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Case ascertainment for an epidemiologic study of Creutzfeldt-Jakob disease (CJD) can be difficult. This report investigates the efficiency of various sources of case ascertainment for CJD. Cases were identified utilizing neuropathologists, hospitals and death certificates from 11 targeted states. For the period of 1986–1988, 247 death certificates indicating a diagnosis of CJD were obtained. Only 26 potential cases were identified without death certificates. The proportion of neuropathologically confirmed cases identified by death certificates only, i.e., which were not identified through any other source, was 42%. Furthermore, 80% of all the neuropathologically confirmed cases were ascertained utilizing death certificates as a source. Of the remaining 20%, 7% were ascertained through neuropathologists only, 10% through hospitals only, 1.5% through a combination of hospitals and neuropathologists, and 1.5% through another source. The false-positive rate for death certificates with neuropathology (which may have been performed after the death certificate was filled out) was estimated to be 8.3%. The results indicate that death certificates were by far the most efficient source for initial ascertainment of potential CJD cases to be followed by verification of diagnosis.

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AimsTo evaluate the hypothesis that sporadic Creutzfeldt-Jakob disease (sCJD) may be transmitted through ocular tonometry.BackgroundThe infectious agent of sCJD may be present in the cornea prior to clinical symptoms. Cornea infectiousness has been documented by cornea transplants in guinea pigs and humans. sCJD is resistant to complete inactivity by conventional sterilization techniques. Thus contact tonometry equipment is not disinfected sufficiently to kill sCJD. We previously hypothesized that contact tonometry is a sCJD risk factor.Study DesignPopulation-based case-control study.Place and Duration of StudyDepartment of Neurology, School of Medicine, Loma Linda University, Loma Linda, CA, USA; 4 years.MethodologyAn 11-state case-control study of pathologically confirmed definite sCJD cases, individually matched controls, and a sample of control surrogates was conducted. Ocular tonometry histories were obtained from case-surrogates, controls, and a sample of control-surrogates.ResultsThe odds ratio (OR) for ever vs never having had an ocular tonometry test was statistically significant for matched and unmatched analyses for 15 through 3 years prior to disease onset, using both control self-responses and control surrogates: ORs were ∞ and 19.4 with 1-sided P-values <0.0001 and 0.003 and ORs=∞ and 11.1 with 1-sided P-values <0.003 and 0.02, respectively. ORs increased as the number of tonometry tests increased during this age period: trend test, 2-sided P-value < 0.0001. For ≥5 vs <5 tonometry tests, the OR was 5.8 (unmatched) and 3.7 (matched), 2-sided P-value<0.00005. Respondents generally could not specify the type of tonometry. There was no indication of increased tonometry testing among cases within 2 years of disease onset.ConclusionsThe a priori hypothesis was supported. Contact tonometry, preferred by ophthalmologists, may be capable of transmitting sCJD. Consideration should be given to using disposable instrument covers after each use. The use the disposable covers or non-contact tonometry is preferable in the absence of effective disinfectant processes at this time.