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Volume 18, Number 10—October 2012


Constant Transmission Properties of Variant Creutzfeldt-Jakob Disease in 5 Countries

Abigail B. Diack, Diane Ritchie, Matthew Bishop, Victoria Pinion, Jean-Philippe Brandel, Stephane Haik, Fabrizio Tagliavini, Cornelia Van Duijn, Ermias D. Belay, Pierluigi Gambetti, Lawrence B. Schonberger, Pedro Piccardo, Robert G. Will1, and Jean C. Manson1Comments to Author 
Author affiliations: The Roslin Institute, Easter Bush, Scotland, UK (A.B. Diack, V. Pinion, J.C. Manson); University of Edinburgh, Edinburgh, Scotland, UK (D. Ritchie, M. Bishop, R.G. Will); Cellule Nationale de Référence des Maladies de Creutzfeldt-Jakob, Universite Pierre et Marie Curie-Paris 6, INSERM, and CNRS, Paris, France (J.-P. Brandel, S. Haik); Fdazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy (F. Tagliavini); Erasmus University Medical School, Rotterdam, the Netherlands (C. Van Duijn); Centers for Disease Control and Prevention, Atlanta, Georgia, USA (E.D. Belay, L.B. Schonberger); Case Western Reserve University, Cleveland, Ohio, USA (P. Gambetti); and Food and Drug Administration, Rockville, Maryland, USA (P. Piccardo)

Main Article


Demographic and clinical features of case-patients with variant CJD from the Netherlands, France, Italy, and United States and 2 reference case-patients from the United Kingdom*

Characteristic The Netherlands France Italy United States United Kingdom
1 2
Case-patient sex F F F F M M
Case-patient age at illness onset, y 24 36 25 22 24 35
Case-patient age at death, y 26 37 27 24 25 36
Disease duration, mo 19 14 27 32 14 12
Early psychiatric symptoms Yes Yes Yes Yes Yes Yes
Persistent painful sensory symptoms Yes No Yes No Yes No
Ataxia Yes Yes Yes Yes Yes Yes
Myoclonus, dystonia, or chorea Yes Yes Yes Yes Yes Yes
Dementia Yes Yes Yes Yes Yes Yes
No typical appearance of sporadic CJD on EEG Yes Yes No Yes Yes Yes
Bilateral symmetric pulvinar high signal on MRI scan of brain Yes No Yes Yes Yes No
Positive tonsil biopsy result ND Yes Yes Yes ND ND
Treatment No specific treatment No specific treatment Quinacrine Quinacrine No specific treatment No specific treatment
History of travel to or residence in the United Kingdom No No No Yes† Yes Yes
Codon 129MM Yes Yes Yes Yes Yes Yes
Type 2B PrP Yes Yes Yes Yes Yes Yes

*CJD, Creutzfeldt-Jakob disease; EEG, electroencephalogram; MRI, magnetic resonance imaging; ND, not done; PrP, prion protein.
†Born in United Kingdom in 1979, moved to United States in 1992.

Main Article

1Joint senior authors.