Creutzfeldt-Jakob Disease

The Toronto Hospital Policy & Procedure Manual

INFECTION CONTROL

SECTION:   DISEASE SPECIFIC INFORMATION
SUBJECT TITLE:   CREUTZFELDT-JAKOB DISEASE
POLICY #:   60.03
ISSUED BY:   INFECTION CONTROL
DATE:   09.90
APPROVED BY:   INFECTION CONTROL COMMITTEE
REVIEW DATE:   03.95 ® 07.97 ®

GUIDELINES

The incidence of Creutzfeldt-Jakob Disease (CJD) is estimated at one case per million per year. CJD is a progressive, degenerative neurologic disorder with a clinically latent stage of up to 30 years (age of onset 50-65) with a duration from the onset of symptoms to death of 6-12 months. The symptoms can include progressive ataxia, myoclonus, dementia with memory loss, vertigo, visual disturbances and EEG changes. From 1979 to 1993, a total of 334 deaths were attributed to CJD in Canada.
CJD is thought to be transmitted by a prion protein which is smaller than a virus. Other prion diseases in man are kuru, resulting from the cannibalistic practice of eating infected human brains and Gerstmann-Straussler-Scheinker disease which is genetic. In sheep and goats, there is a form of prion disease called scrapie.
In 1986, there was an outbreak of bovine spongiform encephalopathy (BSE) in the cattle population in the United Kingdom related to contaminated feed made from scrapie infected sheep and cattle. This practice was banned in 1988, however, in 1996, a cluster of cases (19 to date) of a new variant of CJD (Variant-CJD) was reported in a younger population of patients (<30 years) with clinical and pathological differences from CJD. This raised the question of a link between BSE and V-CJD through the ingestion of contaminated beef and further research is being done at this time.

Mode of Transmission
CJD cases are presently classified as:
Genetic: a familial form (5-15%)
Infectious: iatrogenic transmission: corneal transplants/ dura matter grafts/ pituitary
hormones from CJD infected donors or inadequately sterilized CJD
contaminated neurosurgical stereotactic electrodes (<1%)
Sporadic: from an unknown source (90%)

Tissues of high infectivity: brain, spinal cord, spleen, lymph node, thymus
tonsil, placenta, pituitary gland, dura mater, eye
Tissues of moderate to low infectivity: peripheral nerve, CSF, adrenal gland, liver, lung,
pancreas
Tissues/ fluid unlikely infectious: milk, semen, urine, saliva, faeces, skin, muscle
kidney, blood

Incubation Period
This can vary from 15 months to more than 30 years, depending on the mode of transmission.

Period Of Communicability
Central nervous system tissues (brain/CSF) are infectious throughout symptomatic illness, although other tissues and CSF have been shown to infectious at times. Infectivity during the incubation period is not known, however studies in animals suggest that lymphoid and other organs are probably infectious before any clinical signs.

Treatment
There is no known treatment for this disease.

Diagnosis
Brain biopsy is the definitive method, however specific EEG changes, CT studies which show atrophy, as well as clinical symptoms are used to diagnosis CJD. A new test which is done on the CSF is presently under investigation at this time.

Control Measures

use general BSP practices
dispose of sharps into a sharps container and contaminated waste into a yellow bag
spills of blood or body fluids are to be carefully contained/absorbed and disinfected using a 2.64% solution of sodium hypochlorite (50% dilution of household bleach »20,000 ppm available chlorine) for 1 hour
contained waste fluid must be placed into a plastic bucket labeled CJD for incineration. (Pathology and Autopsy areas)
invasive items used should be disposable if possible e.g. LP needles, OR instruments.
gloves and goggles should be worn when collecting CSF specimens
identify these specimens on the computer as possible or confirmed CJD
the OR and Pathology must be informed in advance if a neurosurgical procedure is booked
refer to Pathology/Autopsy, CPD, Housekeeping and OR for specific policies

Treatment after an exposure

percutaneous exposures to blood, CSF or tissue (especially brain) of known or suspected cases should be bled extensively, irrigated first with soap and water and then with using a solution of 2.64% sodium hypochlorite (50% dilution of household bleach »20,000 ppm available chlorine) or 2 N sodium hydroxide
mucous membranes exposures (splashes into mouth/nose/eye) or should be thoroughly irrigated with water (and soap if possible)
skin exposures with possibly infectious material should be followed by washing with 1 N sodium hydroxide
all incidences must be reported to the Occupational Health unit

Research to date has indicated that exposure to blood is unlikely to present a risk of transmission. Surveillance has suggested that there has been no transmission of CJD to the recipients of blood donations from confirmed CJD cases.
There has been no epidemiological evidence of occupational transmission of CJD, although isolated and sporadic cases have been reported in neurosurgeons and pathology personnel. Airborne transmission of CJD has not been reported, however power saws should not be used due to aerosolization. TTH procedures are based on the guidelines established by the United Kingdom CJD Surveillance Neuropathology Unit. (5)
Inactivation of the CJD Agent
The CJD agent is quite resistant to routine chemical inactivation and sterilization when compared to other organisms and can survive desiccation, freezing and ultraviolet radiation. Scrapie has been shown to survive in the soil for several years.

Sterilization of equipment possibly contaminated with CJD (OR, Pathology, Autopsy)
Physical & Chemical Agents Effective against CJD

Incineration
Sodium Hydroxide NaOH (2 M for 60 minutes) ¹
Formic Acid HCOOH (96 % for 60 minutes)²
Sodium Hypochlorite/Bleach NaOCl (> 20 000 ppm for 120 minutes) ^{2, 3}
Prevacuum Steam Autoclave (134 C for 1 - 18 minute cycle) or
Prevacuum Steam Autoclave (134 C for 6 - 3 minute consecutive cycles)
Standard Gravity Displacement Steam Autoclave (134 C for 60 minutes)
1 NaOH is less corrosive than NaOCl, but is incompatible with aluminum or zinc-containing instruments or surfaces.
2 NaOCl and HCOOH are highly corrosive to metals and care should be exercised in their use.
3 50 % dilution of 5.25 % household bleach.
Physical & Chemical Agents Ineffective against CJD
Alcohols
Dry Heat
Ethylene Oxide (ETO)
Formaldehyde
Formalin
Glutaraldehyde
Hydrogen Peroxide
Iodophors
Peracetic Acid
Phenolics
References:

McGreevy-Steelman V. Creutzfeldt-Jakob disease: Recommendations for infection control. AJIC 1994;22(5):312-318.

Brown P, Carleton Gajdusek D. Survival of scrapie virus after 3 years' internment. Lancet 1991;337:269.

Will RG. Epidemiology of Creutzfeldt-Jakob disease. British Medical Bulletin 1993;49(4):960-970.

Committee on Health Care Issues, American Neurological Association. Precautions in Handling Tissues, Fluids, and Other Contaminated Materials from Patients with Documented or Suspected Creutzfeldt-Jakob Disease. Annals Neurology 1986;19(1):75-77.

Advisory committee on dangerous pathogens (1994). Precautions for work with human and animal transmissible spongiform encephalopathies. HMSO, London.

Ironside RG et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996; 921-925

Budka H et al. Tissue handling in suspected Creutzfeldt-Jakob disease(CJD) and other human spongiform encephalopathies (prion disease) Brain Pathology 1995;5:319-322

Dormont D. How to limit the spread of Creutzfeldt-Jakob Disease. Infect Cont Hosp Epidemiol 1996;17:521-528

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