Creutzfeldt-Jacob disease


Infection, Occupational




Transmissible spongiform encephalopathies; Prion diseases;

Biomedical References

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INITIAL SYMPTOMS: Patients may present with sensory (vague pains and paresthesias) and psychiatric (anxiety and withdrawal) symptoms. Difficulty speaking and walking are later symptoms. [PPID, p. 2148] As of mid-2012, 225 cases of variant Creutzfeldt-Jacob disease (vCJD) had been reported (176 in UK and 26 in France). It is thought that patients ate United Kingdom beef infected with bovine spongiform encephalopathy (BSE). Three patients were infected with vCJD by blood transfusion. [CCDM, p. 484-90] Patients have rapid onset of dementia, rigidity, myoclonus, and death within one year. [Harrison, p. 1221] Also known to cause dementia are subacute and chronic meningitis caused by fungal agents, TB, AIDS, Lyme disease, and syphilis. [Merck Manual, p. 1744] "Ban regarding the feeding of ruminant proteins (such as bone meal) to ruminants yielded control of the BSE epidemic." [ABX Guide] Most of iatrogenic transmission occurred through contaminated cadaveric dura mater allografts (Lyodura), and this was discontinued in 1987. Growth hormone was another source when it was obtained from cadaveric pituitaries. "Contaminated surgical instruments are persuasively documented to have transmitted CJD on only four occasions." [Cecil, p. 2381]


15 months to over 30 years; 10-20 years for vCJD after consuming BSE-contaminated cattle products; [CCDM]


Clinical; EEG (markedly abnormal periodic discharges); Diffusion-weighted MR (abnormalities of cortex and basal ganglia); [Harrison, p. 1221]

ICD-9 Code


Reference Link

CDC - Creutzfeldt-Jakob Disease

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