Publications

epidemiology of creutzfeldt-jakob disease in england and wales.	some aspects of the epidemiology of creutzfeldt-jakob disease in england and wales in the decade 1964-73 were studied with the object of detecting evidence of natural transmission of this slow virus encephalopathy. some geographical clustering and possibility of contact between cases was found.	1975	1097599
a retrospective study of creutzfeldt-jakob disease in england and wales 1970-79. i: clinical features.	a comprehensive search yielded 121 confirmed cases of creutzfeldt-jakob disease who died in england and wales in the decade 1970-79, and 31 probable cases. the clinical features and the results of investigation are described. three subgroups of patients were distinguished by clinical characteristics and contrasting course.	1984	6368752
a retrospective study of creutzfeldt-jakob disease in england and wales 1970-1979. ii: epidemiology.	a comprehensive search yielded 121 confirmed cases of creutzfeldt-jakob disease who died in england and wales in the decade 1970-1979, 31 probable cases and 10 possible cases. descriptive epidemiological data are presented. the average annual incidence was 0.3 cases/million. an unexpected female excess was found. there was no evidence of space-time clustering of cases and no associations with occupation or past medical treatment were apparent. there was statistically significant variation in inc ...	1986	3528394
creutzfeldt-jakob disease in england and wales, 1980-1984: a case-control study of potential risk factors.	an attempt was made to ascertain all cases of creutzfeldt-jakob disease occurring in england and wales during the 5 year period 1980-1984. the mean annual mortality rate was 0.49/million; women were more frequently affected than men. the age-specific mortality rate reached a peak in the seventh decade. a case-control study involving 92 of the 122 definite and probable cases ascertained failed to confirm the reality of previously suspected aetiological agents in the environment. although there wa ...	1988	3066847
geographical distribution of cases of creutzfeldt-jakob disease in england and wales 1970-84.	two hundred and sixty seven patients with a definite or probable diagnosis of creutzfeldt-jakob disease (cjd) were identified in an attempt to ascertain all cases of the disease in england and wales in the period 1970-84. no evidence of space-time clustering of dates and places of onset was found for these cases. two age- and sex-matched controls were selected for 72 of the 122 cases diagnosed in the period 1980-84 and life histories of places of residence were obtained for these cases and the c ...	1990	2199610
epidemiological surveillance of creutzfeldt-jakob disease in the united kingdom.	systematic epidemiological surveillance of creutzfeldt-jakob disease was carried out in england and wales covering the years 1970-1984. the results of this work provide a basis from which to assess any change in the pattern of creutzfeldt-jakob disease following the occurrence of bovine spongiform encephalopathy. the methodology, early results and problems of the current systematic national survey of cjd, initiated in may 1990, are discussed.	1991	1761103
[spongiform encephalopathies with special reference to bovine spongiform encephalopathy].	in switzerland bovine spongiform encephalopathy (bse) was detected for the first time in november 1990. it is a transmissible disease of the central nervous system similar to creutzfeldt-jakob disease (cjd), gerstmann-sträussler-scheinker syndrome (gss) and kuru in man, and, in animals, scrapie in sheep and goats, chronic wasting disease (cwd) in captive mule deer and elk of north america and transmissible mink encephalopathy (tme) of farm reared mink. the infectious agent of the spongiform ence ...	1992	1615298
increasing mortality from creutzfeldt-jakob disease in england and wales since 1979: ascertainment bias from increase in post-mortems?	creutzfeldt-jakob disease (cjd) is a rare and fatal dementing illness. a direct link has been proposed between cattle infected with bovine spongiform encephalopathy (bse) and a newly-identified variant of cjd. one possible explanation for the emergence of this new variant, together with a general increase in death due to classical cjd, would be ascertainment bias, due to an increase in the frequency of post-mortems in death attributed to dementia. this article uses national mortality records to ...	1996	8895949
[the 10 cases which shook england. distinctive features of a new variant of creutzfeldt-jakob syndrome].		1996	8667787
is mad cow disease caused by a bacteria?	transmissible spongioform enchephalopathies (tse's), include bovine spongiform encephalopathy (also called bse or "mad cow disease"), creutzfeldt-jakob disease (cjd) in humans, and scrapie in sheep. they remain a mystery, their cause hotly debated. but between 1994 and 1996, 12 people in england came down with cjd, the human form of mad cow, and all had eaten beef from suspect cows. current mad cow diagnosis lies solely in the detection of late appearing "prions", an acronym for hypothesized, ge ...	2004	15325025
phenotypic heterogeneity and genetic modification of p102l inherited prion disease in an international series.	the largest kindred with inherited prion disease p102l, historically gerstmann-sträussler-scheinker syndrome, originates from central england, with émigrés now resident in various parts of the english-speaking world. we have collected data from 84 patients in the large uk kindred and numerous small unrelated pedigrees to investigate phenotypic heterogeneity and modifying factors. this collection represents by far the largest series of p102l patients so far reported. microsatellite and genealogic ...	2008	18757886