What is the variant form of CJD that the experts in the United Kingdom believe might be related to the BSE outbreak in cattle?
In contrast to the classic form of CJD, the variant form in the United Kingdom predominantly affects younger persons (median age at death around 29 years) and has atypical clinical features. These atypical features include prominent psychiatric or sensory symptoms at the time of clinical presentation or early in the course of the illness, delayed onset of neurologic abnormalities, duration of illness of at least 6 months, and a diffusely abnormal non-diagnostic electroencephalogram. The characteristic neuropathologic profile of variant CJD includes, in both the cerebellum and cerebrum, numerous kuru-type amyloid plaques surrounded by vacuoles and prion protein (PrP) accumulation at high concentration indicated by immunohistochemical analysis. Recently published data indicate that the epidemic of variant CJD in the United Kingdom may have already reached a peak. A listing of monthly updated numbers of variant CJD cases in the United Kingdom is available at: http://www.doh.gov.uk/cjd/cjd