CJD: are there distinct neuropsychological features?
Different faces wearing the same expression: is there a core neuropsychological deficit in sporadic CJD?
As with many diseases originally defined on purely clinicopathological phenomenological grounds, “Creutzfeldt-Jakob disease” (CJD) has been subject to various “lumping” and “splitting” processes over the years.1 The demonstration of laboratory transmissibility and the central role of the prion protein have served to unify the concept of CJD. Sporadic CJD (sCJD) remains, of course, defined by the absence of a known cause, in contradistinction to genetic, iatrogenic, and variant CJD. Despite this background unification, there is notable clinical phenotypic variation in sCJD including age of onset, duration of illness, and presenting …
