Results 1 to 3 of 3

Thread: Does Sporadic CJD differ in terms of clinical course in younger patients?

  1. #1

    Default Does Sporadic CJD differ in terms of clinical course in younger patients?

    Hello guys. I've been wondering if age has an effect on the clinical progression of Sporadic CJD in young patients that differs from the course that older patients go through. Some articles that I have read seem to indicate that young people seem to have a longer clinical course that starts in a similar manner to Variant CJD (psychiatric symptoms being more prominent, though the VV1 phenotype results in Dementia). However, other articles seem to show younger individuals (29 years of age) lapsing into a vegetative state in just 2 weeks. So as a result, I thought I'd post this thread and ask the community here about the subject.

  2. #2
    Distinguished Community Member
    Join Date
    Oct 2006
    Location
    Bacliff, Texas
    Posts
    495

    Default

    Quote Originally Posted by Fishmahboi View Post
    This quote is hidden because you are ignoring this member. Show Quote
    Hello guys. I've been wondering if age has an effect on the clinical progression of Sporadic CJD in young patients that differs from the course that older patients go through. Some articles that I have read seem to indicate that young people seem to have a longer clinical course that starts in a similar manner to Variant CJD (psychiatric symptoms being more prominent, though the VV1 phenotype results in Dementia). However, other articles seem to show younger individuals (29 years of age) lapsing into a vegetative state in just 2 weeks. So as a result, I thought I'd post this thread and ask the community here about the subject.

    it's getting a bit confusing now due to the elderly getting nvCJD (vCJD) and young folks getting sporadic CJD, and the price of poker has gone up now that sporadic CJD has been linked to atypical and typical BSE, atypical and typical Scrapie, and to CWD, but nobody talks of that. psychiatric symptoms were showing up in sporadic cjd victims decades ago, along with kuru type plaques. i think it will depend on the strain of TSE, dose, route, from what species, and the individual. symptoms thus will differ greatly imo. we now have vpspr, sporadic GSS, and sporadic FFI, tied to no genetic factor of any family. UKBSEnvCJD only theory was wrong from the beginning imo. i really don't think they have a clue yet today as to answer your question...imo...kindest regards, terry


    see;

    ''It remains a remote possibility that when older people contract CJD from BSE the resulting phenotype is like sporadic CJD and is distinct from the vCJD phenotype in younger people.''

    Volume 2: Science

    4. The link between BSE and vCJD

    Summary

    4.29 The evidence discussed above that vCJD is caused by BSE seems overwhelming. Uncertainties exist about the cause of CJD in farmers, their wives and in several abattoir workers. It seems that farmers at least might be at higher risk than others in the general population. 1 Increased ascertainment (ie, increased identification of cases as a result of greater awareness of the condition) seems unlikely, as other groups exposed to risk, such as butchers and veterinarians, do not appear to have been affected. The CJD in farmers seems to be similar to other sporadic CJD in age of onset, in respect to glycosylation patterns, and in strain-typing in experimental mice. Some farmers are heterozygous for the methionine/valine variant at codon 129, and their lymphoreticular system (LRS) does not contain the high levels of PrPSc found in vCJD. It remains a remote possibility that when older people contract CJD from BSE the resulting phenotype is like sporadic CJD and is distinct from the vCJD phenotype in younger people.

    4.30 Estimates of the likely scale of a possible epidemic of vCJD are wide-ranging and the subject of much debate. To know the likely number of cases is very important, not least to enable preparations to be made for the care of victims, as well as to be able to draw up guidelines to reduce the risk of transmission from infected but asymptomatic people. Preliminary results of the study examining tonsil and appendix material for signs of infection were not informative in this regard and full results are awaited. A blood test that would allow the widespread screening of the population by a simple method is still being sought.

    BSE INQUIRY


    PRION 2015 CONFERENCE


    O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations

    Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France

    Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods.

    *** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,

    ***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),

    ***is the third potentially zoonotic PD (with BSE and L-type BSE),

    ***thus questioning the origin of human sporadic cases.

    We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.

    ===============

    ***thus questioning the origin of human sporadic cases***

    ===============

    ***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.

    ==============

    https://prion2015.files.wordpress.co...5abstracts.pdf


    PRION 2016 CONFERENCE


    Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.

    Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.

    These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

    http://www.tandfonline.com/doi/abs/1...nalCode=kprn20


    http://www.tandfonline.com/doi/abs/1...nalCode=kprn20


    SPONTANEOUS ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY

    ***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
    https://www.nature.com/articles/srep11573


    ELEPHANT IN THE ROOM $$$

    LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$

    *** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***

    https://www.landesbioscience.com/jou...ache=112223249

    http://www.tandfonline.com/doi/full/...124?src=recsys

    http://www.tandfonline.com/doi/pdf/1...eedAccess=true


    THURSDAY, AUGUST 17, 2017

    Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States revisited 2017

    http://creutzfeldt-jakob-disease.blo...ing-forms.html


    Terry S. Singeltary Sr.

  3. #3
    Distinguished Community Member
    Join Date
    Oct 2006
    Location
    Bacliff, Texas
    Posts
    495

    Default

    http://jnnp.bmj.com/content/79/1/14.long

    http://jnnp.bmj.com/content/88/9/764

    http://www.sciencedirect.com/science...73959811000780

    https://academic.oup.com/brain/artic...205/awl123.pdf

    https://academic.oup.com/brain/artic...104/awl224.pdf

    http://jamanetwork.com/journals/jama...article/784138

    http://neuro.psychiatryonline.org/do...psych.14070160

    I
    n 2008 in Spain, 2 cases of variant Creutzfeldt-Jakob disease
    (vCJD) in first-degree relatives were identified. After
    the death of a 41-year-old man (patient 1) from vCJD, his
    64-year-old mother (patient 2) began showing symptoms of
    anxiety and depression and, 2 months later, a gait disorder
    and progressive dementia. Although the clinical duration
    was relatively short and the early symptoms uncommon in
    comparison to vCJD cases in the United Kingdom, the overall
    clinical phenotype and posterior thalamic hyperintensities
    as seen in an MRI brain scan led to a diagnosis of suspected
    vCJD. Neuropathological examination confirmed the diagnosis
    of vCJD. Both patients were 129MM homozygous,
    had never received a blood transfusion or tissue graft, and
    had lived in the same town within the Castilla-León region
    of Spain (Table 1) (1). The region is a farming area at high
    risk for bovine spongiform encephalopathy (BSE); 3 of the
    5 cases of vCJD reported in Spain came from this region (1).
    The patients had similar eating habits, which included ingestion
    of bovine brain. We conducted a study to determine
    whether these 2 vCJD cases were caused by the BSE agent,
    whether the agent strain was similar to previously characterized
    human vCJD cases, and whether the age of the patients
    would influence the strain characteristics.

    https://wwwnc.cdc.gov/eid/article/23/9/pdfs/17-0159.pdf

    http://vcjd.blogspot.com/2017/06/pri...-p61-vcjd.html

    Summary
    We report a case of variant Creutzfeldt-Jakob disease (vCJD) in a 74-year old man in whom diagnosis was made at necropsy. The occurrence of vCJD in an individual in this age group is unlikely to be an isolated event. Doctors need to be aware that vCJD can arise in elderly patients so that appropriate investigations (including magnetic resonance imaging) can be done, and permission for neuropathological necropsy requested, in suspected cases. This case could also have important implications for public health policy decisions and surveillance programmes that target the younger age range of vCJD cases.

    http://www.thelancet.com/journals/la...504-9/abstract

    http://jnnp.bmj.com/content/early/20...np-2014-309397

    http://www.health.gov.au/internet/ma...alopathies.htm

    http://creutzfeldt-jakob-disease.blo...withjames.html

    http://madcowusda.blogspot.com/2009/...gibbs-and.html


    kind regards, terry

Similar Threads

  1. Infectivity in bone marrow from sporadic CJD patients
    By flatfish in forum Creutzfeldt Jakobs Disease (CJD)
    Replies: 0
    Last Post: 08-11-2017, 11:32 AM
  2. Drug to make you feel younger
    By Jeanie Z in forum Multiple Sclerosis
    Replies: 4
    Last Post: 05-01-2017, 09:01 PM
  3. CREUTZFELDT JAKOB DISEASE CJD TSE PRION they are getting younger, why?
    By flatfish in forum Creutzfeldt Jakobs Disease (CJD)
    Replies: 0
    Last Post: 08-21-2016, 12:39 PM
  4. Replies: 0
    Last Post: 04-23-2016, 08:49 AM
  5. Psychiatric Symptoms in Patients With Sporadic Creutzfeldt-Jakob Disease in Germany
    By flatfish in forum Creutzfeldt Jakobs Disease (CJD)
    Replies: 0
    Last Post: 05-09-2015, 01:02 PM

Posting Permissions

  • You may not post new threads
  • You may not post replies
  • You may not post attachments
  • You may not edit your posts
  •  


BTC Inc's Disclaimer and Privacy Policy

The material on this site is for information & support purposes only, and is not a substitute for medical advice provided by a licensed health care provider. Always consult your doctor before trying anything that you find online.