Behavioural and Psychiatric Features of the Human Prion Diseases: Experience in 368 Prospectively Studied Patients
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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
posted by Terry S. Singeltary Sr. at
12:54 PM
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