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Cause:
Prions, or “proteinaceous
infectious particles” in which normal cellular prion
proteins in the brain fold into abnormal, pathologic forms.
Illness and treatment:
CJD is a rare,
fatal neurodegenerative disease. About 85% of CJD cases are
sporadic (sCJD) while 15% are inherited. Sporadic CJD is
characterized by rapidly progressing dementia, poor balance,
visual changes and/or muscle jerks. Treatment is supportive.
Sources:
The cause of sporadic CJD is
not known. In 1996, a new variant of CJD (vCJD) recognized
in the United Kingdom was associated with cattle infected
with a related infection (“mad cow disease”). To date, no
cases of vCJD have been acquired in the United States.
Prevention:
There are no specific
precautions.
Recent Washington trends:
During 2000 to
2007, 4 to 10 cases were reported each year.
2008:
17 cases were reported, but
death certificates had not been finalized for 2008 as of
publication of this report. The median age of these patients
was 68 years old (range 49–82 years). All cases were
clinically compatible with sporadic CJD with 12 laboratory
confirmed to be sporadic CJD. Purpose of Reporting and
Surveillance
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To
monitor trends in the epidemiology of human prion
diseases in Washington State.
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To
maximize laboratory confirmation of suspected cases and
promote awareness of available resources.
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To
detect the emergence of variant Creutzfeldt-Jakob
Disease (vCJD) or novel prion diseases in the United
States.
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To
prevent potential iatrogenic transmission.
Legal Reporting Requirements
Human
prion diseases are reportable as rare diseases of public
health significance.
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Healthcare providers: immediately notifiable to local
health jurisdiction
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Hospitals: immediately notifiable to local health
jurisdiction
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Laboratories: no requirements for reporting
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Local health jurisdictions: immediately notifiable to
DOH Communicable Disease Epidemiology: 1-877-539-4344
Last
update
November 2009 |
