title: A new variant of Creutzfeldt-Jakob disease in the UK.
abstract: BACKGROUND
  Epidemiological surveillance of Creutzfeldt-Jakob disease (CJD) was reinstituted in the UK in 1990 to identify any changes in the occurrence of this disease after the epidemic of bovine spongiform encephalopathy (BSE) in cattle.
  
  
  METHODS
  Case ascertainment of CJD was mostly by direct referral from neurologists and neuropathologists. Death certificates on which CJD was mentioned were also obtained. Clinical details were obtained for all referred cases, and information on potential risk factors for CJD was obtained by a standard questionnaire administered to patients' relatives. Neuropathological examination was carried out on approximately 70% of suspect cases. Epidemiological studies of CJD using similar methodology to the UK study have been carried out in France, Germany, Italy, and the Netherlands between 1993 and 1995.
  
  
  RESULTS
  Ten cases of CJD have been identified in the UK in recent months with a new neuropathological profile. Other consistent features that are unusual include the young age of the cases, clinical findings, and the absence of the electroencephalogram features typical for CJD. Similar cases have not been identified in other countries in the European surveillance system.
  
  
  CONCLUSIONS
  These cases appear to represent a new variant of CJD, which may be unique to the UK. This raises the possibility that they are causally linked to BSE. Although this may be the most plausible explanation for this cluster of cases, a link with BSE cannot be confirmed on the basis of this evidence alone. It is essential to obtain further information on the current and past clinical and neuropathological profiles of CJD in the UK and elsewhere.
authors.affiliation: National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK.
authors.forename: R G
authors.initials: RG
authors.lastname: Will
authors.forename: J W
authors.initials: JW
authors.lastname: Ironside
authors.forename: M
authors.initials: M
authors.lastname: Zeidler
authors.forename: S N
authors.initials: SN
authors.lastname: Cousens
authors.forename: K
authors.initials: K
authors.lastname: Estibeiro
authors.forename: A
authors.initials: A
authors.lastname: Alperovitch
authors.forename: S
authors.initials: S
authors.lastname: Poser
authors.forename: M
authors.initials: M
authors.lastname: Pocchiari
authors.forename: A
authors.initials: A
authors.lastname: Hofman
authors.forename: P G
authors.initials: PG
authors.lastname: Smith
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000818:Animals; D001921:Brain / Q000473:pathology; D002417:Cattle; D004396:Coloring Agents; D007562:Creutzfeldt-Jakob Syndrome / Q000145:classification / Q000473:pathology / Q000503:physiopathology; D016643:Encephalopathy, Bovine Spongiform / Q000473:pathology / Q000635:transmission; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D009420:Nervous System / Q000473:pathology; D011159:Population Surveillance; D011328:Prions / Q000302:isolation & purification; D012307:Risk Factors; D006113:United Kingdom / Q000453:epidemiology; D015047:Zoonoses
chemical_list: D004396:Coloring Agents; D011328:Prions