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Behavioural and Psychiatric Features of the Human Prion Diseases: Experience in 368 Prospectively Studied Patients

Posted Aug 01 2012 1:54pm
43


Tue 29th, Neurodegeneration: 17:33


Behavioural and Psychiatric Features of the Human Prion Diseases: Experience in 368 Prospectively Studied Patients


Thompson,AGB; Mackay, A; Rudge, P; Lukic, A; Porter, MC; Lowe, J; Gopalakrishnan, GS; Collinge, J; Mead, S


MRC Prion Unit, Department of Neurodegenerative Disease, UCL Institute of Neurology; NHS Highland Mental Health Services, Argyll and Bute Hospital; NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery


Behavioural and psychiatric symptoms (BPS) are major features of the prion diseases with a great impact on patients and carers, but up to now have been subjected to very little systematic clinical research. We have prospectively studied 368 patients, providing an unprecedented opportunity to study these symptoms across the range of prion disease types. The prevalence of BPS was high in all disease types, ranging from 70% in inherited prion disease due to PRNP point mutation (IPD-point) to 96% in variant CJD (vCJD). Prevalence at the onset of disease was more variable, ranging from 24% (IPD-point) to 92% (vCJD). We describe the clinical characteristics and natural history of the commonly occurring symptoms. Observational clinical data on 189 courses of symptomatic drug treatment shows that use of antidepressants, antipsychotics, benzodiazepines and acetyl cholinesterase inhibitors was reported to be beneficial in a substantial proportion of patients (between 15% and 60%) and that no severe adverse events were reported.


We show that specific disease types and PRNP codon 129 genotypes independently predict the presence of psychotic symptoms and the presence of BPS at disease onset, suggesting that prion strain is largely responsible for the heterogeneity seen in these clinical features. However, we also show that, in sporadic CJD, younger age at onset independently predicts the presence of BPS at onset. We discuss the implications of these findings both for clinical practice and for our understanding of the biological mechanisms underlying the complex symptoms of prion disease.


andrew.thompson2@uclh.nhs.uk







Thursday, July 10, 2008


A New Prionopathy update July 10, 2008


snip...


DOES ANYONE BESIDES ME SEE A PATTERN YET ??


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.


SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.


Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.


snip...


Sporadic creutzfeldt-jakob disease in two adolescents





see full text sporadic CJD the big lie;


snip...


IT seems we have come full circle from the 'ORIGINAL 10' i.e. the 1st 10 adolescents in the UKBSEnvCJD only theory. and now we find us at the 1st 10 in USA, or is it the first 10, or the tip of the iceburg, many that went undocumented ??


lets look at the full circle, to date ;





Sunday, August 10, 2008


A New Prionopathy OR more of the same old BSe and sporadic CJD





full text ;






snip...see full text ;




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






Monday, August 9, 2010


Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more Prionbaloney ? http://prionunitusaupdate2008.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html




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






Saturday, March 5, 2011


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






Wednesday, June 27, 2012


First US BSE Case Since 2006 Underscores Need for Vigilance


Neurology Today 21 June 2012






in the url that follows, I have posted


SRM breaches first, as late as 2011.


then


MAD COW FEED BAN BREACHES AND TONNAGES OF MAD COW FEED IN COMMERCE up until 2007, when they ceased posting them.


then,


MAD COW SURVEILLANCE BREACHES.




Friday, May 18, 2012


Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States Friday May 18, 2012






Thursday, June 21, 2012


MEATINGPLACE.COM WAVES MAGIC WAND AND EXPECTS THE USDA MAD COW FOLLIES BSE TO BE GONE






Thursday, June 14, 2012


R-CALF USA Calls USDA Dishonest and Corrupt; Submits Fourth Request for Extension


R-CALF United Stockgrowers of America






Friday, May 25, 2012


R-CALF USDA’s New BSE Rule Eliminates Important Protections Needed to Prevent BSE Spread






Monday, June 18, 2012


R-CALF Submits Incomplete Comments Under Protest in Bizarre Rulemaking “Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products”






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






Monday, July 23, 2012


The National Prion Disease Pathology Surveillance Center July 2012






previous USA PRION UNIT reports ;






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...






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. In particular the L-type Atypical BSE agent might be similarly or even more virulent to humans than the Classical BSE agent. While mankind has been in contact with the major TSE of small ruminants for centuries, there is no epidemiological evidence to suggest that classical scrapie is zoonotic; however, experimental transmission data on humanised mice and non-human primates have been very scarce so far.





Monday, October 10, 2011


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







Tuesday, July 31, 2012



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









Wednesday, April 25, 2012


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







layperson



Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518


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