Creutzfeldt-Jakob Disease (cont.)
How is the Disease Treated?
There is no treatment that can cure or control CJD.
Researchers have tested many drugs, including amantadine, steroids, interferon,
acyclovir, antiviral
agents, and antibiotics. Studies of a variety of other drugs are now in
progress. However, so far none of these treatments has shown any consistent
benefit in humans.
Current treatment for CJD is aimed at alleviating
symptoms and making the patient as comfortable as possible. Opiate drugs can
help relieve pain if it occurs, and the drugs clonazepam and sodium valproate
may help relieve myoclonus. During later stages of the disease, changing the
person's position frequently can keep him or her comfortable and helps prevent
bedsores. A catheter can be used to drain urine if the patient cannot control
bladder function, and intravenous fluids and artificial feeding also may be used.
What Causes Creutzfeldt-Jakob Disease?
Some researchers believe an unusual "slow virus" or
another organism causes CJD. However, they have never been able to isolate a
virus or other organism in people with the disease. Furthermore, the agent that
causes CJD has several characteristics that are unusual for known organisms such
as viruses and bacteria. It is difficult to kill, it does not appear to contain any genetic
information in the form of nucleic acids (DNA or RNA), and it usually has a long
incubation period before symptoms appear. In some cases, the incubation period
may be as long as 40 years. The leading scientific theory at this time maintains
that CJD and the other TSEs are caused by a type of protein called a prion.
Prion proteins occur in both a normal form, which is a harmless protein found
in the body's cells, and in an infectious form, which causes disease. The
harmless and infectious forms of the prion protein have the same sequence of
amino acids (the "building blocks" of proteins) but the infectious form of the
protein takes a different folded shape than the normal protein. Sporadic CJD may
develop because some of a person's normal prions spontaneously change into the
infectious form of the protein and then alter the prions in other cells in a
chain reaction.
Once they appear, abnormal prion proteins aggregate, or clump together.
Investigators think these protein aggregates may lead to the neuron loss and
other brain damage seen in CJD. However, they do not know exactly how this
damage occurs.
About 5 to 10 percent of all CJD cases are inherited.
These cases arise from a mutation, or change, in the gene that controls
formation of the normal prion protein. While prions themselves do not contain
genetic information and do not require genes to reproduce themselves, infectious prions can arise if a mutation
occurs in the gene for the body's normal prion protein. If the prion protein
gene is altered in a person's sperm or egg cells, the mutation can be
transmitted to the person's offspring. Several different mutations in the prion
gene have been identified. The particular mutation found in each family affects
how frequently the disease appears and what symptoms are most noticeable.
However, not all people with mutations in the prion protein gene develop CJD.
Next: How is Creutzfeldt-Jakob disease transmitted? »
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