Infection-related dementia: Creutzfeldt-Jakob disease (CJD)
Creutzfeldt-Jakob disease (CJD) is a rare and fatal brain disorder. Infectious agents, called prions attack the central nervous system and then invade the brain, causing dementia. Early symptoms include minor lapses of memory, mood changes and loss of interest in normally stimulating activities. An infected person may complain of clumsiness, feeling muddled, become unsteady in walking and exhibit slow or slurred speech.
The disease progresses to jerky movements, shakiness and stiffness of limbs, incontinence and loss of the ability to move or speak. Eventually the person will need full nursing care.
People affected by CJD usually die within six months of early symptoms, often from pneumonia. In a minority of patients the disease may take two years to run its course. Very rarely, the disease lasts many years.
Variant CJD, a more aggressive strain of the disease, was identified in the UK in 1996. Symptoms are similar to classic CJD but the disease progresses more rapidly and usually affects people in the 16-52 year age group, whereas classical CJD tends to affect people over 60 years. There is now evidence that variant CJD is caused by eating meat infected with bovine spongiform encephalopathy (BSE), described as "mad cow disease". There have been no cases of variant CJD in New Zealand.
A CJD register was established in 1996, and each year, two to three cases of CJD are reported.
