Rapidly Progressive Dementia Does Not Always Mean Creutzfeldt-Jakob Disease

Sporadic Creutzfeldt-Jakob disease (sCJD) has no known treatment and is invariably fatal. The WHO has established diagnostic criteria for sCJD; however, because of this condition's variable presentation and overlap of features with many other central nervous system (CNS) diseases, diagnosis is often difficult. The National Prion Disease Pathology Surveillance Center (NPDPSC) monitors prion disease occurrence in the U.S. and provides neuropathologic confirmation of autopsy specimens. To study the incidence of CJD misdiagnoses and to identify diseases that constitute true diagnoses, investigators retrospectively analyzed the correlations of clinical data with pathologic findings in tissue samples from 1106 cases that were reported to the NPDP…