Creutzfeldt-Jacob disease
Definition:
A brain disorder involving rapid decrease of mental function
and movement abnormalities caused by damage to the tissues
of the brain from a viral-like organism (a transmissible protein
called a prion).
Causes, incidence, and risk factors:
Creutzfeldt-Jakob disease is an organic
brain syndrome caused by a viral-type organism. The disorder
is rare, occurring in about 2 out of 1,000,000 people. It
usually first appears in mid-life, beginning between ages
20 and 68, with the average age at onset of symptoms being
between 57 and 62.
Cases have occurred in adolescents who have received growth
hormone derived from cadavers, despite the production
process, which is intended to kill all bacterial and viral
material in the product. The causative agent, or prion, is
thought to have been transmitted in the growth hormone (extracted
from the pituitary of cadavers), which explains the abnormally
early appearance of the disease. Cadaver derived growth hormone
has been replaced by synthetically manufactured growth hormone
and contagion is no longer a problem.
Once symptoms appear there is rapid progression of the disorder
with progressive loss of brain function. The lesion and deterioration
of function is similar to that of senile
dementia/Alzheimer's type, but is distinguished by its
very rapid course. There may be a familial tendency to acquire
the disorder.
Early symptoms include personality changes and changes in
coordination. Extreme dementia
is accompanied by muscle
tremors and rigid posture.
The virus associated with Creutzfeldt-Jakob disease is contagious
to humans and primates. Risks include exposure to the virus,
directly or indirectly through contact with contaminated equipment
or tissues (especially corneal
transplant). Other risks include a family history of dementia.
Creutzfeldt-Jacob disease may be related to several other
diseases also thought to be caused by prions, including kuru
(seen in New Guinea headhunters), scrapie (seen in sheep)
and bovine spongiform encephalitis
(seen in cows).
Update Date: 02/09/00
Updated by: J. Gordon Lambert, MD, Associate Medical Director,
Utah Health Informatics and adam.com
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