NR AMOF
AU Will,R.G.; Ironside,J.W.; Zeidler,M.; Cousens,S.N.; Estibeiro,K.; Alperovitch,A.; Poser,S.; Pocchiari,M.; Hofman,A.; Smith,P.G.
TI A new variant of Creutzfeldt-Jakob disease in the UK
QU Lancet 1996 Apr 6; 347(9006): 921-5
KI Lancet. 1996 Apr 27;347(9009):1181. PMID: 8609775 Lancet. 1996 Apr 6;347(9006):916-7. PMID: 8598749 Lancet. 1996 Apr 6;347(9006):917-8. PMID: 8598750 Lancet. 1996 May 11;347(9011):1332. PMID: 8622524 Lancet. 1996 May 11;347(9011):1332. PMID: 8622525 Lancet. 1996 May 11;347(9011):1332-3. PMID: 8622526 Lancet. 1996 May 11;347(9011):1333. PMID: 8622527
PT journal article
AB
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.
FINDINGS: 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.
INTERPRETATION: 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.
IN
Nach dem Ausbruch der BSE-Seuche wurde in England 1990 die Überwachung der Creutzfeldt-Jakob-Krankheit neu organisiert, um möglicherweise in Folge der BSE eintretende Änderungen bei den menschlichen Erkrankungen zu bemerken. Man nutzt hauptsächlich direkte Berichte von Neurologen und Neuropathologen, erhält aber auch Totenscheine mit entsprechenden Vermerken. Bei jedem der Meldestelle bekannt werdenden Fall werden die klinischen Einzelheiten und mögliche Risikofaktoren erfragt. Bei 70% der gemeldeten Fälle erfolgt eine neuropathologische Nachuntersuchung. Vergleichbare epidemiologische Studien wurden 1993-1995 auch in Frankreich, Deutschland, Italien, und den Niederlanden durchgeführt.
In den letzten Monaten (Jahren) traten in England 10 Fälle mit ungewöhnlichem neuropathologischen Profil auf. Die Betroffenen waren ungewöhnlich jung und zeigten nicht die bei der Creutzfeldt-Jakob-Krankheit häufigen Veränderungen des EEG.
ZR 18
MH Adolescent; Adult; Age Factors; Age of Onset; Animal; Brain/pathology; Cattle; *Creutzfeldt-Jakob Syndrome/classification/pathology/physiopathology; Dyes; Encephalopathy, Bovine Spongiform/pathology/transmission; Female; Great Britain/epidemiology; Human; Immunohistochemistry; Male; Nervous System/pathology; Population Surveillance; Prions/isolation & purification; Risk Factors; Support, Non-U.S. Gov't; Zoonoses
AD National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK
SP englisch
PO England
OR Prion-Krankheiten 8