Ordering Guidance

In cases where there is high suspicion of human prion disease supported by clinical or paraclinical (MRI imaging) features, this test should be ordered.

Early in the disease course, or in atypical cases, the disease progression may be slower and include significant clinical overlap (dementia, rigidity, myoclonus) with other potential causes of rapidly progressive dementia, including Alzheimer disease. In the latter case, it would be more appropriate to order RPDE / Rapidly Progressive Dementia Evaluation, Spinal Fluid.

Specimen Required

Supplies: CJD/RPD Evaluation Kit (T966)

Container/Tube:

Preferred: 2 Sarstedt CSF False Bottom Tubes 63.614.625 (2.5 mL)

Acceptable: Sarstedt 72.703.600 (1.5 mL) or Sarstedt 72.694.600 (2 mL)

Specimen Volume: 2 tubes, each containing 1.5 mL to 2.5 mL

Collection Instructions:

1. Perform lumbar puncture and discard the first 1 to 2 mL of cerebrospinal fluid (CSF).

2. Collect two tubes of CSF directly into an acceptable collection tube until the tube is at least 50% full.

3. Send CSF specimen in original collection tube. Do not aliquot.

4. Collection instructions can also be found on Spinal Fluid Specimen Collection Instructions for Creutzfeldt-Jakob Disease and Rapidly Progressive Dementia Evaluations (T974).