ABC of Safety in the Biological Sciences
Creutzfeldt-Jakob disease is a progressive, fatal condition of the central nervous system caused by a prion. The causative agent - a prion - is an aggregate of protein rods that contain either DNA or RNA. Both sexes are affected and the disease commonly occurs in middle life. Dementia appears after a period of vague prodromal symptoms and rapidly progresses to coma and death, usually within two years of onset, although survival for longer periods has been reported.46 Dementia is often accompanied by other neurological disturbances.
Twenty Four cases of transmission of the agent in health care workers (four in medical laboratory personnel) have been documented.47-52 Therefore it is necessary to institute the most stringent safety measures in laboratories and autopsy rooms where the possibility of encountering this agent exists.53
Prions represent the most chemically resistant and untreatable of all infectious diseases. Most known disinfectants are ineffective, these include boiling water, formalin, alcohol, phenol, glutaraldehyde, hydrogen peroxide, ionising or UV radiation,54 or dry heat to 360°C. Enzymes are also ineffective. The methods which are thought to be effective include the following:
Great caution must be exercised to avoid accidental percutaneous inoculation of infected material.56 If accidental puncture does occur the affected area should be treated with one of the recommended skin disinfectants immediately although this may be ineffective. Recommended procedures include iodine or phenol-containing preparations, 0.5 Mol/l sodium hydroxide or 1:3000 potassium permanganate.57
Autopsy and surgical pathology
Because of the nature of the agent necropsy and histological technique must be designed to contain the infected tissue and decontaminate any exposed surfaces.55 Only those persons directly involved in the autopsy should be present and should wear long sleeved gowns, gloves and a mask. The skull and spinal canal should be opened as usual with an oscillating saw, without cutting the brain or spinal cord. All tissues must be regarded as infectious although evidence now suggests that the infective agent is most concentrated in nervous tissue, cerebrospinal fluid and lymphoid tissues.55 The sink in which water from the autopsy table collects should be plugged and all wash water collected. This should be autoclaved or treated with greater than 4 volumes of 5% hypochlorite and left for a minimum of two hours before discarding. Instruments, gloves, drapes, saw blades and any other materials used in the autopsy should be autoclaved as previously described, wherever possible use disposable instruments. Surfaces should be cleaned with an appropriate disinfectant (2mol/l sodium hydroxide), followed by scrubbing with strong detergent in hot water followed by further disinfection. Special attention should be paid to the external disinfection of containers used for the collection of tissues.
In the laboratory a dedicated room or limited section of the laboratory should be used enabling thorough decontamination. Wherever possible disposable items which can be incinerated should be used. All tissues must be considered as fully infectious even after prolonged fixation in formalin and histological processing. All liquids used in processing including alcohols and clearing agents, should be pooled and decontaminated before disposal. Glassware, forceps and tissue carriers can be decontaminated in 5% hypochlorite (note this is an oxidising agent and a corrosive and will attack some metals at this concentration). The microtome blade used to cut the tissue must also be decontaminated with special care taken to avoid cuts. Waste wax from rough cutting can be collected and decontaminated by incineration. The microtome should be well cleaned with 0.5% sodium hypochlorite (at this concentration the solution is not corrosive). The section can be decontaminated with dilute potassium permanganate before staining and blocks should be stored in a sealed air tight container with a visible warning that the contents are infectious.
Tissue from cases of Creutzfeldt-Jakob disease should not be accepted for teaching of gross anatomy and any specimens in pathological teaching collections should carry a warning and be handled with caution.