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Lawrence B. Schonberger, M.D., M.P.H Prion and Public Health Office, Division of High-Consequence Pathogens & Pathology, National Center for Emerging &

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Presentation on theme: "Lawrence B. Schonberger, M.D., M.P.H Prion and Public Health Office, Division of High-Consequence Pathogens & Pathology, National Center for Emerging &"— Presentation transcript:

1 Lawrence B. Schonberger, M.D., M.P.H Prion and Public Health Office, Division of High-Consequence Pathogens & Pathology, National Center for Emerging & Zoonotic Diseases US-Diagnosed Variant Creutzfeldt-Jakob Disease in Two Patients Born and Raised in Saudi Arabia Division of High-Consequence Pathogens & Pathology National Center for Emerging and Zoonotic Infectious Diseases

2 Characteristics of the Two vCJD Patients Patient 1 Patient 2 Birth Country (yr)Saudi Arabia (1970)Saudi Arabia (1983) CitizenshipSaudi ArabiaEgypt Social Class “Professional” Country of vCJD onset (yr)Saudi Arabia (2003)United States (2006) Age at Onset32.5 yrs.22.8 yrs

3 Early Symptoms of the Two vCJD Patients Patient 1Patient 2 Increased irritabilityNumbness & “heat” sensation in Withdrawn shoulders, legs, & thigh AgitatedWithdrawn ParanoiaInsomnia Altered sleep/wake cycleIncreased anxiety (diagnosed by psychiatrist) Unsteady gait, scissor stepsUnsteady gait, ataxia Slurring of speechEmotionally labile, frequent crying “Sensory symptoms”

4 Subsequent Dementia and Duration of the Two vCJD Illnesses Patient 1 Patient 2 Within 10 months of onset:Within 6 months of onset: “Global cognitive impairment”Severe dementia Decreased verbal outputNon-ambulatory Making incomprehensible sounds Increased deep tendon reflexes, ankle and jaw clonus Illness duration: 6.9 years, mostly in akinetic mute state; died in Illness duration: 8.5 months; died in 2006

5 Diagnostic Test Results on the Two vCJD Patients Test Patient 1 Patient 2 EEG Non-specific slow wave activity; no periodic complexes Diffuse slowing; no periodic complexes MRIPositive pulvinar sign PRNP Genetics Genotype 129MM, no mutations Adenoid Biopsy Lymphoid follicles positive for PrP sc ; vCJD staining pattern Brain Biopsy Immunopositive for PrP sc; “Florid plaques” Intense immunopositive deposits of PrP sc ; Pericellular “encrustations” of stellate-shaped cells with PrP sc Results

6 Countries of Residence of 1st US-Diagnosed vCJD Patient by Calendar Years, Age, and Time Intervals Before vCJD Onset Country*Calendar YearsAge (yrs) Intervals before Onset (yrs) Saudi Arabia1970- late United Stateslate 1997 – late – 4.2 Saudi Arabialate 1998 – early 2003 (onset) *Visited United Kingdom for 4 days in late 1997 and France for 1 and/or 2 weeks in 1995 and/or 1996 (Maximum interval before vCJD onset, 8.1 years)

7 Countries of Residence of 2nd US-Diagnosed vCJD Patient by Calendar Years, Age, and Time Intervals Before vCJD Onset Country*Calendar YearsAge (yrs) Intervals before Onset (yrs) Saudi Arabia Egypt1999 – late – – 0.3 United States late 2005 – early 2006 (onset) 22.5 – *Visited the United States for 1½ months in 1989 and since 2001, occasionally during vacations each year for up to about 3 months at a time.

8  Pathologist Pierluigi Gambetti and his staff at the National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio, USA  Professors Robert Will and Richard Knight and their staff at the UK CJD Surveillance Unit, Edinburgh, Scotland, UK  Neurologist Michael Geschwind and Pathologist Stephen DeArmond and their staff at the University of California, San Francisco, USA  State Epidemiologists and their staff at the Arizona, California, North Carolina and Virginia State Departments of Health, USA  Neurologists Shireen A. Qureshi, Saudi Arabia and Alireza Atri, USA  Pathologist Caterina Giannini, Mayo Clinic, Minnesota, USA  Dr. Imad A. Al Jahdall, Chief Preventive Medicine Services Division, Saudi Aramco Medical Services Organization, Dhahran, Saudi Arabia  Colleagues at the Centers for Disease Control and Prevention, Atlanta, Georgia, USA, particularly members of the Prion and Public Health Office, Division of High-Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases. Acknowledgments

9 T2 & Fluid-Attenuated Inversion-Recovery, FLAIR “Pulvinar Sign” MRI on Saudi vCJD Patient

10 Histopathological and Immunohistochemical Findings on Brain Biopsy of First Saudi vCJD Case compared to sCJD, 2004 AC B A.H&E of a florid plaque in the present case. B.Immunostaining showing florid plaques. C.Immunostaining of sCJDMM1 (B and C Mab 3F4).

11 50 µm B: magnification 40X – US Patient PrP IHC Plaque-like and spider-like formations; HE of the location of the plaque-like formations is unremarkable (mAb 3F4)

12 Thank You! For more information please contact Centers for Disease Control and Prevention 1600 Clifton Road NE, Atlanta, GA Telephone: CDC-INFO ( )/TTY: Web: The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention. National Center for Emerging and Zoonotic Infectious Diseases Division of High-Consequence Pathogens & Pathology

13 National Prion Disease Pathology Surveillance Center - Referrals, By Year of Death * Disease almost certainly acquired outside the US (UK or Saudi Arabia ). YearReferralsPrion disease (total)vCJD 1996 & earlier * * Total (59.5%)3*

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