CJD was first identified in the 1920s by German neuroscientists Hans Gerhard Creutzfeldt and Alphons Maria Jakob. Creutzfeldt had described a case of progressive fatal dementia in a female patient that was accompanied by multiple neurological abnormalities. Jakob identified five similar cases.5 Later, review of pathologic material provided confirmation of the diagnosis of CJD in only two of Jakob’s cases.6 For many years, there was debate about the clinical and pathological features of such diseases; but by 1960, the combination of a typical clinical course, a characteristic electroencephalogram (EEG) and neuropathological changes, including spongiform changes in brain tissue were accepted as the major criteria for diagnosing CJD.5

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