Incidences Trends of Creutzfeldt-Jakob Disease in Israel
Incidences Trends of Creutzfeldt-Jakob Disease in Israel
Yacov Balasha, Meir Walkerb, Esther Kahanac,d, Hadeel Nabale, Emilia Anise, Hanna Rosenmannf, Ron Miloc,d, and Amos D. Korczynb
aDepartment of Neurology, Kaplan Medical Center, Rehovot, Israel; bSackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel; cDepartment of Neurology, Barzilai University Medical Center, Ashkelon, Israel; dFaculty of Health Sciences, Ben Gurion University of the Negev, Beer-Sheva, Israel; eDivision of Epidemiology, Ministry of Health, Jerusalem, Israel; fDepartment of Neurology, the Agnes Ginges Center for Human Neurogenetics, Hadassah Hebrew University Medical Center, Jerusalem, Israel
Aims: Sporadic Creutzfeldt-Jakob disease (s-CJD) is a rare, fatal neurodegenerative disorder. Familial cases of Creutzfeldt-Jakob disease (f-CJD) due to mutations in the PRNP gene are even rarer around the world; however, in Israel there is an unusual focus of f-CJD patients carrying the E200K mutation. The number of E200K mutation carriers in Israel is increasing, which raised the suspicion of CJD transmission from person to person. If such transmission does occur, the incidence of s-CJD is expected to increase.
Materials and Methods: Using data from the national CJD registry and official statistics on the Israeli popula- tion, we studied incidence rates of f-CJD and s-CJD for the period from 1985 to 2018 applying the SEER (Surveillance Epidemiology and End Results) statistical packet elaborated in the US National Cancer Institute Results: In total, we identified 621 CJD patients (405 f-CJD and 216 s-CJD) cases. In the cohort of f-CJD patients the mean age-adjusted annual incidence rate over the above-mentioned period was 1.88 ± 0.09 (95% CI: 1.7–2.08) per 1,000,000. In the cohort of s-CJD patents the mean age-adjusted incidence rate over the same period was 0.93 ± 0.06 (95% CI: 0.81– 1.06) per 1,000,000 people. No significant time trends were found according to the permutation test of join- point regression in either of them.
When both cohorts were combined, the mean annual age-adjusted incidence of CJD in Israel was 2.81 ± 0.11 (95% CI: 2.58–3.04) per 1,000,000. From 1985 to 2018, there was a borderline increase in inci- dence of 0.8% per year, (95% CI: 0–1.6, p = 0.37), which is non significant.
Conclusions: Israel has a great predominance of f-CJD compared to s-CJD. The mean incidence of s-CJD in Israel is similar to most countries. Between 1985 and 2018, the annual age adjusted incidence rates for both forms of CJD have remained stable. There is no evidence for transmission of the disease.
Biobank of genetic CJD in Israel
Alice Anane, and Victor Novack
CJD Foundation Israel, and Soroka University Medical Center, Israel
Aims: A sharing longitudinal biobank of samples and values of genetic families with E200K mutation and patients.
Material and Methods: The world prevalence of the disease is 1:500,000–1,000,000 a year. About 85% are sporadic cases, and about 15% are genetic. In Israel, there is the highest prevalence in the world of the genetic form (gCJD), estimated by about 1:200,000 a year, due to a common mutation that induces the risk, at the Libyan-Tunisian community at Israel, which is characterized by many members at each family. The estimation is thousands of families with the genetic risk, concentrate in rather small area of the state of Israel.
My name is Alice Anane, a daughter of a father, who died from this disease at a young age (49). After discovering I and my siblings are carriers, I founded the association in 2008 and I am very active worldwide to advance research for this hor- rible and devastating disease. We unite a community of the genetic families. Our registry counts more than 400 members (representatives of families) and it grows constantly.
Our partner – Prof. Victor Novack, Clinical Epidemiologist. MD, Ph.D. Head of The Research Authority, Supervising Negev Bio Bank (NBB), a high- quality biorepository of biological samples that operates with MIDGAM, a national Israeli infrastructure for biomedical research and includes five Israeli medical centers, which located in different areas and works as biobanking sites. NBB will be in charge of managing and maintaining the samples of CJD from all the sites in its facilities. We invite researchers to apply and initiate new research on our human samples, sharing values of results with us, for conducting AI of Big Data research.
Results: Longitude studies for biomarkers, early diag- nosis and therapeuties for gCJD.
Conclusions: Conducting and sharing data on the same specific samples for longitude studies can bring a revelation for science and for us at the shortest time in the most efficient way.
Cathala et al. (9) reported that at least three ancestors of a family with 14 cases of CJD were shepherds. Most of the family had lived in the same farming region for generations. Mayer et al. (14) noted that sheep raising is traditional in the region where a Slovakian CJD cluster occurred. One patient, a 57-year-old female raised sheep for 12 years prior to onset of CJD and admitting eating raw sheep brain (Orolin D. et al., personal communication, 1978). Lo Russo et al. (15) reported that the places of origin of seven out of eight patients with CJD coincided with the distribution of sheep raised in Central and Southern Italy. Libya has one of the highest rates of consumption of sheep and mutton per capita in the world (16), and Libyan immigrants to Israel were found to have more than 30 times the rate of CJD as immigrants to Israel from other countries (17). Thus, some available data suggest a link between CJD and sheep exposure. However, more direct data relating sheep contact and consumption in CJD patients and controls are needed.
Brown stated that the agent of scrapie has not been detected in sheep muscle. Since mainly muscle is consumed when sheep are eaten, he did not think that sheep meat was a reasonable source of the scrapie agent. However, Pattison and Millson (18) did detect the scrapie virus in the muscle of experimentally infected goats. Very few transmission and virus recovery experiments were done with sheep muscle compared with the number done with other tissues. It should be realized that sheep meat prepared for consumption also contains blood vessels, nervous and lymphatic tissues. Lamb and mutton chops may even contain spinal cord. It has been well established that lymphatic tissues of affected sheep contain scrapie virus from an early age. Even after invading the central nervous system, scrapie agent continues to exist in other tissues (19).
Brown stated that the agent of scrapie has not been detected in sheep muscle. Since mainly muscle is consumed when sheep are eaten, he did not think that sheep meat was a reasonable source of the scrapie agent. However, Pattison and Millson (18) did detect the scrapie virus in the muscle of experimentally infected goats. Very few transmission and virus recovery experiments were done with sheep muscle compared with the number done with other tissues. It should be realized that sheep meat prepared for consumption also contains blood vessels, nervous and lymphatic tissues. Lamb and mutton chops may even contain spinal cord. It has been well established that lymphatic tissues of affected sheep contain scrapie virus from an early age. Even after invading the central nervous system, scrapie agent continues to exist in other tissues (19).
Sunday, October 27, 2013
A Kiss of a Prion: New Implications for Oral Transmissibility
***The occurrence of contact cases raises the possibility that transmission in families may be effected by an unusually virulent strain of the agent.
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vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what if ???
Greetings Friends, Neighbors, and Colleagues,
Saturday, February 2, 2019
CWD GSS TSE PRION SPINAL CORD, Confucius Ponders, What if?
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***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***
REVIEW
***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***
***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<***
***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***
***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<***
***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***
Thursday, March 8, 2018
Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein
MONDAY, NOVEMBER 26, 2018
Sporadic Creutzfeldt-Jakob Disease in a Woman Married Into a Gerstmann-Sträussler-Scheinker Family: An Investigation of Prions Transmission via Microchimerism
“The number of E200K mutation carriers in Israel is increasing, which raised the suspicion of CJD transmission from person to person. If such transmission does occur, the incidence of s-CJD is expected to increase.”
Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD).
posted by Terry S. Singeltary Sr. at
4:11 PM
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