Occupational contamination
In France, the last two victims of variant Creutzfeldt-Jakob disease, who died in 2019 and 2021, were research technicians at the National Research Institute for Agriculture, Food and the Environment (INRAE). Emilie Jaumain, who died in 2019, at the age of 33, had been the victim of a work accident in 2010, during which she had pricked herself with a tool contaminated with infected brain. The efficacy of this route of contamination has been unambiguously demonstrated in primates. Pierrette C., who died in 2021, had been victim of the same type of work accident. After her diagnosis, a moratorium was initiated in all French laboratories on research activities on infectious prions. In March 2022, INRAE recognized the occupational cause of these two deaths. This raises serious questions about the safety of personnel in these laboratories. Indeed, inspections have noted serious failures in the protection of agents in the face of this deadly risk, and the long incubation period of this disease leads to fears of new cases in the future, hence great concern. Other causes
Eating other types of brains such as those from squirrels is not recommended as one person contracted vCJD from eating the brain of a squirrel. Mechanism
Despite the consumption of contaminated beef in the UK being high, vCJD has infected a small number of people. One explanation for this can be found in the genetics of people with the disease.
The type of surgery performed is specific to the type of dystonia that the patient has. A systematic review found that some patients benefit from deep brain stimulation (DBS) surgery, but the studies may have been subject to financial bias. Prevalence
The disease is more commonly found amongst Ashkenazi Jews. The occurrence of torsion dystonia in the Ashkenazi Jewish population as stated by the Department of Epidemiology and Public Health of Yale University School of Medicine in New Haven, Connecticut; "Reports dating to the beginning of this century describe Ashkenazi Jewish (AJ) families with multiple cases of ITD either in siblings (Schwalbe 1908; Bernstein 1912; Abrahamson 1920) or in parents and offspring (Wechsler and Brock 1922; Mankowsky and Czerny 1929; Regensberg 1930). The first comprehensive evaluation of the mode of inheritance of ITD in Jewish and non-Jewish families was described by Zeman and Dyken (1967), who concluded that the disorder was inherited as an autosomal dominant with incomplete penetrance in both populations. Although they concluded that the gene frequency was higher in the AJ population than in non-Jews, no difference in mode of inheritance or disease mechanism was construed." Research
A 1969 study of torsion dystonia patients found an average IQ 10 points higher than controls matched for age, sex and ethnic background. References
Further reading
https://web.archive.org/web/20060903085219/http://www.biochem.arizona.edu/classes/bioc461/Biochem499/AngelaPulsifer/dystonia1.htm
Risch, N. J.; Bressman, S. B.; Deleon, D; Brin, M. F.; Burke, R. E.; Greene, P. E.; Shale, H; Claus, E. B.; Cupples, L. A.; Fahn, S (1990). "Segregation analysis of idiopathic torsion dystonia in Ashkenazi Jews suggests autosomal dominant inheritance".