| Disease/Syndrome |
Creutzfeldt-Jacob disease |
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| Category |
Infection, Occupational |
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| Acute/Chronic |
Chronic |
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| Synonyms |
Subacute spongiform encephalopathy |
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| Biomedical References |
Search PubMed |
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| Comments |
Over 130 cases of variant Creutzfeldt-Jacob disease (vCJD) have been reported. It is thought that patients ate United Kingdom beef infected with bovine spongiform encephalopathy (BSE). The mean age at death is 29 years, and the mean length from onset of the illness until death is 14 months. [CCDM, p. 191-2] There is an abrupt onset of dementia with deterioration over days to weeks leaving the patient mute and disabled within 2-5 months. The EEG is abnormal, but the CSF is usually normal. Patients have myoclonic jerking and startle easily. [Wilson p. 76] Most patients present with dementia. Myoclonus is common, and it is aggravated by stimulation, i.e., "startle myoclonus." About 1/3 of patients present with visual and cerebellar symptoms. Rigidity, nystagmus, tremor, and ataxia are present in about 2/3 of patients. Uncommon findings are seizures, cranial nerve palsies, and muscular atrophy/fasciculations. [PPID, p. 2219-35] Also known to cause dementia are subacute and chronic meningitis caused by fungal agents, TB, AIDS, Lyme disease, and syphilis. [Merck Manual, p. 1866] "To reduce any risk of acquiring vCJD from food, travelers to Europe or other areas with indigenous cases of BSE may consider either avoiding beef and beef products altogether or selecting beef or beef products, such as solid pieces of muscle meat (rather than brains or beef products such as burgers and sausages), that might have a reduced opportunity for contamination with tissues that may harbor the BSE agent." [http://www.cdc.gov/travel/diseases/madcow.htm] |
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| Latency/Incubation |
15 months to over 30 years for iatrogenic cases ; [CCDM, p. 192] |
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| Diagnostic |
Clinical; EEG; MRI scan of brain; Postmortem examination of brain tissue; |
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| ICD-9 Code |
046 |
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| Reference Link |
CDC - Creutzfeldt-Jacob Disease |
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| Related Information in Haz-Map |
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| Symptoms/Findings |
Symptoms/Findings associated with this disease:
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| Job Tasks |
High risk job tasks associated with this disease:
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