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From the Klinik und Poliklinik für Neurologie (Drs. Zerr, Bodemer, and Poser), Georg-August-Universität, Göttingen, Germany; Laboratory of Virology (Drs. Pocchiari, Cardone, and Ladogana), Istituto Superiore di Sanità, Rome, Italy; Department of Pathology (Drs. Collins and Fletcher), University of Melbourne, Australia; U360 INSERM, Hôpital de la Salpetriere (Drs. Brandel and Delasnerie-Laupêtre), Paris, France; Department of Applied Epidemiology (Drs. de Pedro Cuesta and Ruiz Bremón), National Centre for Epidemiology, Carlos III Institute of Health, Madrid, Spain; CJD Surveillance Unit, Western General Hospital, Edinburgh, Scotland (Drs. Knight and Will); Klinisches Institut für Neurologie (Drs. Bernheimer and Budka), Universität Wien, Austria; Istituto Neurologico Nazionale C. Besta, (Dr. Awan), Milan, Italy; Department of Applied Epidemiology, National Centre for Epidemiology, Carlos III Institute of Health and Research Brain Bank (Dr. Cuadrado Corrales), Medical School, Complutensis University, Madrid, Spain; and Service de Biochimie et Biologie Moléculaire (Dr. Laplanche), Hôpital Lariboisière, Paris, France.
Address correspondence and reprint requests to Dr. I. Zerr, Klinik und Poliklinik für Neurologie, Georg-August-Universität Göttingen, Robert-Koch-Str. 40, 37075 Göttingen, Germany; e-mail: 106004.1022{at}compuserve.com
OBJECTIVE: To improve diagnostic criteria for sporadic CreutzfeldtJakob disease (CJD).
METHODS: Pooled data on initial and final diagnostic classification of suspected CJD patients were accumulated, including results of investigations derived from a coordinated multinational study of CJD. Prospective analysis for a comparison of clinical and neuropathologic diagnoses and evaluation of the sensitivity and specificity of EEG and 14-3-3 CSF immunoassay were conducted.
RESULTS: Data on 1,003 patients with suspected CJD were collected using a standard questionnaire. After follow-up was carried out, complete clinical data and neuropathologic diagnoses were available in 805 cases. In these patients, the sensitivity of the detection of periodic sharp wave complexes in the EEG was 66%, with a specificity of 74%. The detection of 14-3-3 proteins in the CSF correlated with the clinical diagnosis in 94% (sensitivity). The specificity (84%) was higher than that of EEG. A combination of both investigations further increased the sensitivity but decreased the specificity.
CONCLUSIONS: Incorporation of CSF 14-3-3 analysis in the diagnostic criteria for CJD significantly increases the sensitivity of case definition. Amended diagnostic criteria for CJD are proposed.
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