Work

When prion disease Isn’t suspected: prion disease as the cause of terminal decline in chronic mixed dementia

Year: 2023

Type: article

Abstract: Alzheimer's Disease (AD) is the most common cause of dementia, although multiple pathologies are found in nearly half of the cases with clinically diagnosed AD. Prion diseases, such as Creutzfeldt-Jakob disease (CJD), are rare causes of dementia and typically manifest as a rapidly progressive dementia, where symptom onset to dementia most often occurs over the course of months. In this brief report, we describe a patient's typically progressive dementia with a precipitous decline at the end of their life who, on neuropathological evaluation, was found to have multiple neurodegenerative proteinopathies as well as spongiform encephalopathy due to CJD. This case of unsuspected CJD highlights a rare, but epidemiologically important, cause of sudden decline in well-established neurodegenerative dementias.

Source: Neurocase

DOI: 10.1080/13554794.2024.2346990

Authors: Sudarshan Krishnamurthy, William Harrison, Suzanne Craft, Samuel N. Lockhart, James R. Bateman

Institutions: Wake Forest University Health Sciences, Wake Forest University

Language: English

Topic: Prion Diseases and Protein Misfolding

Subfield: Molecular Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: green

Funder: National Institute on Aging