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Laboratories of CNS Studies (Drs. Brown, Cervenáková, Goldfarb, and Gajdusek, and G. Graupera and J. Ligan) and Molecular and Cellular Neurobiology (Dr. McCombie), National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD; the Institute for Basic Research in Developmental Disabilities (Dr. Rubenstein and C. Scalici), Staten Island, NY; the Department of Clinical Neurosciences (Dr. Will), Western General Hospital, Edinburgh, UK; the Laboratory of Virology (Dr. Pocchiari), Istituto Superiore di Sanitá, Rome, Italy; the Service of Neurosurgery (Dr. Martinez-Lage), Hospital Universitario, Murcia, Spain; and the Institute of Neurology (Dr. Masullo), Catholic University, Rome, Italy.
We tested DNA from 15 centrally infected cases of iatrogenic Creutzfeldt-Jakob disease (CJD) (dura mater or corneal homografts and stereotactic EEG electrodes), 11 peripherally infected cases (native human growth hormone or gonadotrophin), and 110 control individuals for the presence of mutations in the chromosome 20 amyloid gene. No patient or control had any of the known pathogenic point or insert mutations found in familial disease, but allelic homozygosity at polymorphic codon 129 was present in all but two (92%) of the 26 patients, compared with 54 (50%) of the 110 controls (p < 0.001). Pooled data from all identified and tested cases of iatrogenic disease yielded a worldwide total of 56 patients, of whom all but four were homozygous at codon 129 (p < 0.001). These findings support the thesis that homozygosity at codon 129 enhances susceptibility to iatrogenic infections of both central and peripheral origin, with evident implications for the population of dura mater homograft and pituitary hormone recipients whose lives have been complicated by the possibility of exposure to the infectious agent of CJD.
Address correspondence and reprint requests to Dr. Paul Brown, Building 36, Room 5B21, NIH, Bethesda, MD 20892.
Received June 30, 1993. Accepted for publication in final form August 17,1993.
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