Clinically Unsuspected Prion Disease Among Patients With Dementia Diagnoses in an Alzheimer's Disease Database

American Journal of Alzheimer's Disease and Other Dementias
Ryan A MaddoxErmias D Belay

Abstract

Brain tissue analysis is necessary to confirm prion diseases. Clinically unsuspected cases may be identified through neuropathologic testing. National Alzheimer's Coordinating Center (NACC) Minimum and Neuropathologic Data Set for 1984 to 2005 were reviewed. Eligible patients had dementia, underwent autopsy, had available neuropathologic data, belonged to a currently funded Alzheimer's Disease Center (ADC), and were coded as having an Alzheimer's disease clinical diagnosis or a nonprion 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. Of 6000 eligible patients in the NACC database, 7 (0.12%) were clinically unsuspected but autopsy-confirmed prion disease cases. The proportion of patients with dementia 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.

References

Dec 3, 1999·Brain : a Journal of Neurology·S PoserK Felgenhauer
Jun 20, 2001·Journal of Neurology, Neurosurgery, and Psychiatry·H J TschampaS Poser
Aug 19, 2005·Clinical Infectious Diseases : an Official Publication of the Infectious Diseases Society of America·Ermias D BelayLawrence B Schonberger
Feb 11, 2009·Archives of Neurology·Keith A JosephsRonald C Petersen
Jun 16, 2011·Annals of Neurology·Numthip ChitravasMark L Cohen

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Citations

Jul 1, 2020·Case Reports in Neurology·Yosuke AibaTsuyoshi Ogata

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