Am J Alzheimers Dis Other Demen

BackgroundBrain tissue analysis is necessary to confirm prion diseases. Clinically unsuspected cases may be identified through neuropathologic testing.MethodsNational Alzheimer\ue2\u20ac\u2122s Coordinating Center (NACC) Minimum and Neuropathologic Data Sets for 1984\ue2\u20ac\u201c2005 were reviewed. Eligible patients had dementia, underwent autopsy, had available neuropathologic data, belonged to a currently funded Alzheimer\ue2\u20ac\u2122s Disease Center (ADC), and were coded as having an Alzheimer\ue2\u20ac\u2122s disease clinical diagnosis or a non-prion disease etiology. For the eligible patients with neuropathology indicating prion disease, further clinical information, collected from the reporting ADC, determined whether prion disease was considered before autopsy.ResultsOf 6000 eligible patients in the NACC database, 7 (0.12%) were clinically unsuspected but autopsy confirmed prion disease cases.ConclusionThe proportion of dementia patients with clinically unrecognized but autopsy confirmed prion disease was small. Besides confirming clinically suspected cases, neuropathology is useful to identify unsuspected, clinically atypical cases of prion disease.CC999999/Intramural CDC HHS/United StatesU01 AG016976/AG/NIA NIH HHS/United States2016-12-01T00:00:00Z26320231PMC465173