Creutzfeldt-Jakob disease in a husband and wife |
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Authors: | P Brown L Cervenáková L McShane LG Goldfarb K Bishop F Bastian J Kirkpatrick P Piccardo B Ghetti DC Gajdusek |
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Affiliation: | Laboratory of CNS Studies, NINDS, National Institutes of Health, Bethesda, MD 20892, USA. |
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Abstract: | 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. |
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