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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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