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SLT Lab update: Protein 14-3-3 Testing in Suspect Creutzfeldt-Jakob Disease (CJD)

01-Dec-2015

Special precautions are required when collecting, handling and transporting samples from a suspected case of CJD. The requirements for submitting these types of specimens are outlined below.

Background: Transmissible Spongiform Encephalopathy (TSE) disease refers to a group of diseases, inherited or transmissible that may occur in animals and humans that share common characteristics related to the deterioration of the central nervous system. There is no known treatment, and the outcome is uniformly fatal. This group of diseases includes: Creutzfeldt-Jakob Disease (CJD), Gerstmann-Straussler-Scheinker Syndrome (GSS), Fatal Familial Insomnia (FFI), and Kuru.

The prion proteins associated with these diseases are hardy, remain intact for years in a dried state, and resist all routine sterilization and disinfection procedures commonly used in healthcare facilities. Thus, specimens considered infectious for prion diseases that originate from suspect patients cannot be handled routinely in the laboratory.

Based on epidemiological evidence, the World Health Organization (WHO) recommends that: Blood and /or its components require no special precautions for handling in clinical laboratories. Standard precautions apply. With the exception of Cerebrospinal Fluid (CSF), other body fluids, secretions and excretions need no special handling. Tissues with high concentrations of prion proteins require special precautions. (e.g. cornea, brain, spinal cord).

PROCEDURE FOR CSF COLLECTION FOR PROTIEN 14-3-3 Testing:

PRIOR to collecting the CSF specimen, the attending physician and/or neurologist MUST CALL the RQHR microbiologist on call (via switchboard 766-4444) to discuss testing of samples from high risk patients and to seek approval of 14-3-3 protein testing where deemed appropriate.

In general, the patient must have shown rapid, progressive dementia, with at least one of the following clinical findings:

  • Patient has had an enhanced MRI with changes
  • Patient has EEG changes consistent with possible CJD/TSE
  • Patient shows signs of myoclonus

AFTER obtaining approval, CSF specimen can be collected. Ensure specimen is LABELEDCJD precautions” and requisition is CLEARLY LABELLED to identify the specimen as “CJD/Prion Agent”.

Please note that testing on CSF from patients suspected of prion-mediated diseases will be limited to the cell count as well as bacterial culture and susceptibility testing. Chemistry will NOT perform ANY testing on CSF from patients with suspected CJD.

The Saskatchewan Disease Control Laboratory will forward the specimen for protein 14-3-3 testing to the National Microbiology Laboratory and will not perform any testing on CSF from patients with suspect CJD.

Sincerely,

K. Malejczyk MD, FRCP Medical Microbiology
kathy.malejczyk@rqhealth.ca

Phone: 306-766-4805

(Information has been adapted from Alberta Health Services Laboratory Services “Laboratory Guidelines for Creutzfeldt-Jakob Disease (CJD)”http://www.albertahealthservices.ca/assets/wf/lab/wf-lab-laboratory-guidelines-for-creutzfeldt-jakob-disease.pdf)