Creutzfeldt Jakob Disease (Sendout)

General Information

Lab Name

Creutzfeldt Jakob Disease

Lab Code

RCCJD

Epic Name

Creutzfeldt Jakob Disease

Description

This assay includes testing for the 14-3-3 protein and tau protein by ELISA Immunoassay and RT-QuIC (real-time quaking-induced conversion). RT-QuIC identifies the disease-causing agent. T-tau protein and 14-3-3 protein are indirect markers of neurodenerative disease.

Ordering Requirements:

  • A completed NPDPSC Test Requisition, including physician contact information, must accompany all sample submissions. (See also handling instructions below.)
  • This test requires Laboratory Medicine Resident (LMR) approval. Exception noted in Processing section below.

Synonyms

14 3 3, 14-3-3, 14-3-3 Protein, 14.3.3, CJD, Familial Insomnia (sFI), fCJD, FFI, Gerstmann-Sträussler-Scheinker Syndrome (GSS), Iatrogenic CJD, iCJD, Kuru, Mad Cow Disease, prion, Protein 14-3-3, sCJD, tau, vCJD

Components

Code Name
RCCJDR CJD Result
RCCJDI CJD Interp

Interpretation

Reference Range

See individual components

Ref. Range Notes

Reference Ranges:

RT-QuIC, CSF: Negative
T-tau protein, CSF: 0-1149 pg/mL
14-3-3 protein, CSF: Negative

Guidelines

Ordering & Collection

Specimen Type

CSF, Cerebrospinal Fluid

Collection

5 mL SPINAL FLUID - No visible blood

Note: Contact NPDPSC at (216) 368-0587 prior to submitting blood samples. Blood is non-diagnostic and should only be sent when a familial case is suspected, or if the sample has been requested by NPDPSC.

Forms & Requisitions

Approval Required

Laboratory Medicine Resident approval is required before sending a sample. [Exception noted in Processing section below.]

Handling Instructions

Outside Laboratories: If no concurrent CSF testing is ordered that is to be performed by the UW Department of Laboratory Medicine and Pathology clinical laboratories, clients outside of the University system are encouraged to send samples directly to National Prion Disease Pathology Surveillance Center (Sample Submission Information).

Failure to follow the guidelines below for submitting CJD samples may result in significant processing delays.

  • Approval from the Laboratory Medicine Resident must be obtained prior to sample submission. The ordering provider should contact the paging operator at 206-598-6190 and ask to speak to the General Lab Medicine Resident on-call.
  • After approval, contact UW Client Support Services at (206) 520-4600 or 1-800-713-5198 to notify them of incoming samples. Communicate the name of the approving doctor.
  • Clearly label the requisition and all samples as “Suspected Prion.”
  • Complete the NPDPSC Requisition and submit with the samples.

Quantity

requested: 5 mL CSF
minimum: 2 mL CSF

Processing

LMR Approval Exception for Outside Orders: Outside clients should be discouraged from sending samples for CJD without concurrent CSF orders for in-house testing. However, if the sample has already been shipped to/received by UW from an outside facility, DLMP will accept the order and forward to NPDPSC.

  • LMR approval is not required in this case. The order may be accessioned and sample(s) forwarded to Sendouts for shipment to NPDPSC.
  • Create an HC1 Case to document the error, assign to CSS, and set the status to "Supervisor Review" so that CSS Management can provide feedback to the client.

Specimen Processing Services (SPS)/Microbiology Set up:

  • Page the LMR immediately upon receipt of specimen(s) and prior to aliquoting, testing, or freezing. When paging through operator or text paging, include comment: “urgent CSF, call-back within 15 min.,” in addition to your name, lab location, and call-back number.
    • Create a case in HC1 to document approval and any instructions given by the resident.
    • If no response within 15 minutes, contact the LMR again.
  • If CJD is the only test ordered on CSF, freeze the sample at -70°C.
  • If additional tests are ordered, determine if there is sufficient CSF for CJD testing (2 mL) AND for all other testing.
    • If there is sufficient sample, freeze an aliquot for CJD testing at -70°C and hold all remaining samples at the temperature received.
    • If there is insufficient CSF (or if unsure), hold all samples at the temperature received until directed otherwise by the LMR.
  • Do not setup testing on any CSF from this patient until approved by LMR. Do not send out CJD testing prior to LMR approval.
  • If the LMR approves CJD testing, label all specimens and aliquots of CSF from the same patient as “Suspected Prion Disease.”
  • If CJD order is cancelled, other testing may proceed without special precautions or labels.

Sendouts:

  • Submissions must be accompanied by an NPDPSC request form completed by the ordering provider.
  • Label all sendout specimens and aliquots of CSF from the same patient as “Suspected Prion Disease.”

Stability: Frozen -70°C (preferred): 2 weeks; Refrigerated: 2 weeks.

  • Note: Refrigerated samples are stable for up to 2 weeks after collection (freeze prior to sending to NPDPSC). Any specimens with a significant delay in freezing or prolonged storage at refrigerated or room temperature conditions should be cleared for testing with the National Prion Disease Pathology Surveillance Center. Call NPDPSC at 216-368-0587 to verify acceptability.

Performance

LIS Dept Code

Serology Sendouts (SRSO)

Performing Location(s)

Sendout National Prion Disease Pathology Surveillance Center
216-368-0587

2085 Adelbert Road
Room 418
Cleveland, OH 44106

Frequency

Results available in 7-10 days.

Available STAT?

No

Billing & Coding

CPT codes

0035U, 83520x2

LOINC

36924-9

Interfaced Order Code

UOW2879