Prion Disease Testing


Transmissible spongiform encephalopathies, TSEs.

Testing for prion diseases may be considered for patients with undiagnosed, rapidly progressive dementia.

Patient Preparation
There are no activity restrictions unless by medical direction. Follow the food, fluid, or medication instructions indicated in the individual studies.

Normal Findings
Negative biopsy, normal cerebrospinal fluid (CSF) protein levels, normal electroencephalogram (EEG), normal magnetic resonance imaging (MRI), normal neurological examination.

  • No evidence of prion disease
  • Negative tonsillar biopsy (if variant Creutzfeldt-Jakob disease [vCJD] is being considered), as vCJD is the only type known to involve the lymph nodes, spleen, tonsil, and appendix.Note: A negative biopsy does not rule out vCJD.

Critical Findings and Potential Interventions
Specific infectious organisms are required to be reported to local, state, and national departments of health. Lists of specific organisms may vary among facilities. State health departments provide information regarding reportable diseases, which can be accessed at each state health department Web site. The CDC provides information regarding national notifiable diseases at

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