NR AUBG
AU Kovacs,G.G.; Laszlo,L.; Bakos,A.; Minarovits,J.; Bishop,M.T.; Ströbel,T.; Vajna,B.; Mitrova,E.; Majtenyi,K.
TI Increased incidence of genetic human prion disease in Hungary
QU Neurology 2005 Nov 22; 65(10): 1666-9
PT journal article
AB The authors performed analysis of the prion protein gene (PRNP) in 27 out of 109 confirmed prion disease patients between 1994 and 2004. E200K mutation was found in 17 cases. Another 10 patients, lacking PRNP analysis, showed positive family history. The mean annual incidence (0.27/million) and proportion (25.6%) of genetic prion disease is unusually high in Hungary and might be related to the migration of ancestors from the Slovakian focus.
MH Adult; Aged; Amyloid/*genetics; Brain/pathology/physiopathology; Cohort Studies; DNA Mutational Analysis; Female; Genetic Predisposition to Disease/ethnology/*genetics; Genetic Screening; Humans; Hungary/epidemiology; Incidence; Male; Middle Aged; Mutation/*genetics; Prion Diseases/*epidemiology/ethnology/*genetics; Protein Precursors/*genetics; Research Support, Non-U.S. Gov't; Retrospective Studies; Risk Factors; Slovakia/ethnology
AD National Institute of Psychiatry and Neurology, Hungarian Reference Centre for Human Prion Disease, Budapest, Hungary. kovacsgg@opni.hu
SP englisch
PO USA