Creutzfeldt-Jakob Disease

mad cow disease (2)

Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disease of unknown cause. Patients are usually aged between 50 and 75 and typical clinical features include a rapidly progressive dementia associated, myoclonus and a characteristic electroencephalographic pattern. Neuropathological examination reveals cortical spongiform change, hence the term `spongiform encephalopathy’.

Creutzfeldt-Jakob disease captured public attention in the 1990s when individuals in the United Kingdom developed a form of the disease – variant CJD (vCJD) – after eating meat from cattle suspected of having mad cow disease. However, “”classic”” Creutzfeldt-Jakob disease has not been linked to beef contaminated with mad cow disease. The CJD Foundation is also concerned about the complexity of issues surrounding this brain disease. Our philosophy is to be proactive by establishing collaborations and linkages, communicating with family members, researchers, physicians and political representatives and informing the broader community about CJD.

CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges under a microscope. CJD is the most common of the known human TSEs. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS). o The first person to develop symptoms of what turned out to be vCJD became ill in January 1994.

Most people who have developed vCJD have lived in the UK. Some of the patients had been long-standing residents in Wales, Scotland or Northern Ireland. As of early November 2002, the CJD surveillance unit for the UK reported 129 cases of vCJD, including 93 confirmed and 24 probable cases. In addition, there are 12 cases where vCJD is strongly suspected, but the diagnosis has not yet been definitively confirmed by post mortem analysis. The incidence of Creutzfeldt-Jakob disease (CJD) is monitored in the UK by the National CJD Surveillance Unit (NCJDSU) based at the Western General Hospital in Edinburgh, Scotland.

The Unit brings together a team of clinical neurologists, neuropathologists and scientists specialising in the investigation of this disease. This document is intended to summarise the research in progress at the NCJDSU and also provide some background information about CJD and other human spongiform encephalopathies. We have also provided some links to other resources and contrary points of view available on the Web. About 90 percent of patients die within 1 year. In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances.

As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur. Creutzfeldt-Jakob disease (CJD) is a rare, degenerative brain disorder. Symptoms usually start around age 60. Memory problems, behavior changes, vision problems and poor muscle coordination progress quickly to dementia, coma and death.

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