The National Prion Disease Pathology Surveillance Center

Supported by the CDC and Sponsored by the American Association of Neuropathologists


The National Prion Disease Pathology Surveillance Center (NPDPSC) was established in 1997 at Case Western Reserve University in response to concerns about the transmission to humans of prion diseases, or spongiform encephalopathies, acquired from animals in the United States, as well as transmission between humans during medical procedures. Several countries worldwide also have established surveillance centers to monitor the occurrence of prion diseases, in response to the epidemic of Bovine Spongiform Encephalopathy (BSE), also known as "mad cow disease". Transmissibility to humans of chronic wasting disease (CWD), an endemic prion disease of elk and deer, is also a concern in the US. The NPDPSC is supported by the Centers for Disease Control and Prevention and sponsored by the American Association of Neuropathologists.

Purposes of the Center:

Diagnostic Activities of the Center:

Only frozen brain tissue examination definitely confirms or excludes the diagnosis of prion disease and provides the information to identify the type of prion disease. The immunohistochemical examination provides a definitive diagnosis only when positive. The CSF, blood and MRI examinations provide information that may be very helpful to caring physicians in making a clinical diagnosis.


The National Prion disease Pathology Surveillance Center (NPDPSC) has performed Western blot (WB), histological and immunohistochemical examinations of autopsy tissue from a patient who died in Texas with the diagnosis of suspected variant Creutzfeldt-Jakob disease (vCJD). The results of these tests have confirmed the diagnosis of variant Creutzfeldt - Jakob disease, a prion disease believed to be acquired by eating prion contaminated bovine meat (Diack et al. Prion 2014). According to the available information, the patient has history of travelling extensively in Europe and Middle East. Therefore, the potential source of infection needs to be thoroughly investigated. This is the fourth US case of vCJD diagnosed by the NPDPSC based on tissue examination. In all three previous cases strong evidence indicates that the disease was acquired in the United Kingdom or Saudi Arabia.


Web Table: Cases Examined

prion web table

Test Request Form

prion test request form