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the plenum and plant are avoided unless there is damage to these filters
Because the mam filters are readily accessible m such cabmets, it is an easy
matter to prevent particle disperston during their removal, by prior treatment
of the filter surface, e.g., with hairspray or latex solution.

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39 Brown, P , Gibbs, C J., Amyx, H L , Kingsbury, D T., Rohwer, R G , Sulima,
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41. Kimberlm, R H., Walker, C. A , Mtllson, G. C , Taylor, D M , Robertson, P. A ,
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of the IXth Internatronal Congressof Infecttous and Parastttc Dueases,Mumch
49 Advisory Group on the Management of Patients wtth Spongiform Encephalopathy
(Creutzfeldt-Jakob Dtsease)[CJD] (198 1) Report to the Chief Medrcal Officers of
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50 Wight, A. L (I 993) Prevention of tatrogemc transmtsston of Creutzfeldt-Jakob
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heat treatment systems for processing animal waste of ruminant ortgm, wtth a
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56 Fraser, H , Bruce, M E , Chree, A , McConnell, I , and Wells, G A H (1992)
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60 Amyx, H L , Gibbs, C J , Kingsbury, D. T , and GaJdusek, D C (198 1) Some
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61 Armbrustmacher, V W Personal commumcation cited by Trtford, M. and Basttan,
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64 Esirt, M M (1989) Dzagnostzc Neuropathology Blackwell, Oxford.
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Surveillance of Prion Diseases in Humans
Robert G. Will

1. Introduction
In contrast to ammal priori diseases, the human forms of prion disease are
rare and include Creutzfeldt-Jakob disease (CJD), km-u,Gerstmann-Straussler-
Scheinker syndrome (GSS), and fatal familial insomnia (FFI). Meticulous
epidemiological research has demonstrated that km-u was caused by cross-con-
tammation in the course of ritual canmbahsm (I), whereas advances m molecu-
lar biology have provided powerful evidence that famihal forms of human PI ion
disease, includmg GSS and FFI, are linked to mutations within the priori pro-
tein (PrP) gene (2-4). The cause of sporadic CJD remains unknown despite
both extensive epidemiological research and the remarkable advances m the
understanding of pathogenic mechanisms in priori diseases.
Sporadic CJD accounts for about 85% of all cases of human priori disease
and has been the subJect of extensive media and public interest following the
advent of bovine spongiform encephalopathy (BSE) and attendant concerns
regarding the possrbihty of a risk to public health. In the absence of an m vivo
diagnostic marker, currently the only mechanism of determining whether BSE
has caused disease in the human population is by epidemiological surveillance.
In view of the manifest implications of demonstrating such a link between
animal and human prion diseases, it is appropriate to critically review the cur-
rent methodologies for epidemiological research in CJD and to discuss spe-
cific difficulties in relation to casedefinition, bias, and interpretation of results.
2. Case Ascertainment
Epidemiological surveillance of CJD is complicated by the rarity of the dis-
ease, the absence of any specific diagnostic test, and the apparently random
occurrence of cases.A high level of caseascertainment is nonetheless essential
if meaningful results are to be obtained, which is dependent on the systematic
From Methods m Molecular Medmne Pnon Diseases
Edlted by H Baker and R M Ridley Humana Press Inc , Totowa, NJ

779
120 Will




0.0
30-34
2024 25-29 35-39 4044 45-49 SO.54 55-59 60-64 65-69 70-74 75-79 80-84 85-89




Fig. 1. Age-specific incidence rates in sporadic cases of CJD 1970-1994 (25 yr):
N=573.

identification of incident cases. In the United Kingdom data on CJD has been
accumulated systematically since 1970 (5,6) and has depended on two major
mechanisms of case identification:
1. Direct referral of cases from targeted professional groups: neurologists, neuro-
pathologists, and neurophysiologists.
2. Death certificates coded under the specific rubrics 046.1 and 33 1.9 in the 9th
ICD revision.
2.1. Direct Referral
CJD is usually a dramatic illness. Patients present with rapidly progressive
multifocal neurological dysfunction. The majority develop involuntary
myoclonic movements of the limbs and the terminal stage of akinetic mutism
supervenes within a few weeks, with median survival from first symptom to
death of approx 6 mo. The underlying assumption in the identification of such

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