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From: TSS ()
News & Events - 6 December 2006 Researchers at the Universities of Edinburgh, St Andrews, Bristol and California have identified a key protein which can build up in the nerve cells of a cat's brain and cause mental deterioration. In humans with Alzheimer's disease, this protein creates ‘tangles’ inside the nerve cells which inhibit messages being processed by the brain. The team says that the presence of this protein in cats is proof that they too can develop this type of disease. By carrying out post-mortem examination of cats which have succumbed naturally to the disease, scientists may now be able to uncover vital clues about how the condition develops. This may eventually help scientists to come up with possible treatments. Researchers already thought cats were susceptible to dementia because previous studies had identified thick, gritty plaques on the outside of elderly cats' brain cells which are similar to those found in humans. By pinpointing this second key marker, the Edinburgh-led team says we can be sure that cats can suffer from a feline form of Alzheimer's. Dr Danielle Gunn-Moore, at the University of Edinburgh's Royal (Dick) School of Veterinary Studies, said: “This newly discovered protein is crucial to our understanding of the ageing process in cats. We've known for a long time that cats develop dementia, but this study tells us that the cat's neural system is being compromised in a similar fashion to that we see in human Alzheimer's sufferers. The gritty plaques had only hinted that might be the case - now we know. “The shorter life-span of a cat, compared to humans, allows researchers to more rapidly assess the effects of diet, high blood pressure, and prescribed drugs on the course of the disease. However, we also need to understand more about our geriatric cats for their own benefit, so we can slow down the degeneration the disease brings and keep them as happy cats for as long as possible.” The findings of the study are published in a recent edition of the Journal of Feline Medicine. http://www.ed.ac.uk/news/061206cats.html Late onset cerebellar degeneration in a middle-aged cat Arianna Negrin DVM1, , , Marco Bernardini DVM, Dipl ECVN2, Wolfgang Baumgärtner Dr Med Vet, PhD, Dipl ECVP3 and Massimo Castagnaro DVM, PhD, Dipl ECVP1 1Department of Public Health, Comparative Pathology and Veterinary Hygiene, Faculty of Veterinary Medicine, Università degli Studi di Padova, Viale dell'Università, 16, 35020 Legnaro, Padova, Italy Accepted 24 April 2006. Available online 15 June 2006. http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6WJC-4K66F8J-2&_user=10&_handle=V-WA-A-W-AC-MsSAYZW-UUA-U-AAZDEDCEDY-AAZCCCZDDY-AWYBZZADA-AC-U&_fmt=summary&_coverDate=12%2F31%2F2006&_rdoc=10&_orig=browse&_srch=%23toc%236875%232006%23999919993%23636973!&_cdi=6875&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=9cb579124f38fbfaa6dd03422d3bd9ac Neuropathological study of cerebellar degeneration in prion disease http://www.blackwell-synergy.com/links/doi/10.1046/j.1440-1789.1999.00212.x A 48-year-old woman was admitted to our hospital with a 1-month history of lethargy, loss of appetite, and poor mental performance. On admission, she exhibited inattentiveness, lack of fluent speech, tremor, and dystonic movement of the right hand. Her medical and family history were unremarkable. Neurologic examination showed her to be alert but with mutism. Her mental performance was characterized by poor calculative abilities and sluggish judgment as to person and place. Muscle strength was normal, but with mild axial rigidity. The deep tendon reflexes were increased, but the Babinski sign was not present. A wide-based, shuffling gait was observed. Cerebrospinal fluid analysis was normal. An electroencephalogram (EEG) showed a generalized pattern of theta-delta background slowing activity and periodic synchronized sharp 1/s delta-wave discharge. Findings at cranial computed tomography (CT) were unremarkable (Fig 1A). The initial impression was dementia of unknown origin. Two months later, the patient’s condition had deteriorated to global aphasia and she was bedridden. Myoclonic jerk was present and muscular rigidity developed in all limbs. There was muscle wasting, followed by difficulty in swallowing and incontinence of urination. Initial, noncontrast MR images revealed a subtle increase in signal in the heads of the caudate nuclei, putamina, and thalami on T2-weighted images (Fig 1B–E). An open brain biopsy of the right temporal lobe performed just after MR imaging revealed severe loss of neurons, hypertrophic glial reaction, and vacuolar-spongiform changes in the gray matter (Fig 1F). One year later, the patient was in a vegetative state with poor respiratory control. Noncontrast T2-weighted MR images obtained at this time revealed severe cerebral and cerebellar atrophy, prominent dilatation of the ventricular system, and a global increase in signal intensity in the white matter with shrinkage of the basal ganglia (Fig 1G–J). One and a half years after admission, she was discharged to a chronic-care unit, where she remained in a vegetative state. She died 8 months later. snip... http://www.ajnr.org/cgi/reprint/18/3/583.pdf PATHOLOGY OF PRION DISEASES Creutzfeldt-Jakob disease (CJD) is the most common prion disease of humans, but overall is a rare disease, affecting one in every million people. It affects middle aged or old persons and causes dementia, myoclonus, ataxia and other neurological abnormalities. This phenotype, also known as the Heidenhain variant, is seen in 70% of sporadic CJD. The other 30% have less typical features. The EEG shows perodic sharp wave complexes. CJD is inexorably progressive and fatal within months up to 1 to 2 years. A definitive diagnosis can only be made by microscopic examination of brain tissue showing the characteristic spongy change. Abnormal prions can be detected in brain tissue extracts by ELISA and in tissue sections by immunohistochemistry. A protein called 14-3-3 is elevated in the CSF of patients with CJD. Microtubule associated protein tau was also recently shown to be elevated in the CSF, including in the new variant (see below) and iatrogenic CJD cases in which 14-3-3 remains normal. Determination of tau can be done in routine hospital laboratories. Detection of these proteins provides much needed premortem diagnostic tests for CJD. Both, tau and 14-3-3, are also elevated in other neurological diseases (tau most notably in Alzheimer's disease) but these can be clinically distinguished from CJD. For this reason they are helpful only when the differential diagnosis has been narrowed down and more common neurological conditions such as Alzheimer's disease, stroke, HIV encephalitis, etc, have been excluded. Mutations of the PRNP gene and the codon 129 polymorphism can be detected by DNA analysis of blood and tissues. http://www.neuropathologyweb.org/chapter5/chapter5ePrions.html more here; http://www.google.com/search?num=30&hl=en&lr=&as_qdr=d&edition=us&q=cerebellar+degeneration+cjd&btnG=Search Statement FOR IMMEDIATE RELEASE Media Inquiries: 301-827-6242 The Food and Drug Administration (FDA) has learned from the government It is also important to stress that there is no scientific evidence to FDA notified the U.S. pet food firm, The Pet Pantry International, of The Pet Pantry products were packaged in 50 lb bags, distributed to The product subject to this notification includes Maintenance Diet FDA is working closely with the Pet Pantry International to assure for FDA will continue to provide updates on this case of BSE in Canada as http://www.fda.gov/bbs/topics/NEWS/2003/NEW0910.html It was thought likely that at least some, and probably all, of the cases snip... http://www.bseinquiry.gov.uk/files/ws/s324.pdf cases have been reported in domestic cats), are characterised by 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 snip... Crushed heads (which inevitably involve brain and spinal cord material) http://www.bseinquiry.gov.uk/files/yb/1989/03/17004001.pdf 2. The Parliamentary Secretary said that he was concerned snip... YOU explained that imported crushed heads were extensively used in the http://www.bseinquiry.gov.uk/files/yb/1989/04/14001001.pdf In particular I do not believe one can say that the levels of http://www.bseinquiry.gov.uk/files/yb/1989/04/24003001.pdf http://www.bseinquiry.gov.uk/files/yb/1989/04/25001001.pdf some 100+ _documented_ TSE cats of all types later...tss on occassions, materials obtained from slaughterhouses http://www.bseinquiry.gov.uk/files/yb/1989/05/03007001.pdf Meldrum's notes on pet foods and materials used http://www.bseinquiry.gov.uk/files/yb/1989/05/16001001.pdf http://www.bseinquiry.gov.uk/files/yb/1989/05/16002001.pdf IN CONFIDENCE CJD TO CATS... http://www.bseinquiry.gov.uk/files/yb/1989/05/18002001.pdf Confidential BSE and __________________ http://www.bseinquiry.gov.uk/files/yb/1989/05/22012001.pdf 1st case natural FSE http://www.bseinquiry.gov.uk/files/yb/1990/05/09002001.pdf FSE and pharmaceuticals http://www.bseinquiry.gov.uk/files/yb/1990/05/10005001.pdf confidential cats/dogs and unsatisfactory posture http://www.bseinquiry.gov.uk/files/yb/1990/06/14006001.pdf can't forget about the mad man and his mad cat; Deaths of CJD man and cat linked http://news.bbc.co.uk/1/hi/health/184558.stm In October 1998 the simultaneous occurrence of spongiform encephalopathy http://www.defra.gov.uk/animalh/bse/bse-science/level-4-othertses.htmlImage] Research letters Volume 352, Number 9134 [Image] 3 October1998[Previous] [Next] [Image][Image]Simultaneous occurrence of spongiform encephalopathy in a manand his cat in Italy[Image] Gianluigi Zanusso, Ettore Nardelli, Anna Rosati, GianMaria Fabrizi, SergioFerrari, Antonella Carteri, Franco De Simone, Nicola Rizzuto, SalvatoreMonaco Transmissible spongiform encephalopathies (TSE) encompass inherited,acquired, and sporadic mammalian neurological disorders, and arecharacterised by the conversion of the cellular prion protein (PrP) in aninsoluble and protease-resistant isoform (PrPres). In human TSE, four typesof PrPres have been identified according to size and glycoform ratios, whichmay represent different prion strains. Type-1 and type-2 PrPres areassociated with sporadic Creutzfeldt-Jakob disease (CJD), type 3 withiatrogenic CJD, and type 4 with variant CJD.1,2 There is evidence thatvariant CJD is caused by the bovine spongiform encephalopathy (BSE)-prionstrain.2-4 The BSE strain has been identified in three cats with felinespongiform encephalopathy (FSE), a prion disease which appeared in 1990 inthe UK.5 We report the simultaneous occurrence of sporadic CJD in a man anda 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, andmyoclonus. An electroencephalogram (EEG) showed diffuse theta-deltaactivity. A brain magnetic resonance imaging scan was unremarkable. 10 dayslater, he was speechless and able to follow only simple commands. RepeatEEGs showed periodic triphasic complexes. 2 weeks after admission, he wasmute, 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. Nobites from the cat were recalled. In the next few days, the cat becameataxic, with hindquarter locomotor dysfunction; the ataxia got worse andthere was diffuse myoclonus. The cat was killed in mid-January, 1994. No pathogenic mutations in the patient's PrP gene were found. The patientand the cat were methionine homozygous at codon 129. Histology of thepatient's brain showed neocortical and cerebellar neuronal loss,astrocytosis, and spongiosis (figure A). PrP immunoreactivity showed apunctate pattern and paralleled spongiform changes (figure B). The cat'sbrain showed mild and focal spongiosis in deeper cortical layers of all fourlobes (figure C), vacuolated cortical neurons (figure D), and mildastrogliosis. The cerebellar cortex and the dentate nucleus were gliosed.Immunoreactive PrP showed a punctate pattern in neocortex, allocortex, andcaudate nucleus (figure E). Western blot analysis of control and affectedhuman and cat brain homogenates showed 3 PrP bands of 27-35 kDa. Afterdigestion with proteinase K and deglycosylation, only samples from theaffected patient and cat showed type-1 PrPres, with PrP glycoform ratioscomparable to those observed in sporadic CJD1 (details available fromauthor). [Image] Microscopic sections of patient and cat brains A: Occipital cortex of the patient showing moderate spongiformdegeneration and neuronal loss (haematoxylin and eosin) and B: punctateperineuronal pattern of PrP immunoreactivity; peroxidaseimmunohistochemistry with monoclonal antibody 3F4. C: cat parietal cortexshowing mild spongiform degeneration (haematoxylin and eosin).D:vacuolated neurons (arrow, haematoxylin and eosin), E: peroxidaseimmunohistochemistry with antibody 3F4 shows punctate perineuronaldeposition of PrP in temporal cortex. This study shows a spatio-temporal association between human and felineprion diseases. The clinical features of the cat were different frompreviously reported cases of FSE which were characterised by gradual onsetof behavioural changes preceding locomotor dysfunction and ataxia.5Neuropathological changes were also at variance with the diffuse spongiosisand vacuolation of brainstem neurons, seen in FSE.5 The synaptic pattern ofPrP deposition, similar in the cat and in the patient, was atypical for aBSE-related condition. Evidence of a new type of FSE was further provided bythe detection of a type-1 PrPres, other than the BSE-associated type 4.2Taken together, our data suggest that the same agent strain of sporadic CJDwas involved in the patient and in his cat. It is unknown whether these TSE occurred as the result of horizontaltransmission 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 phenotypicvariablity 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 ofprion 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 vCJDand BSE. Nature 1997; 389: 448-50 [PubMed]. 5 Pearson GR, Wyatt JM, Henderson JP, Gruffydd-Jones TJ. Feline spongiformencephalopathy: a review. Vet Annual 1993; 33: 1-10. ------------------------------------------------------------------------Sezione di Neurologie Clinica, Dipartimento di Scienze Neurologiche e dellaVisione, Università di Verona, Policlinico Borgo Roma, 37134 Verona, Italy(S Monaco; e mail rizzuto@Gorgorna.univr.it); and Istituto ZooprofilatticoSperimentale della Lombardia e dell' Emilia, Brescia =========================================TSS indeed there have been 4 documented cases of TSE in Lions to date. Lion 32 December 98 Born November 86 Lion 33 May 1999 (euthanased) Born November 81. Lion 36 Euthanased August 2000 Born July 87. Deteriorating hind limb Lion 37 Euthanased November 2001 Male, 14 years. Deteriorating hind http://www.defra.gov.uk/animalh/bse/index.html go to the url above, on the bar at the top, click on _statistics_, or go here; http://www.defra.gov.uk/animalh/bse/bse-statistics/level-3-tsestat.html and http://www.defra.gov.uk/animalh/bse/bse-science/level-4-othertses.html http://www.bseinquiry.gov.uk/files/yb/1992/11/13001001.pdf also; Reports on the clinical symptoms presented by these cats give a http://www.neurocenter-bern.ch/tse_e.shtml Subject: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY Greetings BSE-L, is there any other CWD surveys/testing in the UK on their deer? DEER SPONGIFORM ENCEPHALOPATHY SURVEY http://www.bseinquiry.gov.uk/files/yb/1991/11/20004001.pdf http://www.bseinquiry.gov.uk/files/yb/1992/11/04002001.pdf hope they did not go by the wayside as the hound study; http://www.bseinquiry.gov.uk/files/yb/1991/10/18001001.pdf http://www.bseinquiry.gov.uk/files/yb/1993/12/06001001.pdf 37.Putative TSE in hounds - work started 1990 -(see para 41) Robert Higgins, a Veterinary Investigation Officer at Thirsk, 38.I reviewed the literature on hound neuropathology, and 39.Hound ataxia had reportedly been occurring since the 1930's, 40.The inconclusive results in hounds were never confirmed, 41.The hound work could have provided valuable evidence Histopathological support to various other published 42.These included neuropathological examination of material http://www.bseinquiry.gov.uk/witness/htm/stat067.htm nothing to offer scientifically; http://www.bseinquiry.gov.uk/files/yb/1991/10/17001001.pdf maddogs and Englishman http://www.bseinquiry.gov.uk/files/yb/1990/11/28001001.pdf kind regards, ###########bse-l ############ Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY Dear Terry, What happened with the deer was that an association between deer meat Steve Dealler Incubation periods for BSE are proportional to the life expectancy of Steve Dealler, consultant in medical microbiology. TSS ########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ############ http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be07.html Docket Management Docket: 02N-0273 - Substances Prohibited From Use in http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be09.html August 22, 2003 5:11 PM Mad cat disease A second case of feline spongiform encephalopathy (FSE), a disease swissinfo http://www.swissinfo.org/sen/Swissinfo.html?siteSect=113&sid=41558971: Ann N Y Acad Sci. 1982;396:131-43. Links Alzheimer's disease and transmissible virus dementia (Creutzfeldt-Jakob disease).Brown P, Salazar AM, Gibbs CJ Jr, Gajdusek DC. Ample justification exists on clinical, pathologic, and biologic grounds for considering a similar pathogenesis for AD and the spongiform virus encephalopathies. However, the crux of the comparison rests squarely on results of attempts to transmit AD to experimental animals, and these results have not as yet validated a common etiology. Investigations of the biologic similarities between AD and the spongiform virus encephalopathies proceed in several laboratories, and our own observation of inoculated animals will be continued in the hope that incubation periods for AD may be even longer than those of CJD.http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=6758661&query_hl=3&itool=pubmed_DocSum1: Neurology. 1990 Feb;40(2):226-8. Coexistence of Creutzfeldt-Jakob disease and Alzheimer's disease in the same patient.Brown P, Jannotta F, Gibbs CJ Jr, Baron H, Guiroy DC, Gajdusek DC. Laboratory of CNS Studies, NINDS, National Institutes of Health, Bethesda, MD 20892.We report the case of a 73-year-old patient in whom a diagnosis of Creutzfeldt-Jakob disease, suggested by the clinical course, was verified by the neuropathologic finding of widespread spongiform change and astrogliosis, the presence of proteinase-resistant protein in brain extracts, and the experimental transmission of spongiform encephalopathy to primates inoculated with brain tissue. However, neuropathologic examination also revealed a profusion of senile and neuritic plaques and neurofibrillary tangles that reacted with antibody to the amyloid beta-protein characteristic of Alzheimer's disease, but not with antibody to the scrapie amyloid protein characteristic of Creutzfeldt-Jakob disease.http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=2405293&query_hl=9&itool=pubmed_DocSum1: Prog Clin Biol Res. 1989;317:549-57. Links Neuroaxonal dystrophy: an ultrastructural link between subacute spongiform virus encephalopathies and Alzheimer's disease.Liberski PP, Yanagihara R, Gibbs CJ Jr, Gajdusek DC. Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, Bethesda, Maryland 20892.====================Unconventional Viruses and the Origin and Disappearance of Kuru 333334 Physiology or Medicine 1976Table 12. Creutzfeldt-Jakob disease in catsIncubation period(months)Duration(months)Primary passageHuman brainSerial passageCat brain (passage 1)Cat brain (passage 2)This geographic and temporal clustering does not apply, however, to amajority of cases and is unexplained by the 10% of the cases that are familial.Matthews has recently made a similar observation in two clusters in England(50). There are two reports of conjugal disease in which husband and wife diedof CJD within a few years of each other (30, 50).The prevalence of CJD has varied markedly in time and place throughoutthe United States and Europe, but we have noted a trend toward making thediagnosis more frequently in many neurological clinics in recent years, sinceattention has been drawn to the syndrome by its transmission to primates(3, 33). For many large population centers of the United States, Europe,Australia, and Asia, we have found a prevalence approaching one per millionwith an annual incidence and a mortality of about the same magnitude, asthe average duration of the disease is 8 to 12 months. Matthews (50) found anannual incidence of 1.3 per million in one of his clusters, which was over 10times the overall annual incidence for the past decade for England andWales (0.09 per million). Kahana et al. (40) reported the annual incidence ofCJD ranging from 0.4 to 1.9 per million in various ethnic groups in Israel. Theynoted, however, a 30-fold higher incidence of CJD in Jews of Libyan originabove the incidence in Jews of European origin. From recent discussions withour Scandinavian colleagues it is apparent that an annual incidence of at leastone per million applies to Sweden and Finland in recent years.Probable man-to-man transmission of CJD has been reported in a recipientof a cornea1 graft, which was taken from a donor who was diagnosed retrospectivelyto have had pathologically confirmed CJD ( 12). The disease occurred18 months after the transplant, an incubation period just the average forchimpanzees inoculated with human CJD brain tissue (32, 62). From suspensionof brain of the cornea1 graft recipient we succeeded in transmitting CJDto a chimpanzee although the brain had been at room temperature in 10%formol-saline for seven months (26a). More recently we learned that two of ourconfirmed cases of TVD were professional blood donors until shortly beforethe onset of their symptoms. To date, there have been no transmissions of CJDfrom blood of either human patients or animals affected with the experimentallytransmitted disease. However, we have only transfused two chimpanzees eachwith more than 300 ml of human whole blood from a different CJD patientUnconventional Viruses and the origin and Disappearance of KuruFigure 20. Six serial passages of CJD in chimpanzees, starting with brain tissue from abiopsy of a patient (R. R.) with CJD in the United Kingdom (U. K.). Also shown is transmissionof the disease directly from man to the capuchin monkey and marmoset, and fromchimpanzee brain to three species of New World monkeys (squirrel, capuchin, spidermonkeys), and to six Old World species (rhesus, stumptailed, cynomolgus, African green,pigtailed, and sooty mangabey). Incubation periods in the New World monkeys rangedfrom 19 to 47 months, and in the Old World monkeys from 43 to 60 months. The pigtailedmacaque and the sooty mangabey showed positive CJD pathology when sacrificed without336 Physiology or Medicine 1976within the past several months. Finally, the recognition of TVD in a neurosurgeon(27), and more recently in two physicians, has raised the question ofpossible occupational infection, particularly in those exposed to infected humanbrain tissue during surgery, or at postmortem examination (61, 63).The unexpectedly high incidence of previous craniotomy in CJD patientsnoted first by Nevin et al. (51) and more recently by Matthews (50) and byourselves (62), raises the possibility of brain surgery either affording a mode ofentry for the agent or of precipitating the disease in patients already carryinga latent infection. The former unwelcome possibility now seems to be areality with the probable transmission of CJD to two young patients withepilepsy from the use of implanted silver electrodes sterilized with 70%ethanol and formaldehyde vapor after contamination from their use on apatient who had CJD. The patients had undergone such electrode implantationfor stereotactic electroencephalographic localization of the epileptic focusat the time of correctional neurosurgery (3a).Two patients with transmissible virus dementias were not diagnosed clinicallyor neuropathologically as having CJD, but rather as having Alzheimer’sdisease (62). In both cases the disease was familial: in one (Fig. 21) therewere six close family members with the disease in two generations; in the otherboth the patient’s father and sister had died of presenile dementia. Thediseases as transmitted to primates were clinically and pathologically typicalsubacute spongiform virus encephalopathies, and did not have pathologicalfeatures of Alzheimer’s disease in man. More than 30 additional specimens ofbrain tissue from non-familial Alzheimer’s disease have been inoculated intoTVD-susceptible primates without producing disease. Therefore, although weclinical disease. A third passage to the chimpanzee was accomplished using frozen andthawed explanted tissue culture of brain cells that had been growing in vitro for 36 days.Using 10-3, 10-4, and 10-4 dilutions of brain, respectively, the 4th, 5th, and 6th chimpanzeepassages were accomplished. This indicates that the chimpanzee brain contains >50,000infectious doses per gram, and that such infectivity is maintained in brain cells cultivatedin vitro at 37” C for at least one month. The lower left shows transmission of CJD from asecond human patient (J. T.) to a cat with a 30 month incubation and serial passage in thecat with 19 to 24 month incubation.Unconventional Viruses and the Origin and Disappearance of Kum 337Figure 21a. Y family. Brain tissue obtained from patient A. Y. at biopsy induced subacutespongiform encephalopathy in a squirrel monkey 24 months after intracerebral inoculation.The patient, a 48-year old woman who died after a 68 month course of progressive dementia,quite similar in clinical aspects to the progressive dementia from which her fatherand brother had died at 54 and 56 years of age, respectively, was diagnosed clinically andneuropathologically as suffering from Alzheimer’s disease. Her sister is at present incapacitatedby a similar progressive dementia of 4 years’ duration. Although the transmitteddisease in the squirrel monkey was characterized by severe status spongiosis, none was seenin the patient. although amyloid plaques and neurofibrillary tangles were frequent.21b. H family. Brain tissue obtained from patient B. H. at surgical biopsy induced subacutespongiform encephalopathy in a squirrel monkey and a capuchin monkey 29 1/2 monthsand 43 months, respectively, after intracerebral inoculations. The patient, a 57 year oldwoman, has had slowly progressive dementia and deterioration for the past 7 years. Neuropathologicalfindings revealed abundant neurofibrillary tangles and senile plaques and noevidence of status spongiosis. The patient’s father, A. S., had died at age 64 following severalyears of progressive dementia, behavioral change and memory loss. B. H. is presently aliveand institutionalized.338 Physiology or Medicine 1976cannot claim to have transmitted the classical sporadic Alzheimer’s disease toprimates, we are confronted with the anomaly that the familial form ofAlzheimer’s disease has, in these two instances, transmitted as though it wereCJD.The above findings have added impetus to our already extensive studies ofHuntington’s chorea, Alzheimer’s and Pick’s diseases, parkinsonism-dementia,senile dementia, and even “dementia praecox", the organic brain diseaseassociated with late uncontrolled schizophrenia. ......http://nobelprize.org/nobel_prizes/medicine/laureates/1976/gajdusek-lecture.pdfGAH 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. snip... http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf 2005 DEFRADepartment for Environment,Food & Rural Affairs Area 307, London, SW1P 4PQTelephone: 0207 904 6000Direct line: 0207 904 6287E-mail: h.mcdonagh.defra.gsi.gov.uk GTN:FAX: Mr T S SingeltaryP.O. Box 42BacliffTexasUSA 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), Later, at their meeting in June 95, The Committee re-evaluated the hound study to see if any useful results could be Overall, it is clear that SEAC had major concerns about the survey as conducted. As a result it is likely that the For more details see- As this study remains unpublished, my understanding is that the ownership of the data essentially remains with the original researchers. 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. I hope this is helpful Yours sincerely 4 HUGH MCDONAGHBSE CORRESPONDENCE SECTION ============================================ IN STRICT CONFIDENCE TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES http://www.bseinquiry.gov.uk/files/yb/1993/01/05004001.pdf Regarding Alzheimer's disease (note the substantial increase on a yearly basis) http://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://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://www.bseinquiry.gov.uk/files/yb/1990/07/06005001.pdf Human BSE snip... These are not relevant to any possible human hazard from BSE nor to the much more common dementia, Alzheimers. snip... http://www.bseinquiry.gov.uk/files/yb/1990/07/09001001.pdf NOW, ASK yourself how many dogs and cats are ever examined and autopsied for a TSE in the USA ??? Terry S. Singeltary Sr.
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