Brown P, Cervenáková L, McShane L, Goldfarb L G, Bishop K, Bastian F, Kirkpatrick J, Piccardo P, Ghetti B, Gajdusek D C
Laboratory of CNS Studies, NINDS, National Institutes of Health, Bethesda, MD 20892, USA.
Neurology. 1998 Mar;50(3):684-8. doi: 10.1212/wnl.50.3.684.
A 53-year-old man died of sporadic Creutzfeldt-Jakob disease (CJD) after a 1.5-year clinical course. Four and a half years later, his then 55-year-old widow died from CJD after a 1-month illness. Both patients had typical clinical and neuropathologic features of the disease, and pathognomonic proteinase-resistant amyloid protein ("prion" protein, or PrP) was present in both brains. Neither patient had a family history of neurologic disease, and molecular genetic analysis of their PrP genes was normal. No medical, surgical, or dietary antecedent of CJD was identified; therefore, we are left with the unanswerable alternatives of human-to-human transmission or the chance occurrence of sporadic CJD in a husband and wife.
一名53岁男性在经历1.5年的临床病程后死于散发性克雅氏病（CJD）。四年半后，他当时55岁的遗孀在患病1个月后死于CJD。两名患者均具有该疾病典型的临床和神经病理学特征，且在两人的大脑中均存在具有病理诊断意义的抗蛋白酶淀粉样蛋白（“朊病毒”蛋白，或PrP）。两名患者均无神经疾病家族史，且他们的PrP基因分子遗传学分析结果正常。未发现CJD的任何医学、手术或饮食方面的前驱因素；因此，我们只能面对人传人或夫妻双方偶然发生散发性CJD这两个无法解答的可能性。
