U.S. News Man, 88, kills wife in Calif. nursing home
Published: Nov. 22, 2010 at 9:57 AM
SEAL BEACH, Calif., Nov. 22 (UPI) -- A man who had long cared for his ailing wife, whose "mind was gone," ended 70 years of marriage by shooting her in a nursing home, their daughter said.
Roy Charles Laird, 88, killed his wife Clara, 86, Sunday with a single gunshot to the head at the Country Villa Health Care Center in Seal Beach, police told the Los Angeles Times.
"It was a mercy killing," said their daughter, Kathy Palmateer, 68. "Her mind was gone."
As Clara Laird declined steadily from dementia over five years, her husband cared for her himself at home until three months ago. He would visit her at the nursing home three times a day, spoon-feeding her at each meal, friends and family said.
Around noon Sunday, a single gunshot was heard at the nursing home. Within minutes, police surrounded it and called in backup.
"We didn't know if we had a shooter or not," said Seal Beach police Sgt. Steve Bowles.
Police found Clara Laird dead in her bed and her husband in the hallway. He followed them into her room, took a .38-caliber revolver out of his pocket and calmly set it on a table, Bowles said.
Laird was arrested on suspicion of murder.
http://www.upi.com/Top_News/US/2010/11/22/Man-88-kills-wife-in-Calif-nursing-home/UPI-65541290437876/
God bless his soul, and may they both rest in peace. he will not last long now, because his soul died too. Alzheimer's, Prion disease, Dementia, and all neurological disease are truly a nightmare. If you have never witnessed the demise and death of a person from this, long term, or short, then you really should not persecute this man.
AS a layperson, and as someone that has witnessed the demise and death of a close loved one to hvCJD and Alzheimer's, it is a cruel, brutal death, and the loved ones and friends that care for them, a part of them die as well, and you never forget.
I do not condone, or approve of this man choice to end the brutal clutches of Alzheimer's that had a hold of his wife, but i have been there before. ...
BSE101/1 0136
IN CONFIDENCE
CMO
From: Dr J S Metters DCMO
4 November 1992
TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES
http://collections.europarchive.org/tna/20081106170650/http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf
CJD1/9 0185
Ref: 1M51A
IN STRICT CONFIDENCE
From: Dr. A Wight
Date: 5 January 1993
Copies:
Dr Metters
Dr Skinner
Dr Pickles
Dr Morris
Mr Murray
TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES
http://collections.europarchive.org/tna/20080102191246/http://www.bseinquiry.gov.uk/files/yb/1993/01/05004001.pdf
Friday, September 3, 2010
Alzheimer's, Autism, Amyotrophic Lateral Sclerosis, Parkinson's, Prionoids, Prionpathy, Prionopathy, TSE
http://betaamyloidcjd.blogspot.com/2010/09/alzheimers-autism-amyotrophic-lateral.html
http://betaamyloidcjd.blogspot.com/
2010 PRION UPDATE
Thursday, August 12, 2010
Seven main threats for the future linked to prions
http://prionpathy.blogspot.com/2010/08/seven-main-threats-for-future-linked-to.html
http://prionpathy.blogspot.com/
TSS
Friday, October 22, 2010
Peripherally Applied Aß-Containing Inoculates Induce Cerebral ß-Amyloidosis
http://betaamyloidcjd.blogspot.com/2010/10/peripherally-applied-containing.html
Saturday, March 22, 2008
10 Million Baby Boomers to have Alzheimer's in the coming decades
http://betaamyloidcjd.blogspot.com/2008/03/10-million-baby-boomers-to-have.html
see full text Alzheimer's and CJD i.e. TSE, aka mad cow disease
http://betaamyloidcjd.blogspot.com/
Thursday, November 18, 2010
UNITED STATES OF AMERICA VS GALEN J. NIEHUES FAKED MAD COW FEED TEST ON 92 BSE INSPECTION REPORTS FOR APPROXIMATELY 100 CATTLE OPERATIONS
http://bse-atypical.blogspot.com/2010/11/united-states-of-america-vs-galen-j.html
Tuesday, November 02, 2010
IN CONFIDENCE
The information contained herein should not be disseminated further except on the basis of "NEED TO KNOW".
BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992
http://bse-atypical.blogspot.com/2010/11/bse-atypical-lesion-distribution-rbse.html
with saddest regards, terry
Showing posts with label Dementia. Show all posts
Showing posts with label Dementia. Show all posts
Monday, November 22, 2010
Friday, May 7, 2010
Spouses of Dementia Sufferers Have a Six-Fold Increased Risk of Dementia Onset
Spouses of Dementia Sufferers Have a Six-Fold Increased Risk of Dementia Onset:
Husbands Appear at Higher Risk Than Wives
April 30th, 2010
Strictly Embargoed Until 00.01 Hours (EST) Wednesday, May 5, 2010
Contact: Jennifer Beal +44 (0) 1243 770633 Medicalnews@wiley.com
Spouses of Dementia Sufferers Have a Six-Fold Increased Risk of Dementia Onset
Husbands Appear at Higher Risk Than Wives Older married adults whose spouse has dementia are at significantly higher risk for developing dementia themselves, compared to similar older married adults whose spouse never develops dementia. This is the key finding of a study published today in the Journal of the American Geriatrics Society.
Informal dementia caregiving for a spouse is a natural marital obligation, and spousal caregivers often report positive feelings toward caregiving, yet it is difficult, requiring time, energy and usually physical exertion. Dementia caregivers have been shown to provide more assistance, and to report more personal sacrifices and stress, than those who care for physically-impaired elderly without dementia. While there are many published studies showing that dementia caregivers are at higher risk for health problems and depression, none have examined risk for dementia in the caregiver.
2,442 subjects (1,221 married couples) aged 65 and older from Northern Utah, USA, without dementia at onset were studied for up to 12 years to monitor for onset of dementia in husbands, wives or both. During this time, 125 cases of dementia only in the husband were diagnosed, 70 only in the wife, and 30 where both spouses were diagnosed (60 people).
The researchers, led by Dr. Maria Norton of Utah State University, USA, adjusted for socioeconomic status, a significant predictor of many health-related outcomes including dementia to control for shared environmental exposures that might influence risk for dementia in both spouses.
The results showed that incident dementia was significantly associated with older age, and having a spouse with dementia. Participants with a spouse who developed dementia were at a six times increased risk of developing dementia, net of the effect of age, gender, APOE genotype, and socioeconomic status, with higher risk in men (11.9) than women (3.7).
“Future studies are needed to determine how much of this association is due to caregiver stress compared to a shared environment,” said Norton. “On the positive side, the majority of these individuals, with spouses who develop dementia, did not themselves develop dementia, therefore more research is needed to explore which factors distinguish those who are more vulnerable.”
“Given the significant public health concern of Alzheimer’s disease and other dementias, and the upcoming shift in population age composition, continued research into the causes of dementia is urgent,” concluded Norton. __________________________________________________________________
This study is published in the issue of the Journal of the American Geriatrics Society. Media wishing to receive a PDF of this article may contact medicalnews@wiley.com
Full citation: Norton et al; Increased Risk of Dementia When Spouse Has Dementia? The Cache County Study; The Journal of the American Geriatrics Society, May 2010
About the Author: Dr. Maria Norton is based at Utah State University, USA. To arrange an interview with Dr. Norton, please contact Tim Vitale at Utah State University’s Public Relations office on tim.vitale@usu.edu or +1 435-797-1356.
About the Journal: The Journal of the American Geriatrics Society is a comprehensive and reliable source of monthly research and information about common diseases and disorders of older adults. The journal is published by Wiley-Blackwell on behalf of the American Geriatrics Society. For more information, please visit www.blackwellpublishing.com/jgs.
About Wiley-Blackwell: Wiley-Blackwell is the international scientific, technical, medical, and scholarly publishing business of John Wiley & Sons, with strengths in every major academic and professional field and partnerships with many of the world’s leading societies. Wiley-Blackwell publishes nearly 1,500 peer-reviewed journals and 1,500+ new books annually in print and online, as well as databases, major reference works and laboratory protocols. For more information, please visit www.wileyblackwell.com or www.interscience.wiley.com.
Modified On: May 5th, 2010
http://www.americangeriatrics.org/press/id:665
Tuesday, August 26, 2008
Alzheimer's Transmission of AA-amyloidosis: Similarities with Prion Disorders NEUROPRION 2007 FC4.3
http://betaamyloidcjd.blogspot.com/2008/08/alzheimers-transmission-of-aa.html
http://betaamyloidcjd.blogspot.com/2010/03/neurobiology-of-disease-molecular-cross.html
http://betaamyloidcjd.blogspot.com/2010/04/food-combination-and-alzheimer-disease.html
Wednesday, April 14, 2010
Food Combination and Alzheimer Disease Risk A Protective Diet
http://betaamyloidcjd.blogspot.com/2010/04/food-combination-and-alzheimer-disease.html
TSE & HOUNDS
GAH WELLS (very important statement here...TSS)
HOUND STUDY
AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.
http://web.archive.org/web/20010305222642/www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
76 pages on hound study;
http://web.archive.org/web/20030327022236/http://www.bseinquiry.gov.uk/files/sc/seac16/tab04.pdf
I thought that in Britain dogs had contracted BSE, but perhaps not.
not so fast here;
The spongiform changes were not pathognomonic (ie. conclusive proof) for prion disease, as they were atypical, being largely present in white matter rather than grey matter in the brain and spinal cord. However, Tony Scott, then head of electron microscopy work on TSEs, had no doubt that these SAFs were genuine and that these hounds therefore must have had a scrapie-like disease. I reviewed all the sections myself (original notes appended) and although the pathology was not typical, I could not exclude the possibility that this was a scrapie-like disorder, as white matter vacuolation is seen in TSEs and Wallerian degeneration was also present in the white matter of the hounds, another feature of scrapie.
38.I reviewed the literature on hound neuropathology, and discovered that micrographs and descriptive neuropathology from papers on 'hound ataxia' mirrored those in material from Robert Higgins' hound survey. Dr Tony Palmer (Cambridge) had done much of this work, and I obtained original sections from hound ataxia cases from him. This enabled me provisionally to conclude that Robert Higgins had in all probability detected hound ataxia, but also that hound ataxia itself was possibly a TSE. Gerald Wells confirmed in 'blind' examination of single restricted microscopic fields that there was no distinction between the white matter vacuolation present in BSE and scrapie cases, and that occurring in hound ataxia and the hound survey cases.
39.Hound ataxia had reportedly been occurring since the 1930's, and a known risk factor for its development was the feeding to hounds of downer cows, and particularly bovine offal. Circumstantial evidence suggests that bovine offal may also be causal in FSE, and TME in mink. Despite the inconclusive nature of the neuropathology, it was clearly evident that this putative canine spongiform encephalopathy merited further investigation.
40.The inconclusive results in hounds were never confirmed, nor was the link with hound ataxia pursued. I telephoned Robert Higgins six years after he first sent the slides to CVL. I was informed that despite his submitting a yearly report to the CVO including the suggestion that the hound work be continued, no further work had been done since 1991. This was surprising, to say the very least.
41.The hound work could have provided valuable evidence that a scrapie-like agent may have been present in cattle offal long before the BSE epidemic was recognised. The MAFF hound survey remains unpublished.
Histopathological support to various other published MAFF experiments
42.These included neuropathological examination of material from experiments studying the attempted transmission of BSE to chickens and pigs (CVL 1991) and to mice (RVC 1994).
http://www.bseinquiry.gov.uk/witness/htm/stat067.htm
It was thought likely that at least some, and probably all, of the cases in zoo animals were caused by the BSE agent. Strong support for this hypothesis came from the findings of Bruce and others (1994) ( Bruce, M.E., Chree, A., McConnell, I., Foster, J., Pearson, G. & Fraser, H. (1994) Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and species barrier. Philosophical Transactions of the Royal Society B 343, 405-411: J/PTRSL/343/405 ), who demonstrated that the pattern of variation in incubation period and lesion profile in six strains of mice inoculated with brain homogenates from an affected kudu and the nyala, was similar to that seen when this panel of mouse strains was inoculated with brain from cattle with BSE. The affected zoo bovids were all from herds that were exposed to feeds that were likely to have contained contaminated ruminant-derived protein and the zoo felids had been exposed, if only occasionally in some cases, to tissues from cattle unfit for human consumption.
snip...
NEW URL ;
http://collections.europarchive.org/tna/20080102174910/http://www.bseinquiry.gov.uk/files/ws/s324.pdf
SEE LETTER FROM MAFF TO ME IN 2005 ABOUT THE HOUND STUDY ;
2005
DEFRA Department for Environment, Food & Rural Affairs
Area 307, London, SW1P 4PQ Telephone: 0207 904 6000 Direct line: 0207 904 6287 E-mail: h.mcdonagh.defra.gsi.gov.uk
GTN: FAX:
Mr T S Singeltary P.O. Box 42 Bacliff Texas USA 77518
21 November 2001
Dear Mr Singeltary
TSE IN HOUNDS
Thank you for e-mail regarding the hounds survey. I am sorry for the long delay in responding.
As you note, the hound survey remains unpublished. However the Spongiform Encephalopathy Advisory Committee (SEAC), the UK Government's independent Advisory Committee on all aspects related to BSE-like disease, gave the hound study detailed consideration at their meeting in January 1994. As a summary of this meeting published in the BSE inquiry noted, the Committee were clearly concerned about the work that had been carried out, concluding that there had clearly been problems with it, particularly the control on the histology, and that it was more or less inconclusive. However was agreed that there should be a re-evaluation of the pathological material in the study.
Later, at their meeting in June 95, The Committee re-evaluated the hound study to see if any useful results could be gained from it. The Chairman concluded that there were varying opinions within the Committee on further work. It did not suggest any further transmission studies and thought that the lack of clinical data was a major weakness.
Overall, it is clear that SEAC had major concerns about the survey as conducted. As a result it is likely that the authors felt that it would not stand up to r~eer review and hence it was never published. As noted above, and in the detailed minutes of the SEAC meeting in June 95, SEAC considered whether additional work should be performed to examine dogs for evidence of TSE infection. Although the Committee had mixed views about the merits of conducting further work, the Chairman noted that when the Southwood Committee made their recommendation to complete an assessment of possible spongiform disease in dogs, no TSEs had been identified in other species and hence dogs were perceived as a high risk population and worthy of study. However subsequent to the original recommendation, made in 1990, a number of other species had been identified with TSE ( e.g. cats) so a study in hounds was less
critical. For more details see- http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf
As this study remains unpublished, my understanding is that the ownership of the data essentially remains with the original researchers. Thus unfortunately, I am unable to help with your request to supply information on the hound survey directly. My only suggestion is that you contact one of the researchers originally involved in the project, such as Gerald Wells. He can be contacted at the following address.
Dr Gerald Wells, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey, KT 15 3NB, UK
You may also wish to be aware that since November 1994 all suspected cases of spongiform encephalopathy in animals and poultry were made notifiable. Hence since that date there has been a requirement for vets to report any suspect SE in dogs for further investigation. To date there has never been positive identification of a TSE in a dog.
I hope this is helpful
Yours sincerely 4
HUGH MCDONAGH BSE CORRESPONDENCE SECTION
======================================END...TSS
WHAT ABOUT THOSE STUMBLING, STAGGERING, AND BLIND DOGS, the old dog syndrome, just another spontaneous event ???
The signs of canine cognitive dysfunction syndrome or "old dog syndrome" commonly seen in dogs are: lose of house training increased barking or whining increased anxiety or fear signs disorientation-appearing lost or confused,getting stuck behind furniture or in corners, walking in circles, becoming forgetful,walking aimlessly,staring into space, repetitious or compulsive behavior change in sleep patterns-up at night, sleep all day
lack of responsiveness other changes,may not recognize you, their name,may become more docile, more aggressive.. You can liken it to human senility. An article at the petcenter says "CDS is not "normal aging". A number of pathophysiological changes are suspected to play a role in its development. These include: * deposition of amyloid plaques in the cerebral cortex and hippocampal part of the brain * alterations in neurotransmitters, including dopamine * increased levels of monoamine oxidase B (MAOB) in the brain * increased levels of free radicals L-DEPRENYL HYDROCHLORIDE SELEGILINE HYDROCHLORIDE,BRAND NAME: ANIPRYL OR ELDEPRYL is used to help treat canine cognitive dysfunction by increasing brain concentrations of the neurotransmitter dopamine. Hopefully you can see a difference in a month or so. If you don't see a difference in the first month, your vet might tell you to try two pills a day for the next month. ANIPRYL doesn't work for all dogs. A great writeup on L-DEPRENYL can be found at
http://www.petsinfo.org/elderlydogs1.html
"One third of canine CD patients respond extremely well to treatment with deprenyl by regaining their youthful vigor; another one third respond reasonably well; and one third do not respond at all (perhaps there is a variant of CD with different neuropathology). The bottom line is that for any dog that is slowing down to the point that problems become apparent, treatment with deprenyl is the logical route once other organic causes for reduced mental function have been ruled out. Here is a write up on selegline " Selegiline has immune-system-boosting and anti-neurodegenerative effects. ....
Taken consistently in low doses, selegiline tends to extend the life-expectancy of rats by some 20%; enhances drive, libido and endurance; and independently improves cognitive performance in Alzheimer's patients and in some healthy normals. It is used successfully to treat canine cognitive dysfunction syndrome (CDS) in dogs...Selegiline protects the brain's dopamine cells from oxidative stress. " Some also use alpha lipoic acid and r-lipoic acid. powerful antioxidants to help slow down canine cognitive dysfunction. There is a dog food that is rich in antioxidants for CDS but I am assuming if you supplement with your own antioxidants you don't have to worry if your dog likes the food or not. I know my dog Hammy has become very picky and at least if I pill him, I know he is getting his antioxidants.
http://www.thensome.com/cds.htm
Doggie Dementia
Does 14-year-old Fido get lost in his own back yard?
Does he not respond when you call his name?
Does he generally seem confused?
According to Pulse, the official magazine of the Southern California Veterinary Medical Association, just as humans in the 21st Century are living longer, so is man’s best friends—more than 7.3 million dogs in the United States are age 10 or older. And with age dogs become prone to the same age-related diseases as their human companions, including dementia.
A disease of old age affects dogs and humans alike
Kazzy, a 17-year-old Lhasa Apso, is one of the 60 percent of dogs aged 11 to 15 who suffer from one or more symptoms of canine cognitive dysfunction syndrome (CDS), also known to veterinarians as doggie dementia. "He used to be the most incredible watchdog," says his owner, Olivia Feldman-Rich. "But he’s not like that anymore. He’s quite bewildered."
Experts like Dr. Maritza Perez, a veterinarian at West Orange (NJ) Animal Hospital, say that confusion is one of the four major signs of CDS (see sidebar). Dr. Perez says dogs may "pace around in circles, get stuck behind furniture, or they don’t know where the back door is anymore."
Often the most distressing sign of CDS is that, like human patients with Alzheimer’s disease, your pet seems to forget you and your family. "A lot of people notice that when you walk in the door, and this dog that was happy to see you doesn’t get up off the couch or off the floor to greet you," says Dr. Perez. "And he doesn’t come anymore when you call him."
These symptoms, coupled with others debilitating diseases affecting older dogs, such as arthritis, all add up to a serious loss in quality of life for your canine friend. The American Veterinary Medical Association reports that some 500,000 dogs are put to sleep each year because of CDS.
Researchers say that deposits of beta-amyloid plaques in brain tissues are likely to play a role in CDS. These plaques build up and eventually inhibit transmission of the brain’s neural signals. Still, the recognized symptoms of CDS are behavioral, so a diagnosis is exclusionary, meaning it is arrived at only after all other physical and neurological causes are ruled out.
No cure yet, but relief for some dogs
Dr. Perez with a 14 year-old beagle who is on Anipryl.
While scientists search for a permanent cure for CDS, there is one treatment currently FDA-approved for CDS. Selegiline hydrochloride, whose brand name is Anipryl, may give some dogs relief from its symptoms. Researchers speculate that Anipryl works by increasing levels of dopamine, a neurotransmitter. Other treatments are currently being investigated, including diets high in anti-oxidants as well as a new drug, Adrafinil, in one Canadian study.
Dr. Perez says that Anipryl does cause an improvement in many dogs with CDS, meaning relief from at least one of the common symptoms. "We have lots of animals on it and it does work," she says. But it’s not a sure thing—Dr. Perez tried it on her own dog with no effect.
Feldman-Rich is debating putting Kazzy on Anipryl. "I’m hoping that it will give a little more balance to his life and make him a little more aware that he’s still here and we’re still here for him," she says. "I always told him that he couldn’t leave me too soon, and he’s definitely kept up his end of it, but I’d definitely like for him to feel a little more like he’s part of the family."
by Debra Utacia Krol
http://www.sciencentral.com/articles/view.php3?article_id=218391360&cat=1_6
SEE CJD IN MAN AND HIS CAT ;
[Image] Research letters Volume 352, Number 9134 [Image] 3 October 1998 [Previous] [Next]
[Image][Image]
Simultaneous occurrence of spongiform encephalopathy in a man and his cat in Italy
[Image]
Gianluigi Zanusso, Ettore Nardelli, Anna Rosati, GianMaria Fabrizi, Sergio Ferrari, Antonella Carteri, Franco De Simone, Nicola Rizzuto, Salvatore Monaco
Transmissible spongiform encephalopathies (TSE) encompass inherited, acquired, and sporadic mammalian neurological disorders, and are characterised by the conversion of the cellular prion protein (PrP) in an insoluble and protease-resistant isoform (PrPres). In human TSE, four types of PrPres have been identified according to size and glycoform ratios, which may represent different prion strains. Type-1 and type-2 PrPres are associated with sporadic Creutzfeldt-Jakob disease (CJD), type 3 with iatrogenic CJD, and type 4 with variant CJD.1,2 There is evidence that variant CJD is caused by the bovine spongiform encephalopathy (BSE)-prion strain.2-4 The BSE strain has been identified in three cats with feline spongiform encephalopathy (FSE), a prion disease which appeared in 1990 in the UK.5 We report the simultaneous occurrence of sporadic CJD in a man and a new variety of FSE in his cat.
A 60-year-old man, with no unusual dietary habits, was admitted in November, 1993, because of dysarthria, cerebellar ataxic gait, visual agnosia, and myoclonus. An electroencephalogram (EEG) showed diffuse theta-delta activity. A brain magnetic resonance imaging scan was unremarkable. 10 days later, he was speechless and able to follow only simple commands. Repeat EEGs showed periodic triphasic complexes. 2 weeks after admission, he was mute, akinetic, and unable to swallow. He died in early January, 1994.
His 7-year-old, neutered, female shorthaired cat presented in November, 1993, with episodes of frenzy, twitching of its body, and hyperaesthesia. The cat was usually fed on canned food and slept on its owner's bed. No bites from the cat were recalled. In the next few days, the cat became ataxic, with hindquarter locomotor dysfunction; the ataxia got worse and there was diffuse myoclonus. The cat was killed in mid-January, 1994.
No pathogenic mutations in the patient's PrP gene were found. The patient and the cat were methionine homozygous at codon 129. Histology of the patient's brain showed neocortical and cerebellar neuronal loss, astrocytosis, and spongiosis (figure A). PrP immunoreactivity showed a punctate pattern and paralleled spongiform changes (figure B). The cat's brain showed mild and focal spongiosis in deeper cortical layers of all four lobes (figure C), vacuolated cortical neurons (figure D), and mild astrogliosis. The cerebellar cortex and the dentate nucleus were gliosed. Immunoreactive PrP showed a punctate pattern in neocortex, allocortex, and caudate nucleus (figure E). Western blot analysis of control and affected human and cat brain homogenates showed 3 PrP bands of 27-35 kDa. After digestion with proteinase K and deglycosylation, only samples from the affected patient and cat showed type-1 PrPres, with PrP glycoform ratios comparable to those observed in sporadic CJD1 (details available from author).
[Image]
Microscopic sections of patient and cat brains
A: Occipital cortex of the patient showing moderate spongiform degeneration and neuronal loss (haematoxylin and eosin) and B: punctate perineuronal pattern of PrP immunoreactivity; peroxidase immunohistochemistry with monoclonal antibody 3F4. C: cat parietal cortex showing mild spongiform degeneration (haematoxylin and eosin).D: vacuolated neurons (arrow, haematoxylin and eosin), E: peroxidase immunohistochemistry with antibody 3F4 shows punctate perineuronal deposition of PrP in temporal cortex.
This study shows a spatio-temporal association between human and feline prion diseases. The clinical features of the cat were different from previously reported cases of FSE which were characterised by gradual onset of behavioural changes preceding locomotor dysfunction and ataxia.5 Neuropathological changes were also at variance with the diffuse spongiosis and vacuolation of brainstem neurons, seen in FSE.5 The synaptic pattern of PrP deposition, similar in the cat and in the patient, was atypical for a BSE-related condition. Evidence of a new type of FSE was further provided by the detection of a type-1 PrPres, other than the BSE-associated type 4.2 Taken together, our data suggest that the same agent strain of sporadic CJD was involved in the patient and in his cat.
It is unknown whether these TSE occurred as the result of horizontal transmission in either direction, infection from an unknown common source, or the chance occurrence of two sporadic forms.
1 Parchi P, Castellani R, Capellari S, et al. Molecular basis of phenotypic variablity in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39: 767-78 [PubMed].
2 Collinge J, Sidle KCL, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996; 383: 685-90 [PubMed].
3 Bruce ME, Will RG, Ironside JW, et al. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 1997; 389: 498-501 [PubMed].
4 Hill AF, Desbruslais M, Joiner S, et al. The same prion strain causes vCJD and BSE. Nature 1997; 389: 448-50 [PubMed].
5 Pearson GR, Wyatt JM, Henderson JP, Gruffydd-Jones TJ. Feline spongiform encephalopathy: a review. Vet Annual 1993; 33: 1-10.
------------------------------------------------------------------------ Sezione di Neurologie Clinica, Dipartimento di Scienze Neurologiche e della Visione, Università di Verona, Policlinico Borgo Roma, 37134 Verona, Italy (S Monaco; e mail rizzuto@Gorgorna.univr.it); and Istituto Zooprofilattico Sperimentale della Lombardia e dell' Emilia, Brescia
=======================================
Terry S. Singeltary Sr. wrote:
######## Bovine Spongiform Encephalopathy #########
Greetings list members,
ODD that some FELINE in Italy seem to have this same or maybe very similar phenotype of TSE;
In October 1998 the simultaneous occurrence of spongiform encephalopathy in a man and his pet cat was reported. The report from Italy noted that the cat did not display the same clinical features as FSE cases previously seen. Indeed, the presence of a new type of FSE was suggested. The man was diagnosed as having sporadic CJD, and neither case (man nor cat) appeared to be affected by a BSE-related condition.
http://www.defra.gov.uk/animalh/bse/bse-science/level-4-othertses.html
-------- Original Message --------
Subject: FDA BSE Update - Pet Food from Canadian Manufacturer & MAD DOG DATA
Date: Tue, 27 May 2003 08:07:58 -0500
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy
Statement
FOR IMMEDIATE RELEASE Statement May 26, 2003
Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA
FDA BSE Update - Pet Food from Canadian Manufacturer
The Food and Drug Administration (FDA) has learned from the government of Canada that rendered material from a Canadian cow that last week tested positive for bovine spongiform encephalopathy (BSE, also known as mad cow disease ) may have been used to manufacture pet food, specifically dry dog food, some of which was reported to have been shipped to the United States. The Canadian government prevented the BSE positive cow from being processed for human food. Therefore, consumers can be assured that their food does not contain any remnants of the BSE positive cow.
It is also important to stress that there is no scientific evidence to date that dogs can contract BSE or any similar disease. In addition there is no evidence that dogs can transmit the disease to humans.
FDA notified the U.S. pet food firm, The Pet Pantry International, of Carson City, Nevada, when FDA learned that the pet food that the firm received may have included rendered material from the BSE positive cow. The manufacturer of the pet food is Champion Pet Food, Morinville, Alberta. Even though there is no known risk to dogs from eating this dog food, as a prudent measure to help assure that the U.S. stays BSE free The Pet Pantry International is asking its customers who may have purchased the suspect product to hold it for pickup by the distributor so that the dog food will not mistakenly be mixed into cattle or other feeds if any of the dog food is discarded or otherwise not used to feed dogs. The suspect dog food was produced by Champion Pet Food between February 4, 2003, and March 12, 2003.
The Pet Pantry products were packaged in 50 lb bags, distributed to franchises around the country, and sold by home delivery only. There was no retail distribution of the product. Consumers purchase Pet Pantry products by phone or email orders. The product is then delivered by the nearest franchisee directly to the consumer s home.
The product subject to this notification includes Maintenance Diet labeled with a use by date of 17FEB04 and Beef with Barley with a use by date of 05MAR04 . Consumers who have purchased dog food from The Pet Pantry since February of this year are asked to check their present supplies and see if any match the description of the product being removed. If so, consumers are asked to contact The Pet Pantry at 1-800-381-7387 for further information on how to return the product to The Pet Pantry for proper disposal. Consumers are asked not to destroy or discard the product themselves. The Pet Pantry will also use its sales records to contact consumers who purchased the affected product.
FDA is working closely with the Pet Pantry International to assure for proper disposal of the recovered product.
FDA will continue to provide updates on this case of BSE in Canada as additional information becomes available.
http://www.fda.gov/bbs/topics/NEWS/2003/NEW0910.html
It was thought likely that at least some, and probably all, of the cases in zoo animals were caused by the BSE agent. Strong support for this hypothesis came from the findings of Bruce and others (1994) ( Bruce, M.E., Chree, A., McConnell, I., Foster, J., Pearson, G. & Fraser, H. (1994)
Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and species barrier.
Philosophical Transactions of the Royal Society B 343, 405-411: J/PTRSL/343/405 ), who demonstrated that the pattern of variation in incubation period and lesion profile in six strains of mice inoculated with brain homogenates from an affected kudu and the nyala, was similar to that seen when this panel of mouse strains was inoculated with brain from cattle with BSE. The affected zoo bovids were all from herds that were exposed to feeds that were likely to have contained contaminated ruminant-derived protein and the zoo felids had been exposed, if only occasionally in some cases, to tissues from cattle unfit for human consumption.
snip...
http://collections.europarchive.org/tna/20080102174910/http://www.bseinquiry.gov.uk/files/ws/s324.pdf
cases have been reported in domestic cats), are characterised by long asymptomatic incubation periods followed by progressive symptoms and signs of degeneration of the brain, leading eventually to death.
http://www.bsereview.org.uk/download/draft_2.pdf
PET FOODS MAD CATS AND MAD DOGS BSE/TSEs
worse still, there is serious risk the media could get to hear of such a meeting...
snip...
Crushed heads (which inevitably involve brain and spinal cord material) are used to a limited extent but will also form one of the constituent raw materials of meat and bone meal, which is used extensively in pet food manufacturer...
http://collections.europarchive.org/tna/20080102163540/http://www.bseinquiry.gov.uk/files/yb/1989/03/17004001.pdf
2. The Parliamentary Secretary said that he was concerned about the possibility that countries in which BSE had not yet been detected could be exporting raw meat materials (in particular crushed heads) contaminated with the disease to the UK for use in petfood manufacture...
snip...
YOU explained that imported crushed heads were extensively used in the petfood industry...
http://collections.europarchive.org/tna/20080102154438/http://www.bseinquiry.gov.uk/files/yb/1989/04/14001001.pdf
In particular I do not believe one can say that the levels of the scrapie agent in pet food are so low that domestic animals are not exposed...
http://collections.europarchive.org/tna/20081105230259/http://www.bseinquiry.gov.uk/files/yb/1989/04/24003001.pdf
BSE IN PETFOOD
1. The Secretary asked on 19 April whether I was content with the advice in para 3 of the record of the meeting on 17 March with the Parliamentary Secretary (Mr Thompson). The simple answer is ''not entirely''.
2. On occasions, material obtained from slaughterhouses will be derived from sheep affected with scrapie or cattle that may be incubating BSE for use in petfood manufacture. Some of this material must be classified as high risk since it contains brain, spinal cord, spleen or lymphatic glands.
http://collections.europarchive.org/tna/20090505233052/http://www.bseinquiry.gov.uk/files/yb/1989/05/03007001.pdf
Meldrum's notes on pet foods and materials used
http://collections.europarchive.org/tna/20081105230323/http://www.bseinquiry.gov.uk/files/yb/1989/05/16001001.pdf
http://collections.europarchive.org/tna/20080102200123/http://www.bseinquiry.gov.uk/files/yb/1989/05/16002001.pdf
IN CONFIDENCE CJD TO CATS...
It should be noted that under experimental conditions cats succumb to an encephalopathy after intracerebral inoculation of material derived from patients affected with Creutzfeldt-Jakob Disease.
http://collections.europarchive.org/tna/20080103005226/http://www.bseinquiry.gov.uk/files/yb/1989/05/18002001.pdf
Confidential BSE and __________________
3. I have thought very hard about whether the Branch should carry out a similar exercise with meat and meat products for human foods. On balance I do NOT think we should undertake it, but a final decision has not been taken and you may wish to discuss this further. ...
http://web.archive.org/web/20030509205351/http://www.bseinquiry.gov.uk/files/yb/1989/05/22012001.pdf
full text ;
http://caninespongiformencephalopathy.blogspot.com/
http://betaamyloidcjd.blogspot.com/
Wednesday, February 24, 2010
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America 14th
ICID International Scientific Exchange Brochure -
http://transmissiblespongiformencephalopathy.blogspot.com/2010/02/transmissible-spongiform-encephalopathy.html
Transmissible Spongiform Encephalopathy
http://transmissiblespongiformencephalopathy.blogspot.com/
Monday, April 5, 2010
Update on Feed Enforcement Activities to Limit the Spread of BSE April 5, 2010
http://madcowfeed.blogspot.com/2010/04/update-on-feed-enforcement-activities.html
TSS
Husbands Appear at Higher Risk Than Wives
April 30th, 2010
Strictly Embargoed Until 00.01 Hours (EST) Wednesday, May 5, 2010
Contact: Jennifer Beal +44 (0) 1243 770633 Medicalnews@wiley.com
Spouses of Dementia Sufferers Have a Six-Fold Increased Risk of Dementia Onset
Husbands Appear at Higher Risk Than Wives Older married adults whose spouse has dementia are at significantly higher risk for developing dementia themselves, compared to similar older married adults whose spouse never develops dementia. This is the key finding of a study published today in the Journal of the American Geriatrics Society.
Informal dementia caregiving for a spouse is a natural marital obligation, and spousal caregivers often report positive feelings toward caregiving, yet it is difficult, requiring time, energy and usually physical exertion. Dementia caregivers have been shown to provide more assistance, and to report more personal sacrifices and stress, than those who care for physically-impaired elderly without dementia. While there are many published studies showing that dementia caregivers are at higher risk for health problems and depression, none have examined risk for dementia in the caregiver.
2,442 subjects (1,221 married couples) aged 65 and older from Northern Utah, USA, without dementia at onset were studied for up to 12 years to monitor for onset of dementia in husbands, wives or both. During this time, 125 cases of dementia only in the husband were diagnosed, 70 only in the wife, and 30 where both spouses were diagnosed (60 people).
The researchers, led by Dr. Maria Norton of Utah State University, USA, adjusted for socioeconomic status, a significant predictor of many health-related outcomes including dementia to control for shared environmental exposures that might influence risk for dementia in both spouses.
The results showed that incident dementia was significantly associated with older age, and having a spouse with dementia. Participants with a spouse who developed dementia were at a six times increased risk of developing dementia, net of the effect of age, gender, APOE genotype, and socioeconomic status, with higher risk in men (11.9) than women (3.7).
“Future studies are needed to determine how much of this association is due to caregiver stress compared to a shared environment,” said Norton. “On the positive side, the majority of these individuals, with spouses who develop dementia, did not themselves develop dementia, therefore more research is needed to explore which factors distinguish those who are more vulnerable.”
“Given the significant public health concern of Alzheimer’s disease and other dementias, and the upcoming shift in population age composition, continued research into the causes of dementia is urgent,” concluded Norton. __________________________________________________________________
This study is published in the issue of the Journal of the American Geriatrics Society. Media wishing to receive a PDF of this article may contact medicalnews@wiley.com
Full citation: Norton et al; Increased Risk of Dementia When Spouse Has Dementia? The Cache County Study; The Journal of the American Geriatrics Society, May 2010
About the Author: Dr. Maria Norton is based at Utah State University, USA. To arrange an interview with Dr. Norton, please contact Tim Vitale at Utah State University’s Public Relations office on tim.vitale@usu.edu or +1 435-797-1356.
About the Journal: The Journal of the American Geriatrics Society is a comprehensive and reliable source of monthly research and information about common diseases and disorders of older adults. The journal is published by Wiley-Blackwell on behalf of the American Geriatrics Society. For more information, please visit www.blackwellpublishing.com/jgs.
About Wiley-Blackwell: Wiley-Blackwell is the international scientific, technical, medical, and scholarly publishing business of John Wiley & Sons, with strengths in every major academic and professional field and partnerships with many of the world’s leading societies. Wiley-Blackwell publishes nearly 1,500 peer-reviewed journals and 1,500+ new books annually in print and online, as well as databases, major reference works and laboratory protocols. For more information, please visit www.wileyblackwell.com or www.interscience.wiley.com.
Modified On: May 5th, 2010
http://www.americangeriatrics.org/press/id:665
Tuesday, August 26, 2008
Alzheimer's Transmission of AA-amyloidosis: Similarities with Prion Disorders NEUROPRION 2007 FC4.3
http://betaamyloidcjd.blogspot.com/2008/08/alzheimers-transmission-of-aa.html
http://betaamyloidcjd.blogspot.com/2010/03/neurobiology-of-disease-molecular-cross.html
http://betaamyloidcjd.blogspot.com/2010/04/food-combination-and-alzheimer-disease.html
Wednesday, April 14, 2010
Food Combination and Alzheimer Disease Risk A Protective Diet
http://betaamyloidcjd.blogspot.com/2010/04/food-combination-and-alzheimer-disease.html
TSE & HOUNDS
GAH WELLS (very important statement here...TSS)
HOUND STUDY
AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.
http://web.archive.org/web/20010305222642/www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
76 pages on hound study;
http://web.archive.org/web/20030327022236/http://www.bseinquiry.gov.uk/files/sc/seac16/tab04.pdf
I thought that in Britain dogs had contracted BSE, but perhaps not.
not so fast here;
The spongiform changes were not pathognomonic (ie. conclusive proof) for prion disease, as they were atypical, being largely present in white matter rather than grey matter in the brain and spinal cord. However, Tony Scott, then head of electron microscopy work on TSEs, had no doubt that these SAFs were genuine and that these hounds therefore must have had a scrapie-like disease. I reviewed all the sections myself (original notes appended) and although the pathology was not typical, I could not exclude the possibility that this was a scrapie-like disorder, as white matter vacuolation is seen in TSEs and Wallerian degeneration was also present in the white matter of the hounds, another feature of scrapie.
38.I reviewed the literature on hound neuropathology, and discovered that micrographs and descriptive neuropathology from papers on 'hound ataxia' mirrored those in material from Robert Higgins' hound survey. Dr Tony Palmer (Cambridge) had done much of this work, and I obtained original sections from hound ataxia cases from him. This enabled me provisionally to conclude that Robert Higgins had in all probability detected hound ataxia, but also that hound ataxia itself was possibly a TSE. Gerald Wells confirmed in 'blind' examination of single restricted microscopic fields that there was no distinction between the white matter vacuolation present in BSE and scrapie cases, and that occurring in hound ataxia and the hound survey cases.
39.Hound ataxia had reportedly been occurring since the 1930's, and a known risk factor for its development was the feeding to hounds of downer cows, and particularly bovine offal. Circumstantial evidence suggests that bovine offal may also be causal in FSE, and TME in mink. Despite the inconclusive nature of the neuropathology, it was clearly evident that this putative canine spongiform encephalopathy merited further investigation.
40.The inconclusive results in hounds were never confirmed, nor was the link with hound ataxia pursued. I telephoned Robert Higgins six years after he first sent the slides to CVL. I was informed that despite his submitting a yearly report to the CVO including the suggestion that the hound work be continued, no further work had been done since 1991. This was surprising, to say the very least.
41.The hound work could have provided valuable evidence that a scrapie-like agent may have been present in cattle offal long before the BSE epidemic was recognised. The MAFF hound survey remains unpublished.
Histopathological support to various other published MAFF experiments
42.These included neuropathological examination of material from experiments studying the attempted transmission of BSE to chickens and pigs (CVL 1991) and to mice (RVC 1994).
http://www.bseinquiry.gov.uk/witness/htm/stat067.htm
It was thought likely that at least some, and probably all, of the cases in zoo animals were caused by the BSE agent. Strong support for this hypothesis came from the findings of Bruce and others (1994) ( Bruce, M.E., Chree, A., McConnell, I., Foster, J., Pearson, G. & Fraser, H. (1994) Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and species barrier. Philosophical Transactions of the Royal Society B 343, 405-411: J/PTRSL/343/405 ), who demonstrated that the pattern of variation in incubation period and lesion profile in six strains of mice inoculated with brain homogenates from an affected kudu and the nyala, was similar to that seen when this panel of mouse strains was inoculated with brain from cattle with BSE. The affected zoo bovids were all from herds that were exposed to feeds that were likely to have contained contaminated ruminant-derived protein and the zoo felids had been exposed, if only occasionally in some cases, to tissues from cattle unfit for human consumption.
snip...
NEW URL ;
http://collections.europarchive.org/tna/20080102174910/http://www.bseinquiry.gov.uk/files/ws/s324.pdf
SEE LETTER FROM MAFF TO ME IN 2005 ABOUT THE HOUND STUDY ;
2005
DEFRA Department for Environment, Food & Rural Affairs
Area 307, London, SW1P 4PQ Telephone: 0207 904 6000 Direct line: 0207 904 6287 E-mail: h.mcdonagh.defra.gsi.gov.uk
GTN: FAX:
Mr T S Singeltary P.O. Box 42 Bacliff Texas USA 77518
21 November 2001
Dear Mr Singeltary
TSE IN HOUNDS
Thank you for e-mail regarding the hounds survey. I am sorry for the long delay in responding.
As you note, the hound survey remains unpublished. However the Spongiform Encephalopathy Advisory Committee (SEAC), the UK Government's independent Advisory Committee on all aspects related to BSE-like disease, gave the hound study detailed consideration at their meeting in January 1994. As a summary of this meeting published in the BSE inquiry noted, the Committee were clearly concerned about the work that had been carried out, concluding that there had clearly been problems with it, particularly the control on the histology, and that it was more or less inconclusive. However was agreed that there should be a re-evaluation of the pathological material in the study.
Later, at their meeting in June 95, The Committee re-evaluated the hound study to see if any useful results could be gained from it. The Chairman concluded that there were varying opinions within the Committee on further work. It did not suggest any further transmission studies and thought that the lack of clinical data was a major weakness.
Overall, it is clear that SEAC had major concerns about the survey as conducted. As a result it is likely that the authors felt that it would not stand up to r~eer review and hence it was never published. As noted above, and in the detailed minutes of the SEAC meeting in June 95, SEAC considered whether additional work should be performed to examine dogs for evidence of TSE infection. Although the Committee had mixed views about the merits of conducting further work, the Chairman noted that when the Southwood Committee made their recommendation to complete an assessment of possible spongiform disease in dogs, no TSEs had been identified in other species and hence dogs were perceived as a high risk population and worthy of study. However subsequent to the original recommendation, made in 1990, a number of other species had been identified with TSE ( e.g. cats) so a study in hounds was less
critical. For more details see- http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf
As this study remains unpublished, my understanding is that the ownership of the data essentially remains with the original researchers. Thus unfortunately, I am unable to help with your request to supply information on the hound survey directly. My only suggestion is that you contact one of the researchers originally involved in the project, such as Gerald Wells. He can be contacted at the following address.
Dr Gerald Wells, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey, KT 15 3NB, UK
You may also wish to be aware that since November 1994 all suspected cases of spongiform encephalopathy in animals and poultry were made notifiable. Hence since that date there has been a requirement for vets to report any suspect SE in dogs for further investigation. To date there has never been positive identification of a TSE in a dog.
I hope this is helpful
Yours sincerely 4
HUGH MCDONAGH BSE CORRESPONDENCE SECTION
======================================END...TSS
WHAT ABOUT THOSE STUMBLING, STAGGERING, AND BLIND DOGS, the old dog syndrome, just another spontaneous event ???
The signs of canine cognitive dysfunction syndrome or "old dog syndrome" commonly seen in dogs are: lose of house training increased barking or whining increased anxiety or fear signs disorientation-appearing lost or confused,getting stuck behind furniture or in corners, walking in circles, becoming forgetful,walking aimlessly,staring into space, repetitious or compulsive behavior change in sleep patterns-up at night, sleep all day
lack of responsiveness other changes,may not recognize you, their name,may become more docile, more aggressive.. You can liken it to human senility. An article at the petcenter says "CDS is not "normal aging". A number of pathophysiological changes are suspected to play a role in its development. These include: * deposition of amyloid plaques in the cerebral cortex and hippocampal part of the brain * alterations in neurotransmitters, including dopamine * increased levels of monoamine oxidase B (MAOB) in the brain * increased levels of free radicals L-DEPRENYL HYDROCHLORIDE SELEGILINE HYDROCHLORIDE,BRAND NAME: ANIPRYL OR ELDEPRYL is used to help treat canine cognitive dysfunction by increasing brain concentrations of the neurotransmitter dopamine. Hopefully you can see a difference in a month or so. If you don't see a difference in the first month, your vet might tell you to try two pills a day for the next month. ANIPRYL doesn't work for all dogs. A great writeup on L-DEPRENYL can be found at
http://www.petsinfo.org/elderlydogs1.html
"One third of canine CD patients respond extremely well to treatment with deprenyl by regaining their youthful vigor; another one third respond reasonably well; and one third do not respond at all (perhaps there is a variant of CD with different neuropathology). The bottom line is that for any dog that is slowing down to the point that problems become apparent, treatment with deprenyl is the logical route once other organic causes for reduced mental function have been ruled out. Here is a write up on selegline " Selegiline has immune-system-boosting and anti-neurodegenerative effects. ....
Taken consistently in low doses, selegiline tends to extend the life-expectancy of rats by some 20%; enhances drive, libido and endurance; and independently improves cognitive performance in Alzheimer's patients and in some healthy normals. It is used successfully to treat canine cognitive dysfunction syndrome (CDS) in dogs...Selegiline protects the brain's dopamine cells from oxidative stress. " Some also use alpha lipoic acid and r-lipoic acid. powerful antioxidants to help slow down canine cognitive dysfunction. There is a dog food that is rich in antioxidants for CDS but I am assuming if you supplement with your own antioxidants you don't have to worry if your dog likes the food or not. I know my dog Hammy has become very picky and at least if I pill him, I know he is getting his antioxidants.
http://www.thensome.com/cds.htm
Doggie Dementia
Does 14-year-old Fido get lost in his own back yard?
Does he not respond when you call his name?
Does he generally seem confused?
According to Pulse, the official magazine of the Southern California Veterinary Medical Association, just as humans in the 21st Century are living longer, so is man’s best friends—more than 7.3 million dogs in the United States are age 10 or older. And with age dogs become prone to the same age-related diseases as their human companions, including dementia.
A disease of old age affects dogs and humans alike
Kazzy, a 17-year-old Lhasa Apso, is one of the 60 percent of dogs aged 11 to 15 who suffer from one or more symptoms of canine cognitive dysfunction syndrome (CDS), also known to veterinarians as doggie dementia. "He used to be the most incredible watchdog," says his owner, Olivia Feldman-Rich. "But he’s not like that anymore. He’s quite bewildered."
Experts like Dr. Maritza Perez, a veterinarian at West Orange (NJ) Animal Hospital, say that confusion is one of the four major signs of CDS (see sidebar). Dr. Perez says dogs may "pace around in circles, get stuck behind furniture, or they don’t know where the back door is anymore."
Often the most distressing sign of CDS is that, like human patients with Alzheimer’s disease, your pet seems to forget you and your family. "A lot of people notice that when you walk in the door, and this dog that was happy to see you doesn’t get up off the couch or off the floor to greet you," says Dr. Perez. "And he doesn’t come anymore when you call him."
These symptoms, coupled with others debilitating diseases affecting older dogs, such as arthritis, all add up to a serious loss in quality of life for your canine friend. The American Veterinary Medical Association reports that some 500,000 dogs are put to sleep each year because of CDS.
Researchers say that deposits of beta-amyloid plaques in brain tissues are likely to play a role in CDS. These plaques build up and eventually inhibit transmission of the brain’s neural signals. Still, the recognized symptoms of CDS are behavioral, so a diagnosis is exclusionary, meaning it is arrived at only after all other physical and neurological causes are ruled out.
No cure yet, but relief for some dogs
Dr. Perez with a 14 year-old beagle who is on Anipryl.
While scientists search for a permanent cure for CDS, there is one treatment currently FDA-approved for CDS. Selegiline hydrochloride, whose brand name is Anipryl, may give some dogs relief from its symptoms. Researchers speculate that Anipryl works by increasing levels of dopamine, a neurotransmitter. Other treatments are currently being investigated, including diets high in anti-oxidants as well as a new drug, Adrafinil, in one Canadian study.
Dr. Perez says that Anipryl does cause an improvement in many dogs with CDS, meaning relief from at least one of the common symptoms. "We have lots of animals on it and it does work," she says. But it’s not a sure thing—Dr. Perez tried it on her own dog with no effect.
Feldman-Rich is debating putting Kazzy on Anipryl. "I’m hoping that it will give a little more balance to his life and make him a little more aware that he’s still here and we’re still here for him," she says. "I always told him that he couldn’t leave me too soon, and he’s definitely kept up his end of it, but I’d definitely like for him to feel a little more like he’s part of the family."
by Debra Utacia Krol
http://www.sciencentral.com/articles/view.php3?article_id=218391360&cat=1_6
SEE CJD IN MAN AND HIS CAT ;
[Image] Research letters Volume 352, Number 9134 [Image] 3 October 1998 [Previous] [Next]
[Image][Image]
Simultaneous occurrence of spongiform encephalopathy in a man and his cat in Italy
[Image]
Gianluigi Zanusso, Ettore Nardelli, Anna Rosati, GianMaria Fabrizi, Sergio Ferrari, Antonella Carteri, Franco De Simone, Nicola Rizzuto, Salvatore Monaco
Transmissible spongiform encephalopathies (TSE) encompass inherited, acquired, and sporadic mammalian neurological disorders, and are characterised by the conversion of the cellular prion protein (PrP) in an insoluble and protease-resistant isoform (PrPres). In human TSE, four types of PrPres have been identified according to size and glycoform ratios, which may represent different prion strains. Type-1 and type-2 PrPres are associated with sporadic Creutzfeldt-Jakob disease (CJD), type 3 with iatrogenic CJD, and type 4 with variant CJD.1,2 There is evidence that variant CJD is caused by the bovine spongiform encephalopathy (BSE)-prion strain.2-4 The BSE strain has been identified in three cats with feline spongiform encephalopathy (FSE), a prion disease which appeared in 1990 in the UK.5 We report the simultaneous occurrence of sporadic CJD in a man and a new variety of FSE in his cat.
A 60-year-old man, with no unusual dietary habits, was admitted in November, 1993, because of dysarthria, cerebellar ataxic gait, visual agnosia, and myoclonus. An electroencephalogram (EEG) showed diffuse theta-delta activity. A brain magnetic resonance imaging scan was unremarkable. 10 days later, he was speechless and able to follow only simple commands. Repeat EEGs showed periodic triphasic complexes. 2 weeks after admission, he was mute, akinetic, and unable to swallow. He died in early January, 1994.
His 7-year-old, neutered, female shorthaired cat presented in November, 1993, with episodes of frenzy, twitching of its body, and hyperaesthesia. The cat was usually fed on canned food and slept on its owner's bed. No bites from the cat were recalled. In the next few days, the cat became ataxic, with hindquarter locomotor dysfunction; the ataxia got worse and there was diffuse myoclonus. The cat was killed in mid-January, 1994.
No pathogenic mutations in the patient's PrP gene were found. The patient and the cat were methionine homozygous at codon 129. Histology of the patient's brain showed neocortical and cerebellar neuronal loss, astrocytosis, and spongiosis (figure A). PrP immunoreactivity showed a punctate pattern and paralleled spongiform changes (figure B). The cat's brain showed mild and focal spongiosis in deeper cortical layers of all four lobes (figure C), vacuolated cortical neurons (figure D), and mild astrogliosis. The cerebellar cortex and the dentate nucleus were gliosed. Immunoreactive PrP showed a punctate pattern in neocortex, allocortex, and caudate nucleus (figure E). Western blot analysis of control and affected human and cat brain homogenates showed 3 PrP bands of 27-35 kDa. After digestion with proteinase K and deglycosylation, only samples from the affected patient and cat showed type-1 PrPres, with PrP glycoform ratios comparable to those observed in sporadic CJD1 (details available from author).
[Image]
Microscopic sections of patient and cat brains
A: Occipital cortex of the patient showing moderate spongiform degeneration and neuronal loss (haematoxylin and eosin) and B: punctate perineuronal pattern of PrP immunoreactivity; peroxidase immunohistochemistry with monoclonal antibody 3F4. C: cat parietal cortex showing mild spongiform degeneration (haematoxylin and eosin).D: vacuolated neurons (arrow, haematoxylin and eosin), E: peroxidase immunohistochemistry with antibody 3F4 shows punctate perineuronal deposition of PrP in temporal cortex.
This study shows a spatio-temporal association between human and feline prion diseases. The clinical features of the cat were different from previously reported cases of FSE which were characterised by gradual onset of behavioural changes preceding locomotor dysfunction and ataxia.5 Neuropathological changes were also at variance with the diffuse spongiosis and vacuolation of brainstem neurons, seen in FSE.5 The synaptic pattern of PrP deposition, similar in the cat and in the patient, was atypical for a BSE-related condition. Evidence of a new type of FSE was further provided by the detection of a type-1 PrPres, other than the BSE-associated type 4.2 Taken together, our data suggest that the same agent strain of sporadic CJD was involved in the patient and in his cat.
It is unknown whether these TSE occurred as the result of horizontal transmission in either direction, infection from an unknown common source, or the chance occurrence of two sporadic forms.
1 Parchi P, Castellani R, Capellari S, et al. Molecular basis of phenotypic variablity in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39: 767-78 [PubMed].
2 Collinge J, Sidle KCL, Meads J, Ironside J, Hill AF. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 1996; 383: 685-90 [PubMed].
3 Bruce ME, Will RG, Ironside JW, et al. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 1997; 389: 498-501 [PubMed].
4 Hill AF, Desbruslais M, Joiner S, et al. The same prion strain causes vCJD and BSE. Nature 1997; 389: 448-50 [PubMed].
5 Pearson GR, Wyatt JM, Henderson JP, Gruffydd-Jones TJ. Feline spongiform encephalopathy: a review. Vet Annual 1993; 33: 1-10.
------------------------------------------------------------------------ Sezione di Neurologie Clinica, Dipartimento di Scienze Neurologiche e della Visione, Università di Verona, Policlinico Borgo Roma, 37134 Verona, Italy (S Monaco; e mail rizzuto@Gorgorna.univr.it); and Istituto Zooprofilattico Sperimentale della Lombardia e dell' Emilia, Brescia
=======================================
Terry S. Singeltary Sr. wrote:
######## Bovine Spongiform Encephalopathy #########
Greetings list members,
ODD that some FELINE in Italy seem to have this same or maybe very similar phenotype of TSE;
In October 1998 the simultaneous occurrence of spongiform encephalopathy in a man and his pet cat was reported. The report from Italy noted that the cat did not display the same clinical features as FSE cases previously seen. Indeed, the presence of a new type of FSE was suggested. The man was diagnosed as having sporadic CJD, and neither case (man nor cat) appeared to be affected by a BSE-related condition.
http://www.defra.gov.uk/animalh/bse/bse-science/level-4-othertses.html
-------- Original Message --------
Subject: FDA BSE Update - Pet Food from Canadian Manufacturer & MAD DOG DATA
Date: Tue, 27 May 2003 08:07:58 -0500
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy
Statement
FOR IMMEDIATE RELEASE Statement May 26, 2003
Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA
FDA BSE Update - Pet Food from Canadian Manufacturer
The Food and Drug Administration (FDA) has learned from the government of Canada that rendered material from a Canadian cow that last week tested positive for bovine spongiform encephalopathy (BSE, also known as mad cow disease ) may have been used to manufacture pet food, specifically dry dog food, some of which was reported to have been shipped to the United States. The Canadian government prevented the BSE positive cow from being processed for human food. Therefore, consumers can be assured that their food does not contain any remnants of the BSE positive cow.
It is also important to stress that there is no scientific evidence to date that dogs can contract BSE or any similar disease. In addition there is no evidence that dogs can transmit the disease to humans.
FDA notified the U.S. pet food firm, The Pet Pantry International, of Carson City, Nevada, when FDA learned that the pet food that the firm received may have included rendered material from the BSE positive cow. The manufacturer of the pet food is Champion Pet Food, Morinville, Alberta. Even though there is no known risk to dogs from eating this dog food, as a prudent measure to help assure that the U.S. stays BSE free The Pet Pantry International is asking its customers who may have purchased the suspect product to hold it for pickup by the distributor so that the dog food will not mistakenly be mixed into cattle or other feeds if any of the dog food is discarded or otherwise not used to feed dogs. The suspect dog food was produced by Champion Pet Food between February 4, 2003, and March 12, 2003.
The Pet Pantry products were packaged in 50 lb bags, distributed to franchises around the country, and sold by home delivery only. There was no retail distribution of the product. Consumers purchase Pet Pantry products by phone or email orders. The product is then delivered by the nearest franchisee directly to the consumer s home.
The product subject to this notification includes Maintenance Diet labeled with a use by date of 17FEB04 and Beef with Barley with a use by date of 05MAR04 . Consumers who have purchased dog food from The Pet Pantry since February of this year are asked to check their present supplies and see if any match the description of the product being removed. If so, consumers are asked to contact The Pet Pantry at 1-800-381-7387 for further information on how to return the product to The Pet Pantry for proper disposal. Consumers are asked not to destroy or discard the product themselves. The Pet Pantry will also use its sales records to contact consumers who purchased the affected product.
FDA is working closely with the Pet Pantry International to assure for proper disposal of the recovered product.
FDA will continue to provide updates on this case of BSE in Canada as additional information becomes available.
http://www.fda.gov/bbs/topics/NEWS/2003/NEW0910.html
It was thought likely that at least some, and probably all, of the cases in zoo animals were caused by the BSE agent. Strong support for this hypothesis came from the findings of Bruce and others (1994) ( Bruce, M.E., Chree, A., McConnell, I., Foster, J., Pearson, G. & Fraser, H. (1994)
Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and species barrier.
Philosophical Transactions of the Royal Society B 343, 405-411: J/PTRSL/343/405 ), who demonstrated that the pattern of variation in incubation period and lesion profile in six strains of mice inoculated with brain homogenates from an affected kudu and the nyala, was similar to that seen when this panel of mouse strains was inoculated with brain from cattle with BSE. The affected zoo bovids were all from herds that were exposed to feeds that were likely to have contained contaminated ruminant-derived protein and the zoo felids had been exposed, if only occasionally in some cases, to tissues from cattle unfit for human consumption.
snip...
http://collections.europarchive.org/tna/20080102174910/http://www.bseinquiry.gov.uk/files/ws/s324.pdf
cases have been reported in domestic cats), are characterised by long asymptomatic incubation periods followed by progressive symptoms and signs of degeneration of the brain, leading eventually to death.
http://www.bsereview.org.uk/download/draft_2.pdf
PET FOODS MAD CATS AND MAD DOGS BSE/TSEs
worse still, there is serious risk the media could get to hear of such a meeting...
snip...
Crushed heads (which inevitably involve brain and spinal cord material) are used to a limited extent but will also form one of the constituent raw materials of meat and bone meal, which is used extensively in pet food manufacturer...
http://collections.europarchive.org/tna/20080102163540/http://www.bseinquiry.gov.uk/files/yb/1989/03/17004001.pdf
2. The Parliamentary Secretary said that he was concerned about the possibility that countries in which BSE had not yet been detected could be exporting raw meat materials (in particular crushed heads) contaminated with the disease to the UK for use in petfood manufacture...
snip...
YOU explained that imported crushed heads were extensively used in the petfood industry...
http://collections.europarchive.org/tna/20080102154438/http://www.bseinquiry.gov.uk/files/yb/1989/04/14001001.pdf
In particular I do not believe one can say that the levels of the scrapie agent in pet food are so low that domestic animals are not exposed...
http://collections.europarchive.org/tna/20081105230259/http://www.bseinquiry.gov.uk/files/yb/1989/04/24003001.pdf
BSE IN PETFOOD
1. The Secretary asked on 19 April whether I was content with the advice in para 3 of the record of the meeting on 17 March with the Parliamentary Secretary (Mr Thompson). The simple answer is ''not entirely''.
2. On occasions, material obtained from slaughterhouses will be derived from sheep affected with scrapie or cattle that may be incubating BSE for use in petfood manufacture. Some of this material must be classified as high risk since it contains brain, spinal cord, spleen or lymphatic glands.
http://collections.europarchive.org/tna/20090505233052/http://www.bseinquiry.gov.uk/files/yb/1989/05/03007001.pdf
Meldrum's notes on pet foods and materials used
http://collections.europarchive.org/tna/20081105230323/http://www.bseinquiry.gov.uk/files/yb/1989/05/16001001.pdf
http://collections.europarchive.org/tna/20080102200123/http://www.bseinquiry.gov.uk/files/yb/1989/05/16002001.pdf
IN CONFIDENCE CJD TO CATS...
It should be noted that under experimental conditions cats succumb to an encephalopathy after intracerebral inoculation of material derived from patients affected with Creutzfeldt-Jakob Disease.
http://collections.europarchive.org/tna/20080103005226/http://www.bseinquiry.gov.uk/files/yb/1989/05/18002001.pdf
Confidential BSE and __________________
3. I have thought very hard about whether the Branch should carry out a similar exercise with meat and meat products for human foods. On balance I do NOT think we should undertake it, but a final decision has not been taken and you may wish to discuss this further. ...
http://web.archive.org/web/20030509205351/http://www.bseinquiry.gov.uk/files/yb/1989/05/22012001.pdf
full text ;
http://caninespongiformencephalopathy.blogspot.com/
http://betaamyloidcjd.blogspot.com/
Wednesday, February 24, 2010
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America 14th
ICID International Scientific Exchange Brochure -
http://transmissiblespongiformencephalopathy.blogspot.com/2010/02/transmissible-spongiform-encephalopathy.html
Transmissible Spongiform Encephalopathy
http://transmissiblespongiformencephalopathy.blogspot.com/
Monday, April 5, 2010
Update on Feed Enforcement Activities to Limit the Spread of BSE April 5, 2010
http://madcowfeed.blogspot.com/2010/04/update-on-feed-enforcement-activities.html
TSS
Monday, January 4, 2010
Rising Tide: The Impact of Dementia in Canada
Huge wave of dementia cases coming, warns report
CTV.ca News Staff
Date: Mon. Jan. 4 2010 9:52 AM ET
So many Canadians are expected to develop Alzheimer's disease and dementia in the next 30 years that a new case will be diagnosed every two minutes unless preventive measures are taken, a new report says.
The report, released Monday by the Alzheimer Society, says the prevalence of dementia will more than double in the next 30 years.
By 2038, almost three per cent of Canada's population will be affected by dementia, and about 257,800 new cases will be diagnosed per year.
Today, dementia costs Canada about $15 billion a year; those costs could soon increase by 10-fold.
"If nothing changes, this sharp increase in the number of people living with dementia will mean that by 2038, the total costs associated with dementia will reach $153 billion a year," David Harvey, principal spokesperson for the Alzheimer Society project called "Rising Tide: The Impact of Dementia on Canadian Society," said in a statement.
That amounts to a cumulative total of $872 billion over the 30-year period.
Much of the increase in cases can be attributed to the "greying" of Canada. With Canadians living longer and baby boomers aging, there is expected to be a spike in many chronic diseases that come with age, such as heart disease, arthritis and cancer.
But the expected rising rates of dementia are not just about demographics; poor lifestyles also play a role.
It's been well documented that regular physical and mental exercise can delay the onset of dementia, which includes Alzheimer's disease and other progressive diseases that destroy brain cells. For that reason, the report recommends that all Canadians over 65 without dementia increase their physical activity by 50 per cent.
"Prevention is where we need to be starting," Harvey told Canada AM.
"We know that healthy eating and active living are antidotes to dementia."
The "Rising Tide" report calls on government to fund more health promotion to remind Canadians of the benefits of a healthy lifestyle.
"This intervention would reduce the number of people diagnosed with dementia, resulting in a reduction in the pressure on long-term care facilities, community care services and informal caregivers," the report says.
Need for national strategy
Just as important, Harvey says, is the need for Canada's health care system to adapt to accommodate the projected rise in dementia cases.
"Dementia is one of the leading cases of disability amongst older people," Harvey said, noting that the flood of dementia expected in the next 30 years could overwhelm emergency rooms and hospitals.
His group's report calls for more support for informal caregivers -- generally, family members -- who tend to be the ones who care for patients with dementia in the early stages of the disease.
"There are services that can be put in place to support caregivers, and also economic and financial support for caregivers," he said.
By also providing caregivers with skill-building and support programs, caregivers struggling with the overwhelming emotional and financial hardships of providing care may feel better equipped to care for their loved one.
That could go far to delay admission of patients into long-term care facilities, thereby lessening the burden on the health care system.
The report also suggests assigning "system navigators" to each newly diagnosed dementia patient and their caregivers. These case managers would help families navigate the health system to find the right social services for their loved one depending on their stage of dementia.
--------------------------------------------------------------------------------
Some facts about dementia:
The symptoms of dementia include a gradual and continuing decline of memory, changes in judgment or reasoning, mood and behaviour, and an inability to perform familiar tasks. Dementia can strike adults at any age, but has traditionally been diagnosed in people over 65. However, symptoms start much earlier, and an increasing number of people are being diagnosed in their 50s and early 60s. Age is the number one risk factor for dementia Alzheimer's disease, the most common form of dementia, accounts for approximately 64 per cent of all dementias in Canada. Other related dementias include Vascular Dementia, Frontotemporal Dementia, Creutzfeldt-Jakob Disease and Lewy body Dementia. There is no known cure for dementia. However, some medications can delay progression of the disease. Researchers are confident that within five to seven years, there will be treatments that attack the disease process itself, not just the symptoms.
http://www.ctv.ca/servlet/ArticleNews/story/CTVNews/20100104/dementia_surge_100104/20100104?hub=Health
Report Summary Rising Tide: The Impact of Dementia in Canada In this section :
Read a summary of Rising Tide: The Impact of Dementia in Canada
Download Rising Tide: The Impact of Dementia in Canada Rising Tide: the Impact of Dementia on Canadian Society is the final report of an Alzheimer Society project funded by Pfizer Canada, Health Canada, Public Health Agency of Canada, Canadian Institutes of Health Research and Rx&D. The purpose of the report was to:
Estimate the health and economic burden of dementia in Canada over the next 30 years; Analyze the possible effects of intervention scenarios upon this burden; Demonstrate how the proposed interventions could affect the health and economic impacts of dementia in Canada; Review policy options; Make recommendations on how to address the issue. The Findings of Rising Tide1 Health Burden of Dementia for Canada: 2008-2038²
Incidence of Alzheimer's disease and related dementias in Canada: 2008 - 103,700 new cases per year (1 every 5 minutes) 2038 - 257,800 new cases per year (1 every 2 minutes) Prevalence of Alzheimer's disease and related dementias in Canada: 2008 - 480,600 people with dementia (1.5% of Canada's population) 2038 - 1,125,200 people with dementia (2.8% of Canada's population) View the prevalence of dementia by age graph
View the prevalence of dementia by sex graph
Hours of informal care provided annually for people with dementia in Canada 2008 - 231 million hours 2038 - 756 million hours Economic Consequences of Dementia for Canada: 2008-2038²
The Economic Burden of dementia doubles every decade, increasing from $15 billion in 2008 to a startling $153 billion in 2038.
Economic Burden of Dementia (in future dollars) 2008 - $15 billion 2018 - $37 billion 2028 - $75 billion 2038 - $153 billion
Cumulative Consequences of Dementia over a 30-year period
Cumulative data represents the combined total of either the economic costs of dementia per year, or the number of people developing dementia per year, each year between 2008 and 2038. By 2038, the cumulative incidence of dementia will be more than 5.5 million people³, with a cumulative economic cost of $872 billion² (2008 dollars).
Implications – What can Canada do? What Has Been Done Elsewhere
Across the globe, many countries are recognizing the urgent issue of dementia. Australia, Norway, the Netherlands, France, Scotland and the United Kingdom have recently developed specific plans or frameworks for dealing with dementia.
View Alzheimer Disease International's graphs correlating research effort with contributions to mortality and disability.
Intervention Opportunities
Recognizing the urgent need to start turning the tide of dementia, Rising Tide describes four potential intervention scenarios, backed by current evidence that could become critical factors in reducing the impact of dementia.
The report tested the impact of four potential intervention scenarios:
Increasing Physical Activity Delay Onset of Dementia Caregiver Training, Support System Navigation All showed the potential for dramatic reductions in economic impact over the next 30 years.
Note: Rising Tide was undertaken in order to alert the Canadian public and federal, provincial and territorial politicians of the need for policies and approaches to address the looming dementia crisis. In the reports, you will find four suggested interventions. They are not meant to be definitive but to serve as illustrations of how the base case can be used to inform and shape policy in this field. The 5 recommendations in the report were developed through a comprehensive process of consultations with subject experts and stakeholders. The underlying message is that we must act now and that change is possible.
Recommendations
Rising Tide also makes five recommendations that would make up the components of a comprehensive National Dementia Strategy. They include:
An accelerated investment in all areas of dementia research. A clear recognition of the important role played by informal caregivers. An increased recognition of the importance of prevention and early intervention. Greater integration of care and increased use of chronic disease prevention and management. A strengthening of Canada's dementia workforce.
Download a copy of Rising Tide: The Impact of Dementia on Canadian Society.
Endnotes
Rising Tide: Impact of Dementia on Canadian Society is a report based on a study conducted by RiskAnalytica, a leading firm in risk management. RiskAnalytica's Life at Risk® simulation platform was customized for the Rising Tide study based on the latest dementia and health economic research, validated for epidemiological and economic aspects by subject matter experts and checked for data, logic and results. The simulation platform was then run to establish the Base Case, or the findings. Rising Tide: The Impact of Dementia on Canadian Society. Alzheimer Society, 2009. Smetanin, P., Kobak, P., Briante, C., Stiff, D., Sherman, G., and Ahmad, S. Rising Tide: The Impact of Dementia in Canada 2008 to 2038. RiskAnalytica, 2009.
http://www.alzheimer.ca/english/rising_tide/rising_tide_summary.htm
http://www.alzheimer.ca/english/rising_tide/rising_tide_report.htm
SEE FULL REPORT HERE ;
Rising Tide:
The Impact of Dementia on Canadian Society
Executive Summary
http://www.alzheimer.ca/docs/RisingTide/AS%20Rising%20Tide-Executive%20Summary_Eng_FINAL_SecuredVersion.pdf
Saturday, October 31, 2009
Involvement of Dab1 in APP processing and ß-amyloid deposition in sporadic Creutzfeldt–Jakob patients
http://betaamyloidcjd.blogspot.com/2009/10/involvement-of-dab1-in-app-processing.html
SEAC OCTOBER 2009
. Are some commoner types of neurodegenerative disease (including Alzheimer's disease and Parkinson's disease) also transmissible? Some recent scientific research has suggested this possibility
http://www.seac.gov.uk/pdf/hol-response091008.pdf
Thursday, February 26, 2009
'Harmless' prion protein linked to Alzheimer's disease Non-infectious form of prion protein could cause brain degeneration ???
http://betaamyloidcjd.blogspot.com/2009/02/harmless-prion-protein-linked-to.html
CJD1/9 0185
Ref: 1M51A
IN STRICT CONFIDENCE
TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES
1. CMO will wish to be aware that a meeting was held at DH yesterday, 4 January, to discuss the above findings. It was chaired by Professor Murray (Chairman of the MRC Co-ordinating Committee on Research in the Spongiform Encephalopathies in Man), and attended by relevant experts in the fields of Neurology, Neuropathology, molecular biology, amyloid biochemistry, and the spongiform encephalopathies, and by representatives of the MRC and AFRC.
2. Briefly, the meeting agreed that:
i) Dr Ridley et als findings of experimental induction of p amyloid in primates were valid, interesting and a significant advance in the understanding of neurodegeneradve disorders;
ii) there were no immediate implications for the public health, and no further safeguards were thought to be necessary at present; and
iii) additional research was desirable, both epidemiological and at the molecular level. Possible avenues are being followed up by DH and the MRC, but the details will require further discussion.
93/01.05/4.1tss
http://web.archive.org/web/20010305223440/www.bseinquiry.gov.uk/files/yb/1993/01/05004001.pdf
Regarding Alzheimer's disease
(note the substantial increase on a yearly basis)
http://web.archive.org/web/20010305222847/www.bseinquiry.gov.uk/files/yb/1988/07/08014001.pdf
snip...
The pathogenesis of these diseases was compared to Alzheimer's disease at a molecular level...
snip...
http://web.archive.org/web/20010305223234/www.bseinquiry.gov.uk/files/yb/1990/03/12003001.pdf
And NONE of this is relevant to BSE?
There is also the matter whether the spectrum of ''prion disease'' is wider than that recognized at present.
http://web.archive.org/web/20010305223234/www.bseinquiry.gov.uk/files/yb/1990/07/06005001.pdf
http://web.archive.org/web/20010305223234/www.bseinquiry.gov.uk/files/yb/1990/07/09001001.pdf
BSE101/1 0136
IN CONFIDENCE
5 NOV 1992
CMO From: Dr J S Metters DCMO 4 November 1992
TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES
http://web.archive.org/web/20010305223143/www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf
also, see the increase of Alzheimer's from 1981 to 1986
http://web.archive.org/web/20010305222847/www.bseinquiry.gov.uk/files/yb/1988/07/08014001.pdf
Tuesday, August 26, 2008
Alzheimer's Transmission of AA-amyloidosis: Similarities with Prion Disorders NEUROPRION 2007 FC4.3
http://betaamyloidcjd.blogspot.com/2008/08/alzheimers-transmission-of-aa.html
see full text ;
http://betaamyloidcjd.blogspot.com/2009/02/harmless-prion-protein-linked-to.html
Saturday, October 31, 2009
Involvement of Dab1 in APP processing and ß-amyloid deposition in sporadic Creutzfeldt–Jakob patients Copyright © 2009 Published by Elsevier Inc.
http://betaamyloidcjd.blogspot.com/2009/10/involvement-of-dab1-in-app-processing.html
----- Original Message -----
From: "Terry S. Singeltary Sr." To: Sent: Monday, October 12, 2009 9:47 AM Subject: [BSE-L] SEAC Science and Technology Committee's investigation of research funding priorities on behalf of the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy
-------------------- BSE-L@LISTS.AEGEE.ORG --------------------
snip...
. More specific examples of unanswered questions with health implications are:
. Will the eventual elimination of classical scrapie in the EU leave an ecological niche for other TSEs such as BSE or atypical scrapie?
. Is CWD transmissible to humans?
. Can a reliable ante mortem diagnostic blood test for vCJD be developed?
. What is the true prevalence of v CJD infection (as opposed to overt disease) in the UK?
XXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXX
--------------------------------------------------------------------------------
. Are some commoner types of neurodegenerative disease (including Alzheimer's disease and Parkinson's disease) also transmissible? Some recent scientific research has suggested this possibility
--------------------------------------------------------------------------------
XXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXX
. Could cases of protease sensitive prionopathy (PSP) be missed by conventional tests which, in all other TSEs, rely on the resistance of the prion protein in the nervous system that accompanies disease to digestion by protease enzymes?
. Can we develop reliable methods for removing and detecting protein on re-usable surgical instruments?
SNIP...
FULL TEXT ;
Monday, October 12, 2009
SEAC Science and Technology Committee's investigation of research funding priorities on behalf of the Advisory Committee on Dangerous Pathogens TSE 8 October 2009
http://bse-atypical.blogspot.com/2009/10/seac-science-and-technology-committees.html
Tuesday, August 26, 2008
Alzheimer's Transmission of AA-amyloidosis: Similarities with Prion Disorders NEUROPRION 2007 FC4.3
http://betaamyloidcjd.blogspot.com/2008/08/alzheimers-transmission-of-aa.html
----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Monday, June 29, 2009 2:08 PM
Subject: [BSE-L] Beyond the prion principle
-------------------- BSE-L@LISTS.AEGEE.ORG --------------------
News and Views Nature 459, 924-925 (18 June 2009) doi:10.1038/459924a; Published online 17 June 2009
CELL BIOLOGY
Beyond the prion principle
Adriano Aguzzi
It seems that many misfolded proteins can act like prions - spreading disease by imparting their misshapen structure to normal cellular counterparts. But how common are bona fide prions really?
The protein-only hypothesis of prion propagation is steadily gaining ground. First envisaged by John Stanley Griffith1 and later formalized by Stanley Prusiner2, this theory proposes the existence of an infectious agent composed solely of protein. Three reports, two in Nature Cell Biology3,4 and one in The Journal of Cell Biology5, now contend that, far from being confined to the rare prion diseases, prion-like transmission of altered proteins may occur in several human diseases of the brain and other organs.
Prions are now accepted as causing the transmissible spongiform encephalopathies, which include scrapie in sheep, bovine spongiform encephalopathy (BSE, or mad cow disease) and its human variant Creutzfeldt-Jakob disease. The infectious prion particle is made up of PrPSc, a misfolded and aggregated version of a normal protein known as PrPC. Like the growth of crystals, PrPSc propagates by recruiting monomeric PrPC into its aggregates - a process that has been replicated in vitro6 and in transgenic mice7. The breakage of PrPSc aggregates represents the actual replicative event, as it multiplies the number of active seeds8.
Apart from prion diseases, the misfolding and aggregation of proteins into various harmful forms, which are collectively known as amyloid, causes a range of diseases of the nervous system and other organs. The clinical characteristics of amyloidoses, however, gave little reason to suspect a relationship to prion diseases. Hints of prion-like behaviour in amyloid have emerged from studies of Alzheimer's disease and Parkinson's disease. Alzheimer's disease had been suspected to be transmissible for some time: an early report9 of disease transmission to hamsters through white blood cells from people with Alzheimer's disease caused great consternation, but was never reproduced. Much more tantalizing evidence came from the discovery10,11 that aggregates of the amyloid-â (Aâ) peptide found in the brain of people with Alzheimer's disease could be transmitted to the brain of mice engineered to produce large amounts of the Aâ precursor protein APP. Another study12 has shown that healthy tissue grafted into the brain of people with Parkinson's disease acquires intracellular Lewy bodies - aggregates of the Parkinson's disease-associated protein á-synuclein. This suggests prion-like transmission of diseased protein from the recipient's brain to the grafted cells.
These findings10-12 raise a provocative question. If protein aggregation depends on the introduction of 'seeds' and on the availability of the monomeric precursor, and if, as has been suggested13, amyloid represents the primordial state of all proteins, wouldn't all proteins - under appropriate conditions - behave like prions in the presence of sufficient precursor? Acceptance of this concept is gaining momentum. For one thing, an increasing wealth of traits is being found in yeast, fungi and bacteria that can best be explained as prion-like phenomena (see table). And now, Ren and colleagues3 provide evidence for prion-like spread of polyglutamine (polyQ)- containing protein aggregates, which are similar to the aggregates found in Huntington's disease. They show that polyQ aggregates can be taken up from the outside by mammalian cells. Once in the cytosol, the polyQ aggregates can grow by recruiting endogenous polyQ. Clavaguera et al.4 report similar findings in a mouse model of tauopathy, a neurodegenerative disease caused by intraneuronal aggregation of the microtubule-associated tau protein. Injection of mutant human tau into the brain of mice overexpressing normal human tau transmitted tauopathy, with intracellular aggregation of previously normal tau and spread of aggregates to neighbouring regions of the brain. Notably, full-blown tauopathy was not induced in mice that did not express human tau. Assuming that tau pathology wasn't elicited by some indirect pathway (some mice overexpressing mutated human tau develop protein tangles even when exposed to un related amyloid aggregates14), this sequence of events is reminiscent of prions. Finally, Frost and colleagues5 show that extracellular tau aggregates can be taken up by cells in culture. Hence, tau can attack and penetrate cells from the outside, sporting predatory behaviour akin to that of prions.
Yet there is one crucial difference between actual prion diseases and diseases caused by other prion-like proteins (let's call them prionoids) described so far (see table). The behaviour of prions is entirely comparable to that of any other infectious agent: for instance, prions are transmissible between individuals and often across species, and can be assayed with classic microbiological techniques, including titration by bioassay. Accordingly, prion diseases were long thought to be caused by viruses, and BSE created a worldwide panic similar to that currently being provoked by influenza. By contrast, although prionoids can 'infect' neighbouring molecules and sometimes even neighbouring cells, they do not spread within communities or cause epidemics such as those seen with BSE.
So, should any amyloid deserve an upgrade to a bone fide prion status? Currently, amyloid A (AA) amyloidosis may be the most promising candidate for a truly infectious disease caused by a self-propagating protein other than PrPSc. AA amyloid consists of orderly aggregated fragments of the SAA protein, and its deposition damages many organs of the body. Seeds of AA amyloid can be excreted in faeces15, and can induce amyloidosis if taken up orally (at least in geese)16. Also, AA amyloid may be transmitted between mice by transfusion of white blood cells17. So, like entero viruses and, perhaps, sheep scrapie prions, AA amyloid seems to display all the elements of a complete infectious life cycle, including uptake, replication and release from its host.
There are intriguing evolutionary implications to the above findings. If prionoids are ubiquitous, why didn't evolution erect barriers to their pervasiveness? Maybe it is because the molecular transmissibility of aggregated states can sometimes be useful. Indeed, aggregation of the Sup35 protein, which leads to a prion-like phenomenon in yeast, may promote evolutionary adaptation by allowing yeast cells to temporarily activate DNA sequences that are normally untranslated18. Mammals have developed receptors for aggregates, and ironically PrPC may be one of them19, although these receptors have not been reported to mediate protective functions. Therefore, we shouldn't be shocked if instances of beneficial prionoids emerge in mammals as well. ¦
Adriano Aguzzi is at the Institute of Neuropathology, University Hospital of Zurich, CH-8091 Zurich, Switzerland. e-mail: adriano.aguzzi@usz.ch
1. Griffith, J. S. Nature 215, 1043-1044 (1967). 2. Prusiner, S. B. Science 216, 136-144 (1982). 3. Ren, P.-H. et al. Nature Cell Biol. 11, 219-225 (2009). 4. Clavaguera, F. et al. Nature Cell Biol. doi:10.1038/ncb1901 (2009). 5. Frost, B., Jacks, R. L. & Diamond, M. I. J. Biol. Chem. 284, 12845-12852 (2009). 6. Castilla, J., Saá, P., Hetz, C. & Soto, C. Cell 121, 195-206 (2005). 7. Sigurdson, C. J. et al. Proc. Natl Acad. Sci. USA 106, 304-309 (2009). 8. Aguzzi, A. & Polymenidou, M. Cell 116, 313-327 (2004). 9. Manuelidis, E. E. et al. Proc. Natl Acad. Sci. USA 85, 4898-4901 (1988). 10. Kane, M. D. et al. J. Neurosci. 20, 3606-3611 (2000). 11. Meyer-Luehmann, M. et al. Science 313, 1781-1784 (2006). 12. Li, J.-Y. et al. Nature Med. 14, 501-503 (2008). 13. Chiti, F. & Dobson, C. M. Annu. Rev. Biochem. 75, 333-366 (2006). 14. GÖtz, J., Chen, F., van Dorpe, J. & Nitsch, R. M. Science 293, 1491-1495 (2001). 15. Zhang, B. et al. Proc. Natl Acad. Sci. USA 105, 7263-7268 (2008). 16. Solomon, A. et al. Proc. Natl Acad. Sci. USA 104, 10998-11001 (2007). 17. Sponarova, J., NystrÖm, S. N. & Westermark, G. T. PLoS ONE 3, e3308 (2008). 18. True, H. L. & Lindquist, S. L. Nature 407, 477-483 (2000). 19. Laurén, J. et al. Nature 457, 1128-1132 (2009).
PRIONS AND POTENTIAL PRIONOIDS
Disease Protein Molecular transmissibility Infectious life cycle Prion diseases PrPSc Yes Yes Alzheimer's disease Amyloid-ß Yes Not shown Tauopathies Tau Yes Not shown Parkinson's disease a-Synuclein Host-to-graft Not shown AA amyloidosis Amyloid A Yes Possible Huntington's disease Polyglutamine Yes Not shown Phenotype Protein Molecular transmissibility Infectious life cycle Suppressed translational termination (yeast) Sup35 Yes Not shown Heterokaryon incompatibility (filamentous fungi) Het-s Yes Not shown Biofilm promotion (bacteria) CsgA Yes Not shown In humans and animals, infectious prion diseases are caused by PrPSc, which spreads by recruiting its monomeric precursor PrPC into aggregates. Aggregates then multiply by breakage, a process that is termed molecular transmissibility. Other proteins involved in disease and in phenotypes of fungi and bacteria, can also undergo self-sustaining aggregation, but none of these 'prionoid' proteins behaves like typical infectious agents, nor do any of them enact a complete infectious life cycle - with the possible exception of AA amyloid. Correction In the News & Views article "Immunology: Immunity's ancient arms" by Gary W. Litman and John P. Cannon (Nature 459, 784-786; 2009), the name of the fi rst author of the Nature paper under discussion was misspelt. The author's name is P. Guo, not Gou as published.
© 2009 Macmillan Publishers Limited. All rights reserved
http://www.nature.com/nature/journal/v459/n7249/full/459924a.html
http://betaamyloidcjd.blogspot.com/2009/10/involvement-of-dab1-in-app-processing.html
Thursday, February 26, 2009
'Harmless' prion protein linked to Alzheimer's disease Non-infectious form of prion protein could cause brain degeneration ???
http://betaamyloidcjd.blogspot.com/2009/02/harmless-prion-protein-linked-to.html
Saturday, March 22, 2008
10 Million Baby Boomers to have Alzheimer's in the coming decades 2008 Alzheimer's disease facts and figures
http://betaamyloidcjd.blogspot.com/2008/03/association-between-deposition-of-beta.html
Alzheimer's and CJD
http://betaamyloidcjd.blogspot.com/
Saturday, January 2, 2010
Human Prion Diseases in the United States January 1, 2010 ***FINAL***
http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html
Friday, January 01, 2010
Human Prion Diseases in the United States PART 1
http://creutzfeldt-jakob-disease.blogspot.com/2010/01/human-prion-diseases-in-united-states.html
my comments to PLosone here ;
http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd
TSS
CTV.ca News Staff
Date: Mon. Jan. 4 2010 9:52 AM ET
So many Canadians are expected to develop Alzheimer's disease and dementia in the next 30 years that a new case will be diagnosed every two minutes unless preventive measures are taken, a new report says.
The report, released Monday by the Alzheimer Society, says the prevalence of dementia will more than double in the next 30 years.
By 2038, almost three per cent of Canada's population will be affected by dementia, and about 257,800 new cases will be diagnosed per year.
Today, dementia costs Canada about $15 billion a year; those costs could soon increase by 10-fold.
"If nothing changes, this sharp increase in the number of people living with dementia will mean that by 2038, the total costs associated with dementia will reach $153 billion a year," David Harvey, principal spokesperson for the Alzheimer Society project called "Rising Tide: The Impact of Dementia on Canadian Society," said in a statement.
That amounts to a cumulative total of $872 billion over the 30-year period.
Much of the increase in cases can be attributed to the "greying" of Canada. With Canadians living longer and baby boomers aging, there is expected to be a spike in many chronic diseases that come with age, such as heart disease, arthritis and cancer.
But the expected rising rates of dementia are not just about demographics; poor lifestyles also play a role.
It's been well documented that regular physical and mental exercise can delay the onset of dementia, which includes Alzheimer's disease and other progressive diseases that destroy brain cells. For that reason, the report recommends that all Canadians over 65 without dementia increase their physical activity by 50 per cent.
"Prevention is where we need to be starting," Harvey told Canada AM.
"We know that healthy eating and active living are antidotes to dementia."
The "Rising Tide" report calls on government to fund more health promotion to remind Canadians of the benefits of a healthy lifestyle.
"This intervention would reduce the number of people diagnosed with dementia, resulting in a reduction in the pressure on long-term care facilities, community care services and informal caregivers," the report says.
Need for national strategy
Just as important, Harvey says, is the need for Canada's health care system to adapt to accommodate the projected rise in dementia cases.
"Dementia is one of the leading cases of disability amongst older people," Harvey said, noting that the flood of dementia expected in the next 30 years could overwhelm emergency rooms and hospitals.
His group's report calls for more support for informal caregivers -- generally, family members -- who tend to be the ones who care for patients with dementia in the early stages of the disease.
"There are services that can be put in place to support caregivers, and also economic and financial support for caregivers," he said.
By also providing caregivers with skill-building and support programs, caregivers struggling with the overwhelming emotional and financial hardships of providing care may feel better equipped to care for their loved one.
That could go far to delay admission of patients into long-term care facilities, thereby lessening the burden on the health care system.
The report also suggests assigning "system navigators" to each newly diagnosed dementia patient and their caregivers. These case managers would help families navigate the health system to find the right social services for their loved one depending on their stage of dementia.
--------------------------------------------------------------------------------
Some facts about dementia:
The symptoms of dementia include a gradual and continuing decline of memory, changes in judgment or reasoning, mood and behaviour, and an inability to perform familiar tasks. Dementia can strike adults at any age, but has traditionally been diagnosed in people over 65. However, symptoms start much earlier, and an increasing number of people are being diagnosed in their 50s and early 60s. Age is the number one risk factor for dementia Alzheimer's disease, the most common form of dementia, accounts for approximately 64 per cent of all dementias in Canada. Other related dementias include Vascular Dementia, Frontotemporal Dementia, Creutzfeldt-Jakob Disease and Lewy body Dementia. There is no known cure for dementia. However, some medications can delay progression of the disease. Researchers are confident that within five to seven years, there will be treatments that attack the disease process itself, not just the symptoms.
http://www.ctv.ca/servlet/ArticleNews/story/CTVNews/20100104/dementia_surge_100104/20100104?hub=Health
Report Summary Rising Tide: The Impact of Dementia in Canada In this section :
Read a summary of Rising Tide: The Impact of Dementia in Canada
Download Rising Tide: The Impact of Dementia in Canada Rising Tide: the Impact of Dementia on Canadian Society is the final report of an Alzheimer Society project funded by Pfizer Canada, Health Canada, Public Health Agency of Canada, Canadian Institutes of Health Research and Rx&D. The purpose of the report was to:
Estimate the health and economic burden of dementia in Canada over the next 30 years; Analyze the possible effects of intervention scenarios upon this burden; Demonstrate how the proposed interventions could affect the health and economic impacts of dementia in Canada; Review policy options; Make recommendations on how to address the issue. The Findings of Rising Tide1 Health Burden of Dementia for Canada: 2008-2038²
Incidence of Alzheimer's disease and related dementias in Canada: 2008 - 103,700 new cases per year (1 every 5 minutes) 2038 - 257,800 new cases per year (1 every 2 minutes) Prevalence of Alzheimer's disease and related dementias in Canada: 2008 - 480,600 people with dementia (1.5% of Canada's population) 2038 - 1,125,200 people with dementia (2.8% of Canada's population) View the prevalence of dementia by age graph
View the prevalence of dementia by sex graph
Hours of informal care provided annually for people with dementia in Canada 2008 - 231 million hours 2038 - 756 million hours Economic Consequences of Dementia for Canada: 2008-2038²
The Economic Burden of dementia doubles every decade, increasing from $15 billion in 2008 to a startling $153 billion in 2038.
Economic Burden of Dementia (in future dollars) 2008 - $15 billion 2018 - $37 billion 2028 - $75 billion 2038 - $153 billion
Cumulative Consequences of Dementia over a 30-year period
Cumulative data represents the combined total of either the economic costs of dementia per year, or the number of people developing dementia per year, each year between 2008 and 2038. By 2038, the cumulative incidence of dementia will be more than 5.5 million people³, with a cumulative economic cost of $872 billion² (2008 dollars).
Implications – What can Canada do? What Has Been Done Elsewhere
Across the globe, many countries are recognizing the urgent issue of dementia. Australia, Norway, the Netherlands, France, Scotland and the United Kingdom have recently developed specific plans or frameworks for dealing with dementia.
View Alzheimer Disease International's graphs correlating research effort with contributions to mortality and disability.
Intervention Opportunities
Recognizing the urgent need to start turning the tide of dementia, Rising Tide describes four potential intervention scenarios, backed by current evidence that could become critical factors in reducing the impact of dementia.
The report tested the impact of four potential intervention scenarios:
Increasing Physical Activity Delay Onset of Dementia Caregiver Training, Support System Navigation All showed the potential for dramatic reductions in economic impact over the next 30 years.
Note: Rising Tide was undertaken in order to alert the Canadian public and federal, provincial and territorial politicians of the need for policies and approaches to address the looming dementia crisis. In the reports, you will find four suggested interventions. They are not meant to be definitive but to serve as illustrations of how the base case can be used to inform and shape policy in this field. The 5 recommendations in the report were developed through a comprehensive process of consultations with subject experts and stakeholders. The underlying message is that we must act now and that change is possible.
Recommendations
Rising Tide also makes five recommendations that would make up the components of a comprehensive National Dementia Strategy. They include:
An accelerated investment in all areas of dementia research. A clear recognition of the important role played by informal caregivers. An increased recognition of the importance of prevention and early intervention. Greater integration of care and increased use of chronic disease prevention and management. A strengthening of Canada's dementia workforce.
Download a copy of Rising Tide: The Impact of Dementia on Canadian Society.
Endnotes
Rising Tide: Impact of Dementia on Canadian Society is a report based on a study conducted by RiskAnalytica, a leading firm in risk management. RiskAnalytica's Life at Risk® simulation platform was customized for the Rising Tide study based on the latest dementia and health economic research, validated for epidemiological and economic aspects by subject matter experts and checked for data, logic and results. The simulation platform was then run to establish the Base Case, or the findings. Rising Tide: The Impact of Dementia on Canadian Society. Alzheimer Society, 2009. Smetanin, P., Kobak, P., Briante, C., Stiff, D., Sherman, G., and Ahmad, S. Rising Tide: The Impact of Dementia in Canada 2008 to 2038. RiskAnalytica, 2009.
http://www.alzheimer.ca/english/rising_tide/rising_tide_summary.htm
http://www.alzheimer.ca/english/rising_tide/rising_tide_report.htm
SEE FULL REPORT HERE ;
Rising Tide:
The Impact of Dementia on Canadian Society
Executive Summary
http://www.alzheimer.ca/docs/RisingTide/AS%20Rising%20Tide-Executive%20Summary_Eng_FINAL_SecuredVersion.pdf
Saturday, October 31, 2009
Involvement of Dab1 in APP processing and ß-amyloid deposition in sporadic Creutzfeldt–Jakob patients
http://betaamyloidcjd.blogspot.com/2009/10/involvement-of-dab1-in-app-processing.html
SEAC OCTOBER 2009
. Are some commoner types of neurodegenerative disease (including Alzheimer's disease and Parkinson's disease) also transmissible? Some recent scientific research has suggested this possibility
http://www.seac.gov.uk/pdf/hol-response091008.pdf
Thursday, February 26, 2009
'Harmless' prion protein linked to Alzheimer's disease Non-infectious form of prion protein could cause brain degeneration ???
http://betaamyloidcjd.blogspot.com/2009/02/harmless-prion-protein-linked-to.html
CJD1/9 0185
Ref: 1M51A
IN STRICT CONFIDENCE
TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES
1. CMO will wish to be aware that a meeting was held at DH yesterday, 4 January, to discuss the above findings. It was chaired by Professor Murray (Chairman of the MRC Co-ordinating Committee on Research in the Spongiform Encephalopathies in Man), and attended by relevant experts in the fields of Neurology, Neuropathology, molecular biology, amyloid biochemistry, and the spongiform encephalopathies, and by representatives of the MRC and AFRC.
2. Briefly, the meeting agreed that:
i) Dr Ridley et als findings of experimental induction of p amyloid in primates were valid, interesting and a significant advance in the understanding of neurodegeneradve disorders;
ii) there were no immediate implications for the public health, and no further safeguards were thought to be necessary at present; and
iii) additional research was desirable, both epidemiological and at the molecular level. Possible avenues are being followed up by DH and the MRC, but the details will require further discussion.
93/01.05/4.1tss
http://web.archive.org/web/20010305223440/www.bseinquiry.gov.uk/files/yb/1993/01/05004001.pdf
Regarding Alzheimer's disease
(note the substantial increase on a yearly basis)
http://web.archive.org/web/20010305222847/www.bseinquiry.gov.uk/files/yb/1988/07/08014001.pdf
snip...
The pathogenesis of these diseases was compared to Alzheimer's disease at a molecular level...
snip...
http://web.archive.org/web/20010305223234/www.bseinquiry.gov.uk/files/yb/1990/03/12003001.pdf
And NONE of this is relevant to BSE?
There is also the matter whether the spectrum of ''prion disease'' is wider than that recognized at present.
http://web.archive.org/web/20010305223234/www.bseinquiry.gov.uk/files/yb/1990/07/06005001.pdf
http://web.archive.org/web/20010305223234/www.bseinquiry.gov.uk/files/yb/1990/07/09001001.pdf
BSE101/1 0136
IN CONFIDENCE
5 NOV 1992
CMO From: Dr J S Metters DCMO 4 November 1992
TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES
http://web.archive.org/web/20010305223143/www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf
also, see the increase of Alzheimer's from 1981 to 1986
http://web.archive.org/web/20010305222847/www.bseinquiry.gov.uk/files/yb/1988/07/08014001.pdf
Tuesday, August 26, 2008
Alzheimer's Transmission of AA-amyloidosis: Similarities with Prion Disorders NEUROPRION 2007 FC4.3
http://betaamyloidcjd.blogspot.com/2008/08/alzheimers-transmission-of-aa.html
see full text ;
http://betaamyloidcjd.blogspot.com/2009/02/harmless-prion-protein-linked-to.html
Saturday, October 31, 2009
Involvement of Dab1 in APP processing and ß-amyloid deposition in sporadic Creutzfeldt–Jakob patients Copyright © 2009 Published by Elsevier Inc.
http://betaamyloidcjd.blogspot.com/2009/10/involvement-of-dab1-in-app-processing.html
----- Original Message -----
From: "Terry S. Singeltary Sr." To: Sent: Monday, October 12, 2009 9:47 AM Subject: [BSE-L] SEAC Science and Technology Committee's investigation of research funding priorities on behalf of the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy
-------------------- BSE-L@LISTS.AEGEE.ORG --------------------
snip...
. More specific examples of unanswered questions with health implications are:
. Will the eventual elimination of classical scrapie in the EU leave an ecological niche for other TSEs such as BSE or atypical scrapie?
. Is CWD transmissible to humans?
. Can a reliable ante mortem diagnostic blood test for vCJD be developed?
. What is the true prevalence of v CJD infection (as opposed to overt disease) in the UK?
XXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXX
--------------------------------------------------------------------------------
. Are some commoner types of neurodegenerative disease (including Alzheimer's disease and Parkinson's disease) also transmissible? Some recent scientific research has suggested this possibility
--------------------------------------------------------------------------------
XXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXXX
. Could cases of protease sensitive prionopathy (PSP) be missed by conventional tests which, in all other TSEs, rely on the resistance of the prion protein in the nervous system that accompanies disease to digestion by protease enzymes?
. Can we develop reliable methods for removing and detecting protein on re-usable surgical instruments?
SNIP...
FULL TEXT ;
Monday, October 12, 2009
SEAC Science and Technology Committee's investigation of research funding priorities on behalf of the Advisory Committee on Dangerous Pathogens TSE 8 October 2009
http://bse-atypical.blogspot.com/2009/10/seac-science-and-technology-committees.html
Tuesday, August 26, 2008
Alzheimer's Transmission of AA-amyloidosis: Similarities with Prion Disorders NEUROPRION 2007 FC4.3
http://betaamyloidcjd.blogspot.com/2008/08/alzheimers-transmission-of-aa.html
----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Monday, June 29, 2009 2:08 PM
Subject: [BSE-L] Beyond the prion principle
-------------------- BSE-L@LISTS.AEGEE.ORG --------------------
News and Views Nature 459, 924-925 (18 June 2009) doi:10.1038/459924a; Published online 17 June 2009
CELL BIOLOGY
Beyond the prion principle
Adriano Aguzzi
It seems that many misfolded proteins can act like prions - spreading disease by imparting their misshapen structure to normal cellular counterparts. But how common are bona fide prions really?
The protein-only hypothesis of prion propagation is steadily gaining ground. First envisaged by John Stanley Griffith1 and later formalized by Stanley Prusiner2, this theory proposes the existence of an infectious agent composed solely of protein. Three reports, two in Nature Cell Biology3,4 and one in The Journal of Cell Biology5, now contend that, far from being confined to the rare prion diseases, prion-like transmission of altered proteins may occur in several human diseases of the brain and other organs.
Prions are now accepted as causing the transmissible spongiform encephalopathies, which include scrapie in sheep, bovine spongiform encephalopathy (BSE, or mad cow disease) and its human variant Creutzfeldt-Jakob disease. The infectious prion particle is made up of PrPSc, a misfolded and aggregated version of a normal protein known as PrPC. Like the growth of crystals, PrPSc propagates by recruiting monomeric PrPC into its aggregates - a process that has been replicated in vitro6 and in transgenic mice7. The breakage of PrPSc aggregates represents the actual replicative event, as it multiplies the number of active seeds8.
Apart from prion diseases, the misfolding and aggregation of proteins into various harmful forms, which are collectively known as amyloid, causes a range of diseases of the nervous system and other organs. The clinical characteristics of amyloidoses, however, gave little reason to suspect a relationship to prion diseases. Hints of prion-like behaviour in amyloid have emerged from studies of Alzheimer's disease and Parkinson's disease. Alzheimer's disease had been suspected to be transmissible for some time: an early report9 of disease transmission to hamsters through white blood cells from people with Alzheimer's disease caused great consternation, but was never reproduced. Much more tantalizing evidence came from the discovery10,11 that aggregates of the amyloid-â (Aâ) peptide found in the brain of people with Alzheimer's disease could be transmitted to the brain of mice engineered to produce large amounts of the Aâ precursor protein APP. Another study12 has shown that healthy tissue grafted into the brain of people with Parkinson's disease acquires intracellular Lewy bodies - aggregates of the Parkinson's disease-associated protein á-synuclein. This suggests prion-like transmission of diseased protein from the recipient's brain to the grafted cells.
These findings10-12 raise a provocative question. If protein aggregation depends on the introduction of 'seeds' and on the availability of the monomeric precursor, and if, as has been suggested13, amyloid represents the primordial state of all proteins, wouldn't all proteins - under appropriate conditions - behave like prions in the presence of sufficient precursor? Acceptance of this concept is gaining momentum. For one thing, an increasing wealth of traits is being found in yeast, fungi and bacteria that can best be explained as prion-like phenomena (see table). And now, Ren and colleagues3 provide evidence for prion-like spread of polyglutamine (polyQ)- containing protein aggregates, which are similar to the aggregates found in Huntington's disease. They show that polyQ aggregates can be taken up from the outside by mammalian cells. Once in the cytosol, the polyQ aggregates can grow by recruiting endogenous polyQ. Clavaguera et al.4 report similar findings in a mouse model of tauopathy, a neurodegenerative disease caused by intraneuronal aggregation of the microtubule-associated tau protein. Injection of mutant human tau into the brain of mice overexpressing normal human tau transmitted tauopathy, with intracellular aggregation of previously normal tau and spread of aggregates to neighbouring regions of the brain. Notably, full-blown tauopathy was not induced in mice that did not express human tau. Assuming that tau pathology wasn't elicited by some indirect pathway (some mice overexpressing mutated human tau develop protein tangles even when exposed to un related amyloid aggregates14), this sequence of events is reminiscent of prions. Finally, Frost and colleagues5 show that extracellular tau aggregates can be taken up by cells in culture. Hence, tau can attack and penetrate cells from the outside, sporting predatory behaviour akin to that of prions.
Yet there is one crucial difference between actual prion diseases and diseases caused by other prion-like proteins (let's call them prionoids) described so far (see table). The behaviour of prions is entirely comparable to that of any other infectious agent: for instance, prions are transmissible between individuals and often across species, and can be assayed with classic microbiological techniques, including titration by bioassay. Accordingly, prion diseases were long thought to be caused by viruses, and BSE created a worldwide panic similar to that currently being provoked by influenza. By contrast, although prionoids can 'infect' neighbouring molecules and sometimes even neighbouring cells, they do not spread within communities or cause epidemics such as those seen with BSE.
So, should any amyloid deserve an upgrade to a bone fide prion status? Currently, amyloid A (AA) amyloidosis may be the most promising candidate for a truly infectious disease caused by a self-propagating protein other than PrPSc. AA amyloid consists of orderly aggregated fragments of the SAA protein, and its deposition damages many organs of the body. Seeds of AA amyloid can be excreted in faeces15, and can induce amyloidosis if taken up orally (at least in geese)16. Also, AA amyloid may be transmitted between mice by transfusion of white blood cells17. So, like entero viruses and, perhaps, sheep scrapie prions, AA amyloid seems to display all the elements of a complete infectious life cycle, including uptake, replication and release from its host.
There are intriguing evolutionary implications to the above findings. If prionoids are ubiquitous, why didn't evolution erect barriers to their pervasiveness? Maybe it is because the molecular transmissibility of aggregated states can sometimes be useful. Indeed, aggregation of the Sup35 protein, which leads to a prion-like phenomenon in yeast, may promote evolutionary adaptation by allowing yeast cells to temporarily activate DNA sequences that are normally untranslated18. Mammals have developed receptors for aggregates, and ironically PrPC may be one of them19, although these receptors have not been reported to mediate protective functions. Therefore, we shouldn't be shocked if instances of beneficial prionoids emerge in mammals as well. ¦
Adriano Aguzzi is at the Institute of Neuropathology, University Hospital of Zurich, CH-8091 Zurich, Switzerland. e-mail: adriano.aguzzi@usz.ch
1. Griffith, J. S. Nature 215, 1043-1044 (1967). 2. Prusiner, S. B. Science 216, 136-144 (1982). 3. Ren, P.-H. et al. Nature Cell Biol. 11, 219-225 (2009). 4. Clavaguera, F. et al. Nature Cell Biol. doi:10.1038/ncb1901 (2009). 5. Frost, B., Jacks, R. L. & Diamond, M. I. J. Biol. Chem. 284, 12845-12852 (2009). 6. Castilla, J., Saá, P., Hetz, C. & Soto, C. Cell 121, 195-206 (2005). 7. Sigurdson, C. J. et al. Proc. Natl Acad. Sci. USA 106, 304-309 (2009). 8. Aguzzi, A. & Polymenidou, M. Cell 116, 313-327 (2004). 9. Manuelidis, E. E. et al. Proc. Natl Acad. Sci. USA 85, 4898-4901 (1988). 10. Kane, M. D. et al. J. Neurosci. 20, 3606-3611 (2000). 11. Meyer-Luehmann, M. et al. Science 313, 1781-1784 (2006). 12. Li, J.-Y. et al. Nature Med. 14, 501-503 (2008). 13. Chiti, F. & Dobson, C. M. Annu. Rev. Biochem. 75, 333-366 (2006). 14. GÖtz, J., Chen, F., van Dorpe, J. & Nitsch, R. M. Science 293, 1491-1495 (2001). 15. Zhang, B. et al. Proc. Natl Acad. Sci. USA 105, 7263-7268 (2008). 16. Solomon, A. et al. Proc. Natl Acad. Sci. USA 104, 10998-11001 (2007). 17. Sponarova, J., NystrÖm, S. N. & Westermark, G. T. PLoS ONE 3, e3308 (2008). 18. True, H. L. & Lindquist, S. L. Nature 407, 477-483 (2000). 19. Laurén, J. et al. Nature 457, 1128-1132 (2009).
PRIONS AND POTENTIAL PRIONOIDS
Disease Protein Molecular transmissibility Infectious life cycle Prion diseases PrPSc Yes Yes Alzheimer's disease Amyloid-ß Yes Not shown Tauopathies Tau Yes Not shown Parkinson's disease a-Synuclein Host-to-graft Not shown AA amyloidosis Amyloid A Yes Possible Huntington's disease Polyglutamine Yes Not shown Phenotype Protein Molecular transmissibility Infectious life cycle Suppressed translational termination (yeast) Sup35 Yes Not shown Heterokaryon incompatibility (filamentous fungi) Het-s Yes Not shown Biofilm promotion (bacteria) CsgA Yes Not shown In humans and animals, infectious prion diseases are caused by PrPSc, which spreads by recruiting its monomeric precursor PrPC into aggregates. Aggregates then multiply by breakage, a process that is termed molecular transmissibility. Other proteins involved in disease and in phenotypes of fungi and bacteria, can also undergo self-sustaining aggregation, but none of these 'prionoid' proteins behaves like typical infectious agents, nor do any of them enact a complete infectious life cycle - with the possible exception of AA amyloid. Correction In the News & Views article "Immunology: Immunity's ancient arms" by Gary W. Litman and John P. Cannon (Nature 459, 784-786; 2009), the name of the fi rst author of the Nature paper under discussion was misspelt. The author's name is P. Guo, not Gou as published.
© 2009 Macmillan Publishers Limited. All rights reserved
http://www.nature.com/nature/journal/v459/n7249/full/459924a.html
http://betaamyloidcjd.blogspot.com/2009/10/involvement-of-dab1-in-app-processing.html
Thursday, February 26, 2009
'Harmless' prion protein linked to Alzheimer's disease Non-infectious form of prion protein could cause brain degeneration ???
http://betaamyloidcjd.blogspot.com/2009/02/harmless-prion-protein-linked-to.html
Saturday, March 22, 2008
10 Million Baby Boomers to have Alzheimer's in the coming decades 2008 Alzheimer's disease facts and figures
http://betaamyloidcjd.blogspot.com/2008/03/association-between-deposition-of-beta.html
Alzheimer's and CJD
http://betaamyloidcjd.blogspot.com/
Saturday, January 2, 2010
Human Prion Diseases in the United States January 1, 2010 ***FINAL***
http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html
Friday, January 01, 2010
Human Prion Diseases in the United States PART 1
http://creutzfeldt-jakob-disease.blogspot.com/2010/01/human-prion-diseases-in-united-states.html
my comments to PLosone here ;
http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd
TSS
Subscribe to:
Posts (Atom)