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13 Infectious and Sporadic Prion Diseases

Robert G. Will, Michael P. Alpers, Dominique Dormont, Lawrence B. Schonberger


Creutzfeldt-jakob disease (CJD) was first identified in 1921 by Jakob (Jakob 1921), who referred to a previous case described by Creutzfeldt in 1920 (Creutzfeldt 1920). These original cases were clinically heterogeneous, and review of pathologic material provided confirmation of the diagnosis of CJD in only two of the original five cases (Masters and Gajdusek 1982). Over subsequent decades, the nosology of CJD was confused and confusing. Wilson regarded CJD as a “dumping ground for several rare cases of presenile dementia” (Wilson 1940). Although an important monograph by Kirschbaum (1968) listed the clinical and pathologic features of all 150 cases identified before 1965, it included cases such as Creutzfeldt’s original case, which would not now fulfill clinical or pathologic criteria for the diagnosis of CJD. In 1954 (Jones and Nevin 1954) and 1960 (Nevin et al. 1960), Nevin and Jones described the typical clinical course, the “characteristic” electroencephalogram (EEG), and neuropathologic changes, including spongiform change, which in combination are now recognized as the paradigm features of sporadic CJD.

In the late 1950s and early 1960s, with remarkable perseverance in difficult conditions, Gajdusek and colleagues investigated kuru, a fatal ataxic syndrome restricted to the Okapa area of the highlands of Papua New Guinea (Gajdusek and Zigas 1957). The similarity of the neuropathologic findings in scrapie and kuru was recognized in 1959 by Hadlow (Hadlow 1959) with the implication that kuru, like scrapie, might be transmissible in the laboratory. Successful laboratory transmission of kuru in 1966 (Gajdusek et al. 1966) was followed in 1968...

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