Kulczycki Jerzy
I Klinika Neurologiczna, Instytutu Psychiatrii i Neurologii w Warszawie.
Przegl Epidemiol. 2006;60 Suppl 1:63-7.
Some recent views on ethiopathogenesis and epidemiology of four main forms of CJD, based on up to-day experiences and expectations for the future, are presented. The sporadic form of the disease (sCJD) displays a stable morbidity--ca. 1 case/1 million population yearly. The reasons of its so constant appearance remain still unknown. The familial forms of CJD (fCJD) depending upon more than 40 mutations in PRNP gene known today are inherited as autosomal dominant train. The clinical and neuropathological phenotype of patients belonging to various families are usually very different. The best epidemiological prognosis is attributed to iatrogenic form of CJD (iCJD), since both the medical errors causative of the disease and methods of avoiding of them are now very good recognized. The serious fears in many countries raises variant CJD (vCJD), connected etiologically with BSE, because of unknown duration of its incubation period and regular chronic involvement by prions reticulo-lymphatic tissue in infected persons.
本文基于目前的经验和对未来的期望，介绍了关于克雅氏病（CJD）四种主要形式的病因发病机制和流行病学的一些最新观点。散发性克雅氏病（sCJD）的发病率稳定，约为每年1例/100万人口。其持续出现的原因仍然不明。目前已知，家族性克雅氏病（fCJD）取决于PRNP基因中的40多种突变，呈常染色体显性遗传。不同家族患者的临床和神经病理表型通常差异很大。医源性克雅氏病（iCJD）的流行病学预后最佳，因为导致该病的医疗失误及其避免方法目前已得到很好的认识。变异型克雅氏病（vCJD）在许多国家引起了严重担忧，它在病因上与牛海绵状脑病（BSE）有关，因为其潜伏期未知，且感染人群的朊病毒会持续慢性侵犯网状淋巴组织。
