CJD - Creutzfeldt-Jakob Disease

Article published on 7th May 2008

Creutzfeldt-Jakob Disease (CJD) is so called because it was first diagnosed by two German neurologists, Hans Gerhard Creutzfeldt and Alfons Maria Jakob.

Like fatal familial insomnia (FFI) and Gerstmann-Straussler-Scheinker disease (GSS), CJD is a prion disease. Prions are abnormal and infectious proteins that cause a form of dementia by invading the brain having already attacked the central nervous system. Prions are very robust and cannot be destroyed by usual methods of sterilisation.

The presence of prions in the brain causes irreparable damage its cellular structure, effectively killing whole sections of the brain and leaving it with a spongy appearance when viewed through a microscope hence its medical name spongiform encephalopathy.

The early symptoms of CJD can include mood swings, memory lapses and a general disinterest in concerns and processes that the infected person would normally find interesting or important. Within a few weeks the sufferer will become increasingly clumsy and may also become muddled and complain of feeling unsteady while walking. The person may also start to slur his or her speech.

As the illness progresses infected persons will begin to lose awareness of their surroundings and may lose the ability to speak or move. At this stage they will likely need full time and full-spectrum nursing care.

People suffering from CJD are unlikely to live for more than half a year after the first symptoms have been diagnosed, although in a minority of cases the disease can take a few years to run its course.

Providing that they are looked after with appropriate care and attention, there is no evidence to suggest that people with CJD feel any pain and there are medications that can help alleviate some physical symptoms (like tremors).

Many critical illness policies now cover CJD as a specified illness, meaning that a lump sum can be paid out upon diagnosis.

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