© 2002 Journal of Neurology Neurosurgery and Psychiatry
EDITORIAL COMMENTARY
Creutzfeldt-Jakob disease
CJD: are there distinct neuropsychological features?
NCJDSU, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK; R.Knight@ed.ac.uk
Different faces wearing the same expression: is there a core neuropsychological deficit in sporadic CJD?
Keywords: Creutzfeldt-Jakob disease; sporadic; PRNP genotype; cognitive dysfunction; neuropsychological tools
| The first 150 words of the full text of this article appear below. |
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 symptom(s) in particular. Research has attempted to correlate these variations with molecular factors such as the PRNP genotype and the prion protein structure.2 If sCJD is indeed a "spontaneous" disease due to a chance protein structural change or somatic PRNP mutation, then the different presentations may simply reflect the usual function of the initially affected part of the
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J. Neurol. Neurosurg. Psychiatry 2002 73: 686-694.[Abstract] [Full Text] [PDF]
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