Clinically Unsuspected Prion Disease among Patients with Dementia Diagnoses in an Alzheimer’s Disease Database

Details

• Alternative Title:
  Am J Alzheimers Dis Other Demen
• Personal Author:
  Maddox, R.A. ; Blase, J.L. ; Mercaldo, N.D. ; Harvey, A.R. ; Schonberger, L.B. ; Kukull, W.A. ; Belay, E. D.
• Description:
  Background
  
  Brain tissue analysis is necessary to confirm prion diseases. Clinically unsuspected cases may be identified through neuropathologic testing.
  
  Methods
  
  National Alzheimer’s Coordinating Center (NACC) Minimum and Neuropathologic Data Sets for 1984–2005 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 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.
  
  Results
  
  Of 6000 eligible patients in the NACC database, 7 (0.12%) were clinically unsuspected but autopsy confirmed prion disease cases.
  
  Conclusion
  
  The 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.
  
  
• Subjects:
  Aged Aged, 80 And Over Alzheimer Disease Alzheimer’s Disease Article Autopsy Creutzfeldt-Jakob Disease Creutzfeldt-Jakob Syndrome Dementia Diagnosis Female Gerstmann-Straussler-Scheinker Disease Humans Male Middle Aged Prion Disease Registries

  More +
• Source:
  Am J Alzheimers Dis Other Demen. 30(8):752-755.
• Pubmed ID:
  26320231
• Pubmed Central ID:
  PMC4651736
• Document Type:
  Journal Article
• Funding:
  CC999999/Intramural CDC HHS/United States ; U01 AG016976/AG/NIA NIH HHS/United States
• Volume:
  30
• Issue:
  8
• Collection(s):
  CDC Public Access
• Main Document Checksum:
  urn:sha256:68ac2c6a23d9c7952d9cfd88cd7e59ecf18208b8ed4b20a4dad757ffa696ace5
• Download URL:
  https://stacks.cdc.gov/view/cdc/36506/cdc_36506_DS1.pdf
• File Type:
  [PDF - 435.78 KB ]