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Keywords:

  • bovine spongiform encephalopathy;
  • Creutzfeldt-Jakob disease;
  • epidemiology;
  • prion disease;
  • surgery

Objective

Galicia is the Spanish region in which most bovine spongiform encephalopathy cases have been registered. Meixoeiro Hospital is included in the Galician Health Service (SERGAS). The aim of the study was to analyze the clinical and epidemiological characteristics of Creutzfeldt-Jakob disease (CJD) in the health area of Meixoeiro Hospital and to identify possible specific risk factors to the general public.

Methods

All incident cases of CJD were identified in the health area of Meixoeiro Hospital (187,877 inhabitants) over a 14-year period, 1997–2010, and classified according to WHO diagnostic criteria. We obtained clinical detail and epidemiological information on all cases. Crude and age-specific incidence rates were calculated. A review of surgical or invasive medical procedures was undertaken.

Results

We diagnosed 12 patients with CJD, 10 sporadic CJD (sCJD), and two genetic CJD (gCJD). No iatrogenic or variant CJD was detected. According to Poisson distribution, 3.9 CJD cases would be expected for our area over the 14 years researched. The average yearly mortality rate from CJD was 4.6 cases per million (3.8 from sCJD and 0.8 from gCJD). Eight patients (67%) underwent at least one surgical or invasive medical procedure. Sixteen of twenty-seven (59%) of these procedures were undertaken in Meixoeiro Hospital.

Conclusions

The incidence of CJD in the health area of Meixoeiro Hospital is three times higher than expected. The hypothesis that at least some cases of sCJD are apparently because of covert transmission or zoonosis events should not be formally refuted and might explain the high rate found.