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Volume 18, Number 6—June 2012
CME ACTIVITY - Synopsis

Iatrogenic Creutzfeldt-Jakob Disease, Final Assessment

Paul BrownComments to Author , Jean-Philippe Brandel, Takeshi Sato, Yosikazu Nakamura, Jan MacKenzie, Robert G. Will, Anna Ladogana, Maurizio Pocchiari, Ellen W. Leschek, and Lawrence B. Schonberger
Author affiliations: Centre à l’Energie Atomique, Fontenay-aux-Roses, France (P. Brown); Institut National de la Santé et de la Recherche Médicale, Paris, France (J.-P. Brandel); Nanohana Clinic, Tokyo, Japan (T. Sato); Jichi Medical University, Yakushiji, Japan (Y. Nakamura); Western General Hospital, Edinburgh, Scotland, UK (J. MacKenzie, R.G. Will); Istituto Superiore de Sanità, Rome, Italy (A. Ladogana, M. Pocchiari); National Institutes of Health, Bethesda, Maryland, USA (E.W. Leschek); Centers for Disease Control and Prevention, Atlanta, Georgia, USA (L.B. Schonberger)

Main Article

Table 3

Comparison of PRNP codon 129 genotype frequencies and incubation periods in growth hormone– and dura mater–associated cases of iatrogenic CJD*

Category MM VV Homozygotes Heterozygotes
Population
Healthy Caucasian, %† 40 10 50 50
European, with sporadic CJD, % 67 17 84 16
Healthy Japanese, % 92 0 92 8
Japanese, with sporadic CJD, (%) 97 1 98 2
Infection source
Growth hormone
France (111)
Genotype frequency, % 54 15 69 31
Incubation period, y 12 9 11 17
United Kingdom (28)
Genotype frequency, % 4 50 54 46
Incubation period, y 21 18 20 23
United States (11)
Genotype frequency, % 55 18 73 27
Incubation period, y 21 18 20 23
Combined total (150)
Genotype frequency, % 45 22 67 33
Incubation period, y 13 12 13 17
Dura mater
Japan (54)‡
Genotype frequency, % 96 0 96 4
Incubation period, y 16 NA 16 13
Countries other than Japan (54)§
Genotype frequency, % 65 15 80 20
Incubation period, y 12 12 12 16
Combined total (108)
Genotype frequency, % 81 7 88 12
Incubation period, y 14 12 14 16

*CJD, Creutzfeldt-Jakob disease; M, methionine; V, valine; NA, not applicable. All values are rounded to the nearest whole number.
†Based on several large-scale population studies (5–9).
‡Personal communication from M. Yamama, Department of Neurology, Kanazawa University Hospital, Kanazawa, Japan.
§Cases from France (11), Spain (11), Germany (10), Italy (8), the Netherlands (5), and 1 or 2 cases from each of 6 other countries with Caucasian populations.

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Page created: May 17, 2012
Page updated: May 17, 2012
Page reviewed: May 17, 2012
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