Confronting CJD and other Prion Diseases: Science, Sense, Support
Program Description
This video presentation addresses several issues about Creutzfeldt-Jakob disease, including clinical presentation and course, pathogenesis, differential diagnosis, transmissibility, surveillance and resources available to physicians and families.
Target Audience and Educational Goal
The target audience for this program includes neurologists and other health care professionals involved in the management of patients with CJD or dementing illnesses.
Learning Objectives
After participating in this CME activity, physicians should have a better understanding of the disease process in CJD and related prion diseases, including the following:
Clinical features, including:
• Patient presentations and comments by family members
• Typical symptoms, time course
• Differential diagnosis
• Possible early clues
• Uses and limitations of various diagnostic techniques: radiology,
EEG, laboratory tests
• Criteria for “Definite,” “Probable,” and “Possible”
CJD
Pathology and pathogenesis:
• Microscopic appearance of spongiform encephalopathy
• Nature of normal prions
• How prions undergo pathologic conformational change
• How the prion hypothesis explains sporadic, inherited or acquired
occurrence
Variation in the clinical presentation explained by differences in prion molecular
structure:
• Polymorphisms
• Prion protein typing
• Classification of clinical variants of sporadic CJD
• Mutations, familial cases, genetic testing
Acquired prion diseases:
• Modes of transmission
• Variation in presentation related to type of exposure: ingestion,
direct inoculation into brain, dural graft, growth hormone treatment
• Variant CJD and bovine spongiform encephalopathy
• Clinical presentation of vCJD
• The “fear factor”
• Chronic wasting disease of deer and elk
Resources available to patients and physicians:
• Importance of tissue examination in all suspected cases
• Services offered by the National Prion Disease Pathology Surveillance
Center
• Assistance and support for families through the Creutzfeldt-Jakob
Disease Foundation
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