Creutzfeldt-Jakob disease in old age
Journal Title: Postępy Nauk Medycznych - Year 2010, Vol 23, Issue 4
Abstract
This review covers the problem of Creutzfeldt-Jakob disease (CJD) in the old age. A short inquiry performed for the purpose of this review revealed that in the Austrian database, there were 17 cases between 70-75 years old; 10 cases: 76-79 years old; 11 cases 80-85 years old but only 1 case 86 years old. In the Netherlands, out of a total of 150 CJD patients for years 1998-2008, they were 39 cases between 70 and 75 years of age (or 28 if age 70 is not included); 15 cases: 76-79 years of age; 9 cases: 80-85 years of age but non for 86 year of age or older. In the UK, 42% of 1089 sCJD patients were aged 70 or over at death during the period May 1990-November 2009, comprising 33% of sCJD cases aged 70-79 and 9% of cases aged 80 or over. Analysis of the codon 129 polymorphism in the prion protein gene (PRNP) in the UK sCJD cases found overall 63% were methionine homozygotes (MM), 18% were valine homozygotes (VV) and 19% were heterozygotes (MV). In the cases aged 70 or over at death, the distribution was 68% MM, 16% VV and 16% MV.The Polish data are very interesting in this respect: there are 6 of 30 cases of sCJD over 70 (20%); the 129 codon data are available for 3, 2 x MM, 1 x MV.The literature on the subject of this review is very limited.
Authors and Affiliations
Paweł Liberski, Caspar Jansen, Beata Sikorska, James Ironside
Keywords
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