人朊病毒病的临床、病理及动物传递的实验研究

Surgical pathological and experimental transmission studies of human prion disease.

根据本文报告经病理证实的人朊病毒病１４例。Ｃｒｅｕｔｚｆｅｌｄｔ－Ｊａｋｏｂ氏病（ＣＪＤ）９例、Ｇｅｒｓｔｍａｎｎ－Ｓｔｒａｕｓｓｌｅｒ－Ｓｃｈｅｉｎｋｅｒ氏综合（ＧＳＳ）５例。５例免疫组化染色阳性。３例ＣＪＤ脑匀浆进行Ｃ５７ＢＬ／６ｃｌａ、ＢＱ１ｂ／ｃ及ｗｉｓｔｅｒ鼠脑内接种．１例于第二代发病，余为第三代发病。资料提示：１．国人ＣＪＤ相对发病较多；二、国内确有ＧＳＳ值得注意；三、免疫组化相当敏感；四、ＣＪＤ脑活检组织可成功地进行实验的动物传递。

Fourteen cases of human prion disease were pathologically confirmed. 9 of them were Creutzfeldt -Jakob disease (CJD), the other 5 were Gerstmann -Straussler syndrome (GSS). Immunohistochemical staining using anti-human Prp antiserum was carried out in 5 biopsies cases and positive reaction was received. Successful transmission with biopsied brain homoqenete from 3 patients with CJD to mice (C_(57)BL/6oLa and Balb/c) and rats was seen,and occurence of disease was found on the 2nd passage in one case, on the third passage in the other two cases. It is suggested that: (1) acute onset of CJD would be predominant in our nation compared with onset of this disease in foreign countries;(2) it should be noted that there is GSS in our nation;(3) immunostaining using PrP antibody might be a sensitive method to detect this disease; (4)biopsied brain tissues from patiens with CJD could be successfuly transmitted to experimental animals.