a retrospective study was started to trace all 564 registered hGH recipients who were treated before May 1985. Until January 1995, none of these was suspected of having CJD.10 Since 1993 prospective surveillance for all forms of human prion disease has been carried out in the Netherlands and, apart from the patient described above, a further two patients with iatrogenic CJD have been identified, who developed the disease after dura mater transplantation.11





An incubation period as long as 38 years had never been reported for iatrogenic CJD. Huillard d'Aignaux et al7 studied the incubation period in 55 patients with hGH related CJD in a cohort of 1361 French hGH recipients. The median incubation period was between 9 and 10 years. Under the most pessimistic model, the upper limit of the 95% confidence interval varied between 17 and 20 years. Although the infecting dose cannot be quantified, it can be speculated that the long incubation period in our patient is partly explained by the administration of a limited amount of hGH. This hypothesis is supported by experimental models, in which higher infecting doses usually produce shorter incubation periods.6 Since our patient was one of the first in the world to receive hGH, this case indicates that still more patients with iatrogenic CJD can be expected in the coming years. Another implication of our study is that CJD can develop even after a low dose of hGH. This case once more testifies that worldwide close monitoring of any form of iatrogenic CJD is mandatory.





ACKNOWLEDGEMENTS





We are grateful to M Jansen PhD MD for his search for the origin of the growth hormone and P P Taminiau MD. CJD surveillance in the Netherlands is carried out as part of the EU Concerted Action on the Epidemiology of CJD and the the EU Concerted Action on Neuropathology of CJD, both funded through the BIOMED II programme, and is supported by the Dutch Ministry of Health. This surveillance would not have been possible without the cooperation of all Dutch neurologists and geriatricians. ........................................





Authors' affiliations





E A Croes, G Roks*, C M van Duijn, Genetic Epidemiology Unit, Department of Epidemiology and Biostatistics, Erasmus University Medical Centre Rotterdam, PO Box 1738, 3000 DR Rotterdam, Netherlands


P C G Nijssen, Department of Neurology, St Elisabeth Hospital, PO Box 90151, 5000 LC Tilburg, Netherlands


G H Jansen, Department of Pathology, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX Utrecht, Netherlands


*Also the Department of Neurology, St Elisabeth Hospital


Correspondence to: Professor C M van Duijn, Genetic Epidemiology Unit, Department of Epidemiology and Biostatistics, Erasmus University Medical Centre Rotterdam, PO Box 1738, 3000 DR Rotterdam, Netherlands; vanduijn@epib.fgg.eur.nl


Received 27 December 2001 In revised form 1 March 2002 Accepted 12 March 2002


Competing interests: none declared





REFERENCES






1 Duffy P, Wolf J, Collins G, et al. Possible person-to-person transmission of Creutzfeldt-Jakob disease. N Engl J Med 1974;290:692-3.


2 Bernoulli C, Siegfried J, Baumgartner G, et al. Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery. Lancet 1977;i:478-9.


3 Koch TK, Berg BO, De Armond SJ, et al. Creutzfeldt-Jakob disease in a young adult with idiopathic hypopituitarism: possible relation to the administration of cadaveric human growth hormone. N Engl J Med 1985;313:731-3.


4 Cochius JI, Burns RJ, Blumbergs PC, et al. Creutzfeldt-Jakob disease in a recipient of human pituitary-derived gonadotrophin. Aust NZ J Med 1990;20:592-3.


5 Thadani V, Penar PL, Partington J, et al. Creutzfeldt-Jakob disease probably acquired from a cadaveric dura mater graft: case report. J Neurosurg 1988;69:766-9.


6 Brown P, Preece M, Brandel JP, et al. Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology 2000;55:1075-81.


7 Huillard d'Aignaux J, Costagliola D, Maccario J, et al. Incubation period of Creutzfeldt-Jakob disease in human growth hormone recipients in France. Neurology 1999;53:1197-201.


8 Billette de Villemeur T, Deslys JP, Pradel A, et al. Creutzfeldt-Jakob disease from contaminated growth hormone extracts in France. Neurology 1996;47:690-5.


9 Roos RA, Wintzen AR, Will RG, et al. Een patiënt met de ziekte van Creutzfeldt-Jakob na behandeling met humaan groeihormoon. Ned Tijdschr Geneeskd 1996;140:1190-3.


10 Wientjens DP, Rikken B, Wit JM, et al. A nationwide cohort study on Creutzfeldt-Jakob disease among human growth hormone recipients. Neuroepidemiology 2000;19:201-5.


11 Croes EA, Jansen GH, Lemstra AF, et al. The first two patients with dura mater associated Creutzfeldt-Jakob disease in the Netherlands. J Neurol 2001;248:877-81.
























P.4.4





Possible iatrogenic Creutzfeldt-Jakob Disease in an adult male 50 years after treatment with human chorionic gonadotrophin






Brian Appleby1, Paul Brown2 1Johns Hopkins University School of Medicine, USA; 2CEA/DSV/iMETI/SEPIA, France





Background: Known causes of iatrogenic Creutzfeldt-Jakob disease (iCJD) include cadaverous corneal transplants, dural mater grafts, human growth hormone (hGH), neurosurgical depth electrodes, and neurosurgical instrument contamination. Four cases of iCJD from human gonadotrophin have been described to date, all of whom have been women.






Objectives: To present a case of possible iCJD from human chorionic gonadotrophin (hCG) and review data from four other cases Methods: Case report and descriptive analysis






Results: A 62-year-old Caucasian man developed ataxia that resulted in frequent falls and an initial diagnosis of benign positional vertigo. Further workup including brain magnetic resonance imaging (MRI), electroencephalogram (EEG), and a lumbar puncture were unrevealing. A cerebrospinal 14-3-3 protein analysis was indeterminate. At the end of the third month of his illness, he developed short-term amnesia, disorientation, and confabulation. A repeat EEG showed generalized slowing without evidence of periodic sharp wave complexes and a repeat 14-3-3 analysis was positive. A second brain MRI showed hyperintensity in the basal ganglia on diffusion- weighted images. He died following a four-month illness. Severe vacuolization was noted on microscopic examination and Western blot analyses detected type II prion proteins. Genomic analyses detected a silent polymorphism at codon 117 and valine homozygousity at codon 129 of the prion protein gene. Further review of his medical records revealed a history of cryptorchidism and treatment with hCG as a child in the 1940’s-1950’s.






Discussion: This case report describes a possible case of iCJD from hCG injections and is unique in that the patient was male and the incubation period approached 50 years. His clinical presentation, EEG findings, and codon 129 homozygousity are similar to previously described cases.















Tuesday, November 23, 2010





Prosecutors call for prison terms for CJD growth hormone doctors France -














Tuesday, May 04, 2010



Review of the Human Pituitary Trust Account and CJD










Monday, February 01, 2010


Import Alert 57-20 and 84-03 Human Dura Mater and risk factors there from due to Creutzfeldt Jakob Disease (CJD)










Saturday, January 26, 2008



CJD HGH BODY SNATCHERS Saturday, January 26, 2008 CJD HGH BODY SNATCHERS HORMONE DRUGS LED TO CJD DEATH











Friday, March 25, 2011


Detection of Prion Protein in Urine-Derived Injectable Fertility Products by a Targeted Proteomic Approach










Tuesday, January 10, 2012


ESHRE position statement concerning prion detection in urinary gonadotropin formulations











16 YEAR OLD SPORADIC FFI ?








Monday, January 14, 2013


Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe











Sunday, February 10, 2013


Creutzfeldt-Jakob disease (CJD) biannual update (February 2013) Infection report/CJD











the great baby food debate about BSE and CJD









Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.







RESTRICTED - POLICY


CJD IN ADOLESCENTS


snip...


3. The first case is that of CJD in a 19 year old boy. This is already publicly known and was the subject of a ''World In Action'' programme over the summer. The second case is in a 17 year old girl, and is the one I reported to the Minister in my minute of 22 September. This patient is still alive, but CJD has been confirmed by brain biopsy. This will be the first time in which this case had been made public.


95/10.25/6.1


T E D EDDY












SEAC HAS STILL FAILED TO EXPLAIN THIS ;





Epidemiologic implications of Creutzfeldt-Jakob disease in a 19 year-old girl





Journal European Journal of Epidemiology Publisher Springer Netherlands ISSN 0393-2990 (Print) 1573-7284 (Online) Issue Volume 1, Number 1 / March, 1985





P. Brown1, F. Cathala2, R. Labauge3, M. Pages3, J. C. Alary3 and H. Baron


(1) Laboratory of CNS Studies, NINCDS, National Institutes of Health, 20205 Bethesda, Maryland, USA (2) Laboratoire de Neurovirologie, Hôpital de la Salpêtrière, Paris, France (3) Départment de Neurologie, Centre Hospitalier Universitaire, Montpellier, France





Abstract A histopathologically-verified, clinically typical case of Creutzfeldt-Jakob disease (CJD) is described in a 19 year-old girl. Only 3 previous cases of CJD have been reported in adolescents, and one of these was iatrogenically transmitted, while another was familial. Epidemiologic investigation of the present case excluded a familial component, and provided no evidence for iatrogenic or natural case-to-case transmission, or of other environmental sources of viral contamination. Young patients such as this one serve to emphasize the obscurity that still sourrounds the epidemiology of CJD, and invite serious reconsideration of the possibilities of transmission by undetected virus carriers, or of the agent as a natural resident of human cells, replication of which might be triggered by non-infective (e.g., traumatic or mutational) environmental events. Key words Creutzfeldt-Jakob disease - Epidemiology










P. Brown1, F. Cathala2, R. Labauge3, M. Pages3, J. C. Alary3 and H. Baron






(1) Laboratory of CNS Studies, NINCDS, National Institutes of Health, 20205 Bethesda, Maryland, USA (2) Laboratoire de Neurovirologie, Hôpital de la Salpêtrière, Paris, France (3) Départment de Neurologie, Centre Hospitalier Universitaire, Montpellier, France







Abstract A histopathologically-verified, clinically typical case of Creutzfeldt-Jakob disease (CJD) is described in a 19 year-old girl. Only 3 previous cases of CJD have been reported in adolescents, and one of these was iatrogenically transmitted, while another was familial. Epidemiologic investigation of the present case excluded a familial component, and provided no evidence for iatrogenic or natural case-to-case transmission, or of other environmental sources of viral contamination. Young patients such as this one serve to emphasize the obscurity that still sourrounds the epidemiology of CJD, and invite serious reconsideration of the possibilities of transmission by undetected virus carriers, or of the agent as a natural resident of human cells, replication of which might be triggered by non-infective (e.g., traumatic or mutational) environmental events. Key words Creutzfeldt-Jakob disease - Epidemiology



















2. Sporadic CJD normally occurs in people in their 50s and 60s although it can occur more rarely in younger age groups. Until this year the youngest case of sporadic CJD in the UK had been in a 34 year old. Other countries, howver, have reported sporadic CJD in teenagers. Those we know about are;




* in the USA, a 16 year old in 1978;




* in France, a 19 year old in 1982;




* in Canada, a 14 year old of UK origin in 1988;




* in Poland cases in people aged 19, 23, and 27 were identified in a retrospective study (published 1991), having been originally misdiagnosed with a viral encephalitis;




* Creutzfeldt's first patient in 1920 was aged 23.




full text ;












snip...





J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd





Original articles





Sporadic creutzfeldt-jakob disease in two adolescents





K Murray 1, D L Ritchie 1, M Bruce 2, C A Young 3, M Doran 3, J W Ironside 4 and R G Will 4* 1 NationalCJD Surveillance Unit, United Kingdom 2 Neuropathogenesis Unit, United Kingdom 3 Walton Centre for Neurology and Neurosurgery, United Kingdom 4 National CJD Surveillance Unit, United Kingdom





* To whom correspondence should be addressed. E-mail: r.g.will@ed.ac.uk.





Accepted 15 April 2007





Abstract





Background: Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional.





Methods: Data from the systematic study of sporadic CJD in the UK are available from 1970 onwards. Clinical and pathological data are reviewed in order to identify atypical cases, including those at the extremes of the age range of sporadic CJD. Detailed analysis of atypical cases is undertaken and in selected cases laboratory transmission studies are carried out in order to provide information on the characteristics of the infectious agent.





Results: In the UK two cases of sporadic CJD in adolescents have been identified, dying aged 16 and 20 years. The first case predated the epidemic of bovine spongiform encephalopathy and the characteristics of the second case, including laboratory transmission studies, are consistent with a diagnosis of sporadic rather than variant CJD.





Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.


















see full text ;















TSS

Creutzfeldt-Jakob disease (CJD) biannual update (February 2013) Infection report/CJD

Infection report/CJD





Creutzfeldt-Jakob disease (CJD) biannual update (February 2013)





This six-monthly report provides an update on reports of incidents of potential iatrogenic (healthcare-acquired) exposures to CJD. The data is correct as of 31st December 2012. For numbers of CJD case reports, readers should consult data provided by the National CJD Research and Surveillance Unit (NCJDRSU), Edinburgh [1]. The latest yearly analysis of vCJD reports (onsets and deaths) is also available from the NCJDRSU website [2].





Reports of incidents of potential iatrogenic exposure to CJD via surgery: 2000 to 2012





A surgical incident occurs when a patient with or at ‘increased risk’ of CJD has undergone surgery without the appropriate infection control guidance being followed [3]. This could occur if an asymptomatic patient undergoes surgery during the incubation period of CJD, or because information for those potentially at risk of CJD is not available at the time of surgery. If this happens, surgical instruments may be contaminated with the infectious agent that causes CJD. These instruments could then pose a transmission risk when they are re-used on other patients.





In June 2010 a distinction was made between surgical incidents and CJD reports. Only CJD cases (or patients at ‘increased risk’ of CJD) who have undergone surgical procedures which are thought to pose a possible transmission risk (i.e. within the likely infectious incubation period, and involving medium or high risk procedures) are categorised as 'surgical incidents'. Other procedures, either earlier in the incubation period, or involving low infectivity tissues, are categorised as 'CJD reports'. If the investigation of a surgical incident identifies any instruments that are considered to be potentially contaminated with the infectious agent, and that could still pose an infection risk to other patients, the Panel advises that these instruments should be removed from general use or refurbished. These instruments may be quarantined, kept for exclusive use on the index patient, refurbished (endoscopes only) or destroyed.





Since 2000 there have been 92 incidents in which instruments have been permanently removed from general use or refurbished (endoscopes only).





Table 1 shows the number of CJD surgical incidents and reports notified to the CJD Incidents Panel by the diagnosis of the index patient from 2000 to 2012. Advice has been issued for 7 surgical incidents and 25 surgical reports that were notified to the CJD Incidents Panel in 2012.





Health Protection Report Vol 7 No. 6 - 8 February 2013





Table 1. Number of CJD Surgical Incidents/Reports Notified to the CJD Incidents Panel:





2000- 2012





Index patient


status


2000


2001


2002


2003


2004


2005


2006


2007


2008


2009


2010


2011


2012


Total I





snip...





TOTAL 16 38 56 50 45 56 63 27 33 29 23 4 13 40 7 25 456 59





Health Protection Report Vol 7 No. 6 - 8 February 2013





Surgical incidents resulting in ‘at risk’ patients





The Panel may advise contacting and informing patients of their possible exposure to CJD following a surgical incident. These patients should be considered 'at risk of CJD for public health purposes' and are asked to take certain precautions (i.e. not to donate blood, other tissues or organs, and to inform their medical and dental carers prior to any invasive procedures) in order to reduce the risk of transmitting the CJD agent.





The diagnosis of the index patient; the timing of the procedure relative to the development of clinical CJD; the tissue that instruments were in contact with during the procedure on the index patient; and the number of cycles of re-use and decontamination the instruments have been through following the procedure on the index case – all influence the possible risk to subsequent patients.





The threshold level of risk at which patients are considered to be ‘at increased risk’ of CJD is 1%, in addition to the background risk in the UK population. This risk threshold is based on risk assessment models, using precautionary assumptions. The 1% threshold level is used as a cut off for implementing public health precautions and is not intended to be a precise measure of an individual patient's risk. A similar threshold is used for identifying other patients who have been exposed to possible CJD risks following surgical, blood, plasma and tissue incidents.





From 2000 to 31st December 2012, there have been 29 surgical incidents in which the Panel has advised that 192 patients should be considered to have an increased risk of CJD.





Patient denotifications





Following changes in the assessment of tissue infectivity and procedural risks in 2005 and 2009, the Panel has advised that 38 patients in 14 surgical incidents who were originally considered (and notified) as being ‘at risk' of CJD should no longer be considered ‘at risk', and should be denotified.





The Panel has received confirmation that of the 34 patients originally notified of their exposure (out of the 38 originally considered to be ‘at risk'), 26 patients have been informed that they are no longer considered ‘at risk' and eight patients died before they could be denotified.





Relating to surgical instruments there are 15 surgical incidents in which 154 patients are still considered to be at increased risk of CJD. Currently, 119 of these 'at risk' patients are alive and notified of their increased risk of CJD. Local decisions have been taken not to notify two patients in these incidents.





Monitoring of patients 'at increased risk' of CJD





The CJD Incidents Panel and the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy Risk Management Subgroup (formerly the ACDP TSE Working Group) have identified a range of individuals and groups who may have been exposed to an increased risk of CJD as a consequence of their medical care (see table 2 below). The risks of iatrogenic CJD transmission to these different individuals are very uncertain, but potentially devastating. The CJD Incidents Panel has advised that these individuals should be informed of their risk and asked to follow public health precautions to avoid transmitting the infection to others.





It is important to follow up these individuals to help determine the risks of CJD spreading to patients through different routes. Follow up involves a range of activities and is carried out by different organisations. At core, follow up aims to ascertain whether any people who may have been exposed to increased CJD risks go on to develop CJD.





Health Protection Report Vol 7 No. 6 - 8 February 2013





Table 2. Summary of the Health Status of All Individuals ‘At Increased Risk’ of CJD/vCJD,





2000-2012





Source: CJD Panel Secretariat





*Data for recipients of human derived growth hormone as at 30/06/2012





a These are minimum figures. Central reporting for bleeding disorder patients is incomplete, and seven patients have opted out of the central UKHCDO database. A small number of ‘at risk’ growth hormone recipients are not included in the Institute of Child Health study. Not all of ‘at risk’ growth hormone recipients have been notified. There is no central record of who has been informed.





b An asymptomatic infection is when an individual does not exhibit any of the signs and symptoms of CJD in life but abnormal prion protein indicative of CJD infection has been found in tissue obtained from them. In these cases the abnormal prion protein was identified during post mortem after the individuals had died of other causes.





c One patient was notified by proxy.





d Four of these were notified by proxy.





e Two of these were notified by proxy.





e Includes patients who were notified by proxy.







snip...







Total for all ‘at risk’ groups a 6,143 >2,198 >1,788 74 2







Health Protection Report Vol 7 No. 6 - 8 February 2013







References





1. The National Creutzfeldt-Jakob Disease Research and Surveillance Unit, The University of Edinburgh. CJD statistics. Available at: http://www.cjd.ed.ac.uk/data.html





2. The National Creutzfeldt-Jakob Disease Research and Surveillance Unit, The University of Edinburgh.Incidence of variant Creutzfeldt-Jakob disease onsets and deaths in the UK January 1994 - May 2011.Edinburgh: NCJDSU, 18 May 2011. Available at: http://www.cjd.ed.ac.uk/documents/report20.pdf





3. Transmissible spongiform encephalopathy agents: safe working and the prevention of infection. The ACDP TSE Risk Management Subgroup.




















Thursday, January 17, 2013



TSE guidance, surgical, dental, blood risk factors, Part 4 Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings (updated January 2013)












Tuesday, July 31, 2012



11 patients may have been exposed to fatal disease Creutzfeldt-Jakob Disease CJD Greenville Memorial Hospital












Thursday, August 02, 2012



CJD case in Saint John prompts letter to patients Canada CJD case in Saint John prompts letter to patients












Friday, February 10, 2012



Creutzfeldt-Jakob disease (CJD) biannual update (2012/1) potential iatrogenic (healthcare-acquired) exposure to CJD, and on the National Anonymous Tonsil Archive












Monday, November 26, 2012



Aerosol Transmission of Chronic Wasting Disease in White-tailed Deer












Thursday, December 29, 2011



Aerosols An underestimated vehicle for transmission of prion diseases?






please see more on Aerosols and TSE prion disease here ;













Saturday, February 12, 2011



Another Pathologists dies from CJD, another potential occupational death ?



another happenstance of bad luck, a spontaneous event from nothing, or friendly fire ???














Tuesday, December 14, 2010



Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings part 4, Annex A1, Annex J,



UPDATE DECEMBER 2010















Tuesday, September 14, 2010



Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)














Thursday, September 02, 2010



NEUROSURGERY AND CREUTZFELDT-JAKOB DISEASE Health Law, Ethics, and Human Rights The Disclosure Dilemma














Thursday, August 12, 2010



USA Blood products, collected from a donor who was at risk for vCJD, were distributed July-August 2010













Sunday, August 01, 2010



Blood product, collected from a donors possibly at increased risk for vCJD only, was distributed USA JULY 2010
















Thursday, July 08, 2010



Nosocomial transmission of sporadic Creutzfeldt–Jakob disease: results from a risk-based assessment of surgical interventions Public release date: 8-Jul-2010













Thursday, July 08, 2010



GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010












Wednesday, June 02, 2010



CJD Annex H UPDATE AFTER DEATH PRECAUTIONS Published: 2 June 2003 Updated: May 2010














Tuesday, May 11, 2010



Current risk of iatrogenic Creutzfeld–Jakob disease in the UK: efficacy of available cleaning chemistries and reusability of neurosurgical instruments














Tuesday, May 04, 2010



Review of the Human Pituitary Trust Account and CJD Issue 20 January 2010














Tuesday, March 16, 2010



Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Part 4 REVISED FEB. 2010














Monday, August 17, 2009



Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J,K, AND D Published: 2009














Monday, July 20, 2009



Pre-surgical risk assessment for variant Creutzfeldt-Jakob disease (vCJD) risk in neurosurgery and eye surgery units














Friday, July 17, 2009



Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units Volume 3 No 28; 17 July 2009














Sunday, May 10, 2009



Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)














Thursday, January 29, 2009



Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999-2008 (WARNING TO Neurosurgeons and Ophthalmologists) Volume 15, Number 2-February 2009 Research














Wednesday, August 20, 2008



Tonometer disinfection practice in the United Kingdom: A national survey














Tuesday, August 12, 2008



Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)














Monday, December 31, 2007



Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation














Subject: CJD: update for dental staff



Date: November 12, 2006 at 3:25 pm PST



1: Dent Update. 2006 Oct;33(8):454-6, 458-60.



CJD: update for dental staff.














Saturday, January 16, 2010



Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al



Evidence For CJD/TSE Transmission Via Endoscopes



From Terry S. Singletary, Sr flounder@wt.net 1-24-3













Thursday, October 25, 2012



Current limitations about the cleaning of luminal endoscopes and TSE prion risk factors there from



Article in Press














2011 TO 2012 UPDATE



Saturday, December 3, 2011



Candidate Cell Substrates, Vaccine Production, and Transmissible Spongiform Encephalopathies



Volume 17, Number 12—December 2011














Sunday, June 26, 2011



Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque














Monday, February 7, 2011



FDA’s Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ???














Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis

















full text with source references ;
















Are some commoner types of neurodegenerative disease (including Alzheimer's disease and Parkinson's disease) also transmissible? Some recent scientific research has suggested this possibility




Singeltary submission ;






Wednesday, May 16, 2012



Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?



Proposal ID: 29403












Wednesday, January 5, 2011



ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011 Prions



David W. Colby1,* and Stanley B. Prusiner1,2





















U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001

















Monday, January 14, 2013



Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe











Monday, December 31, 2012



Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State, 2006–2011-2012













Tuesday, December 25, 2012



CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25, 2012














Tuesday, June 26, 2012


Creutzfeldt Jakob Disease Human TSE report update North America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012


type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the rise in Canada and the USA












Wednesday, June 13, 2012


MEXICO IS UNDER or MIS DIAGNOSING CREUTZFELDT JAKOB DISEASE AND OTHER PRION DISEASE SOME WITH POSSIBLE nvCJD












TSS