Abstract
Creutzfeldt-Jakob disease (CJD) surveillance relies on autopsy and neuropathologic evaluation. The 1990–2000 CJD autopsy rate in California was 21%. Most neurologists were comfortable diagnosing CJD (83%), but few pathologists felt comfortable diagnosing CJD (35%) or performing autopsy (29%). Addressing obstacles to autopsy is necessary to improve CJD surveillance.
Highlights
Transmissible spongiform encephalopathies (TSEs) are rare, progressively fatal, neurodegenerative illnesses
Our analysis suggests that autopsy rates for Creutzfeldt-Jakob disease (CJD) in California are low
The results of our surveys, which attempted to discern the reasons for this low rate, imply that both neurologists and pathologists have similar perceptions of the value of obtaining histopathologic evaluation for CJD but for different reasons
Summary
Transmissible spongiform encephalopathies (TSEs) are rare, progressively fatal, neurodegenerative illnesses. Pathologic review of brain tissue obtained by biopsy or autopsy is the only means of confirming a diagnosis of CJD. We summarize responses generated from a statewide survey of neurologists and pathologists regarding the challenges to diagnosing CJD and variant CJD, including obtaining autopsy in suspected cases.
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