CREUTZFELDT-JAKOB DISEASE AMONG LIBYAN JEWS

被引：23

作者：

KORCZYN, AD

机构：

[1] Department of Neurology and Department of Physiology and Pharmacology Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv

来源：

EUROPEAN JOURNAL OF EPIDEMIOLOGY | 1991年 / 7卷 / 05期

关键词：

CREUTZFELDT-JAKOB DISEASES; PRION DISEASE; JEWS; LIBYA; GENETICS; PATHOPHYSIOLOGY;

D O I：

10.1007/BF00143127

中图分类号：

R1 [预防医学、卫生学];

学科分类号：

1004 ; 120402 ;

摘要：

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 prion protein, (predisposed by the mutation). Later, the disease progresses through cell-to-cell transmission.

引用

页码：490 / 493

页数：4

共 17 条

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