Prion Disease Section
The Public Health Agency of Canada’s Prion Disease Section (PDS) provides national reference services in support of health care professionals dealing with suspected cases of Creutzfeldt-Jakob Disease (CJD) and the Canadian Cruetzfeldt-Jakob Surveillance System (CJDSS). The diagnostic approach for CJD relies upon a standardized clinical presentation and supporting investigations such as the presence of increased amounts of indirect protein markers of disease found in patients’ cerebrospinal fluid (CSF). The PDS provides ELISA based tests of CSF for 14-3-3 and tau proteins. These indirect markers have useful sensitivities and specificities for CJD, but are not fully diagnostic. The PDS also provides a new test, known as end-point quaking-induced conversion (EP-QuIC), able to detect the disease associated isoform of the prion protein in CFS samples.
The PDS supports further investigation of CJD cases is by providing accredited sequence analysis of PRNP (the encoding the prion protein). Sequence analyses confirm or exclude the presence of mutations linked to inherited prion diseases. In addition, neuropathology services are available through the Canadian CJDSS and the University of Ottawa Neuropathology Services. Information on PDS services are available by calling the Winnipeg laboratory at 204-789-6078. The CJDSS can be reached at 1-888-489-2999 (toll free).