NPDPSCNational Prion Disease Pathology Surveillance Center (Case Western Reserve University, Cleveland, OH)
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The panel of diagnostic tests for CJD used for many years at the NPDPSC includes level determinations of 14-3-3 and Tau proteins in the CSF.
The clinical and histopathological features of this case fit well with those of the sCJDMMl subtype, as confirmed by the genetic and molecular data carried out by the NPDPSC.
Two more sCJD cases of patients native and resident to Puerto Rico were listed in the NPDPSC data bank.
Because testing brain tissue permits the most definitive diagnosis of all forms of CJD and identification of emerging forms of the disease, including vCJD, CDC encourages physicians to arrange for brain autopsies in all decedents with suspected or diagnosed CJD and to use the free services of NPDPSC to assess the neuropathology of these patients.
Examination of fixed brain tissue sent to NPDPSC demonstrated no lesions indicative of CJD, and immunohistochemical testing with antibody to the prion protein did not demonstrate the granular deposits seen in prion diseases.
Because the confirmation of CJD and the detection of a new prion disease require neuropathologic study of brain tissue, physicians are encouraged to contact NPDPSC (http:// www.