235th American Chemical Society National Meeting In New Orleans

Sunday, April 6



12:00 p.m.


Alligator blood may put the bite on antibiotic-resistant infections



Despite their reputation for deadly attacks on humans and pets, alligators are wiggling their way toward a new role as potential lifesavers in medicine. Scientists report that proteins in gator blood may provide powerful new antibiotics to help fight infections associated with diabetic ulcers, severe burns, and "superbugs" that are resistant to conventional medication. See corresponding news release, abstract, and nontechnical summary for AGFD 32.



Kermit K. Murray, Ph.D., Professor in the Department of Chemistry at Louisiana State. Lancia N.F. Darville is a doctoral student in the Department of Chemistry at Louisiana State University in Baton Rouge, La.



2:30 p.m.


Expert foresees 10 more years of R&D to make solar energy competitive



Despite oil prices that hover around $100 a barrel, it may take at least 10 or more years of intensive research to reduce the cost of solar energy to levels competitive with petroleum, according to a leading expert on the topic. See corresponding news release, abstract, and nontechnical summary for PRES 063.



Harry Gray, Ph.D., is the Arnold O. Beckman Professor of Chemistry and Founding Director of the Beckman Institute at the California Institute of Technology.



Paul Alivisatos, Ph.D., of the University of California at Berkeley and co-editor of the ACS journal Nano Letters, will describe potential advantages of future solar cells using nanoscale materials, and address some of difficulties that need to be overcome.



3:30 p.m.


Isle of Stability



Those exploring the uncharted seas at the fringes of the Periodic Table of the Elements have landed on one long-sought island - the fabled Island of Stability, home of a new genre of superheavy chemical elements sought for more than three decades. Researchers now are eying other islands on the more-distant fringes of the periodic table. See corresponding news release, abstract, and nontechnical summary for NUCL 007.



Yuri Oganessian, Ph.D., is with the Joint Institute for Nuclear Research in Dubna, Russia. Ken Moody, Ph.D., is a nuclear chemist at the Lawrence Livermore National Laboratory in Livermore, CA.



4:00 p.m.


Meteorites delivered the "seeds" of Earth's left-hand life



Desert heat, a little water and meteorite impacts may have been enough to cook up one of the first prerequisites for life: The dominance of "left-handed" amino acids, the building blocks of life on this planet. Ronald Breslow will describe how our amino acid signature came from outer space. See corresponding news release, abstract, and nontechnical summary for ORGN 001.



Monday, April 7



9:00 a.m.


As nanotech goes mainstream, "toxic socks" raise concerns
















Valued for its antibacterial and odor-fighting properties, nanoparticle silver is becoming the star attraction in a range of products from socks to bandages to washing machines. But as silver's benefits propel it to the forefront of consumer nanomaterials, scientists are recommending a closer examination of the unforeseen environmental and health consequences of nanosilver. Paul Westerhoff, Ph.D. and Troy M. Benn will discuss the research. See corresponding news release, abstract, and nontechnical summary for ENVR 031.



Paul Westerhoff, Ph.D., is a Professor in the Department of Civil and Environmental Engineering at Arizona State University in Tempe, Ariz. Troy M. Benn is a Graduate Researcher in the Department of Civil and Environmental Engineering at Arizona State University in Tempe, Ariz.



10:00 a.m.


Fungus fight: Researchers battle against dangerous corn toxin



At the 235th national meeting of the American Chemical Society in New Orleans, scientists presented advances towards the production of corn less susceptible to aflatoxin contamination. The new varieties could contribute to the reduction of the worldwide threat of the deadly toxin, improve food quality in developing countries and increase corn yield for food in the United States. See corresponding news release, abstract, and nontechnical summary for AGFD 081.



Bruce Hammond, Ph.D., is a researcher in the Product Safety Center at Monsanto.



11:00 a.m.


Protecting a life-saving blood product from human form of mad cow disease



Amid concern that recipients of certain blood transfusions may risk infection with a deadly protein responsible for the human form of mad cow disease, researchers in Canada now report development of a special filter that quickly and effectively removes the protein from blood. The use of the device will significantly decrease the risk of acquiring variant Creutzfeldt-Jakob Disease (vCJD), the human form of mad cow, through blood transfusions, the researchers say. See corresponding news release, abstract, and nontechnical summary for I&EC 064.



Patrick V. Gurgel, Ph.D., Senior Research Scientist in the Department of Chemical and Biomolecular Engineering at PromMetic Life Sciences in Mont-Royal, Quebec, Canada.



Tuesday, April 8



9:00 a.m.


DVDs and CD-ROMs That Thwart Global Warming



Chemists report that carbon dioxide removed from smokestack emissions in order to slow global warming could become a valuable raw material for the production of DVDs, beverage bottles and other products made from polycarbonate plastics. See corresponding news release, abstract, and nontechnical summary for FUEL 150 and FUEL 155.



[Tentative] Thomas E. M??ller, Ph.D., is a Professor at the Institute for Technical Chemistry and Macromolecular Chemistry at RWTH Aachen University in Aachen, Germany.



Toshiyasu Sakakura, Ph.D., is a researcher with the National Institute of Advanced Industrial Science and Technology in Tsukuba, Japan.



10:00 a.m.


Biochemical signals associated with atherosclerosis may damage other organs



In a finding that challenges conventional medical knowledge, researchers report that plaques formed in during atherosclerosis, or hardening of the arteries, are associated with certain harmful chemical reactions that can contribute to damage in the lungs, liver, and other organs. The study suggests that the effects of the disease are more widespread than previously believed. See corresponding news release, abstract, and nontechnical summary for BIOL 152.



Rita K. Upmacis, Ph.D., is an Associate Research Professor in the Department of Pathology at Weill Medical College of Cornell University, New York, NY.



1:00 p.m.


How sweet it is: "Revolutionary" process points to sugar-fueled cars



Chemists are describing development of a "revolutionary" process for converting plant sugars into hydrogen, which could be used to cheaply and efficiently power vehicles equipped with hydrogen fuel cells without producing any pollutants. See corresponding news release, abstract, and nontechnical summary for FUEL 213.



Y.-H. Percival Zhang, Ph.D., is an Associate Professor in the Biological Systems Engineering Department at Virginia Tech in Blacksburg, Va.



2:00 p.m.


Anshul Samar, the "Elementeo Kid"



Age seems to be no obstacle when it comes to starting a business. That's the case with 14-year-old Anshul Samar, CEO of Elementeo, who invented a board game that aims to teach chemistry to students (elementeo/) in a fun, unusual way.







The American Chemical Society - the world's largest scientific society - is a nonprofit organization chartered by the U.S. Congress and a global leader in providing access to chemistry-related research through its multiple databases, peer-reviewed journals and scientific conferences. Its main offices are in Washington, D.C., and Columbus, Ohio.

Using math and physics to investigate mad cow disease

Contact: Andy Fell

ahfellucdavis

530-752-4533

University of California - Davis (USA)


Using math and physics to investigate mad cow disease (bovine spongiform encephalopathy or BSE) and similar diseases caused by infectious proteins called prions is the aim of research by physicists Daniel Cox, Rajiv Singh and colleagues at UC Davis.

The researchers are using mathematical models to study issues such as the incubation time, prion 'strains' and treatment or detection strategies.


Diseases such as BSE in cattle, Creutzfeld-Jakob disease in humans and chronic wasting disease in deer are all apparently caused by prions, misfolded versions of a normal brain protein.

Similar diseases have been found in other animals including cats, mink and rodents, and prion-type proteins have even been found in yeast.



Prions seem to cause disease by triggering normal versions of the same protein to spontaneously fold up the wrong way, creating growing mats and tangles.



The UC Davis researchers have developed mathematical models to simulate this process. The models reproduce how prions collect around an original 'seed' prion, and how these clumps subsequently break up and spread around the brain and nervous system.



Predictions from the models compare well with the course of actual disease in both small and large animals, Cox said.



Cox and colleagues are now using the model system to investigate the minimal requirements for prions to cause disease; how 'strains' of prions can exist and what effect they have; and potential treatment strategies.



Media contact: * Daniel Cox, Physics, 530-752-1789, coxphysics.ucdavis (Cox is best contacted by email.)

Silencing The Cause Of Mad Cow Disease

BSE (more commonly known as mad cow disease) and CJD, which is a related disease in humans that can occur spontaneously, be inherited, or be acquired (in some cases probably from cows with BSE), are fatal neurodegenerative diseases. It is thought that these diseases are caused by accumulation in the brain of an abnormally folded version (PrPsc) of a natural protein (PrPc). There are currently no therapies for the treatment of these diseases, making this an area of active investigation.



In a study appearing in the December issue of the Journal of Clinical Investigation, Alexander Pfeifer and colleagues from the University of Bonn, Germany, show that in mice silencing of the gene encoding PrPc suppresses the accumulation of PrPsc. In vitro, silencing the gene encoding PrPc, using a technique known as RNA interference (RNAi), in already diseased neurons suppressed the accumulation of PrPsc. Similarly, in mice engineered to express the gene silencing therapeutic in a varying proportion of their neurons, the accumulation of PrPsc was markedly delayed, with the delay in accumulation of PrPsc being directly correlated with the proportion of neurons in the brain expressing the gene silencing therapeutic. This study therefore provides hope that RNAi might provide a new approach for the development of a therapeutic to treat individuals and animals with neurodegenerative disorders such as CJD and BSE. However, as Qingzhong Kong from Case Western Reserve University says in an accompanying commentary "Much more research is needed before RNAi can be harnessed to treat these neurodegenerative disorders."






TITLE: Lentivector-mediated RNAi efficiently suppresses prion protein and prolongs survival of scrapie-infected mice



AUTHOR CONTACT:

Alexander Pfeifer,

University of Bonn, Bonn, Germany.



Hans Kretzschmar

University of Munich, Munich, Germany.



ACCOMPANYING COMMENTARY

TITLE: RNAi: a novel strategy for the treatment of prion diseases



AUTHOR CONTACT:

Qingzhong Kong

Case Western Reserve University, Cleveland, Ohio, USA.



Contact: Karen Honey


Journal of Clinical Investigation

Monthly Creutzfeldt Jakob Disease Statistics, UK

The UK 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 30 September 2005


Summary of vCJD cases


Deaths

Deaths from definite vCJD (confirmed): 108

Deaths from probable vCJD (without neuropathological confirmation):
42

Deaths from probable vCJD (neuropathological confirmation pending): 1

Number of deaths from definite or probable vCJD (as above): 151


Alive

Number of probable vCJD cases still alive: 6

Total number of definite or probable vCJD (dead and alive): 157


The next table will be published on Monday 7th November 2005


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).


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.


UK Dept of Health

National CJD Surveillance Unit Publishes 13th Annual Report For 2004, UK

The Thirteenth Annual Report of the National Creutzfeldt-Jakob
Disease Surveillance Unit was published today. The report looks back
over the period from May 1990 when the Unit was set up to 31 December
2004. The report outlines the Unit's work in the clinical
surveillance of sporadic, variant (vCJD) and iatrogenic CJD.


Also included in the report are details of a study on the potential
risk factors for variant and sporadic CJD and the work of the
National Care Team in arranging care and advice to the families of
CJD patients.


The main issues highlighted are:



153 cases of definite or probable vCJD had been identified in the UK,
with 148 deaths reported up to 31 December 2004. Analysis of the
incidence of vCJD onsets and deaths from January 1994 to December
2004 provides statistically significant evidence that a peak in the
incidence of vCJD has been passed.


The Transfusion Medicine Epidemiology Review had identified the first
case of vCJD associated with blood transfusion in 2003 in which a
blood recipient had developed symptoms of vCJD 6 1/2 years after
receiving a transfusion of red cells donated 3 1/2 years before the
donor developed symptoms of vCJD.


The incidence of vCJD across the UK continues to show a "North -
South" difference, although slightly less than previously reported,
with a higher incidence being maintained in the North of the UK. The
underlying reason for this finding is not clear.


It remains the case that the only statistically significant
geographic cluster of vCJD cases in the UK was in Leicestershire.
This cluster was first identified in July 2000. All geographically
associated cases of vCJD are considered for investigation according
to a protocol which involves the NCJDSU, the Health Protection
Agency, The Scottish Centre for Infection and Environmental Health
(SCIEH), and local public health physicians.


There is no evidence of any difference between the UK and other
European countries in the incidence of sporadic CJD, nor any
significant variation in recorded mortality within the UK.


The NCJDSU is jointly funded by the Department of Health and the
Scottish Executive Health Department.


The report is available on the NCJDSU website at
cjd.ed.ac.uk

Food - New method to asses its risk

Risk and food are on the same plate: World food safety experts offer new methods to assess risk


SEATTLE (USA) - New methods are in the pipeline to improve the safety of the world's food supply, and the need is imminent, said the director of the National Food Safety and Toxicology Center at Michigan State University.



Ewen C.D. Todd is organizing the symposium 'Food Safety and Risk Assessment: New Approaches to Microbiological Problems' at the American Association for the Advancement of Science annual meeting today in Seattle. Todd emphasizes that microbiological foodborne hazards, such as listeriosis, are cause for concern today, due to many changes:



The globalization and urbanization of developing countries, some without adequate infrastructure systems to handle food and water safety.



Growing number of countries reporting increased numbers of foodborne illness through better surveillance systems.



Problems with produce contamination, extending the outlook of food risk.



Concerns with bioterrorism and possible threats to food security.



New patterns of food production, distribution and consumption, including increased travel, eating away from home, and the higher demand for a wide variety of food (issues of trade).



Recent recalls and industry losses such as the Pilgrim's Pride Listeria outbreak of 2002 and mad cow disease affecting Canadian and U.S. beef supplies in 2003 and 2004.


'Scientific risk-based policy is overtaking the cultural and political debate about food,' said Todd, referring to the United States' recent single case of mad cow disease.

'Countries are creating policies based on risk, not on culture. The current systems of testing and release of products have not proven very effective in reducing foodborne illness.'



Risk assessments are the gathering of quantitative data on the prevalence (whether it occurs) and concentration (how much is present) of foodborne illness for specific foods.


Modules from farm to fork help create a mathematical picture about risk for contamination. The resulting models can predict the number of illnesses that can occur, and how managers can change parameters - and thus food safety strategies - to control the risk. The concept is relatively new for the food industry - so it's imperative that the global community understand the issue and methods, Todd said.


Todd has gathered together world expert speakers on risk assessment of food to hash out approaches for countries as diverse as Malaysia and the United States - which, as with many countries, currently have different approaches to assess risk for foodborne illness. In addition to Todd, the speakers include:



Jorgen Schlundt of the World Health Organization: The Food and Agriculture Organization of the United Nations, along with WHO, is in the process of drafting risk assessment strategies for 21 foods.














Todd co-authored the FAO/WHO assessment on Listeria monocytogenes. (Biggest issues: What do countries do with these? How do they use the assessments?)



Karen Hulebak of the U.S. Department of Agriculture: Hulebak will represent the Codex Alimentarius Commission from the perspective of the Committee on Food Hygiene. (Biggest issue: How will the Codex standards affect trade?)



Karen Dodds of Health Canada: While Canada has a management system in place, factors such as bioterrorism and mad cow disease may impact food labeling and trade. Todd was the head of the Contaminated Foods Section of Health Canada for many years. (Biggest issue: How does Canada's management approach fit into the new standards?)



Isabel Walls of International Life Sciences Institute, Risk Science Institute: ILSI is drafting a final report that describes risk to different types of populations, including the sensitive immuno-compromised population.

They might require messages to not eat certain foods based on risk assessment findings.

Todd serves on the expert team to reduce listeriosis. (Biggest issue: Can you relax the zero tolerance policy for certain foods, and refocus resources on foods that cause illness?)



Leon Gorris of Unilever: Gorris will provide industry perspective on risk assessment, and how it can aid in product development and design. (Biggest issue: What is the cost to industry, and thus to consumers to control risk?)



Todd is a member of MSU's Environmental Science and Policy Program, a groundbreaking new effort that gathers the university's vast, multidisciplinary resources to best position students, scientists and society for a future filled with change and a need for balance.



For information on the program, access the Web site at environment.msu



According to the U.S. Centers for Disease Control and Prevention, there are 76 million cases of foodborne illness per year. This is the equivalent of 1 in 4 U.S. citizens acquiring a foodborne illness each year. The National Food Safety & Toxicology Center at Michigan State University, celebrating its fifth year of operation, is committed to reducing food-related disease on a global level through research, education and outreach.



Contact: Tom Oswald

oswaldmsu

517-355-2281

Michigan State University

Improving Understanding Of The Spread Of Infectious Prions

Researchers at the University of California, San Diego School of Medicine have identified the motors that move non-infectious prion proteins (PrPC) - found within many mammalian cells - up and down long, neuronal transport pathways. Identifying normal movement mechanisms of PrPC may help researchers understand the spread of infectious prions within and between neurons to reach the brain, and aid in development of therapies to halt the transport.



Their study is published in the February 18 edition of the journal Cell.



The small prion protein is found in the cell membrane of brain neurons. The misfolded or infectious form of this protein (also called "scrapie"), is responsible for "mad cow" disease and has also been implicated in Creutzfeldt-Jakob disease in humans. Non-infectious and scrapie forms interact to produce disease; so, in order to help uncover how the infection is spread within and among neuron cells to the brain, the UCSD scientists studied the movement mechanism of normal PrPC in mouse neuronal cells.



"Our work unraveling the normal mechanism of movement of this prion protein will help us understand how the devastating pathogenic versions found in mad cow disease and other prion diseases are formed and transmitted in the brain. Intriguingly, our work may also shed light on what goes wrong in other neurodegenerative diseases such as Alzheimer's disease," said principal investigator Larry Goldstein, PhD, professor of Cellular and Molecular Medicine, Howard Hughes Medical Institute investigator and director of the UC San Diego Stem Cell Program.



It is known that normal prion proteins and infectious prions need to interact in order for prion pathogenesis to occur, though not how or why these interactions occur. Discovering the transport mechanisms of prions is one key to the puzzle of how the two types of proteins interact, and an important question in transport regulation has been how motor activity is controlled in cells.



The prion protein cargo travels on long microtubule tracks along the peripheral and central nervous system nerves toward the terminus, or synapse, in membrane-bound sacs called vesicles. Intracellular transport is often bi-directional, because cargoes regularly reverse their course en route to their final destinations.



The researchers identified the motors driving these vesicles as anterograde Kinesin-1 - which moves only toward the synapse - and dynein, which is retrograde, moving away from the synapse. These two motor proteins assemble on the PrPC vesicles to "walk" them back and forth along the microtubules.



Secondly, they discovered that the back and forth cargo movement is modulated by regulatory factors, rather than by any structural changes to the motor-cargo associations. The study data show that the activity of Kinesin-1 and dynein are tightly coupled, with PrPC vesicles moving at different velocities and for varied lengths along axons. However, the type and amounts of these motor assemblies remain stably associated with stationary as well as moving vesicles, and normal retrograde transport by Kinesin-1 is independent of dynein-vesicle attachment.



The UCSD study of the mechanisms behind normal vesicle movement along the axons in mouse cells might also shed light on other neurodegenerative disease. While Alzheimer's is not generally considered an infectious disease like mad cow disease, emerging data suggest that Tau, amyloid-beta, and alpha-synuclein - proteins implicated in Alzheimer's and Parkinson's disease - have self-propagating fibril structures with prion-like characteristics.



"Whether these toxic molecules spread along neuronal transport pathways in ways similar to the normal prion protein is unknown," said first author Sandra E. Encalada, PhD, of the UCSD Department of Cellular and Molecular Medicine. "But characterization of these normal mechanisms might lead to a way to control movement of intracellular aggregates, and perhaps to therapies for many neurodegenerative diseases."



Additional contributors to the study include Lukasz Szpankowski, of the UCSD bioinformatics graduate program and the Howard Hughes Medical Institute, and Chun-hong Xia, UCSD Department of Cellular and Molecular Medicine, now at UC Berkeley.



The study was supported in part by the National Institutes of Health's National Institute on Aging.

Potential treatments for Creutzfeldt-Jakob Disease (CJD)

Creutzfeldt-Jakob disease and other human prion diseases are invariably fatal and there is currently no proven treatment for the underlying process. There are however a number of potential treatments in development or under consideration. It must be stressed that, to date, no treatment has been shown conclusively to slow or halt the disease process in humans with any form of CJD. There has been media coverage of two potential treatments in particular: Quinacrine and Pentosan Polysulphate.


Quinacrine


Dr Prusiner's group from San Francisco published an article in Proceedings of the National Academy of Sciences on 14/08/01 entitled 'Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease'. This article provided evidence of inhibition of the formation of the disease associated form of prion protein in scrapie infected neuroblastoma cells by a number of compounds, with quinacrine and chlorpromazine exhibiting the greatest potency.


The first paragraph of the article concludes "Beacause quinacrine and chlorpromazine have been used in humans for many years as anti-malarial and anti-psychotic drugs respectively, and are known to pass the blood brain barrier, we suggest that they are immediate candidates for the treatment of Creutzfeldt-Jakob disease and other prion diseases". More recently, Barret et al published a paper concerning laboratory experimental work on quinacrine, suggesting that its use as a treatment for CJD was questionable.


There was a report in The Mail on Sunday, on Sunday 12 August, entitled "Briton 'cured' in CJD drug trial". The article described a 20-year-old female with variant CJD who was treated in a pioneer drug trial in San Francisco and in whom the family reported clear neurological improvement.


Any finding that might lead to an effective treatment of CJD is to be welcomed. However, laboratory findings are simply indications of possible therapeutic value and the real test is whether a compound is actually effective (and safe) in human beings with disease.

Further work is essential in order to establish whether these drugs can provide an effective treatment and proper assessment needs to be carried out in the context of a well designed clinical trial. A preliminary trial was undertaken by the National Prion Clinic/MRC in London and a more extensive MRC trial is due to start in 2004. Further information on this will be circulated to all neurologists in the near future.


Relatives of patients currently suffering from CJD (including vCJD) may wish to obtain further information on this subject, with a view to consideration of initiating treatment with these compounds. Information on the current and proposed clinical trials can be obtained from the National Prion Clinic. Quinacrine and chlorpromazine are available in the UK, but neither are licensed for use in the treatment of human prion diseases and would need to be prescribed in the context of a trial, or on a named patient basis.

A suggested treatment regimen for quinacrine is 200mg 6 hourly for 5 doses followed by 100mg tds. Quinacrine (Mepacrine hydrochloride) is available from BCM Special in Nottingham, telephone number 0800 952 1010. The role and dosage regimen of chlorpromazine is uncertain and patients who have been treated to date have often received quinacrine alone.


Other treatments include: Pentosan Polysulphate - Risks of Intraventricular PPS - Benefits of Intraventricular PPS


Dr RSG Knight, NCJDSU

Mad cow disease could spread through urine, Swiss study

Researchers from the University Hospital of Zurich have found that prions can be spread through urine. Prions are proteins that cause mad cow disease, CJD and scrapie.


You can read about this study in the journal Science.


Lead researcher, Adriano Aguzzi, said that prions could be found in the urine of scrapie infected mice with kidney inflammation. Mice without kidney inflammation (infected with scrapie) had no prions in their urine.


Hence, they concluded that prions could be transmitted through urine.


Before this study, it was thought that the only way to infect an animal or human with BSE or CJD is through contaminated foods or feeds.


Perhaps we should monitor the urine, not just animal remains, say the researchers.


Written by:

Editor: us

Mad cow in US confirmed by British experts

The U.S. Department of Agriculture says new laboratory tests have confirmed the nation's first case of Mad Cow Disease. The announcement came Thursday after more than 20 countries suspended imports of U.S. beef, citing health reasons.


The U.S. Department of Agriculture said tests by British experts have confirmed an earlier analysis, by American scientists, on a cow traced to a dairy farm in the northwestern state of Washington.



The department said the British laboratory will conduct final tests to confirm its own findings that the cow had Bovine Spongiform Encephalopathy, a brain-wasting illness commonly known as mad cow disease.



But the department said it fully expects final results will be consistent with those of the tests already performed.



Cows usually become ill after eating animal feed that contains parts from BSE-infected cattle, especially the spine and brain. Humans can contract a deadly variant of BSE, known as Creutzfeldt-Jakob disease, by eating contaminated meat.


Creutzfeldt-Jakob has killed more than 100 people worldwide since it was discovered in Britain in 1986.



U.S. officials say they are trying to find the source of the infection and to determine whether any more cows have been infected with BSE.



U.S. Agriculture Secretary Ann Veneman, who announced initial test results on Tuesday, says the nation's food supply is safe and the risk to human health is minimal. More than 20 countries, including Japan, Mexico and South Korea, the three largest importers of U.S. beef, have so far suspended all imports.



But some scientists point out that cattle feed containing animal parts has been officially banned in the United States since 1997. They say the fact that a cow could become infected in spite of the ban shows glaring weaknesses in the government's attempts to protect the food supply.



In any event, U.S. agricultural officials are taking no chances and have ordered the recall of 4,700 kilograms of meat that passed through the same slaughterhouse as the infected cow.



Unfortunately for U.S. meat exporters, foreign governments have also decided they ought to take no chance with the health of their citizens.

Fresh VCJD Outbreak Feared As Third Blood Infected Patient Dies

Post mortem examination of a third person infected with variant Creutzfeldt-Jakob Disease (vCJD) through blood transfusion has confirmed the presence of the fatal disease.


Professor John Collinge of the Medical Research Council (MRC) Prion Unit has been tracking 66 people known to have accidentally received infected blood by transfusion. Of these, three are now confirmed to have died from vCJD and 39 from unrelated causes. The remaining 24 are now thought to have a significant risk of contracting the disease.


An update on the study was reported in The Lancet.


Prof Collinge said on BBC Radio 4's Today Programme earlier today that because three people have now died from this small group, the risk of contracting vCJD through blood transfusion is likely to be "substantial".


Humans can get vCJD through two routes: by ingesting meat infected with Bovine Spongiform Encephalopathy (BSE, or "mad cow" disease) or from infected humans who give blood or contaminate surgical instruments. This new evidence suggests that human to human transmission leads to much earlier development of full blown vCJD, unlike infection via the BSE route which can lie dormant for up to 50 years.


The danger to the public is that people who ate BSE infected meat as long as 20 years ago could be carrying the vCJD precursor "prions" and passed it on through blood donation before testing was brought in. The donor may not show symptoms for another 20 years, but the recipient may well show symptoms much earlier which is why there are renewed fears of a resurgence of cases following this latest news.


vCJD is a fatal, degenerative brain disease with no cure, although some experimental treatments exist. In humans it is transmitted through infection by prions. Unlike more common forms of infection, prions are not viruses or bacteria but "naked" proteins composed of the same amino acid building blocks as normal brain cell proteins. They have a different shape to normal brain cell proteins and infection spreads because they gradually convert the surrounding proteins into their own shape. This causes brain cells to malfunction and eventually die. The brains of people who die from vCJD and of cows who have died of BSE resemble a swiss cheese filled with spongy masses.


The presence of potential vCJD infectious prions can only be confirmed by brain tissue sampling or post mortem examination (for example of the tonsils), making diagnosis before symptoms of full blown vCJD emerge extremely difficult.


Click here to learn more about prions (NIH).

Chronix Biomedical Partners With University Of Calgary To Commercialize Serum DNA-Based Blood Test For Mad Cow Disease

Chronix Biomedical today announced that it is partnering with the University of Calgary to develop a commercial version of its serum DNA-based blood test for the early detection of bovine spongiform encephalopathy (BSE), also known as mad cow disease. Chronix researchers have published data demonstrating that the company's proprietary techniques can accurately detect BSE in cattle before any disease symptoms are evident. Currently BSE can only be definitively diagnosed with a post-mortem brain biopsy. Development of the commercial BSE assay is supported by a grant from the Alberta Livestock and Meat Agency (ALMA) and Genome Canada. Chronix intends to begin offering BSE testing services to the cattle industry once the commercial test is finalized and validated.


Chronix's technology identifies disease-specific genetic fingerprints based on circulating DNA that is released into the bloodstream by damaged and dying cells. The BSE test represents the first commercial application of Chronix's technology, which is primarily being developed for the detection and monitoring of human diseases including cancer and chronic conditions. Chronix recently published studies showing that it can identify the presence or absence of active disease in multiple sclerosis patients and that its approach can accurately detect early stage breast cancer with high diagnostic sensitivity and specificity. Further validating the Chronix technology, researchers will disclose additional breast and prostate cancer study results in an upcoming oral presentation at the 2010 ASCO Annual Meeting.


"The technology from Chronix is the first to demonstrate the potential to achieve our goal of an accurate, cost-effective assay that will revolutionize BSE testing, making it economically and logistically feasible to screen all cattle in the food chain before BSE symptoms appear," said Christoph Sensen, Ph.D., Professor at the University of Calgary Faculty of Medicine and Principal Investigator of the grant research program. "This effort is critically important because the risk of introducing BSE into the food chain continues to worry consumers and hinder international trade."


Chronix's proprietary serum DNA blood tests are based on a growing body of publications showing that circulating DNA fragments can be identified and analyzed to provide a diagnostic window into changes associated with specific diseases. These changes can be used to track the presence of disease months or years before symptoms appear


Howard Urnovitz, Ph.D., Chief Executive Officer of Chronix stated: "Our technology for the first time makes possible the early detection of BSE in an accurate, timely and cost-effective way. We appreciate the visionary support of ALMA and Genome Canada and look forward to working with our colleagues at the University of Calgary to develop a commercial BSE assay. Chronix is also currently developing additional serum DNA-based blood tests for human applications in cancer and chronic neurological conditions."


BSE was among the first diseases that Chronix showed could be detected using its novel approach. In a 2009 publication in Nucleic Acids Research, scientists working with Chronix demonstrated that their blood test based on circulating DNA sequences could identify the presence of BSE and a related condition long before symptoms were evident.


ASCO abstract #10505: Comparative analysis of the chromosomal origins of circulating nucleic acids in breast and prostate cancer, June 7, 2010.


Source

Chronix Biomedical

Farmed Fish May Pose Risk For Mad Cow Disease

University of Louisville neurologist Robert P. Friedland, M.D., questions the safety of eating farmed fish in the June issue of the Journal of Alzheimer's Disease, adding a new worry to concerns about the nation's food supply.



Friedland and his co-authors suggest farmed fish could transmit Creutzfeldt Jakob disease--commonly known as mad cow disease--if they are fed byproducts rendered from cows. The scientists urge government regulators to ban feeding cow meat or bone meal to fish until the safety of this common practice can be confirmed.



"We have not proven that it's possible for fish to transmit the disease to humans. Still, we believe that out of reasonable caution for public health, the practice of feeding rendered cows to fish should be prohibited," Friedland said. "Fish do very well in the seas without eating cows," he added.



Creutzfeldt Jakob disease is an untreatable, universally-fatal disease that can be contracted by eating parts of an animal infected with bovine spongiform encephalopathy (BSE or mad cow disease). An outbreak in England attributed to infected beef prompted most countries to outlaw feeding rendered cow material to other cattle because the disease is so easily spread within the same species.



The risk of transmission of BSE to humans who eat farmed fish would appear to be low because of perceived barriers between species. But, according to the authors, it is possible for a disease to be spread by eating a carrier that is not infected itself. It's also possible that eating diseased cow parts could cause fish to experience a pathological change that allows the infection to be passed between the two species.



"The fact that no cases of Creutzfeldt Jakob disease have been linked to eating farmed fish does not assure that feeding rendered cow parts to fish is safe. The incubation period of these diseases may last for decades, which makes the association between feeding practices and infection difficult. Enhanced safeguards need to be put in place to protect the public," Friedland said.



There have been 163 deaths from Creutzfeldt Jakob disease in the United Kingdom attributed to eating infected beef. Bovine spongiform encephalopathy has been identified in nine Canadian and three U.S. cattle.

Japan could learn from US food safety problems

The discovery in late December of the first U.S. case of bovine spongiform encephalopathy, or mad cow disease, has sparked discussions on the integration of U.S. departments and agencies in charge of food safety.


The U.S. House of Representatives subcommittee on realignment and rationalization of government departments and agencies said the U.S. food safety regulatory regime was a patchwork system, indicating the complexity of the current state of food safety control departments and agencies.



Few people understand how the 12 U.S. departments and agencies, including the Agriculture Department (DOA) and the Food and Drug Administration (FDA), go about implementing regulations on food safety.



The latest U.S. General Accounting Office (GAO) report proposes an integration of the related departments and bureaus or the establishment of an independent organization because in addition to simplifying and rationalizing administrative procedures, an independent organization can respond to emergencies quickly when an infectious disease breaks out or terrorists strike.



The poor handling of the first U.S. case of mad cow disease was one factor prompting discussions on a drastic review of the food safety control system in the United States.



Immediately after the discovery, the DOA, which tried to track down infected cows, and the FDA, which inspected fodder and processed foods made from cattle, separately inspected the farm where the BSE case was found.



The U.S. Congress criticized the dual inspections as administrative sectionalism.



Midway through the inspection, the DOA called off the follow-up check of infected cows and cows imported from the same overseas farm.



Sources close to the livestock industry said the check was canceled due to difficulties in coordinating inspections by the DOA and other related organizations because their authorities were not unified.



To continue reading this article, please go to this web page of The Daily Yomuiri Online

Dept of Health urges calm over vCJD warning, UK

Around 4,000 people have received letters warning them that they might have been infected with the human form of vCJD through contaminated blood plasma.


However, the department of health is stressing that risk of being infected are minimal and people should not be unduly worried by receiving the letter.


Chief Medical Officer Sir Liam Donaldson said: "Throughout our handling of the issue of vCJD we have adopted a highly precautionary approach, taking a series of steps as new evidence became available to maximise the protection of the public."


He added: "This risk assessment continues this approach and identifies three groups of patients who need to know that they may be at a small increased risk of developing vCJD than the rest of the population who ate beef during the 1980s and 1990s."


The government has also set-up a dedicated NHS direct phone line to help deal with any enquires or worries that people may have over the issue.


The majority of those who may have a greater risk of infection are sufferers of haemophilia and were given British clotting agents before safety measures were introduced in 1999.


hda-online.uk

Large Scale Tonsilectomy Study Would Quantify UK CJD Prevalence

A study in this week's issue of THE LANCET describes how two different types of analysis used in conjunction on samples of tonsil tissue is the 'gold standard' method for confirming clinical variant CJD, and that a large-scale screening programme of tonsil tissue is the only way of identifying the true incidence of vCJD infection.


Variant Creutzfeldt-Jakob disease (vCJD) is thought to be caused by dietary or other exposure to bovine spongiform encephalopathy (BSE) prions; 142 people have died of vCJD, although the prevalence of preclinical or subclinical prion infection in the UK is currently unknown.


Since clinical variant CJD is uniformly associated with tonsillar prion infection, Adam Frosh, John Collinge (MRC Prion Unit, Institute of Neurology, London, UK) and colleagues screened 2000 anonymous surgical tonsillectomy specimens for the presence of the rogue prion protein that causes vCJD. The investigators used two techniques to assess each specimen for the abnormal prion.


Although no positive cases of vCJD were identified, the investigators caution that this negative result cannot provide reassurance that relevant community infection is unlikely because of the fairly small sample size, demographic and age-related factors, and unknown test sensitivity during the prolonged incubation period.


Professor Collinge comments: "Our findings suggest that a national large-scale prospective prevalence study of tonsillectomy specimens is necessary. This study should examine fresh frozen specimens since any positive findings can be confirmed by transmission studies to indicator mice. However, the proportion of tonsillectomies done in patients born before the specified bovine offal ban is falling, thus reducing the opportunity to derive meaningful results".


In an accompanying commentary (p 1196), Markus Glatzel (University Hospital of Zurich, Switzerland) concludes: "Previous vCJD prevalence studies, based solely on the histological detection of prion protein accumulation, have detected one positive out of 8318 tested samples, and three positives out of 12,674 tested samples, respectively, which gives estimated prevalences of 120 per million and 237 per million inhabitants. These projections caused substantial turmoil, not least because the lack of frozen tissue precluded further biochemical analysis and additional assays aimed at showing prion infectivity in samples that tested positive. With a protocol like the one used by Frosh and colleagues these difficulties could have been avoided. Therefore, Frosh's protocol should be viewed as the gold standard for vCJD prevalence studies, for now".


The Lancet ISSUE: 2-8 October 2004

Variant CJD And Blood Transfusion

Until recently the risk of developing CJD as a consequence of a blood transfusion was a theoretical concern. However, in December 2003 a patient died from vCJD after receiving a blood transfusion from a donor who subsequently also had vCJD. Since then, three further patients have been identified. One patient died of an unrelated condition, but a post mortem examination established that the abnormal form of the prion protein was present in their body. This suggested that the prion protein had been transmitted via the blood transfusion but had not yet caused a brain illness.


Although we hope ultimately that the numbers of individuals who develop vCJD from blood transfusions will be small, we still do not know how many people in the UK could be incubating vCJD from this route of infection. Our understanding of these matters will be improved when a screening test is developed so that abnormal prion protein can be detected in the blood of at risk individuals or used to screen blood before it is transfused into someone else. Until this happens only precautionary measures can be put in place in an attempt to reduce the risk of exposure.


When a person is diagnosed with CJD, their health history is examined to see if they have had surgery or donated blood in the past. The risk to public health is then determined and anyone who is identified to be at an increased risk of contracting CJD (for instance, through receiving a blood transfusion from that individual) is informed via their GP of the fact that they may have been exposed. As a consequence, a small but increasing number of people who are deemed to have an additional risk of developing vCJD are being identified. Despite the fact that these people have been exposed we still have little idea as to the exact extent of their actual risk making it difficult to give overall advice about what they should expect.


One major concern has been that vCJD caused by prion protein infection from blood might be different to the neurological illness vCJD caused by the infection as a result of eating BSE infected meat. There are examples in the studies of the different prion protein disease where changes in the "strain" of the prion protein occur when the protein has been passed from one person to the next. The recent study suggests that no change in the character of the prion protein occurs as it is passed from one human to the next via blood. These preliminary results are reassuring to some extent but we are still some way from understanding this very complex area.


Dr Angus Kennedy (Consultant Neurologist)

Chairman

CJD Support Network

UK government Ministers link CJD case to blood donor for first time

Fears about the safety of Britain's blood supply were intensified yesterday after ministers disclosed the first case of variant CJD linked with a blood transfusion.


A patient with vCJD who died in the autumn of this year had received blood from a donor in 1996 who went on to develop the disease.


It is the first evidence of what doctors have long feared - that the incurable brain disease passed to humans through consumption of BSE-infected cattle could be transmitted via blood. Animal experiments showed that it was a theoretical possibility but there had been no hard evidence until now.

The donor, who had shown no sign of the infection when he gave the blood, died in 1999. A further 15 people who received blood from donors who went on to develop vCJD are being traced and will be told they may be at risk.


Health professionals will also try to trace several hundred more people who received blood or blood products from pooled donations, such as haemophiliacs. The blood in their cases is diluted with that from other donors, so they are thought to be at lower risk.

In a statement to the Commons, John Reid said that the case was the first in the world of possible transmission from person to person by blood. But it was also possible that both individuals had acquired vCJD separately by eating BSE infected meat.


'The possibility of this being transfusion-related cannot be discounted because it is impossible to be sure which was the route of infection. It is because this is the first report from anywhere in the world of possible transmission of vCJD from person to person via blood that I thought it right to come to the despatch box to inform the house on a precautionary basis.'



Ministers were told of the case last week after post-mortem tests on the brain of the recipient confirmed the presence of vCJD. Mr Reid said he had discussed the issue with Sir Liam Donaldson, the Government's chief medical officer, before yesterday's announcement.



There is no screening test for vCJD but evidence from post-mortem tests has shown that signs of the disease may be present in body tissues such as the tonsils and appendix for several years before symptoms develop, suggesting they could pass on the disease long before they fall ill.

Sir Liam said that there was no way of deciding whether the donor and recipient had acquired vCJD independently or one had infected the other. 'But the conclusion reached by me and my advisers is that blood transmission cannot be ruled out and it must be taken very seriously.'


No details of the donor or recipient of the blood were released yesterday at the family's request. Ministers and health officials are worried about the potential impact of the discovery on the blood service.

Hospitals need 9,000 units of blood a day to carry out surgery, some of it life-saving, and depend on voluntary donors and patients willingly accepting transfusions.



Precautions to ensure the safety of blood have been taken since the late 1990s, because of the theoretical possibility that vCJD could be transmitted in blood, but the new case is expected to lead to extra measures.

Efforts are also to be stepped up to reduce the use of blood in surgery. Sir Liam said Britain's blood service was safer than in many other countries which relied on paid donations.



White cells have been removed from donated blood, believed to be the likeliest carrier of the infectious agent of vCJD, since 1998 in a process known as leucodepletion, and all plasma for the manufacture of blood products has been sourced from outside the UK. But 10 of the 15 people who received blood from donors and later developed vCJD did so before leucodepletion was introduced.



Sir Liam said that 24 million units of blood had been given since 1996, when vCJD was first identified in humans, and there had been only one incident of possible vCJD transmission via blood so far.

Blood transfusions were not free of risks and errors caused an average of 12 deaths a year.

'The risks of a shortage of blood have to be weighed very carefully because we are talking about real life and death situations and not about the margins of science and what we hope is a very rare disease,' he said.



The health department said 23 people who developed vCJD claimed to have been blood donors in the past.



Frances Hall, secretary of the Human BSE Foundation, whose son Peter died from vCJD in 1996, said yesterday's news came as a shock. 'We'll have to hope that leucodepletion is effective,' she said.

How Is Creutzfeldt-Jakob Disease (CJD) Diagnosed? Diagnosing Creutzfeldt-Jakob Disease (CJD)

Only a brain biopsy or an examination of brain tissue after death during autopsy can confirm (diagnose) the presence of Creutzfeldt-Jakob disease (CJD). However, doctors often can make an accurate diagnosis based on the patient's medical and personal history, a neurological exam, and certain diagnostic tests.


A neurologist will carry out the tests to rule out other conditions with similar symptoms, such as Alzheimer's disease, Parkinson's disease or a brain tumor.


The neurological exam is likely to reveal characteristic symptoms as:

muscle twitching and spasms
abnormal reflexes

What Is Creutzfeldt-Jakob Disease (CJD)? What Is Mad Cow Disease?


What Are The Signs And Symptoms Of Creutzfeldt-Jakob Disease (CDJ)? What Are The Complications Of CJD?


What Are The Causes Of Creutzfeldt-Jakob Disease (CJD)?


What Are The Risk Factors Of Creutzfeldt-Jakob Disease (CJD)?


How Is Creutzfeldt-Jakob Disease (CJD) Transmitted? Can CJD Be Transmitted From Person To Person?


How Is Creutzfeldt-Jakob Disease (CJD) Diagnosed? Diagnosing Creutzfeldt-Jakob Disease (CJD)


What Are The Treatment Options For Creutzfeldt-Jakob Disease (CJD)? Prevention Of CJD

coordination problems
people with CJD also may have areas of blindness and changes in visual-spatial perception

Tests to help detect CJD:

Electroencephalogram (EEG). Using electrodes placed on the scalp, this test measures the brain's electrical activity. People with CJD and vCJD show a characteristically abnormal pattern.
Magnetic resonance imaging (MRI) . It uses radio waves and a magnetic field to create cross-sectional images of the head and body. It is especially useful in diagnosing brain disorders.
Spinal fluid tests. Cerebral spinal fluid surrounds and cushions the brain and spinal cord. In a test called a lumbar puncture (also known as a spinal tap) doctors use a needle to withdraw a small amount of this fluid for testing. The presence of a particular protein in spinal fluid is often an indication of CJD or vCJD.
Tonsil biopsy. Scientists have learned that tissue from the tonsils tends to harbor evidence of vCJD. Examination of a sample of tonsil tissue may help diagnose vCJD. However, this method seems less reliable for other forms of CJD.
Genetic test. This is a simple blood test to see if there is a mutation (fault) in the gene that produces normal protein. A positive result may indicate inherited prion disease.
Brain biopsy. It is done under general anesthetic. A surgeon drills a tiny hole into the skull and removes a small piece of brain tissue using a very thin needle.


As a brain biopsy carries the risk of causing brain damage or seizures, it is only performed in a few cases, where there is a concern that the patient does not have CJD but some other treatable condition.


What Is Creutzfeldt-Jakob Disease (CJD)? What Is Mad Cow Disease?
What Are The Signs And Symptoms Of Creutzfeldt-Jakob Disease (CDJ)? What Are The Complications Of CJD?
What Are The Causes Of Creutzfeldt-Jakob Disease (CJD)?
What Are The Risk Factors Of Creutzfeldt-Jakob Disease (CJD)?
How Is Creutzfeldt-Jakob Disease (CJD) Transmitted? Can CJD Be Transmitted From Person To Person?
How Is Creutzfeldt-Jakob Disease (CJD) Diagnosed? Diagnosing Creutzfeldt-Jakob Disease (CJD)
What Are The Treatment Options For Creutzfeldt-Jakob Disease (CJD)? Prevention Of CJD

Written by Stephanie Brunner

Driving Medical Research Via YouTube

YouTube, the online video community that allows people to discover, watch and share originally created videos, has teamed up with scientists at The University of California, San Francisco to tap the video sharing platform to drive medical research.



The move is the latest step by one of the world's leading cadres of neuroscientists to engage the general public and physicians in the fight against neurodegenerative diseases, while helping caregivers cope with these devastating illnesses.



The channel is intended to increase awareness among patients, their families -- and physicians -- about the various forms of dementia, with the goal of promoting earlier diagnoses and getting more patients into research studies and clinical trials. The site is also intended to educate caregivers, and provide support through caregiver testimonials.



To support the effort, the UCSF team is also reaching out with two other forms of online communication. They've created an electronic badge, or "widget," containing links to the YouTube channel and the UCSF Memory and Aging Center web site that will allow people to spread the word about the initiative via email and websites, including disease-education associations. They have also created a Facebook group, "Defeat Dementia."



"The YouTube channel and these other forms of online communications will enable us to engage a broad audience in the fight against these illnesses," says Bruce Miller, MD, director of the UCSF Memory and Aging Center. "All of the dementias - including Alzheimer's disease, frontotemporal dementia, Creutzfelt Jakob disease, Huntington's disease and Parkinson's disease with Lewy Bodies -- share common features. They all are illnesses in which normal proteins are misprocessed.



"One goal is to increase awareness about the earliest signs of some of the less well known diseases, including FTD and CJD. If we can promote accurate diagnoses of patients, we can get them into clinical trials sooner. We believe that early intervention with novel therapies will be key to stalling and halting these diseases."



The channel is the latest outcome of the "Fight for Mike," an initiative by Silicon Valley leaders to save the life of one man - former Apple Computer Inc. and Netscape Computer Corp. marketing wunderkind Michael Homer - that has broadened to a mission to advance scientists' understanding of Homer's rare, fatal illness, Creutzfeldt-Jakob disease. Understanding the disease, the UCSF scientists believe, will accelerate advances against the more common neurodegenerative diseases, including Alzheimer's disease.
















Homer was diagnosed with CJD last spring and is being treated at UCSF. The Fight for Mike is led by two of his best friends, Silicon Valley investor Ron Conway and Intuit chairman William V. Campbell.



The idea to create the video-sharing channel, itself, resulted from a brainstorming session involving UCSF physicians and Silicon Valley entrepreneurs that was hosted last fall by YouTube CEO and co-founder Chad Hurley, a prot?©g?© of Homer's.



"Mike Homer is one of the great people to have helped build Silicon Valley," says Conway. "His extraordinary energy, creativity and passion helped drive the success of major companies and start ups. He also has been a superb father, husband and friend. The Fight for Mike is intended to honor his spirit and drive the medical research underway at UCSF to cure CJD and related diseases. We're hoping the YouTube channel will support this effort."



The Fight for Mike, started last June, so far has raised more than $7 million for CJD research at UCSF, where Stanley B. Prusiner, MD -- who was awarded the Nobel Prize in Physiology or Medicine in 1997 for discovering the protein, known as prion (PREE-on) that causes CJD -- leads a major research enterprise focused on all neurodegenerative diseases.



The funding has already had an impact on several fronts -- including the establishment of stable research funding for Homer's physician, Michael Geschwind, MD, PhD, a premier clinician-researcher on the diagnosis and treatment of prion disorders; the recruitment of Michael Silber, PhD, a former leader in the pharmaceutical industry, to establish a Drug Discovery program; and research strategies to identify new drugs.



The idea for the YouTube channel was an outgrowth of broad thinking.



The Homer family had experienced what others had before them -- the challenges of navigating the medical system to get a diagnosis of a relatively rare neurodegenerative disease and, later, implementing the necessary care-giving strategies in their home. A public forum dedicated to educating the public about all aspects of CJD - and the other neurodegenerative diseases - could address this need.



The decision was made to create a web page dedicated to CJD on the UCSF Memory and Aging Center website. A YouTube channel, the group proposed, could host video for the page as well as well as video on other forms of dementia, including FTD, Huntington's disease and Alzheimer's disease. It could also serve as a portal to drive families and physicians to the UCSF Memory and Aging Center website for further information.



"I hope that the UCSF channel will provide scientists, researchers, and physicians a valuable communication tool as they search for solutions, and patients and their families another way to help cope with these tragic diseases," says YouTube's Hurley. "Mike is an inspirational friend, and I am grateful for the efforts that many have made in the quest to advance medical research of neurodegenerative diseases."



The "Fight for Mike" allocated $200,000 for UCSF to hire a company to create the CJD site on the Memory and Aging Center website. It will serve as a template for the creation of disease-specific site on each form of dementia in the coming years.



The CJD web site includes UCSF physician-researchers and nurses discussing CJD, information for caregivers, testimonials by caregivers, advice for physicians (infection control, interpreting tests, finding services); and the science of proteins and prions (Nobel laureate Prusiner, protein models, scientific talks, lab procedures, etc.) geared to basic and translational scientists.







UCSF is a leading university dedicated to promoting health worldwide through advanced biomedical research, graduate-level education in the life sciences and health professions, and excellence in patient care.



For more information:



UCSF YouTube Channel


UCSF Memory and Aging Center web site


UCSF MAC CJD webpage


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Recipients of blood since 1980 cannot donate blood vCJD risk

UK - Recipients of blood transfusions are to be excluded from donating blood in the future as a further precautionary measure against the possible risk of transmission of variant Creutzfeldt Jakob Disease (vCJD), Health Secretary John Reid announced today.


This follows the government announcement in December last year of the first report of a possible transmission of vCJD from person to person via blood. This remains a possibility and not a proven causal connection.


The new precautionary measure to change the eligibility for blood donation will be implemented from April 5th this year. It will exclude people who confirm they have received a transfusion after 1 January 1980 because it is generally accepted that there will have been no exposure to BSE in the UK before than date.


Health Secretary John Reid said:


'I must stress that the risk attached to this group of blood donors is of course uncertain but we are taking these measures as a precaution, as the risk may be slightly higher than for the population as a whole.


'Excluding these donors will inevitably lead to a reduction in the supply of blood available for transfusions. Whilst the National Blood Service estimates a loss of 52,000 donors, I am pleased to report that they have put in place measures to help conpensate for these losses and hospitals are being encouraged to make best possible use of blood.'


Mr Reid also announced that Chief Medical Officer Sir Liam Donaldson is to draw up a strategy to ensure more appropriate and effective use of blood in the NHS. This strategy will build on the current 'Better Blood Transfusion - More Appropriate Use' initiative, securing further improvments in conserving blood stocks through better use of blood in hospitals.


The Health Secretary said:


'We are following a highly precautionary approach. Although people may have concerns about the implications of this announcement, I would emphasise again that this action is being taken because of an uncertain but slight risk. People should, indeed, continue to have a blood transfusion when it is really necessary. Any slight risk associated with receiving blood must be balanced against the significant risk of not receiving that blood when it is most needed.


'People who can should continue to give blood. Blood donation is a safe procedure and people should continue to donate blood regularly. We place great value on those who already donate and would welcome new donors.'


Notes to editor


In light of the incident reported on December 17 2003, the Chief Medical Officer, Sir Liam Donaldson, asked the expert advisory committee on the Microbiological Safety of Blood and Tissues for Transplantation (MSBT) to consider whether there was a need for any further precautionary measures to be taken. The MSBT recommended that on the basis of evidence available and taking a precautionary approach, the UK should implement the policy to exclude people who have received blood transfusions. CMO advised government to accept this recommendation from MSBT.



Today's announcement sets out the latest in a number of measures that have already been implemented to minimise the possible risk of vCJD being passed through blood:



Since 1997 all cases of vCJD that are reported to the National CJD Surveillance Unit and diagnosed as having 'probable' vCJD, result in a search of the National Blood Service blood donor records. If the patient has given blood, subsequently any stocks of that blood are immediately destroyed.


Since 1998, plasma derivatives, such as clotting factors, have been prepared from plasma imported from the USA.


Since October 1999, white blood cells (which may carry the greatest risk of transmitting vCJD) have been removed from all blood used for transfusion.


In August 2002 we announced that fresh frozen plasma for treating babies and young children born after 1 January 1996 would be obtained from the USA.


In December 2002, the Department of Health completed its purchase of the largest remaining independent US plasma collector, Life Resources Incorporated. This secures long-term supplies of non-UK blood plasma for the benefit of NHS patients.


Contact Media Centre

Phone Press Officer

020 7210 4860/5287/5282

From the UK Department of Health

Research on prion removal technology presented to FDA Advisory Committee

At the request of the Food and Drug Administration (FDA) Transmissible Spongiform Encephalopathies (TSEs) Advisory Committee, Pall Corporation (NYSE: PLL) today presented the latest research results on its prion technology to remove TSE infectivity from blood. The Pall LeukotrapR Affinity Prion Reduction Filter system is the only Council of Europe (CE) marked technology shown to remove prions from red blood cells, the most commonly transfused blood component. Samuel Coker. PhD, Principal Scientist and Technical Director of Pall Medical, who has been spearheading the research effort, gave the Committee a first-hand look at the validation studies on the performance characteristics of the prion reduction technology filter that were used to earn its CE mark in Europe. He also presented new results on quality control measures for blood banks to use upon implementation of prion reduction as a means to prevent transfusion-transmitted variant Creutzfeldt-Jakob Disease (vCJD).


TSEs, also called prion diseases, are fatal neurodegenerative diseases that include vCJD, the human form of bovine spongiform encephalopathy (BSE), or "mad cow disease." The presentation, part of the Advisory Committee's discussion on labeling claims for TSE clearance studies for blood component filters, is a continuation of Pall Corporation's dialogue with U.S. regulatory and health authorities on protecting the blood supply from prions that can cause vCJD.


Studies Validating Filter Performance


Dr. Coker presented research results on the ability of the filter to remove all types of prions from red cells including scrapie (a TSE affecting sheep); mouse infected with human variant CJD (mvCJD) and human sporadic CJD (sCJD). Sporadic CJD is the most common form of CJD affecting people.


He provided results of bioassays to provide a quantitative method to determine log removal of infectious prion in hamsters. (Hamsters are recognized as an optimal animal model for studying prion diseases.) These studies found that the technology was effective in reducing infectious prions in the human sCJD, scrapie and mvCJD models.


"These results demonstrate robust performance of the Leukotrap Affinity Prion Reduction Filter in removing different strains of prions," said Dr. Coker.


Dr. Coker reviewed results of studies on the safety of the product and details on the measurement of the quality of red cells post prion reduction, including survival, maintaining their integrity and therapeutic value (oxygen carrying properties) and other metabolic activities. On all counts measured, the studies found that the safety of processed red cells was not impaired.


Implementing Prion Reduction into Routine Blood Banking Practice


He also presented results of a new study that identified a surrogate marker to measure prion removal in order to enable quality control in the blood banking process. A surrogate marker is required since concentrations of infectious prion in blood are too low to measure using currently available methods. After review of a number of potential markers, the studies validated that reduction of a plasma protein present in blood and blood components directly correlated with reduction of infectious prion.















Dr. Coker explained, "These findings provide a feasible means for quality control for blood banks in their prion reduction process since the plasma protein can easily be measured with currently available analytical methods."


Studies on the efficacy of the filter's removal of prions under different processing conditions, which can vary by blood center and country in their use of anticoagulants, red cell storage solutions, temperatures, etc., were also reviewed. The studies showed about 99.9 percent prion removal across all conditions.


"The data show that the Leukotrap® Affinity Prion Reduction Filter is consistently effective in removing different strains of prions over a broad range of processing conditions," added Dr. Coker.


The Pall Leukotrap® Affinity Prion Reduction Filter system is available in Europe, where the vast majority of vCJD cases have occurred, as a CE marked medical device. The National Blood Service in England and the Irish Blood Transfusion Service are currently evaluating the technology for integration into routine blood processing to help prevent transfusion transmission of infectious prions that can cause vCJD. The Pall prion reduction technology is designed to reduce leukocytes (white blood cells) and prions in a single step.


About Pall Corporation


Pall Corporation is the global leader in the rapidly growing field of filtration, separations and purification. Pall's business is organized around two broad markets: Life Sciences and Industrial. The Company provides leading-edge products to meet the demanding needs of customers in biotechnology, pharmaceutical, transfusion medicine, semiconductor, water purification, aerospace and broad industrial markets. Total revenues for fiscal 2005 were $1.9 billion. The Company headquarters is in East Hills, New York with extensive operations throughout the world. Visit Pall at pall .


Marcia Katz

marcia_katzpall

Pall Corporation

pall

Prion Fingerprints Detected With Glowing Molecule

An effective and sensitive new method for detecting and characterizing prions, the infectious compounds behind diseases like mad cow disease, is now being launched by researchers at Linkoping University in Sweden, among other institutions.


Mad cow disease (BSE), which has caused the death of more than 200,000 cattle and 165 people in the U.K., has now abated. But other prion disorders are on the rise, and there is concern that new strains will infect humans. Prions are not readily transmittable from species to species, but once they have broken through the species barrier they can rapidly adapt and become contagious within the species. Intensive work is now underway to find new, more sensitive methods for detecting these potentially deadly protein structures and distinguish between various strains.


The method now being presented in the journal Nature Methods is based on a fluorescent molecule, a so-called conjugated polymer, which was developed at Linkoping University.


The research team infected genetically identical laboratory mice with BSE, scrapie (which afflicts sheep), and CWD (chronic wasting disease or "mad elk disease," which is epidemic in the central U.S.) for several generations in a row. Gradually new strains of prions emerge, making the diseases more fatal to the mice. Tissue samples from mice were examined using the fluorescent molecule, which seeks out and binds with prions. This is signaled by a shift in color. By tweaking the molecule, the team has been able to get it to show different colors depending on the structure of the prion?­each prion strain emits its own optical fingerprint.


This is an important difference compared with other techniques used to find prions, such as antibodies and the well-known stain Congo red.


The technique has also proven to work well on tissue sections from dead animals, such as cows infected with BSE. Now the scientists want to move on and look for alternative sample-taking methods for diagnosing and tracking prion diseases in humans in early stages.


The method would then be useful for screening blood products, since there is a risk that people can be carriers of prions without having any symptoms of disease. In the U.K. it was discovered that 66 people had received blood from blood donors who were infected with the human form of BSE (a variant of Creutzfeldt-Jakob's disease, vCJD), and among them, four individuals have been shown to be infected (source: Health Protection Agency, Jan. 2007).


"Using our methods, we can directly see the structure of the prions and thereby deduce the disease," says Peter Nilsson, one of the lead authors of the article. Nilsson developed the technique as a doctoral student at Link?¶ping University and now, as a post-doctoral fellow with Professor Adrian Aguzzi's research team in Z??rich, has been applying the technology to prion diseases. After New Year's he will assume a post-doc position at Link?¶ping.


"For us researchers it is truly exciting to use this technique to understand more about both prions and other defectively folded proteins that give rise to similar disorders, such as Alzheimer's," says Peter Hammarstr?¶m, co-author and research director of the prion laboratory at Link?¶ping.


Another co-author is Kurt W??thrich, the 2002 Nobel laureate in chemistry.


The article "Prion strain discrimination using luminescent conjugated polymers" by Christina J Sigurdson, K Peter R Nilsson, Simone Hornemann, Guiseppe Manco, Magdalini Polymenidou, Petra Schwartz, Mario Leclerc, Per Hammarstr?¶m, Kurt W??thrich, and Adriano Aguzzi was published in Nature Methods online on November 18 and will appear in the December issue of the printed journal.


VETENSKAPSRADET (THE SWEDISH RESEARCH COUNCIL)

Regeringstgatan 56

103 78 Stockholm

vr.se

FSA Board to consider advice to Ministers on BSE testing system, UK

A Food Standards Agency Board meeting will take place in public on 15 August 2005 to consider what advice the Agency will give to Ministers on a BSE testing system, trialled earlier this year as part of a managed transition towards replacing the Over Thirty Months Rule.



The Over Thirty Months (OTM) Rule is the BSE control set up in 1996 that automatically bans older cattle from entering the food chain. The principal food safety measure - the Specified Risk Material (SRM) controls, which removes over 99% of any infectivity in cattle - will remain in place, along with the Feed Ban control.


In July last year the Agency advised Ministers that the OTM Rule was no longer proportionate to the risk, but should not change until an independent group had advised that a robust BSE testing system was in place. The Government accepted the advice in December and asked the Agency to advise on a robust system prior to rule change. As a result, an Independent Group, chaired by Professor Patrick Wall, was set up by the FSA to oversee and advise on testing….
CONTINUES …. THE FOOD STANDARDS AGENCY, UK

Monthly Creutzfeldt Jakob (vCJD) disease statistics, UK

The UK 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: 1st of August


Summary of vCJD cases


Deaths


Deaths from definite vCJD (confirmed): 107


Deaths from probable vCJD (without neuropathological confirmation): 42


Deaths from probable vCJD (neuropathological confirmation pending): 1


Number of deaths from definite or probable vCJD (as above): 150


Alive


Number of probable vCJD cases still alive: 7


Total number of definite or probable vCJD (dead and alive): 157
The next table will be published on Monday 1st of September 2005


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).


Related links

Download CJD Statistics 01/08/2005 (PDF, 12K)


UK Dept of Health

Prevalence Of Variant CJD Agent In Britain Remains Uncertain

First results from a large tissue survey in Britain of the agent that causes variant Creutzfeldt-Jakob disease (vCJD) are unable so far to establish that the prevalence is lower than that given by previous estimates, concludes a study published on bmj today.


Although the risk of dietary exposure to the agent that causes vCJD has been virtually eliminated, uncertainty remains about how many people carry the infectious agent and will eventually develop the disease.


Calculations based on cases of vCJD to 2004 predicted between 10 and 190 further clinical cases over the next few decades. However, a study of appendix and tonsil tissues predicted a much higher level of cases - between 520 and 13,000.


To resolve this discrepancy, and ensure that proportionate public health measures are implemented, a research team led by Jonathan Clewley and Noel Gill at the Health Protection Agency in London, set out to establish a more accurate prevalence of disease-associated prion protein in the population of Britain.


They tested 63,007 anonymous tonsil samples from people of all ages who had had their tonsils removed between 2004 and 2008 in hospitals throughout England and Wales.


Of these, 12,753 were from people born between 1961 and 1985 (the group in which most vCJD cases have arisen) and 19,908 were from people born between 1986 and 1995 who would have also been exposed to meat or meat products contaminated with the agent that causes bovine spongiform encephalopathy (BSE) in cattle.


Samples were screened for the presence of the prion protein (PrPCJD) - known to accumulate to relatively high levels in the tonsils of people with vCJD. Two sensitive immunological tests were used and any sample that showed reactivity underwent more comprehensive and specific testing to confirm whether or not the protein was present.


None of the samples was positive for the presence of the protein, suggesting that the prevalence of vCJD carriers in Britain may be lower than that given by previous estimates, with an upper limit of 289 per million in people born between 1961 and 1985, say the authors. This result is, however, still consistent with the earlier study of appendix tissue that showed a prevalence of 292 per million, they add.


They call for more data through continuing to archive and test tonsils, and other anonymous tissue samples, from people born before 1996. This will help guide a proportionate public health response to limit the threat of healthcare associated transmission of vCJD, they conclude.


An accompanying editorial argues that the chance that no one in the UK is incubating the disease is unlikely, but that the limited number of cases and diagnostic problems make accurate measurement difficult.


Maurizio Pocchiari, a neurology expert based in Rome, suggests that repeating surveys of prion protein in tissue specimens may not yield further information unless a more sensitive and specific test is developed. Therefore, the precautionary measures already in place must be maintained to avoid transmission of vCJD between humans and surveillance of disease in the UK and in the rest of Europe should remain alive.
He also says that public health authorities in other countries should not carry out such studies, as an enormous number of samples would be needed to yield useful information because exposure to the BSE agent in the rest of the world is probably much lower than in the UK.



Link to Paper

Link to Editorial


Source

British Medical Journal

Monthly creutzfeldt jakob disease (CJD) statistics in UK

The UK 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 results can be seen by clicking on the link below.


Summary of vCJD cases


Deaths


Deaths from definite vCJD (confirmed): 104


Deaths from probable vCJD (without neuropathological confirmation): 37


Deaths from probable vCJD (neuropathological confirmation pending): 0


Number of deaths from definite or probable vCJD (as above): 141


Alive


Number of definite/probable vCJD cases still alive: 5


Total number of definite or probable vCJD (dead and alive): 146


The next table will be published on Monday 7th June 2004


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).


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
v
****spongiform change and extensive PrP deposition with florid plaques, throughout the cerebrum and cerebellum.


Related links

Download Monthly Stats (PDF, 3K)

dh.uk/assetRoot/04/08/14/35/04081435.pdf


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