Creutzfeldt-Jakob Disease
From ArticleWorld
Creutzfeldt-Jakob disease (CJD) is a rare, incurable brain disorder that is one of several transmissible spongiform encephalopathies, meaning that it causes holes to show up in the brain (like a sponge) and results in a progressive dementia that is ultimately fatal. It is the most common spongiform encephalopathy in humans.
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Cause
CJD disease is caused by the transmission of what is called a “prion”. A prion is an abnormally-structured transmissible cellular protein found in brain tissue. Similar prion diseases occur in humans and in other animals. Prions are even less advanced than viruses. The prion is dangerous because it can change from a stable water-soluble shape to a dangerous, insoluble state that clumps with other prion proteins and destroys brain cells. The infected patient can die in a matter of months or can live as long as two years.
Symptoms
The individual with CJD begins with dementia that progresses rapidly and includes memory loss, hallucinations and personality changes. Speech becomes impaired, gait becomes uncoordinated and the individual can have seizures. Ultimately the rapidly progressing dementia leads to death.
Transmission
While CJD is the most common human prion condition, it occurs in only one out of a million people. It affects adults between the ages of 45 and 75. Although no test is available to detect the presence of the disease, it was found in 2004 to be transmissible via blood transfusions. This prompted the British government to ban the donation of blood in anyone who received a blood transfusion since January, 1980.
Transmission of this defective protein can occur through human growth hormone products, corneal grafts, brain implants or heredity. Spontaneous cases of CJD have been shown to occur in some cases.
Diagnosis
The diagnosis of CJD can be difficult as it can mimic other types of dementia. The electroencephalogram can show characteristic findings, cerebrospinal fluid can show abnormal proteins and the MRI scan can be abnormal. Tissue samples of the brain would show the characteristic spongy appearance of the brain.