Barriers to Creutzfeldt-Jakob Disease Autopsies, California

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.

Barriers to Autopsy: Creutzfeldt-Jakob Disease in New York State

“…Physicians surveyed by colleagues at the California Department of Health Services were similar to New York State neurologists and pathologists in how they rated the importance of autopsy in confi rming CJD and vCJD, in concern for infection control, and in having facilities available to perform an autopsy [14] . More New York State physicians than California physicians saw family reluctance as an important barrier [14] . Other researchers are comparing noninvasive tests such as magnetic resonance imaging to pathological fi ndings in the brain at autopsy [15,16] .…”

“…Questionnaires for neurologists and pathologists (including neuropathologists) were developed in consultation with staff at the Centers for Disease Control and Prevention and the California Department of Health Services who conducted a similar survey [14] . Surveys included physician ratings of barriers to brain autopsy that had been identifi ed from the literature and through personal experience of public health practitioners involved in this research.…”

A case of sporadic Creutzfeldt – Jakob disease and discussion on diagnostic biomarkers

“…Histopathology represents the only method to certify the diagnosis of CJD. Instead of brain biopsy, which can result in serious complication, autopsy remains the preferred method for collecting brain tissue (21). The classic pathology of sporadic CJD is represented by massive neuronophagia with vacuolization (spongiform degeneration) and reactive astrocyte proliferation.…”

Colorado Surveillance Program for Chronic Wasting Disease Transmission to Humans

“…These cases underscore the importance of a thorough clinical, neuropathological, and genetic evaluation before a neurological or neuropsychiatric disorder in persons exposed to potentially infected game can be attributed to CWD. Although significant barriers can exist to obtaining autopsies on suspected Creutzfeldt-Jakob disease cases, 13,14 postmortem brain examinations with referral to the National Prion Disease Pathology Surveillance Center are crucial to evaluating patients with prion disease.…”