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Neurology 2002;59:543-548
© 2002 American Academy of Neurology

Sporadic Creutzfeldt–Jakob disease and surgery

A case–control study using community controls

H. J.T. Ward, MFPHM, D. Everington, MSc, E. A. Croes, MD, A. Alperovitch, MD, N. Delasnerie–Lauprêtre, MD, I. Zerr, MD, S. Poser, MD, C. M. van Duijn, PhD and for the European Union (EU) Collaborative Study Group of Creutzfeldt–Jakob Disease (CJD)*

From the National CJD Surveillance Unit (Dr. Ward and D. Everington), Western General Hospital, Edinburgh, UK; Department of Epidemiology and Biostatistics (Drs. van Duijn and Croes), Erasmus University Medical Centre Rotterdam, the Netherlands; U360 INSERM (Drs. Alperovitch and Delasnerie–Lauprêtre), Hôpital de la Salpetrière, Paris, France; and Neurologische Klinik (Drs. Zerr and Poser), Georg-August Universität, Göttingen, Germany.

Address correspondence and reprint requests to Dr. H.J.T. Ward, National CJD Surveillance Unit, Western General Hospital, Edinburgh, EH4 2XU, UK; e-mail: h.ward{at}ed.ac.uk

Background: The cause of sporadic Creutzfeldt–Jakob disease (CJD) is unknown. Previous studies found a link with a history of surgery but had methodologic problems.

Objective: To help elucidate medical and associated risk factors for sporadic CJD as part of the 1993 to 1995 European Union collaborative studies of CJD.

Methods: Medical and associated risk factors from 326 patients with sporadic CJD, taken from population-based studies performed between 1993 and 1995 in France, Germany, the Netherlands, and the UK, were compared with 326 community controls recruited by telephone in 2000.

Results: A history of surgery was significantly associated with the risk of sporadic CJD (odds ratio [OR]: 1.8; 95% CI: 1.2 to 2.6), which was not dependent on the number of surgical procedures, and was stronger in females (OR: 2.5; 95% CI: 1.5 to 4.0). Gynecologic (OR: 1.5; 95% CI: 1.0 to 2.3) and "other" operations (any operation other than neurologic, eye, ear, gallbladder, gastrointestinal, and gynecologic operations, tonsillectomy, and appendectomy) (OR: 1.5; 95% CI: 1.1 to 2.1) were associated with risk of CJD. Tonsillectomy (OR: 0.3; 95% CI: 0.2 to 0.5) and appendectomy (OR: 0.6; 95% CI: 0.4 to 0.8) were observed less frequently in cases. An increased risk was also found with a history of ear piercing in females (OR: 1.6; 95% CI: 1.1 to 2.5) and psychiatric visit(s) (OR: 2.6; 95% CI: 1.5 to 4.3).

Conclusions: These results support the hypothesis that cases of sporadic CJD may result from hitherto unrecognized surgical contamination events. However, because of the limits of the study design, the rarity of the disease, and the potential for bias, the results should be interpreted with caution.




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