Creutzfeldt-Jacob Disease presents with vague sensory/psychiatric symptoms and difficulty speaking/walking. As of mid-2012, 225 cases of variant Creutzfeldt-Jacob disease (vCJD) had been reported. It is thought that patients ate United Kingdom beef infected with bovine spongiform encephalopathy.
CASES/YEAR
3 (US); 60 (Global)
OTHER NAMES
Transmissible spongiform encephalopathies; Prion diseases;
INCUBATION
15 months to over 30 years; 10-20 years for vCJD after consuming BSE-contaminated cattle products; [CCDM]
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. 2294]
PRECAUTIONS
Standard; "Use disposable instruments or special sterilization/disinfection for
surfaces, objects contaminated with neural tissue if CJD or vCJD
suspected and has not been R/O; No special burial procedures." [CDC 2007 Guideline for Isolation Precautions]
COMMENTS
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. [Harrisons, p. 1007] Also known to cause dementia are subacute and chronic meningitis caused by fungal agents, TB, AIDS, Lyme disease, and syphilis. [Merck Manual, p. 1875] "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 six occasions." [Cecil, p. 2468]
DIAGNOSTIC
Clinical; EEG (markedly abnormal periodic discharges); Diffusion-weighted MR (abnormalities of cortex and basal ganglia); [Harrisons, p. 1007]
SIGNS & SYMPTOMS
-
>fatigue, weakness
-
N confusion, delirium
-
N headache
-
N paresthesia
-
N seizure
-
*cranial neuropathy
-
*pneumonia
-
*weight loss
ENTRY
Ingestion, Needle (Includes Drug Abuse), Scalpel or Transfusion
RESERVOIR
Cattle, Goats and Sheep, Human
RISK FACTORS
- Travel to endemic area
- Work in a medical or research lab
TREATMENT
No specific treatment; [CCDM]
REFERENCES FOR CASES/YEAR
1. (US) 1/20 X global cases/yr;
2. (Global) Annual incidence of all forms of prion disease (sporadically, genetically, or infectiously) = 1 case/million; <1% of these are acquired by infection; <200 people have contracted variant CJD; [Cecil, p. 2380-1] Calculate: World population = 6000 million; therefore 6000 cases per year and 1% or 60 cases per year acquired as infections;