NR AGRU
AU Korczyn,A.D.
TI Creutzfeldt-Jakob disease among Libyan Jews
QU European Journal of Epidemiology 1991 Sep; 7(5): 490-3
PT journal article; review; review, tutorial
AB The focus of CJD among Jews of Libyan origin has been recognized for two decades, but the reasons underlying it were unknown. Prevailing views suggested transmission from sheep infected with scrapie. However, recent data show that in fact CJD in this ethnic group is a genetically determined disease due to a point mutation on the codon 200 of the prion protein gene. The clinical characteristics of CJD in this group, and particularly the less common periodic activity in the EEG, are reviewed. New findings include peripheral neuropathy of the demyelinating type in two cases, presumably due to involvement of Schwann cells. The pathophysiology of the disease includes, presumably, a focal post-translational modification of the prior protein, (predisposed by the mutation). Later, the disease progresses through cell-to-cell transmission.
ZR 19
MH Adult; Aged; Creutzfeldt-Jakob Syndrome/*ethnology/genetics; Human; Israel/epidemiology; *Jews; Libya/ethnology; Middle Age
AD Department of Neurology, Sackler Faculty of Medicine, Tel Aviv University, Israel.
SP englisch
PO Italien
OR Prion-Krankheiten 5