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Neuroepidemiology 2008;31:229–240

Surgery and Risk of Sporadic Creutzfeldt-Jakob Disease in Denmark and Sweden: Registry-Based Case-Control Studies

Mahillo-Fernandez I.a · de Pedro-Cuesta J.a · Bleda M.J.a · Cruz M.b · Mølbak K.c · Laursen H.d · Falkenhorst G.c · Martínez-Martín P.a · Siden Å.b
aDepartment of Applied Epidemiology, National Center for Epidemiology, and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Carlos III Institute of Health, Madrid, Spain; bDepartment of Clinical Neurosciences, Neurology Division, Karolinska Institutet, Stockholm, Sweden; cDepartment of Epidemiology, Statens Serum Institute, and dNeuropathology Laboratory, Rigshospitalet, Copenhagen, Denmark
email Corresponding Author

 goto top of outline Key Words

  • Creutzfeldt-Jakob disease, case-control studies
  • Creutzfeldt-Jakob disease, risk assessment
  • Denmark, prion diseases
  • Sweden, prion diseases

 goto top of outline Abstract

Background: Epidemiologic evidence of surgical transmission of sporadic Creutzfeldt-Jakob disease (sCJD) remains controversial. Methods: From Danish and Swedish registries we selected 167 definite and probable sCJD cases (with onset between 1987 and 2003) and 3,059 controls (835 age-, sex-, and residence-matched, and 2,224 unmatched). Independent of case/control status, surgical histories were obtained from National Hospital Discharge Registries. Surgical procedures were categorized by body system group and lag time to onset of sCJD. Exposure frequencies were compared using logistic regression. Results: A history of any major surgery, conducted ≥20 years before sCJD onset, was more common in cases than both matched (OR = 2.44, 95% CI = 1.46–4.07) and unmatched controls (OR = 2.25, 95% CI = 1.48–3.44). This observation was corroborated by a linear increase in risk per surgical discharge (OR = 1.57, 95% CI = 1.13–2.18; OR = 1.50, 95% CI = 1.18–1.91). Surgery of various body systems, including peripheral vessels, digestive system and spleen, and female genital organs, was significantly associated with increased sCJD risk. Conclusions: A variety of major surgical procedures constitute a risk factor for sCJD following an incubation period of many years. A considerable number of sCJD cases may originate from health care-related accidental transmission.

Copyright © 2008 S. Karger AG, Basel

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 goto top of outline Author Contacts

Jesús de Pedro Cuesta
Centro Nacional de Epidemiología, Instituto de Salud Carlos III
Calle Sinesio Delgado 6, Pabellón 12
ES–28029 Madrid (Spain)
Tel. +34 91 8222 650, Fax +34 91 3877 815, E-Mail

 goto top of outline Article Information

EUROSURGYCJD group members: Danish team: Gerhard Falkenhorst, Henning Laursen and Kåre Mølbak; Finnish team: Jussi Kovanen; Swedish team: Mabel Cruz and Åke Siden; Spanish team: Javier Almazán, María J. Bleda, Miguel Calero, Ignacio Mahillo Fernandez, Pablo Martínez Martín, Jesús de Pedro Cuesta (coordinator) and Alberto Rábano.

Received: April 23, 2008
Accepted: July 31, 2008
Published online: October 9, 2008
Number of Print Pages : 12
Number of Figures : 2, Number of Tables : 5, Number of References : 38

 goto top of outline Publication Details


Vol. 31, No. 4, Year 2008 (Cover Date: November 2008)

Journal Editor: Feigin V.L. (Auckland)
ISSN: 0251–5350 (Print), eISSN: 1423–0208 (Online)

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