Description
CJD is a rare cause of dementia and is
characterised clinically by a rapidly progressive
cognitive decline, myoclonus and multifocal
neurological signs. A periodic EEG and a positive
14-3-3 CSF assay are accurate clinical diagnostic
tests but neuropathology is essential or a definite
diagnosis. The nature of the causative agent is
unknown but many scientists believe that the prion
protein is the most likely candidate. New variant
CJD is caused by the BSE agent and is known to
have a ected 32 persons since 1995. The future
number of cases is dif icult to predict at present.
