A case cluster of variant Creutzfeldt-Jakob disease linked to the Kingdom of Saudi Arabia.
As of mid-2016, 231 cases of variant Creutzfeldt-Jakob disease-the human form of a prion disease of cattle, bovine spongiform encephalopathy-have been reported from 12 countries. With few exceptions, the affected individuals had histories of extended residence in the UK or other Western European countries during the period (1980-96) of maximum global risk for human exposure to bovine spongiform encephalopathy. However, the possibility remains that other geographic foci of human infection exist, identification of which may help to foreshadow the future of the epidemic. We report results of a quantitative analysis of country-specific relative risks of infection for three individuals diagnosed with variant Creutzfeldt-Jakob disease in the USA and Canada. All were born and raised in Saudi Arabia, but had histories of residence and travel in other countries. To calculate country-specific relative probabilities of infection, we aligned each patient's life history with published estimates of probability distributions of incubation period and age at infection parameters from a UK cohort of 171 variant Creutzfeldt-Jakob disease cases. The distributions were then partitioned into probability density fractions according to time intervals of the patient's residence and travel history, and the density fractions were combined by country. This calculation was performed for incubation period alone, age at infection alone, and jointly for incubation and age at infection. Country-specific fractions were normalized either to the total density between the individual's dates of birth and symptom onset ('lifetime'), or to that between 1980 and 1996, for a total of six combinations of parameter and interval. The country-specific relative probability of infection for Saudi Arabia clearly ranked highest under each of the six combinations of parameter × interval for Patients 1 and 2, with values ranging from 0.572 to 0.998, respectively, for Patient 2 (age at infection × lifetime) and Patient 1 (joint incubation and age at infection × 1980-96). For Patient 3, relative probabilities for Saudi Arabia were not as distinct from those for other countries using the lifetime interval: 0.394, 0.360 and 0.378, respectively, for incubation period, age at infection and jointly for incubation and age at infection. However, for this patient Saudi Arabia clearly ranked highest within the 1980-96 period: 0.859, 0.871 and 0.865, respectively, for incubation period, age at infection and jointly for incubation and age at infection. These findings support the hypothesis that human infection with bovine spongiform encephalopathy occurred in Saudi Arabia.
1 Canadian Creutzfeldt-Jakob Disease Surveillance System, Centre for Foodborne, Environmental and Zoonotic Infectious Diseases, Public Health Agency of Canada, Ottawa, ON K1A 0K9, Canada michael.coulthart@canada.ca.
Michael B
MB
Coulthart
2 Memory and Aging Center, Box 1207, University of California, San Francisco (UCSF), San Francisco, CA 94143-1207, USA.
Michael D
MD
Geschwind
3 Consultant Neurologist, Dhahran Health Center, Dhahran, Saudi Arabia.
Shireen
S
Qureshi
4 Department of Neurology, Parkinson's and Movement Disorders Program, University of California Irvine, Irvine, CA 92697, USA.
Nicolas
N
Phielipp
5 Zoonoses Division, Centre for Foodborne, Environmental and Zoonotic Infectious Diseases, Public Health Agency of Canada, Ottawa, ON K1A 0K9, Canada.
Alex
A
Demarsh
6 Division of High Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA 30333, USA.
Joseph Y
JY
Abrams
6 Division of High Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA 30333, USA.
Ermias
E
Belay
7 Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA.
Pierluigi
P
Gambetti
8 Eastern Ontario Regional Laboratory Association, Ottawa Hospital, Ottawa, ON K1Y 4E9, Canada.
Gerard H
GH
Jansen
7 Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA.
Anthony E
AE
Lang
6 Division of High Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA 30333, USA.
Lawrence B
LB
Schonberger
D000328:Adult; D017668:Age of Onset; D000818:Animals; D002417:Cattle; D015331:Cohort Studies; D007562:Creutzfeldt-Jakob Syndrome / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology* / Q000378:metabolism; D004196:Disease Outbreaks; D016643:Encephalopathy, Bovine Spongiform / Q000453:epidemiology / Q000635:transmission*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D000072002:Prion Proteins / Q000378:metabolism; D012307:Risk Factors; D012529:Saudi Arabia / Q000453:epidemiology; D006113:United Kingdom / Q000453:epidemiology; D055815:Young Adult
D000072002:Prion Proteins
Saudi Arabia; bovine spongiform encephalopathy; prion diseases; variant Creutzfeldt-Jakob disease
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