Abstract
Introduction: Human prion diseases are a group of rare encephalopathies resulting in rapidly progressive dementia and ultimately death. While there are no effective treatments for any form of prion disease, prompt and efficient diagnosis is essential to prevent the spread of the self-propagating protein, which may occur through aerosols, and avoid unnecessary or invasive testing. Diagnosis relies largely on physical examination, with many nonspecific findings, and laboratory testing, which has wide ranges of reported accuracy and high false positive rates with diseases such as Alzheimer’s dementia.
Highlights
Human prion diseases are a group of rare encephalopathies resulting in rapidly progressive dementia and death
Human prion disease is a group of rare, spongiform encephalopathies caused by alteration of a naturally occurring protein resulting in rapidly progressive dementia, neurologic symptoms, and death.[1,2,3,4]
All died within two years of admission and had paraclinical tests suggestive of Creutzfeldt-Jakob Disease (CJD)
Summary
Human prion diseases are a group of rare encephalopathies resulting in rapidly progressive dementia and death. While there are no effective treatments for any form of prion disease, prompt and efficient diagnosis is essential to prevent the spread of the self-propagating protein, which may occur through aerosols, and avoid unnecessary or invasive testing. While there are no effective treatments for any form of CJD, prompt diagnosis is essential to prepare both the patient and family for the inevitable outcomes, to prevent unnecessary testing, and to prevent iatrogenic spread of the self-propagating, aberrant prion protein.[6,7,8,9] While most commonly spread through direct contact with instrumentation contaminated with central nervous system (CNS) contents, it has been demonstrated that aerosolized prion protein can infect mice with high efficiency.[10] The spread of CJD is rare but deadly, and the safest and most unambiguous cleaning of surgical equipment requires incineration.[8] To ensure proper decontamination and safety precautions, a difficult diagnosis must first be made
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