Clinical use
Investigation of CSF for CJD.
Background
Transmissible spongiform encephalopathy (TSE) otherwise known as prion disease are rare, fatal degenerative diseases affecting the central nervous system. They occur in humans and certain mammals. These include Creutzfeldt-Jacob Disease (CJD, vCJD), bovine spongiform encephalopathy (BSE) in cattle and scrapie in sheep.
TSEs are caused by unconventional infectious agents thought to be infectious protein particles known as prions. Transmission of TSEs to humans has occurred from both human and bovine sources, resulting in iatrogenic CJD and variant CJD (vCJD).
TSEs are in many ways unique and exhibit biological properties that are different from other microbiological agents. They exhibit an unusual resistance to conventional chemical and physical decontamination methods. They are not significantly affected by disinfectants like formalin and infectivity persists after standard autoclaving (e.g. 134ºc for 3 minutes).
Although TSEs are not contagious, they are experimentally transmissible by inoculation and in some cases by oral challenge. TSE agents are formally classified as Hazard Group (HG3) but the containment measures required when working with them may not necessarily fully meet containment level 3 (CL3) because of the agent’s unique feature and the risks identified as determined by local risk assessment.
Patient preparation
Warning
The following MUST be done prior to performing the lumbar puncture. It is the responsibility of the requesting clinician to contact to inform, and arrange transport of the specimen to:
The National CJD Research & Surveillance Unit (NCJDRSU), University of Edinburgh, 0131 537 1980
If TSE / CJD is suspected the laboratory at James Cook MUST also be contacted prior to sending samples and ALL samples must include TSE / CJD in the clinical details to ensure the safety of laboratory staff.
Details of how to do this can be found on the CJD website.
Once arrangements have been made with the CJD Unit in Edinburgh, this needs to be communicated to the Microbiology Lab at James Cook to allow for preparation prior to collection.
Specimen requirements
Sterile white top container
Minimum volume
0.5ml
Limitations and restrictions
Samples must be received in a sterile container prior to antibiotic treatment if possible
Turnaround time
10 – 15 working days
Analysing laboratory
Mary Andrews/Kim Burns (Tel no: 0131 465-9524-office), Room GU312, Ground Floor, Chancellor’s Building, 40 Little France Crescent, Edinburgh, EH16 4SB
Additional information
If TSE / CJD is suspected the laboratory MUST be contacted prior to sending samples and ALL samples must include TSE / CJD in the clinical details to ensure the safety of laboratory staff.
Cell count, Gram’s stain and culture will be performed at James Cook University Hospital by the Microbiology department at Containment Level 3.
TSE investigation will be referred to the National CJD Research & Surveillance Unit, Edinburgh.
